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Functional Respiratory Disorders
Review article Supported by a grant from Zeneca Pharmaceuticals
Functional respiratory disorders Lavjay Butani, MD and Edward J O’Connell, MD
Learning Objectives: Reading this article will enable health care providers to recognize and to diagnose paroxysmal sneezing, sighing dyspnea, habit cough, and vocal cord dysfunction and to reinforce their knowledge of the epidemiology, etiopathology, clinical features, and treatment of these disorders. Data Sources: The literature was reviewed using a MEDLINE search for information relating to the above-mentioned disorders. Indexing terms used included psychogenic wheezing, vocal cord dysfunction, functional respiratory disorders, sighing dyspnea, paroxysmal sneezing, habit cough, and psychogenic stridor. Review was restricted to English language articles from 1966 onward, with crossreferencing to obtain older references. Study Selection: All human studies that clearly identified the above-mentioned disorders as being nonorganic on the basis of historic and appropriate laboratory evaluation were reviewed. No studies were rejected on the basis of subject age, although special emphasis was given to articles concerning children and adolescents (⬍18 years old). Of all initially identified studies, 95% fulfilled the inclusion criteria. Results: Functional respiratory disorders are common and affect mostly children, adolescents, and young adults, resulting in considerable morbidity and contributing significantly to patient and physician cost and frustration. A history of a psychiatric disorder with temporally related psychogenic stressors is frequently found. Professionals disagree on the technical classification of some of these conditions (ie, psychosomatic versus somatoform), but there is agreement that treatment directed toward underlying stressors should be the cornerstone of therapy. Conclusions: Functional respiratory disorders must be considered in patients with atypical symptoms, especially those resistant to conventional therapy. Possible psychogenic stressors must be inquired into and, when identified, treated in a multidisciplinary manner. This may involve reassurance regarding the absence of significant organic abnormality, counseling, and occasional recourse to formal psychiatric intervention. Ann Allergy Asthma Immunol 1997;79:91–101.
INTRODUCTION In today’s demanding and strife-torn society with extraordinary emphasis on From the Section of Pediatric Allergy and Immunology, Mayo Clinic and Mayo Foundation, Rochester, Minnesota. Publication of this article is made possible through an educational grant from Zeneca Pharmaceuticals. Nothing in this publication implies that Mayo Foundation endorses any products of Zeneca Pharmaceuticals. Received for publication April 17, 1997. Accepted for publication in revised form June 22, 1997.
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perfectionism and overachievement, it is not surprising that nonorganic disorders are being encountered more frequently. Although psychosomatic and somatoform disorders were recognized and described extensively many decades ago,1,2 little mention of them is found in contemporary medical literature. Modern concepts regarding conversion disorders (characterized by the loss of or alteration in physical function resembling a physical disorder in
the absence of organ dysfunction) stem from Freud.1 Their evolution was explained by the existence of an unconscious intrapsychic conflict resulting from a past traumatic event. The person, unable to confront this conflict consciously, would “convert” this psychic energy into a more readily expressed physical symptom, often with symbolic significance. This would allow the person to rid the mind of the distressing affect (the primary gain), yet allow its partial expression in a disguised manner; therefore, it was believed that conversion disorders were a form of maladaptive interpersonal communication. In comparison, the term “psychosomatic disorder,” first used by Johann Christian Heinroth in 1818, refers to disorders characterized by demonstrable organic lesions such as asthma or pathophysiologic processes such as migraine with exacerbations temporally related to “psychologically meaningful environmental stimuli,” for example, personal stressors. The respiratory disorders described below, whether believed to be manifestations of a conversion disorder or psychosomatic (somatoform) illness are encountered and misdiagnosed too often to be ignored. This review is the result of our recent evaluation of several patients with symptoms suggestive of nonorganic disorders. PAROXYSMAL SNEEZING Sneezing, or sternutation, is a normal physiologic response directed toward removal of irritants from the nasal mucosa. The sneezing reflex, initiated by irritation of sensory nerve endings of the trigeminal nerve,3 is well described in the literature. Intractable paroxys-
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mal sneezing is a well-recognized entity first described by Shilkret.4 Since that time, this condition, aptly defined as the “sudden violent sneezing of unusual frequency and duration that is generally psychogenic and is resistant to usual treatment,”5 has been mentioned in several clinical vignettes. To date, 29 cases have been recorded,6 25 of which presented in the context of stressors such as sexual abuse7 and obsessive premorbid personality,8 to name two. Symptoms in the other four patients were attributed to organic processes, including temporal lobe epilepsy3 (the occurrence of sneezing was described as part of the preictal aura by Gowers9 and later confirmed by the electrocorticographic stimulation studies of Penfield and Kristiansen10), tubercular cervical lymphadenitis11 triethanolamine sensitivity,12 and multifactorial causes.13 Other causes that have been implicated include allergies, local nasal disturbances (eg, septal deviation),11 light,14 sexual excitement,15 chilling, pregnancy,13 organic neurologic processes such as “mild encephalitis,” and poliomyelitis.11 In an attempt to explain the pathogenesis of paroxysmal sneezing, Crue16 postulated that the trigeminal system acts as a central neuronal pool in the brain stem and upper spinal cord which is influenced by input from
higher centers (ie, the cerebral cortex and cerebellum, thus accounting for the association with psychogenic stresses), peripheral sensory stimuli (explaining symptoms related to light exposure), and parasympathetic overflow (as in orgasmic situations). Little is known about the incidence of paroxysmal sneezing in the community because most of the information is limited to isolated case reports; however, most of the affected persons are adolescent females.6 Patients present with a history of persistent and recurrent bouts of sneezing (up to 30 to 100 per minute), with each bout often lasting for hours to days. The sneeze, or pseudosneeze,17 is described as having an abbreviated inspiratory phase, short nasal grunting sound, and little or no aerosolization of mucosal secretions. Typically, during these episodes, patients keep their eyes open, which is unusual in a physiologic sneeze reflex; they do not appear distressed and are able to converse, eat, and carry on their activities without hindrance. Furthermore, symptoms have not been documented to persist during sleep. Precipitating stressors are frequently identified, and a history of psychopathology often surfaces. Physical examination findings are noncontributory, but occasional excessive tearing4 and nasal mucosal or tur-
Figure 1. Respiratory tracing from young woman with organic heart disease (mitral stenosis). The tracing, which reads from left to right, covers a 10-minute period. No deep respirations or sighs are present (from White PD, Hahn RG23).
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binate erythema4,17,18 have been noted. Laboratory investigations such as rhinoscopy, allergy skin testing, sinus radiography, complete blood cell count, and total IgE are invariably normal. Response to pharmacologic treatment, expectedly, is poor. Treatments that have been tried, with inconsistent benefit, have included nasal and oral decongestants, epinephrine given subcutaneously, corticosteroids given orally and intranasally, antihistamines, and even cauterization of the “Fleiss nasogenital area” of the nasal mucosa.15 Reports have also described short-term relief in patients with the use of oral diazepam19 and haloperidol.8 Therapy directed at understanding and attempting to resolve underlying unconscious conflicts through insight is inherently more successful, with at least shortterm benefit. Psychotherapy,5,17 operant conditioning,4,20 hypnosis,4,5,18,21 and family therapy have all been used with success, although the lack of controlled trials and long-term follow-up have not allowed assessment of lasting benefit. SIGHING DYSPNEA The term “sighing dyspnea,” first used by Maytum and Willius22 in 1934, refers to a well-recognized medical entity of the early 1900s.23 Perhaps because of the lack of recognition of its existence in more recent times, the only description of this uncommon condition in contemporary medical literature is in a 1993 review by Perin et al.24 Dyspnea (a subjective feeling of distress or difficulty in breathing) associated with a sigh (an audible inspiration and expiration under the influence of some emotion)25 was described by Steell26 in 1906 and believed to be related to fatty degeneration of the heart muscle. The suggestion that “sighing is never due primarily to heart disease but always to fatigue or nervousness or other such factor” came from the study by White and Hahn,23 who inquired about this symptom in 650 persons. They found frequent sighing in 19% of a control group, in 3% of patients with organic heart dis-
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Figure 2. Respiratory tracing from a middle-aged woman with marked effort syndrome. Frequent and deep sighing respirations, characteristic of sighing dyspnea, are observed (from White PD, Hahn RG23).
ease, in 80% of patients with “effort syndrome,” and in 74% of those with coexistent organic heart disease and effort syndrome (Figs 1, 2). Patients with sighing dyspnea are usually in the 2nd to 4th decade of life, although occasionally it has been described in children as young as 5 years. Women in the active years of life22 were the largest proportion of cases in the original reports.23 Maytum and Willius22,27 gave a vivid description of symptoms of sighing dyspnea: patients complain of shortness of breath associated with difficulty in “getting their breath,” inability in obtaining a “satisfying breath” or “full breath,” or “breathing below a certain level.” Symptoms usually occur in attacks, although in some cases mild dyspnea may be present all the time. A typical attack, which can last for up to several hours, consists of a series of deep sighing respirations that are greatly increased in depth but without a change in respiratory rate. Patients appear distressed, frequently using the accessory muscles of respiration. Associated symptoms include a feeling of weight, constriction, or oppression in the chest, aerophagia, belching, palpitations, and ill-defined chest pains. During severe attacks, patients may appear extremely ill and anxious,
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grasp their throat or chest, and run to a window to abort the sensation of suffocation. At times, they must make several attempts before they are able to obtain a deep breath, which when done affords marked relief. In prolonged attacks, tetanic spasms have also been reported.22,27 Attacks usually appear at rest and rarely with physical activity, unlike organic heart disease. In nearly all patients, frequent sighing and yawning are noted between attacks. Although these patients complain of shortness of breath, there is no true dyspnea. When asked to try to reproduce their “dyspnea,” they sigh and call the examiner’s attention to this “difficulty,” emphasizing the feeling of satisfaction that follows when a full breath is taken. Symptoms are often preceded by stressful situations and tend to recur in the presence of new stressors. Physical examination and laboratory findings are normal, and the diagnosis is made on the basis of the history and observation of the characteristic sighing pattern (Figs 1, 2). The etiopathogenic trigger for sighing dyspnea initially was believed to be mild anoxemia22 that resulted from increased nervous and muscular tension related to strain and fatigue, causing involuntary restriction of respiratory excursions. Fatigue of the respiratory center28 was thought to en-
sue and to produce a sensation of dyspnea. Organic causes reportedly associated with sighing dyspnea—and which should be ruled out by appropriate investigations if additional signs and symptoms indicate their presence—include asthma, coronary artery disease, acute left ventricular failure,22 thyroid disorders,23 and sinusitis.24 Reassurance and counseling regarding the benign nature of this condition, relaxation techniques, and identification and possible resolution of emotional stressors are an intrinsic part of the healing process. No results of longterm follow-up studies are available to assess the natural history of this disorder. HABIT COUGH Although cough is a common symptom that serves an important function, its persistence is often a sign of an underlying abnormality. Also, cough itself may result in complications, both physical (laryngeal irritation, vomiting, rib fractures, pneumothorax and pneumomediastinum, and rupture of the rectus abdominus) and emotional (exhaustion, sleep deprivation, and social withdrawal). A study of 72 infants and children with intractable cough referred to an otolaryngology clinic revealed the five commonest causes to be cough-variant asthma (31.9% of patients), sinusitis (24%), gastroesophageal reflux (15%), aberrant innominate artery (12.5%), and psychogenic (10%).29 Table 1 lists some of the common causes of chronic cough in children. Psychogenic, habit, or involuntary cough has been reported almost exclusively in children and adolescents30 and is without any striking predilection for sex. The cough is described as a croupy cough produced by a sharp intake of air and followed by a short explosive expiration, resulting in a characteristic barking or honking sound. Patients are described as being happy and unperturbed by their symptoms. The cough is more disturbing to parents and is disruptive in school, resulting in frequent school absenteeism. Typically, the cough starts after an up-
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Table 1. Etiology of Chronic Cough in Children Etiology Cough-variant asthma Infectious Congenital
Psychogenic Traumatic Irritants Otologic Neoplastic
Cardiac
Clinical Condition Sinusitis, pertussis, parapertussis, tuberculosis, diphtheria Vascular rings, bronchogenic cyst, tracheomalacia, subglottic stenosis, tracheal web or stenosis, gastroesophageal reflux, vocal cord paralysis, tracheoesophageal fistula Foreign body aspiration Smoke exposure, environmental pollutants Foreign body in ear canal, cerumen impaction Laryngeal hemangioma, vocal cord papillomas, bronchial adenomas, mediastinal adenopathy Congestive heart failure with pulmonary edema
Modified from Holinger LD, Sanders AD.29 (Used with permission of the American Laryngological, Rhinological and Otological Society.)
