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Cough syncope: Case presentation and review
BRIEF COMMUNICATION
Cough syncope: Case presentation and review Doftald W. Aaronson, M.D., Richard N. Rower, Roy Patterson, M.D.