per respiratory illness. After resolution of the acute illness, an isolated and explosive daytime cough persists, with cessation of symptoms during sleep and pleasurable social activities. Patients complain of “something in their throat,” or a “tickle,” and sometimes adopt a chin-on-chest posture, with a hand held against the throat as if to support the larynx (Fig 3)31 The cough persists for various periods of time, especially in the absence of appropriate intervention, with frequent exacerbations.32 Symptoms worsen on contact with medical professionals and in the presence of other people and can be reproduced in the physician’s office on request. Psychosocial behaviors may be identified, ranging from school phobia33 to attention seeking and anxiety. Rarely has coexistent psychopathology been formally diagnosed. The results of physical examination, pulmonary function tests, chest radiography, allergy testing, and bronchoscopy are normal. Response to antitussive medications has been dismal. Treatment in mild cases is usually simple: explanation of the nature of the problem33 (reassuring patients that they are not voluntarily faking the symptoms and that there is no serious underlying organic disease), with dis-
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cussion of potential solutions to underlying stressors. The cough should be explained to the patient as arising from a vicious cycle of an initial irritant that is no longer present but which set up a pattern of coughing that is causing more irritation.32 For more persistent and severe symptoms, innovative behavioral techniques have been tried, including nasal breathing while keeping a flat button in the mouth (to avoid dryness of the throat, which was thought to trigger the cough) (an approach of uncertain safety because of the potential risk of aspiration of the button);34 biofeedback;30 suggestion therapy32 (explanation of symptoms and repeated expression of confidence by the therapist through verbal suggestion to suppress the cough while using a distractor, most typically dilute nebulized saline); and aversive techniques,35,36 the most
Figure 3. Characteristic “chin-on-chest” posture of patient with habit cough (from Weinberg EG31).
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publicized of which is the bed sheet method. A bed sheet is wrapped tightly around a patient’s chest, with a double knot tied over the sternum, ostensibly to provide support to the weakened chest muscles believed to be responsible for the persistent cough. The sheet is worn at all times by the patient, with additional intervention in the form of commands such as “stop it” or “no more cough” if the patient makes an effort to start coughing. The true rationale behind the intervention is explained to the parents but is concealed from the patient. Cohlan and Stone35 reported success in 33 of 35 children who received treatment with this approach. Data on long-term follow-up of patients receiving such forms of suggestion therapy are scant but nevertheless reassuring. Lokshin et al32 reported nine patients treated with verbal suggestive therapy, for whom longterm follow-up was available in seven (median follow up, 3.6 years). Of these seven patients, six had complete resolution of their symptoms after a single session, and minor self-controlled symptoms persisted in the other patient. VOCAL CORD DYSFUNCTION The clinical syndrome of vocal cord dysfunction, described for the first time in the mid-1800s,37–39 and also referred to as “pseudoasthma,” “hysterical croup,” and “nonorganic acute upper airway obstruction,” is the most studied entity in this group of disorders. Interest in vocal cord dysfunction was rejuvenated after the case report by Patterson et al40 in 1974. No better description of this can be found than that by Osler,41 “spasm of the muscles may occur with violent inspiratory efforts and great distress, and may even lead to cyanosis . . .. Extraordinary cries may be produced, either inspiratory or expiratory.” Paradoxical vocal cord motion, the laryngoscopic equivalent of vocal cord dysfunction, refers to the abnormal movement of the vocal cords, which are 180 degrees out of phase with the respiratory cycle, that is, adduction of the cords on inspiration with either ab-
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duction (in most cases) or adduction during expiration.42 Normal persons have a fairly constant glottic opening, with mild increase in the aperture during inspiration, whereas persons with asthma have been noted to have mild inspiratory and mid-expiratory narrowing of the glottis and supraglottis, providing the so-called laryngeal positive end-expiratory pressure, which is thought to be of physiologic significance in preventing atelectasis.43,44 Little is known about the epidemiology of vocal cord dysfunction. A study from the National Jewish Center for Immunology and Respiratory Medicine found vocal cord dysfunction in 9.6% of 50 consecutive patients referred for “refractory” asthma.45 An additional 32.6% had coexistent asthma and vocal cord dysfunction. A consistent sex bias has been noted, with more women affected, but this difference is less significant in children.46 Typically, vocal cord dysfunction is diagnosed in adolescent women with medical backgrounds42,45,47 or histories of asthma or psychiatric illness.47 One of the earliest mentions of vocal cord dysfunction in the psychiatric literature is that by Janet: “hysterical asphyxia, resulting from various disturbances in the respiratory mechanism, does not seem to us to be capable, in general, of bringing about death. A moment comes when as-
phyxia brings on fainting; that is, the arrest of the higher functions of the brain, and the respiration, being no longer impeded by these higher functions, is restored owing to the automatism of the bulb.”48 In a literature review of 48 patients with vocal cord dysfunction, 94% had a psychiatric disorder, the most common being conversion disorder (52%).46 A psychopathologic cause is found less frequently in children than adults, but there is a greater incidence of familial stressors (emphasis on competitive sports and overachievement). In one study, a 30% prevalence of sexual abuse in childhood was also noted in women with vocal cord dysfunction.49 This further supports the above-mentioned contention of an unconscious intrapsychic conflict of a sexual nature as a possible trigger. Physicians managing patients with vocal cord dysfunction recognize the important contributory effect of psychologic stresses on clinical symptoms. Psychiatrists differ in their formal categorization of vocal cord dysfunction, with some classifying it as a conversion disorder40,50 on the basis of the Freudian psychodynamic concept of intrapsychic conflict referred to above and others considering it as a psychosomatic illness46,51 due to the undeniable presence of an organic pathologic process, that is, paradoxical
Figure 4. Flow volume loops demonstrating pattern of A, extrathoracic obstruction with blunting of the inspiratory (Insp) component as seen in vocal cord dysfunction and, B, intrathoracic obstruction with a plateau in the expiratory (Exp) phase of the tracing typical of asthma. RV, residual volume; TLC, total lung capacity (from Kryger M, Bode F, Antic R, Anthonisen N. Diagnosis of obstruction of the upper and central airways. Am J Med 1976;61:85–93).
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Table 2. Comparison of Features of Asthma and Vocal Cord Dysfunction Feature Age Sex Premorbid status
Commonest symptom Symptom attributes
Precipitating factors
Physical examination Respiratory distress Neck position Auscultation Investigations Chest radiograph Eosinophilia A-a gradient Spirometry Laryngoscopy Therapy Acute
Long-term
Asthma Not specific Male and female equally affected Not specific
Wheezing Worse at night Expiratory difficulty Rapid onset and gradual resolution No decrease with distraction Infections Exercise Cold weather Allergies Stress
Adolescents Females affected more frequently than males Higher incidence of psychopathology, sexual abuse, personality disorder Stridor Least at night Inspiratory distress Rapid onset and rapid resolution Easily distractible Stress Unusual triggers—sports, odors, medical personnel
Universal Extension Expiratory rhonchi
Variable Flexion Inspiratory stridor
Hyperinflation May be present Sometimes increased Prolonged expiration (decreased FEV1) Normal/mild glottic narrowing
Normal Absent Normal Prolonged inspiratory phase (normal FEV1) Paradoxical vocal cord motion
Supportive Nebulized -agonists Corticosteroids Inhaled steroids Lifestyle modification
Relaxation therapy Inhaled helium
vocal cord motion. There also is growing concern among physicians about the possibility of an occult and purely organic basis for symptoms in a subset of patients. In 1987, Patton et al52 reported on a 61-year-old woman with paradoxical vocal cord motion who had a posterior fossa arachnoid cyst and whose symptoms resolved after surgical removal of the cyst. In 1982, Kellman and Leopold53 described a patient with vocal cord dysfunction in the setting of a possible brain stem abnormality manifested by several cranial nerve palsies. They also described a patient, presented to them by another physician, with paradoxical vocal cord
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Vocal Cord Dysfunction
Speech therapy Reassurance Psychiatric evaluation
motion and persistent aspiration pneumonia due to an organic brain syndrome occurring after viral encephalitis. Supporting the above hypothesis is an older study by Haglund et al,54 in which 18 patients with a provisional diagnosis of functional aphonia/dysphonia (based on normal findings on neurologic and phoniatric examinations) were reevaluated. Four were found to have incomplete closure of the posterior part of their vocal cords during phonation, five others had an oval-shaped adduction insufficiency, and the other nine had complete adduction of the cords. Electromyographic studies of the vocal and cricothyroid
muscles revealed the presence of neurogenic lesions in ten patients in the form of conduction blocks and giant potentials. These patients probably represent a minor subset, but one that must always be kept in mind during the evaluation process. Patients with vocal cord dysfunction present with symptoms similar to those in asthma and croup. Stridor (inspiratory, expiratory, or biphasic) is the commonest symptom (83% of patients),46 followed by wheezing. A history of a preceding upper respiratory illness is not infrequent and is the trigger that directs the patient’s attention toward the larynx.53 Older patients can describe the wheezing as “coming from the throat.”42,45,55 Twenty-one percent of the patients have concomitant dysphonia, with occasional reports of dysphagia45 and multiple somatoform complaints.40 Distraction maneuvers such as coughing,40,56 panting,57 phonation, and verbal suggestion techniques have been associated with a decrease in respiratory distress. Symptoms have been brought on by unusual triggers, including stress,40 odors,45 competitive sports, and even the presence of medical personnel in the patient’s room. The symptoms rarely occur during sleep,45 have a rapid onset and equally rapid resolution, and are characteristically unresponsive to medications.47 Most patients have been through several hospitalizations, emergency room visits,40 and, occasionally, multiple intubations and tracheostomies.45,46,58 It is not unusual for patients to have been given long-term treatment with oral corticosteroids for a presumed diagnosis of steroid-dependent asthma, with consequent adverse side effects.46 On examination, the degree of respiratory distress varies, from some patients reportedly being in an inappropriate panic state to others showing la belle indifference.47,56 An inappropriately increased respiratory rate (for the presumed degree of airway obstruction) has been noted in several reports, with some patients frankly hyperventilating. Patients frequently appear to flex their neck59 during the attack, a
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maneuver that is not physiologic because the posture associated with the greatest diameter of the larynx is extension. Patients are rarely cyanotic, and there are only a few reports of patients in acute respiratory failure. Chest auscultation reveals stridor or wheezing that, if listened to carefully, will be heard best over the larynx, with symmetric distal transmission.46 Laboratory findings are normal. Important negative findings include absence of hyperinflation on chest radiography (present in 73% of patients with asthma),55 absence of eosinophilia,45 and an arterial blood gas result of normal PO2 and respiratory alkalosis due to hyperventilation. There have been reports occasionally of hypoxemia,46 but with a normal A-a gradient45,55 (reflecting lack of ventilationperfusion mismatch), and rarely of hypercarbia and acidosis.47,58 Spirometry often helps in differentiating asthma from vocal cord dysfunction. Unlike asthma, where there is blunting of the expiratory loop of the flow volume curve (because of expiratory bronchial constriction), in vocal cord dysfunction, it is in the inspiratory part of the flow volume curve that a plateau is seen, indicating the presence of an extrathoracic airway obstruction that causes difficulty with getting a breath “in” rather than expelling it (Fig 4). This is not specific for vocal cord dysfunction and may be seen in other pathologic conditions that cause upper airway obstruction. Conflicting results have been reported with the use of methacholine46 during spirometry, but most patients have a negative response. When a patient with paradoxical vocal cord motion occasionally is found to have a positive methacholine challenge, it has been attributed to coexistent asthma45 and expectedly adds to the confusion. Some of the differentiating features between asthma and vocal cord dysfunction are listed in Table 2. The reference standard for diagnosing vocal cord dysfunction is laryngoscopic visualization of paradoxical motion of the vocal cords (Fig 5).42,45 This should be performed during an
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Figure 5. Laryngoscopic appearance of the vocal cords in vocal cord dysfunction. The characteristic triangular-shaped chink is seen in the posterior part of the glottic aperture during inspiration (from Rusakow LS, Blager FB, Barkin RC, White CW. Acute respiratory distress due to vocal cord dysfunction in cystic fibrosis. J Asthma 1991;28:443– 6).
acute episode (because it may be episodic)45 without sedation, anesthesia, or inhaled ipratropium,47 all of which may interfere with the findings. As mentioned above, the characteristic finding is adduction of the vocal cords during inspiration, creating a diamondshaped chink in the glottic aperture posteriorly.42,45,50 This cannot be induced voluntarily by a patient.60 Because of the anecdotal nature of the literature, it is not surprising that various types of pharmacotherapy have been tried in an uncontrolled setting with equally varying results. When a physician is reasonably certain of the diagnosis, acute episodes can be treated most effectively with supplemental oxygen, reassurance, and relaxation techniques. Assisting the patient in extending the neck to increase airway diameter often helps in reducing distress. Anxiolytics may have some role in the acute setting. Attacks may also be aborted by administering continuous positive airway pressure or intermittent positive pressure ventilation61 by bag and mask, asking the patient to relax and to give up voluntary control of breathing. This is believed to work by helping reduce aberrant vagal discharges from higher neurologic centers to the vocal cords, which may be the responsible factor for the paradoxical motion of the vocal cords. Use of inhaled helium and oxygen mixtures (80% helium plus 20%
oxygen) is recommended for more severe attacks42 and can easily be administered using a conventional nebulizer. Because helium has a lower density than oxygen, it decreases airflow turbulence, relieving the patient’s sense of dyspnea. Rarely has intubation or tracheostomy been thought to be essential.46 Important tenets of maintenance therapy include stopping treatment with unnecessary and potentially harmful medications such as corticosteroids and using speech therapy42,45 and formal psychiatric counseling. The goal of speech therapy is to decrease laryngeal tone, which can be achieved by training patients in techniques of abdominal breathing, and to encourage them to concentrate on the expiratory phase of the respiratory cycle during an acute episode. Additional techniques to reduce laryngeal tone and to relieve upper airway obstruction, for example, biofeedback,45 hypnosis,62 and panting, have been used with success. Counseling to help patients identify and cope with underlying psychosocial conflicts remains the cornerstone of long-term therapy. Even if underlying psychodynamic conflicts cannot be avoided, patients can be taught to express their emotions in a more direct and adaptive manner42 and, thereby, eliminate the need for the unconscious to “convert” these conflicts into maladaptive physical symp-
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toms. Formal psychiatric intervention with psychotherapy and psychotropic medications may be necessary to deal with a frank psychopathologic condition.40,42,45,60 The results of short-term follow-up of patients with vocal cord dysfunction appear to indicate an excellent response to intervention, although few data are available on long-term outcome. In the only study to address this issue, three patients with vocal cord dysfunction and asthma were followed over a 10-year period and all proved refractory to therapy,63 with persistent adduction of the vocal cords on inspiration; one of the patients needed tracheostomy. Whether this is representative of the entire group of patients with vocal cord dysfunction or reflects the outcome of patients at the severe end of the clinical spectrum is not known.64 SUMMARY The frequent association of respiratory disorders with psychiatric disorders is not surprising in view of the bidirectional interaction of breathing with emotions and behavior.65 Breathing— being the mark of the first sign of life— has fundamental roots in the human psyche. Little wonder that disorders involving the mechanics of breathing are frequently the only sign of psychogenic conflicts. In general, nonorganic disorders are diagnosed more frequently in adolescent women when the stress of puberty is paramount. A history of psychiatric disorders with temporally related stressors is frequently obtained. These disorders mimic organic conditions, for which they are frequently mistaken, with consequent overinvestigation and expensive, unnecessary treatment. Most of them can be diagnosed or suspected on the basis of a meticulously taken history and physical examination without recourse to expensive investigations. Treatment, which should be directed toward the primary initiating event, involves counseling and psychiatric evaluation. Short-term benefits are apparent, but long-term follow-up is needed to assess lasting improvement.
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Our goal is to increase awareness among physicians about the existence of these disorders, to enable easy diagnosis, to avoid extensive and inappropriate investigations, and to make appropriate referrals for speedy initiation of treatment. REFERENCES 1. Barsky AJ. Somatoform disorders. In: Kaplan HI, Sadock BJ, eds. Comprehensive textbook of psychiatry/V. vol. 1, 5th ed. Baltimore: Williams & Wilkins, 1989:1009 –27. 2. Kaplan HI, Oken D, Lipsitt DR, et al. Psychological factors affecting physical condition (psychosomatic disorders) (Parts 1–14). In: Kaplan HI, Sadock BJ, eds. Comprehensive textbook of psychiatry/V. vol. 2, 5th ed. Baltimore: Williams & Wilkins, 1989: 1155–279. 3. Kofman O. Paroxysmal sneezing. Can Med Assoc J 1964;91:154 –7. 4. Shilkret HH. Psychogenic sneezing and yawning. Psychosom Med 1949; 11:127– 8. 5. Kaplan MJ, Lanoff G. Intractable paroxysmal sneezing. A clinical entity defined with case reports. Ann Allergy 1970;28:24 –7. 6. Keating MU, O’Connell EJ, Sachs MI. Intractable paroxysmal sneezing in an adolescent. Ann Allergy 1989;62: 429 –31. 7. Aggarwal J, Portnoy J. Intractable sneezing with a specific psychogenic origin. Ann Allergy 1986;56:345– 6. 8. Davison K. Pharmacological treatment for intractable sneezing. Br Med J (Clin Res Ed) 1982;284:1163– 4. 9. Gowers WR. Epilepsy and other chronic convulsive diseases. 2nd ed. Philadelphia: Blakiston Company, 1901:200. 10. Penfield W, Kristiansen K. Epileptic seizure patterns: a study of the localizing value of initial phenomena in focal cortical seizures. Springfield, Illinois: Charles C Thomas, 1951. 11. Shapiro SL. Paroxysmal sneezing. Eye, Ear, Nose Throat Monthly 1967; 46:1532– 8. 12. Herman JJ. Intractable sneezing due to IgE-mediated triethanoloamine sensitivity. J Allergy Clin Immunol 1983; 71:339 – 44. 13. Co S. Intractable sneezing: case report and literature review. Arch Neurol 1979;36:111–2.
14. Everett HC. Sneezing in response to light. Neurology 1964;14:483–90. 15. Everett HC. Paroxysmal sneezing following orgasm (answer). JAMA 1972; 219:1350 –1. 16. Crue B, Todd E, Carregal E, et al. Cranial neuralgia. In: Vinken P, Bruyn G, eds. Handbook of clinical neurology, vol 5. Amsterdam: North Holland Publishing Company, 1968:287. 17. Bergman GE, Hiner LB. Psychogenic intractable sneezing in children. J Pediatr 1984;105:496 – 8. 18. Murray N, Bierer J. Prolonged sneezing: a case report. Psychosom Med 1951;13:56 – 8. 19. Gervis JH. Pharmacological treatment for intractable sneezing [Letter]. Br Med J (Clin Res Ed) 1982;284:1560. 20. School girl still sneezes, but feels fine. Miami: AP Newspaper Report; 1967. Cited by Gallia LJ, Roscoe G. Intractable sneezing. Trans Pa Acad Ophthalmol Otolaryngol 1981;34:164 – 8. 21. Elkins M, Milstein JJ. Hypnotherapy of pseudo-sneezing: a case report. Am J Clin Hypnosis 1962;4:273–5. 22. Maytum CK, Willius FA. Abnormal respiration of functional origin. Proc Staff Meet Mayo Clin 1934;9:308 –11. 23. White PD, Hahn RG. The symptom of sighing in cardiovascular diagnosis. With spirographic observations. Am J Med Sci 1929;177:179 – 88. 24. Perin PV, Perin RJ, Rooklin AR. When a sigh is just a sigh . . . and not asthma. Ann Allergy 1993;71:478 – 80. 25. Stedman TL. Stedman’s medical dictionary: a vocabulary of medicine and its allied sciences, with pronunciations and derivations. 23rd ed. Baltimore: Williams & Wilkins, 1976. 26. Steell G. Text book on diseases of the heart. Manchester: University Press, 1906. 27. Maytum CK. Sighing dyspnea: clinical syndrome. J Allergy 1938;10:50 –5. 28. Haldane JS. Respiration. New Haven, CT: Yale University Press, 1922:427. 29. Holinger LD, Sanders AD. Chronic cough in infants and children: an update. Laryngoscope 1991;101: 596 – 605. 30. Riegel B, Warmoth JE, Middaugh SJ, et al. Psychogenic cough treated with biofeedback and psychotherapy. A review and case report. Am J Phys Med Rehabil 1995;74:155– 8. 31. Weinberg EG. ‘Honking’: psychogenic cough tic in children. S Afr Med J 1980;57:198 –200.
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32. Lokshin B, Lindgren S, Weinberger M, Koviach J. Outcome of habit cough in children treated with a brief session of suggestion therapy. Ann Allergy 1991;67:579 – 82. 33. Shuper A, Mukamel M, Mimouni M, et al. Psychogenic cough. Arch Dis Child 1983;58:745–7. 34. Bernstein L. A respiratory tic: “the barking cough of puberty.” Report of a case treated successfully. Laryngoscope 1963;73:315–9. 35. Cohlan SQ, Stone SM. The cough and the bedsheet. Pediatrics 1984;74:11–5. 36. Lavigne JV, Davis AT, Fauber R. Behavioral management of psychogenic cough: alternative to the “bedsheet” and other aversive techniques. Pediatrics 1991;87:532–7. 37. Dunglison RD. The practice of medicine. Philadelphia: Lea and Blanchard, 1842:258. 38. MacKenzie M. Use of laryngoscopy in diseases of the throat. Philadelphia: Lindsey and Blackeston, 1869: 246 –50. 39. Flint A. Principles and practice of medicine. Philadelphia: Henry C. Lea, 1868:267– 8. 40. Patterson R, Schatz M, Horton M. Munchausen’s stridor: non-organic laryngeal obstruction. Clin Allergy 1974;4:307–10. 41. Osler W. The principles and practice of medicine, designed for the use of practitioners and student of medicine. 6th ed. New York: D. Appleton, 1906: 1081. 42. Martin RJ, Blager FB, Gay ML, Wood RP II. Paradoxic vocal cord motion in presumed asthmatics. Semin Respir Med 1986;8:332–7. 43. Collett PW, Brancatisano T, Engel LA. Changes in the glottic aperture during bronchial asthma. Am Rev Respir Dis 1983;128:719 –23.
44. O’Hollaren MT. Masqueraders in clinical allergy: laryngeal dysfunction causing dyspnea. Ann Allergy 1990; 65:351– 6. 45. Brugman SM, Newman K. Vocal cord dysfunction. Medical/Scientific Update 1993;11:1– 6. 46. Lacy TJ, McManis SE. Psychogenic stridor. Gen Hosp Psychiatry 1994;16: 213–23. 47. Niven RM, Roberts T, Pickering CA, Webb AK. Functional upper airways obstruction presenting as asthma. Respir Med 1992;86:513– 6. 48. Janet P. The major symptoms of hysteria; fifteen lectures given in the medical school of Harvard University. 2nd ed. New York: Hafner Publishing Company, 1965:248. 49. Freedman MR, Rosenberg SJ, Schmaling KB. Childhood sexual abuse in patients with paradoxical vocal cord dysfunction. J Nerv Ment Dis 1991; 179:295– 8. 50. Christopher KL, Wood RP II, Eckert RC, et al. Vocal-cord dysfunction presenting as asthma. N Engl J Med 1983; 308:1566 –70. 51. Maricle R. Vocal-cord dysfunction presenting as asthma [Letter]. N Engl J Med 1983;309:1190 –1. 52. Patton H, DiBenedetto R, Downing E, et al. Paradoxic vocal cord syndrome with surgical cure. South Med J 1987; 80:256 – 8. 53. Kellman RM, Leopold DA. Paradoxical vocal cord motion: an important cause of stridor. Laryngoscope 1982; 92:58 – 60. 54. Haglund S, Knutsson E, Martensson A. An electromyographic study of the vocal and cricothyroid muscles in functional dysphonia. Acta Otolaryngol (Stockh) 1974;77:140 –9. 55. Downing ET, Braman SS, Fox MJ, Corrao WM. Factitious asthma. Phys-
56. 57. 58.
59.
60.
61. 62. 63.
64.
65.
iological approach to diagnosis. JAMA 1982;248:2878 – 81. Neel EU, Posthumus DL. Nonorganic upper airway obstruction. J Adolesc Health Care 1983;4:178 –9. Pitchenik AE. Functional laryngeal obstruction relieved by panting. Chest 1991;100:1465–7. Couser JI Jr, Berman JS. Respiratory muscle fatigue from functional upper airway obstruction. Chest 1989;96: 689 –90. Lund DS, Garmel GM, Kaplan GS, Tom PA. Hysterical stridor: a diagnosis of exclusion. Am J Emerg Med 1993;11:400 –2. Heiser JM, Kahn ML, Schmidt TA. Functional airway obstruction presenting as stridor: a case report and literature review. J Emerg Med 1990;8: 285–9. Collett PW, Brancatisano T, Engel LA. Spasmodic croup in the adult. Am Rev Respir Dis 1983;127:500 – 4. Smith MS. Acute psychogenic stridor in an adolescent athlete treated with hypnosis. Pediatrics 1983;72:247– 8. Hayes JP, Nolan MT, Brennan N, FitzGerald MX. Three cases of paradoxical vocal cord adduction followed up over a 10-year period. Chest 1993; 104:678 – 80. Patterson DL, O’Connell EJ. Vocal cord dysfunction: what have we learned in 150 years? Insights Allergy 1994;9:1–12. Ley R. An introduction to the psychophysiology of breathing. Biofeedback Self Regul 1994;19:95– 6.
Request for reprints should be addressed to: Edward J O’Connell, MD Section of Pediatric Allergy & Immunology 200 First St SW Rochester, MN 55905
CME Examination No 007-008 Questions 1–20, Butani L and EJ O’Connell. 1997;79;91–101. CME Test Questions 1. Functional respiratory disorders are diagnosed most frequently in a. elderly males.
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b. preschool children. c. pregnant females. d. middle-aged executive males. e. adolescent females.
2. All the following are considered nonorganic respiratory disorders except a. vocal cord paralysis. b. paroxysmal sneezing.
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c. sighing dyspnea. d. pseudoasthma. e. habit cough. Principles of management of patients with functional respiratory disorders may include all the following except a. reassurance. b. evaluation of and coping with potential stressors. c. psychotherapy. d. extensive laboratory investigations to rule out all possible organic causes. e. meticulous history and physical examination. The following profile is shared by functional respiratory disorders except a. resolution of symptoms with distraction maneuvers. b. patients frequently have a medical background. c. abnormal premorbid personality. d. poor response to conventional medical therapy. e. continuous symptoms that significantly disrupt dayto-day functioning. The following is true of habit cough: a. has a characteristic honking sound. b. is paroxysmal with frequent post-tussive emesis. c. is a variant of cough-type asthma. d. responds well to antitussive medications. e. usually presents along with fever and symptoms of upper respiratory illness. The reference standard for diagnosing vocal cord dysfunction is a. paradoxical vocal cord motion on laryngoscopy. b. positive methacholine challenge test. c. abnormal chest radiographic findings. d. blunting of the inspiratory component of the flow volume loop.
7.
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e. blunting of the expiratory component of the flow volume loop. All the following are frequently seen in vocal cord dysfunction except a. stridor. b. wheezing. c. hypoxemia. d. relief with inhaled mixture of helium-oxygen. e. flexed neck posture of patients. Therapies indicated for acute/ chronic relief in vocal cord dysfunction include the following except a. inhaled helium-oxygen mixture. b. hypnosis. c. relaxation therapy. d. speech therapy. e. vagotomy. All the following are helpful in differentiating vocal cord dysfunction from asthma except a. lack of eosinophilia. b. normal A-a gradient on blood gas analysis. c. blunting of the inspiratory component of the flow volume loop. d. normal chest radiograph. e. consistently negative methacholine challenge. Symptom characteristics in vocal cord dysfunction include a. worse at night. b. result in marked and predictable respiratory distress. c. not reduced by distracting the patient. d. relieved by bronchodilator therapy. e. may be preceded by an upper respiratory illness. Laryngoscopic findings in vocal cord dysfunction are a. abduction of the vocal cords in inspiration. b. adduction of the vocal cords on inspiration. c. vocal polyps.
12.
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d. diamond-shaped chink in the anterior aspect of the vocal cords in inspiration. e. normal vocal cord motion during exacerbations. Psychogenic sneezing characteristically a. is intermittent in nature. b. has a short inspiratory component with an explosive expiratory phase. c. is associated with a higher than normal incidence of cat allergy. d. can be diagnosed on the basis of increased IgE levels in nasal secretions. e. is associated with profound nasal erythema and irritation. Which of the following is true regarding sighing dyspnea? a. is most often seen in elderly white males. b. can be reproduced by patients in the physician’s office on request. c. improves with bronchodilator therapy. d. is caused by pulmonary edema due to congestive heart failure. e. rarely occurs at rest. The following is true of habit cough: a. it is caused by persistent pertussis infection. b. the symptoms are under the patient’s conscious and voluntary control. c. a neck radiograph is essential for diagnosis. d. it improves with biofeedback techniques. e. it has an excellent longterm resolution rate. Symptoms of vocal cord dysfunction may be manifestations of an underlying a. organic lesion. b. somatoform disorder. c. conversion disorder. d. all the above. e. none of the above.
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16. The epidemiology of vocal cord dysfunction in children differs from that in adults in a. a higher prevalence of formal psychopathology. b. a lower prevalence of familial stressors. c. a higher prevalence of dysfunctional families with emphasis on overachievement. d. none of the above. e. all the above. 17. Which of the following is inappropriate in managing patients with vocal cord dysfunction? a. referral for psychiatric evaluation. b. advocating relaxation therapy. c. aggressive trial of corticosteroids. d. reassuring patients regarding the absence of organic lesions.
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e. providing coping skills to deal with stressful situations. 18. The commonest symptom of vocal cord dysfunction is a. wheezing. b. stridor. c. cough. d. dyspnea. e. palpitations. 19. Which of the following is not true of vocal cord dysfunction? a. It may coexist with asthma. b. A positive methacholine challenge rules out the diagnosis. c. It is rarely associated with such significant distress as to need mechanical ventilation. d. Diagnosis can frequently be made on the basis of the history.
e. Paradoxical vocal cord motion is the laryngoscopic counterpart of vocal cord dysfunction. 20. Which of the following is true about functional respiratory disorders? a. They are rare in the general population. b. Diagnosis is one of exclusion. c. Psychopathology can frequently be identified in patients. d. Treatment should be relegated completely to psychiatrists. e. Sufficient long-term data exist regarding excellent long-term outlook for these patients.
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Functional respiratory disorders Lavjay Butani, MD and Edward J O’Connell, MD
Learning Objectives: Reading this article will enable health care providers to recognize and to diagnose paroxysmal sneezing, sighing dyspnea, habit cough, and vocal cord dysfunction and to reinforce their knowledge of the epidemiology, etiopathology, clinical features, and treatment of these disorders. Data Sources: The literature was reviewed using a MEDLINE search for information relating to the above-mentioned disorders. Indexing terms used included psychogenic wheezing, vocal cord dysfunction, functional respiratory disorders, sighing dyspnea, paroxysmal sneezing, habit cough, and psychogenic stridor. Review was restricted to English language articles from 1966 onward, with crossreferencing to obtain older references. Study Selection: All human studies that clearly identified the above-mentioned disorders as being nonorganic on the basis of historic and appropriate laboratory evaluation were reviewed. No studies were rejected on the basis of subject age, although special emphasis was given to articles concerning children and adolescents (⬍18 years old). Of all initially identified studies, 95% fulfilled the inclusion criteria. Results: Functional respiratory disorders are common and affect mostly children, adolescents, and young adults, resulting in considerable morbidity and contributing significantly to patient and physician cost and frustration. A history of a psychiatric disorder with temporally related psychogenic stressors is frequently found. Professionals disagree on the technical classification of some of these conditions (ie, psychosomatic versus somatoform), but there is agreement that treatment directed toward underlying stressors should be the cornerstone of therapy. Conclusions: Functional respiratory disorders must be considered in patients with atypical symptoms, especially those resistant to conventional therapy. Possible psychogenic stressors must be inquired into and, when identified, treated in a multidisciplinary manner. This may involve reassurance regarding the absence of significant organic abnormality, counseling, and occasional recourse to formal psychiatric intervention. Ann Allergy Asthma Immunol 1997;79:91–101.
INTRODUCTION In today’s demanding and strife-torn society with extraordinary emphasis on From the Section of Pediatric Allergy and Immunology, Mayo Clinic and Mayo Foundation, Rochester, Minnesota. Publication of this article is made possible through an educational grant from Zeneca Pharmaceuticals. Nothing in this publication implies that Mayo Foundation endorses any products of Zeneca Pharmaceuticals. Received for publication April 17, 1997. Accepted for publication in revised form June 22, 1997.
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perfectionism and overachievement, it is not surprising that nonorganic disorders are being encountered more frequently. Although psychosomatic and somatoform disorders were recognized and described extensively many decades ago,1,2 little mention of them is found in contemporary medical literature. Modern concepts regarding conversion disorders (characterized by the loss of or alteration in physical function resembling a physical disorder in
the absence of organ dysfunction) stem from Freud.1 Their evolution was explained by the existence of an unconscious intrapsychic conflict resulting from a past traumatic event. The person, unable to confront this conflict consciously, would “convert” this psychic energy into a more readily expressed physical symptom, often with symbolic significance. This would allow the person to rid the mind of the distressing affect (the primary gain), yet allow its partial expression in a disguised manner; therefore, it was believed that conversion disorders were a form of maladaptive interpersonal communication. In comparison, the term “psychosomatic disorder,” first used by Johann Christian Heinroth in 1818, refers to disorders characterized by demonstrable organic lesions such as asthma or pathophysiologic processes such as migraine with exacerbations temporally related to “psychologically meaningful environmental stimuli,” for example, personal stressors. The respiratory disorders described below, whether believed to be manifestations of a conversion disorder or psychosomatic (somatoform) illness are encountered and misdiagnosed too often to be ignored. This review is the result of our recent evaluation of several patients with symptoms suggestive of nonorganic disorders. PAROXYSMAL SNEEZING Sneezing, or sternutation, is a normal physiologic response directed toward removal of irritants from the nasal mucosa. The sneezing reflex, initiated by irritation of sensory nerve endings of the trigeminal nerve,3 is well described in the literature. Intractable paroxys-
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mal sneezing is a well-recognized entity first described by Shilkret.4 Since that time, this condition, aptly defined as the “sudden violent sneezing of unusual frequency and duration that is generally psychogenic and is resistant to usual treatment,”5 has been mentioned in several clinical vignettes. To date, 29 cases have been recorded,6 25 of which presented in the context of stressors such as sexual abuse7 and obsessive premorbid personality,8 to name two. Symptoms in the other four patients were attributed to organic processes, including temporal lobe epilepsy3 (the occurrence of sneezing was described as part of the preictal aura by Gowers9 and later confirmed by the electrocorticographic stimulation studies of Penfield and Kristiansen10), tubercular cervical lymphadenitis11 triethanolamine sensitivity,12 and multifactorial causes.13 Other causes that have been implicated include allergies, local nasal disturbances (eg, septal deviation),11 light,14 sexual excitement,15 chilling, pregnancy,13 organic neurologic processes such as “mild encephalitis,” and poliomyelitis.11 In an attempt to explain the pathogenesis of paroxysmal sneezing, Crue16 postulated that the trigeminal system acts as a central neuronal pool in the brain stem and upper spinal cord which is influenced by input from
higher centers (ie, the cerebral cortex and cerebellum, thus accounting for the association with psychogenic stresses), peripheral sensory stimuli (explaining symptoms related to light exposure), and parasympathetic overflow (as in orgasmic situations). Little is known about the incidence of paroxysmal sneezing in the community because most of the information is limited to isolated case reports; however, most of the affected persons are adolescent females.6 Patients present with a history of persistent and recurrent bouts of sneezing (up to 30 to 100 per minute), with each bout often lasting for hours to days. The sneeze, or pseudosneeze,17 is described as having an abbreviated inspiratory phase, short nasal grunting sound, and little or no aerosolization of mucosal secretions. Typically, during these episodes, patients keep their eyes open, which is unusual in a physiologic sneeze reflex; they do not appear distressed and are able to converse, eat, and carry on their activities without hindrance. Furthermore, symptoms have not been documented to persist during sleep. Precipitating stressors are frequently identified, and a history of psychopathology often surfaces. Physical examination findings are noncontributory, but occasional excessive tearing4 and nasal mucosal or tur-
Figure 1. Respiratory tracing from young woman with organic heart disease (mitral stenosis). The tracing, which reads from left to right, covers a 10-minute period. No deep respirations or sighs are present (from White PD, Hahn RG23).
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binate erythema4,17,18 have been noted. Laboratory investigations such as rhinoscopy, allergy skin testing, sinus radiography, complete blood cell count, and total IgE are invariably normal. Response to pharmacologic treatment, expectedly, is poor. Treatments that have been tried, with inconsistent benefit, have included nasal and oral decongestants, epinephrine given subcutaneously, corticosteroids given orally and intranasally, antihistamines, and even cauterization of the “Fleiss nasogenital area” of the nasal mucosa.15 Reports have also described short-term relief in patients with the use of oral diazepam19 and haloperidol.8 Therapy directed at understanding and attempting to resolve underlying unconscious conflicts through insight is inherently more successful, with at least shortterm benefit. Psychotherapy,5,17 operant conditioning,4,20 hypnosis,4,5,18,21 and family therapy have all been used with success, although the lack of controlled trials and long-term follow-up have not allowed assessment of lasting benefit. SIGHING DYSPNEA The term “sighing dyspnea,” first used by Maytum and Willius22 in 1934, refers to a well-recognized medical entity of the early 1900s.23 Perhaps because of the lack of recognition of its existence in more recent times, the only description of this uncommon condition in contemporary medical literature is in a 1993 review by Perin et al.24 Dyspnea (a subjective feeling of distress or difficulty in breathing) associated with a sigh (an audible inspiration and expiration under the influence of some emotion)25 was described by Steell26 in 1906 and believed to be related to fatty degeneration of the heart muscle. The suggestion that “sighing is never due primarily to heart disease but always to fatigue or nervousness or other such factor” came from the study by White and Hahn,23 who inquired about this symptom in 650 persons. They found frequent sighing in 19% of a control group, in 3% of patients with organic heart dis-
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Figure 2. Respiratory tracing from a middle-aged woman with marked effort syndrome. Frequent and deep sighing respirations, characteristic of sighing dyspnea, are observed (from White PD, Hahn RG23).
ease, in 80% of patients with “effort syndrome,” and in 74% of those with coexistent organic heart disease and effort syndrome (Figs 1, 2). Patients with sighing dyspnea are usually in the 2nd to 4th decade of life, although occasionally it has been described in children as young as 5 years. Women in the active years of life22 were the largest proportion of cases in the original reports.23 Maytum and Willius22,27 gave a vivid description of symptoms of sighing dyspnea: patients complain of shortness of breath associated with difficulty in “getting their breath,” inability in obtaining a “satisfying breath” or “full breath,” or “breathing below a certain level.” Symptoms usually occur in attacks, although in some cases mild dyspnea may be present all the time. A typical attack, which can last for up to several hours, consists of a series of deep sighing respirations that are greatly increased in depth but without a change in respiratory rate. Patients appear distressed, frequently using the accessory muscles of respiration. Associated symptoms include a feeling of weight, constriction, or oppression in the chest, aerophagia, belching, palpitations, and ill-defined chest pains. During severe attacks, patients may appear extremely ill and anxious,
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grasp their throat or chest, and run to a window to abort the sensation of suffocation. At times, they must make several attempts before they are able to obtain a deep breath, which when done affords marked relief. In prolonged attacks, tetanic spasms have also been reported.22,27 Attacks usually appear at rest and rarely with physical activity, unlike organic heart disease. In nearly all patients, frequent sighing and yawning are noted between attacks. Although these patients complain of shortness of breath, there is no true dyspnea. When asked to try to reproduce their “dyspnea,” they sigh and call the examiner’s attention to this “difficulty,” emphasizing the feeling of satisfaction that follows when a full breath is taken. Symptoms are often preceded by stressful situations and tend to recur in the presence of new stressors. Physical examination and laboratory findings are normal, and the diagnosis is made on the basis of the history and observation of the characteristic sighing pattern (Figs 1, 2). The etiopathogenic trigger for sighing dyspnea initially was believed to be mild anoxemia22 that resulted from increased nervous and muscular tension related to strain and fatigue, causing involuntary restriction of respiratory excursions. Fatigue of the respiratory center28 was thought to en-
sue and to produce a sensation of dyspnea. Organic causes reportedly associated with sighing dyspnea—and which should be ruled out by appropriate investigations if additional signs and symptoms indicate their presence—include asthma, coronary artery disease, acute left ventricular failure,22 thyroid disorders,23 and sinusitis.24 Reassurance and counseling regarding the benign nature of this condition, relaxation techniques, and identification and possible resolution of emotional stressors are an intrinsic part of the healing process. No results of longterm follow-up studies are available to assess the natural history of this disorder. HABIT COUGH Although cough is a common symptom that serves an important function, its persistence is often a sign of an underlying abnormality. Also, cough itself may result in complications, both physical (laryngeal irritation, vomiting, rib fractures, pneumothorax and pneumomediastinum, and rupture of the rectus abdominus) and emotional (exhaustion, sleep deprivation, and social withdrawal). A study of 72 infants and children with intractable cough referred to an otolaryngology clinic revealed the five commonest causes to be cough-variant asthma (31.9% of patients), sinusitis (24%), gastroesophageal reflux (15%), aberrant innominate artery (12.5%), and psychogenic (10%).29 Table 1 lists some of the common causes of chronic cough in children. Psychogenic, habit, or involuntary cough has been reported almost exclusively in children and adolescents30 and is without any striking predilection for sex. The cough is described as a croupy cough produced by a sharp intake of air and followed by a short explosive expiration, resulting in a characteristic barking or honking sound. Patients are described as being happy and unperturbed by their symptoms. The cough is more disturbing to parents and is disruptive in school, resulting in frequent school absenteeism. Typically, the cough starts after an up-
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Table 1. Etiology of Chronic Cough in Children Etiology Cough-variant asthma Infectious Congenital
Psychogenic Traumatic Irritants Otologic Neoplastic
Cardiac
Clinical Condition Sinusitis, pertussis, parapertussis, tuberculosis, diphtheria Vascular rings, bronchogenic cyst, tracheomalacia, subglottic stenosis, tracheal web or stenosis, gastroesophageal reflux, vocal cord paralysis, tracheoesophageal fistula Foreign body aspiration Smoke exposure, environmental pollutants Foreign body in ear canal, cerumen impaction Laryngeal hemangioma, vocal cord papillomas, bronchial adenomas, mediastinal adenopathy Congestive heart failure with pulmonary edema
Modified from Holinger LD, Sanders AD.29 (Used with permission of the American Laryngological, Rhinological and Otological Society.)
per respiratory illness. After resolution of the acute illness, an isolated and explosive daytime cough persists, with cessation of symptoms during sleep and pleasurable social activities. Patients complain of “something in their throat,” or a “tickle,” and sometimes adopt a chin-on-chest posture, with a hand held against the throat as if to support the larynx (Fig 3)31 The cough persists for various periods of time, especially in the absence of appropriate intervention, with frequent exacerbations.32 Symptoms worsen on contact with medical professionals and in the presence of other people and can be reproduced in the physician’s office on request. Psychosocial behaviors may be identified, ranging from school phobia33 to attention seeking and anxiety. Rarely has coexistent psychopathology been formally diagnosed. The results of physical examination, pulmonary function tests, chest radiography, allergy testing, and bronchoscopy are normal. Response to antitussive medications has been dismal. Treatment in mild cases is usually simple: explanation of the nature of the problem33 (reassuring patients that they are not voluntarily faking the symptoms and that there is no serious underlying organic disease), with dis-
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cussion of potential solutions to underlying stressors. The cough should be explained to the patient as arising from a vicious cycle of an initial irritant that is no longer present but which set up a pattern of coughing that is causing more irritation.32 For more persistent and severe symptoms, innovative behavioral techniques have been tried, including nasal breathing while keeping a flat button in the mouth (to avoid dryness of the throat, which was thought to trigger the cough) (an approach of uncertain safety because of the potential risk of aspiration of the button);34 biofeedback;30 suggestion therapy32 (explanation of symptoms and repeated expression of confidence by the therapist through verbal suggestion to suppress the cough while using a distractor, most typically dilute nebulized saline); and aversive techniques,35,36 the most
Figure 3. Characteristic “chin-on-chest” posture of patient with habit cough (from Weinberg EG31).
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publicized of which is the bed sheet method. A bed sheet is wrapped tightly around a patient’s chest, with a double knot tied over the sternum, ostensibly to provide support to the weakened chest muscles believed to be responsible for the persistent cough. The sheet is worn at all times by the patient, with additional intervention in the form of commands such as “stop it” or “no more cough” if the patient makes an effort to start coughing. The true rationale behind the intervention is explained to the parents but is concealed from the patient. Cohlan and Stone35 reported success in 33 of 35 children who received treatment with this approach. Data on long-term follow-up of patients receiving such forms of suggestion therapy are scant but nevertheless reassuring. Lokshin et al32 reported nine patients treated with verbal suggestive therapy, for whom longterm follow-up was available in seven (median follow up, 3.6 years). Of these seven patients, six had complete resolution of their symptoms after a single session, and minor self-controlled symptoms persisted in the other patient. VOCAL CORD DYSFUNCTION The clinical syndrome of vocal cord dysfunction, described for the first time in the mid-1800s,37–39 and also referred to as “pseudoasthma,” “hysterical croup,” and “nonorganic acute upper airway obstruction,” is the most studied entity in this group of disorders. Interest in vocal cord dysfunction was rejuvenated after the case report by Patterson et al40 in 1974. No better description of this can be found than that by Osler,41 “spasm of the muscles may occur with violent inspiratory efforts and great distress, and may even lead to cyanosis . . .. Extraordinary cries may be produced, either inspiratory or expiratory.” Paradoxical vocal cord motion, the laryngoscopic equivalent of vocal cord dysfunction, refers to the abnormal movement of the vocal cords, which are 180 degrees out of phase with the respiratory cycle, that is, adduction of the cords on inspiration with either ab-
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duction (in most cases) or adduction during expiration.42 Normal persons have a fairly constant glottic opening, with mild increase in the aperture during inspiration, whereas persons with asthma have been noted to have mild inspiratory and mid-expiratory narrowing of the glottis and supraglottis, providing the so-called laryngeal positive end-expiratory pressure, which is thought to be of physiologic significance in preventing atelectasis.43,44 Little is known about the epidemiology of vocal cord dysfunction. A study from the National Jewish Center for Immunology and Respiratory Medicine found vocal cord dysfunction in 9.6% of 50 consecutive patients referred for “refractory” asthma.45 An additional 32.6% had coexistent asthma and vocal cord dysfunction. A consistent sex bias has been noted, with more women affected, but this difference is less significant in children.46 Typically, vocal cord dysfunction is diagnosed in adolescent women with medical backgrounds42,45,47 or histories of asthma or psychiatric illness.47 One of the earliest mentions of vocal cord dysfunction in the psychiatric literature is that by Janet: “hysterical asphyxia, resulting from various disturbances in the respiratory mechanism, does not seem to us to be capable, in general, of bringing about death. A moment comes when as-
phyxia brings on fainting; that is, the arrest of the higher functions of the brain, and the respiration, being no longer impeded by these higher functions, is restored owing to the automatism of the bulb.”48 In a literature review of 48 patients with vocal cord dysfunction, 94% had a psychiatric disorder, the most common being conversion disorder (52%).46 A psychopathologic cause is found less frequently in children than adults, but there is a greater incidence of familial stressors (emphasis on competitive sports and overachievement). In one study, a 30% prevalence of sexual abuse in childhood was also noted in women with vocal cord dysfunction.49 This further supports the above-mentioned contention of an unconscious intrapsychic conflict of a sexual nature as a possible trigger. Physicians managing patients with vocal cord dysfunction recognize the important contributory effect of psychologic stresses on clinical symptoms. Psychiatrists differ in their formal categorization of vocal cord dysfunction, with some classifying it as a conversion disorder40,50 on the basis of the Freudian psychodynamic concept of intrapsychic conflict referred to above and others considering it as a psychosomatic illness46,51 due to the undeniable presence of an organic pathologic process, that is, paradoxical
Figure 4. Flow volume loops demonstrating pattern of A, extrathoracic obstruction with blunting of the inspiratory (Insp) component as seen in vocal cord dysfunction and, B, intrathoracic obstruction with a plateau in the expiratory (Exp) phase of the tracing typical of asthma. RV, residual volume; TLC, total lung capacity (from Kryger M, Bode F, Antic R, Anthonisen N. Diagnosis of obstruction of the upper and central airways. Am J Med 1976;61:85–93).
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Table 2. Comparison of Features of Asthma and Vocal Cord Dysfunction Feature Age Sex Premorbid status
Commonest symptom Symptom attributes
Precipitating factors
Physical examination Respiratory distress Neck position Auscultation Investigations Chest radiograph Eosinophilia A-a gradient Spirometry Laryngoscopy Therapy Acute
Long-term
Asthma Not specific Male and female equally affected Not specific
Wheezing Worse at night Expiratory difficulty Rapid onset and gradual resolution No decrease with distraction Infections Exercise Cold weather Allergies Stress
Adolescents Females affected more frequently than males Higher incidence of psychopathology, sexual abuse, personality disorder Stridor Least at night Inspiratory distress Rapid onset and rapid resolution Easily distractible Stress Unusual triggers—sports, odors, medical personnel
Universal Extension Expiratory rhonchi
Variable Flexion Inspiratory stridor
Hyperinflation May be present Sometimes increased Prolonged expiration (decreased FEV1) Normal/mild glottic narrowing
Normal Absent Normal Prolonged inspiratory phase (normal FEV1) Paradoxical vocal cord motion
Supportive Nebulized -agonists Corticosteroids Inhaled steroids Lifestyle modification
Relaxation therapy Inhaled helium
vocal cord motion. There also is growing concern among physicians about the possibility of an occult and purely organic basis for symptoms in a subset of patients. In 1987, Patton et al52 reported on a 61-year-old woman with paradoxical vocal cord motion who had a posterior fossa arachnoid cyst and whose symptoms resolved after surgical removal of the cyst. In 1982, Kellman and Leopold53 described a patient with vocal cord dysfunction in the setting of a possible brain stem abnormality manifested by several cranial nerve palsies. They also described a patient, presented to them by another physician, with paradoxical vocal cord
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Vocal Cord Dysfunction
Speech therapy Reassurance Psychiatric evaluation
motion and persistent aspiration pneumonia due to an organic brain syndrome occurring after viral encephalitis. Supporting the above hypothesis is an older study by Haglund et al,54 in which 18 patients with a provisional diagnosis of functional aphonia/dysphonia (based on normal findings on neurologic and phoniatric examinations) were reevaluated. Four were found to have incomplete closure of the posterior part of their vocal cords during phonation, five others had an oval-shaped adduction insufficiency, and the other nine had complete adduction of the cords. Electromyographic studies of the vocal and cricothyroid
muscles revealed the presence of neurogenic lesions in ten patients in the form of conduction blocks and giant potentials. These patients probably represent a minor subset, but one that must always be kept in mind during the evaluation process. Patients with vocal cord dysfunction present with symptoms similar to those in asthma and croup. Stridor (inspiratory, expiratory, or biphasic) is the commonest symptom (83% of patients),46 followed by wheezing. A history of a preceding upper respiratory illness is not infrequent and is the trigger that directs the patient’s attention toward the larynx.53 Older patients can describe the wheezing as “coming from the throat.”42,45,55 Twenty-one percent of the patients have concomitant dysphonia, with occasional reports of dysphagia45 and multiple somatoform complaints.40 Distraction maneuvers such as coughing,40,56 panting,57 phonation, and verbal suggestion techniques have been associated with a decrease in respiratory distress. Symptoms have been brought on by unusual triggers, including stress,40 odors,45 competitive sports, and even the presence of medical personnel in the patient’s room. The symptoms rarely occur during sleep,45 have a rapid onset and equally rapid resolution, and are characteristically unresponsive to medications.47 Most patients have been through several hospitalizations, emergency room visits,40 and, occasionally, multiple intubations and tracheostomies.45,46,58 It is not unusual for patients to have been given long-term treatment with oral corticosteroids for a presumed diagnosis of steroid-dependent asthma, with consequent adverse side effects.46 On examination, the degree of respiratory distress varies, from some patients reportedly being in an inappropriate panic state to others showing la belle indifference.47,56 An inappropriately increased respiratory rate (for the presumed degree of airway obstruction) has been noted in several reports, with some patients frankly hyperventilating. Patients frequently appear to flex their neck59 during the attack, a
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maneuver that is not physiologic because the posture associated with the greatest diameter of the larynx is extension. Patients are rarely cyanotic, and there are only a few reports of patients in acute respiratory failure. Chest auscultation reveals stridor or wheezing that, if listened to carefully, will be heard best over the larynx, with symmetric distal transmission.46 Laboratory findings are normal. Important negative findings include absence of hyperinflation on chest radiography (present in 73% of patients with asthma),55 absence of eosinophilia,45 and an arterial blood gas result of normal PO2 and respiratory alkalosis due to hyperventilation. There have been reports occasionally of hypoxemia,46 but with a normal A-a gradient45,55 (reflecting lack of ventilationperfusion mismatch), and rarely of hypercarbia and acidosis.47,58 Spirometry often helps in differentiating asthma from vocal cord dysfunction. Unlike asthma, where there is blunting of the expiratory loop of the flow volume curve (because of expiratory bronchial constriction), in vocal cord dysfunction, it is in the inspiratory part of the flow volume curve that a plateau is seen, indicating the presence of an extrathoracic airway obstruction that causes difficulty with getting a breath “in” rather than expelling it (Fig 4). This is not specific for vocal cord dysfunction and may be seen in other pathologic conditions that cause upper airway obstruction. Conflicting results have been reported with the use of methacholine46 during spirometry, but most patients have a negative response. When a patient with paradoxical vocal cord motion occasionally is found to have a positive methacholine challenge, it has been attributed to coexistent asthma45 and expectedly adds to the confusion. Some of the differentiating features between asthma and vocal cord dysfunction are listed in Table 2. The reference standard for diagnosing vocal cord dysfunction is laryngoscopic visualization of paradoxical motion of the vocal cords (Fig 5).42,45 This should be performed during an
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Figure 5. Laryngoscopic appearance of the vocal cords in vocal cord dysfunction. The characteristic triangular-shaped chink is seen in the posterior part of the glottic aperture during inspiration (from Rusakow LS, Blager FB, Barkin RC, White CW. Acute respiratory distress due to vocal cord dysfunction in cystic fibrosis. J Asthma 1991;28:443– 6).
acute episode (because it may be episodic)45 without sedation, anesthesia, or inhaled ipratropium,47 all of which may interfere with the findings. As mentioned above, the characteristic finding is adduction of the vocal cords during inspiration, creating a diamondshaped chink in the glottic aperture posteriorly.42,45,50 This cannot be induced voluntarily by a patient.60 Because of the anecdotal nature of the literature, it is not surprising that various types of pharmacotherapy have been tried in an uncontrolled setting with equally varying results. When a physician is reasonably certain of the diagnosis, acute episodes can be treated most effectively with supplemental oxygen, reassurance, and relaxation techniques. Assisting the patient in extending the neck to increase airway diameter often helps in reducing distress. Anxiolytics may have some role in the acute setting. Attacks may also be aborted by administering continuous positive airway pressure or intermittent positive pressure ventilation61 by bag and mask, asking the patient to relax and to give up voluntary control of breathing. This is believed to work by helping reduce aberrant vagal discharges from higher neurologic centers to the vocal cords, which may be the responsible factor for the paradoxical motion of the vocal cords. Use of inhaled helium and oxygen mixtures (80% helium plus 20%
oxygen) is recommended for more severe attacks42 and can easily be administered using a conventional nebulizer. Because helium has a lower density than oxygen, it decreases airflow turbulence, relieving the patient’s sense of dyspnea. Rarely has intubation or tracheostomy been thought to be essential.46 Important tenets of maintenance therapy include stopping treatment with unnecessary and potentially harmful medications such as corticosteroids and using speech therapy42,45 and formal psychiatric counseling. The goal of speech therapy is to decrease laryngeal tone, which can be achieved by training patients in techniques of abdominal breathing, and to encourage them to concentrate on the expiratory phase of the respiratory cycle during an acute episode. Additional techniques to reduce laryngeal tone and to relieve upper airway obstruction, for example, biofeedback,45 hypnosis,62 and panting, have been used with success. Counseling to help patients identify and cope with underlying psychosocial conflicts remains the cornerstone of long-term therapy. Even if underlying psychodynamic conflicts cannot be avoided, patients can be taught to express their emotions in a more direct and adaptive manner42 and, thereby, eliminate the need for the unconscious to “convert” these conflicts into maladaptive physical symp-
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toms. Formal psychiatric intervention with psychotherapy and psychotropic medications may be necessary to deal with a frank psychopathologic condition.40,42,45,60 The results of short-term follow-up of patients with vocal cord dysfunction appear to indicate an excellent response to intervention, although few data are available on long-term outcome. In the only study to address this issue, three patients with vocal cord dysfunction and asthma were followed over a 10-year period and all proved refractory to therapy,63 with persistent adduction of the vocal cords on inspiration; one of the patients needed tracheostomy. Whether this is representative of the entire group of patients with vocal cord dysfunction or reflects the outcome of patients at the severe end of the clinical spectrum is not known.64 SUMMARY The frequent association of respiratory disorders with psychiatric disorders is not surprising in view of the bidirectional interaction of breathing with emotions and behavior.65 Breathing— being the mark of the first sign of life— has fundamental roots in the human psyche. Little wonder that disorders involving the mechanics of breathing are frequently the only sign of psychogenic conflicts. In general, nonorganic disorders are diagnosed more frequently in adolescent women when the stress of puberty is paramount. A history of psychiatric disorders with temporally related stressors is frequently obtained. These disorders mimic organic conditions, for which they are frequently mistaken, with consequent overinvestigation and expensive, unnecessary treatment. Most of them can be diagnosed or suspected on the basis of a meticulously taken history and physical examination without recourse to expensive investigations. Treatment, which should be directed toward the primary initiating event, involves counseling and psychiatric evaluation. Short-term benefits are apparent, but long-term follow-up is needed to assess lasting improvement.
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Our goal is to increase awareness among physicians about the existence of these disorders, to enable easy diagnosis, to avoid extensive and inappropriate investigations, and to make appropriate referrals for speedy initiation of treatment. REFERENCES 1. Barsky AJ. Somatoform disorders. In: Kaplan HI, Sadock BJ, eds. Comprehensive textbook of psychiatry/V. vol. 1, 5th ed. Baltimore: Williams & Wilkins, 1989:1009 –27. 2. Kaplan HI, Oken D, Lipsitt DR, et al. Psychological factors affecting physical condition (psychosomatic disorders) (Parts 1–14). In: Kaplan HI, Sadock BJ, eds. Comprehensive textbook of psychiatry/V. vol. 2, 5th ed. Baltimore: Williams & Wilkins, 1989: 1155–279. 3. Kofman O. Paroxysmal sneezing. Can Med Assoc J 1964;91:154 –7. 4. Shilkret HH. Psychogenic sneezing and yawning. Psychosom Med 1949; 11:127– 8. 5. Kaplan MJ, Lanoff G. Intractable paroxysmal sneezing. A clinical entity defined with case reports. Ann Allergy 1970;28:24 –7. 6. Keating MU, O’Connell EJ, Sachs MI. Intractable paroxysmal sneezing in an adolescent. Ann Allergy 1989;62: 429 –31. 7. Aggarwal J, Portnoy J. Intractable sneezing with a specific psychogenic origin. Ann Allergy 1986;56:345– 6. 8. Davison K. Pharmacological treatment for intractable sneezing. Br Med J (Clin Res Ed) 1982;284:1163– 4. 9. Gowers WR. Epilepsy and other chronic convulsive diseases. 2nd ed. Philadelphia: Blakiston Company, 1901:200. 10. Penfield W, Kristiansen K. Epileptic seizure patterns: a study of the localizing value of initial phenomena in focal cortical seizures. Springfield, Illinois: Charles C Thomas, 1951. 11. Shapiro SL. Paroxysmal sneezing. Eye, Ear, Nose Throat Monthly 1967; 46:1532– 8. 12. Herman JJ. Intractable sneezing due to IgE-mediated triethanoloamine sensitivity. J Allergy Clin Immunol 1983; 71:339 – 44. 13. Co S. Intractable sneezing: case report and literature review. Arch Neurol 1979;36:111–2.
14. Everett HC. Sneezing in response to light. Neurology 1964;14:483–90. 15. Everett HC. Paroxysmal sneezing following orgasm (answer). JAMA 1972; 219:1350 –1. 16. Crue B, Todd E, Carregal E, et al. Cranial neuralgia. In: Vinken P, Bruyn G, eds. Handbook of clinical neurology, vol 5. Amsterdam: North Holland Publishing Company, 1968:287. 17. Bergman GE, Hiner LB. Psychogenic intractable sneezing in children. J Pediatr 1984;105:496 – 8. 18. Murray N, Bierer J. Prolonged sneezing: a case report. Psychosom Med 1951;13:56 – 8. 19. Gervis JH. Pharmacological treatment for intractable sneezing [Letter]. Br Med J (Clin Res Ed) 1982;284:1560. 20. School girl still sneezes, but feels fine. Miami: AP Newspaper Report; 1967. Cited by Gallia LJ, Roscoe G. Intractable sneezing. Trans Pa Acad Ophthalmol Otolaryngol 1981;34:164 – 8. 21. Elkins M, Milstein JJ. Hypnotherapy of pseudo-sneezing: a case report. Am J Clin Hypnosis 1962;4:273–5. 22. Maytum CK, Willius FA. Abnormal respiration of functional origin. Proc Staff Meet Mayo Clin 1934;9:308 –11. 23. White PD, Hahn RG. The symptom of sighing in cardiovascular diagnosis. With spirographic observations. Am J Med Sci 1929;177:179 – 88. 24. Perin PV, Perin RJ, Rooklin AR. When a sigh is just a sigh . . . and not asthma. Ann Allergy 1993;71:478 – 80. 25. Stedman TL. Stedman’s medical dictionary: a vocabulary of medicine and its allied sciences, with pronunciations and derivations. 23rd ed. Baltimore: Williams & Wilkins, 1976. 26. Steell G. Text book on diseases of the heart. Manchester: University Press, 1906. 27. Maytum CK. Sighing dyspnea: clinical syndrome. J Allergy 1938;10:50 –5. 28. Haldane JS. Respiration. New Haven, CT: Yale University Press, 1922:427. 29. Holinger LD, Sanders AD. Chronic cough in infants and children: an update. Laryngoscope 1991;101: 596 – 605. 30. Riegel B, Warmoth JE, Middaugh SJ, et al. Psychogenic cough treated with biofeedback and psychotherapy. A review and case report. Am J Phys Med Rehabil 1995;74:155– 8. 31. Weinberg EG. ‘Honking’: psychogenic cough tic in children. S Afr Med J 1980;57:198 –200.
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32. Lokshin B, Lindgren S, Weinberger M, Koviach J. Outcome of habit cough in children treated with a brief session of suggestion therapy. Ann Allergy 1991;67:579 – 82. 33. Shuper A, Mukamel M, Mimouni M, et al. Psychogenic cough. Arch Dis Child 1983;58:745–7. 34. Bernstein L. A respiratory tic: “the barking cough of puberty.” Report of a case treated successfully. Laryngoscope 1963;73:315–9. 35. Cohlan SQ, Stone SM. The cough and the bedsheet. Pediatrics 1984;74:11–5. 36. Lavigne JV, Davis AT, Fauber R. Behavioral management of psychogenic cough: alternative to the “bedsheet” and other aversive techniques. Pediatrics 1991;87:532–7. 37. Dunglison RD. The practice of medicine. Philadelphia: Lea and Blanchard, 1842:258. 38. MacKenzie M. Use of laryngoscopy in diseases of the throat. Philadelphia: Lindsey and Blackeston, 1869: 246 –50. 39. Flint A. Principles and practice of medicine. Philadelphia: Henry C. Lea, 1868:267– 8. 40. Patterson R, Schatz M, Horton M. Munchausen’s stridor: non-organic laryngeal obstruction. Clin Allergy 1974;4:307–10. 41. Osler W. The principles and practice of medicine, designed for the use of practitioners and student of medicine. 6th ed. New York: D. Appleton, 1906: 1081. 42. Martin RJ, Blager FB, Gay ML, Wood RP II. Paradoxic vocal cord motion in presumed asthmatics. Semin Respir Med 1986;8:332–7. 43. Collett PW, Brancatisano T, Engel LA. Changes in the glottic aperture during bronchial asthma. Am Rev Respir Dis 1983;128:719 –23.
44. O’Hollaren MT. Masqueraders in clinical allergy: laryngeal dysfunction causing dyspnea. Ann Allergy 1990; 65:351– 6. 45. Brugman SM, Newman K. Vocal cord dysfunction. Medical/Scientific Update 1993;11:1– 6. 46. Lacy TJ, McManis SE. Psychogenic stridor. Gen Hosp Psychiatry 1994;16: 213–23. 47. Niven RM, Roberts T, Pickering CA, Webb AK. Functional upper airways obstruction presenting as asthma. Respir Med 1992;86:513– 6. 48. Janet P. The major symptoms of hysteria; fifteen lectures given in the medical school of Harvard University. 2nd ed. New York: Hafner Publishing Company, 1965:248. 49. Freedman MR, Rosenberg SJ, Schmaling KB. Childhood sexual abuse in patients with paradoxical vocal cord dysfunction. J Nerv Ment Dis 1991; 179:295– 8. 50. Christopher KL, Wood RP II, Eckert RC, et al. Vocal-cord dysfunction presenting as asthma. N Engl J Med 1983; 308:1566 –70. 51. Maricle R. Vocal-cord dysfunction presenting as asthma [Letter]. N Engl J Med 1983;309:1190 –1. 52. Patton H, DiBenedetto R, Downing E, et al. Paradoxic vocal cord syndrome with surgical cure. South Med J 1987; 80:256 – 8. 53. Kellman RM, Leopold DA. Paradoxical vocal cord motion: an important cause of stridor. Laryngoscope 1982; 92:58 – 60. 54. Haglund S, Knutsson E, Martensson A. An electromyographic study of the vocal and cricothyroid muscles in functional dysphonia. Acta Otolaryngol (Stockh) 1974;77:140 –9. 55. Downing ET, Braman SS, Fox MJ, Corrao WM. Factitious asthma. Phys-
56. 57. 58.
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61. 62. 63.
64.
65.
iological approach to diagnosis. JAMA 1982;248:2878 – 81. Neel EU, Posthumus DL. Nonorganic upper airway obstruction. J Adolesc Health Care 1983;4:178 –9. Pitchenik AE. Functional laryngeal obstruction relieved by panting. Chest 1991;100:1465–7. Couser JI Jr, Berman JS. Respiratory muscle fatigue from functional upper airway obstruction. Chest 1989;96: 689 –90. Lund DS, Garmel GM, Kaplan GS, Tom PA. Hysterical stridor: a diagnosis of exclusion. Am J Emerg Med 1993;11:400 –2. Heiser JM, Kahn ML, Schmidt TA. Functional airway obstruction presenting as stridor: a case report and literature review. J Emerg Med 1990;8: 285–9. Collett PW, Brancatisano T, Engel LA. Spasmodic croup in the adult. Am Rev Respir Dis 1983;127:500 – 4. Smith MS. Acute psychogenic stridor in an adolescent athlete treated with hypnosis. Pediatrics 1983;72:247– 8. Hayes JP, Nolan MT, Brennan N, FitzGerald MX. Three cases of paradoxical vocal cord adduction followed up over a 10-year period. Chest 1993; 104:678 – 80. Patterson DL, O’Connell EJ. Vocal cord dysfunction: what have we learned in 150 years? Insights Allergy 1994;9:1–12. Ley R. An introduction to the psychophysiology of breathing. Biofeedback Self Regul 1994;19:95– 6.
Request for reprints should be addressed to: Edward J O’Connell, MD Section of Pediatric Allergy & Immunology 200 First St SW Rochester, MN 55905
CME Examination No 007-008 Questions 1–20, Butani L and EJ O’Connell. 1997;79;91–101. CME Test Questions 1. Functional respiratory disorders are diagnosed most frequently in a. elderly males.
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b. preschool children. c. pregnant females. d. middle-aged executive males. e. adolescent females.
2. All the following are considered nonorganic respiratory disorders except a. vocal cord paralysis. b. paroxysmal sneezing.
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c. sighing dyspnea. d. pseudoasthma. e. habit cough. Principles of management of patients with functional respiratory disorders may include all the following except a. reassurance. b. evaluation of and coping with potential stressors. c. psychotherapy. d. extensive laboratory investigations to rule out all possible organic causes. e. meticulous history and physical examination. The following profile is shared by functional respiratory disorders except a. resolution of symptoms with distraction maneuvers. b. patients frequently have a medical background. c. abnormal premorbid personality. d. poor response to conventional medical therapy. e. continuous symptoms that significantly disrupt dayto-day functioning. The following is true of habit cough: a. has a characteristic honking sound. b. is paroxysmal with frequent post-tussive emesis. c. is a variant of cough-type asthma. d. responds well to antitussive medications. e. usually presents along with fever and symptoms of upper respiratory illness. The reference standard for diagnosing vocal cord dysfunction is a. paradoxical vocal cord motion on laryngoscopy. b. positive methacholine challenge test. c. abnormal chest radiographic findings. d. blunting of the inspiratory component of the flow volume loop.
7.
8.
9.
10.
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e. blunting of the expiratory component of the flow volume loop. All the following are frequently seen in vocal cord dysfunction except a. stridor. b. wheezing. c. hypoxemia. d. relief with inhaled mixture of helium-oxygen. e. flexed neck posture of patients. Therapies indicated for acute/ chronic relief in vocal cord dysfunction include the following except a. inhaled helium-oxygen mixture. b. hypnosis. c. relaxation therapy. d. speech therapy. e. vagotomy. All the following are helpful in differentiating vocal cord dysfunction from asthma except a. lack of eosinophilia. b. normal A-a gradient on blood gas analysis. c. blunting of the inspiratory component of the flow volume loop. d. normal chest radiograph. e. consistently negative methacholine challenge. Symptom characteristics in vocal cord dysfunction include a. worse at night. b. result in marked and predictable respiratory distress. c. not reduced by distracting the patient. d. relieved by bronchodilator therapy. e. may be preceded by an upper respiratory illness. Laryngoscopic findings in vocal cord dysfunction are a. abduction of the vocal cords in inspiration. b. adduction of the vocal cords on inspiration. c. vocal polyps.
12.
13.
14.
15.
d. diamond-shaped chink in the anterior aspect of the vocal cords in inspiration. e. normal vocal cord motion during exacerbations. Psychogenic sneezing characteristically a. is intermittent in nature. b. has a short inspiratory component with an explosive expiratory phase. c. is associated with a higher than normal incidence of cat allergy. d. can be diagnosed on the basis of increased IgE levels in nasal secretions. e. is associated with profound nasal erythema and irritation. Which of the following is true regarding sighing dyspnea? a. is most often seen in elderly white males. b. can be reproduced by patients in the physician’s office on request. c. improves with bronchodilator therapy. d. is caused by pulmonary edema due to congestive heart failure. e. rarely occurs at rest. The following is true of habit cough: a. it is caused by persistent pertussis infection. b. the symptoms are under the patient’s conscious and voluntary control. c. a neck radiograph is essential for diagnosis. d. it improves with biofeedback techniques. e. it has an excellent longterm resolution rate. Symptoms of vocal cord dysfunction may be manifestations of an underlying a. organic lesion. b. somatoform disorder. c. conversion disorder. d. all the above. e. none of the above.
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16. The epidemiology of vocal cord dysfunction in children differs from that in adults in a. a higher prevalence of formal psychopathology. b. a lower prevalence of familial stressors. c. a higher prevalence of dysfunctional families with emphasis on overachievement. d. none of the above. e. all the above. 17. Which of the following is inappropriate in managing patients with vocal cord dysfunction? a. referral for psychiatric evaluation. b. advocating relaxation therapy. c. aggressive trial of corticosteroids. d. reassuring patients regarding the absence of organic lesions.
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e. providing coping skills to deal with stressful situations. 18. The commonest symptom of vocal cord dysfunction is a. wheezing. b. stridor. c. cough. d. dyspnea. e. palpitations. 19. Which of the following is not true of vocal cord dysfunction? a. It may coexist with asthma. b. A positive methacholine challenge rules out the diagnosis. c. It is rarely associated with such significant distress as to need mechanical ventilation. d. Diagnosis can frequently be made on the basis of the history.
e. Paradoxical vocal cord motion is the laryngoscopic counterpart of vocal cord dysfunction. 20. Which of the following is true about functional respiratory disorders? a. They are rare in the general population. b. Diagnosis is one of exclusion. c. Psychopathology can frequently be identified in patients. d. Treatment should be relegated completely to psychiatrists. e. Sufficient long-term data exist regarding excellent long-term outlook for these patients.
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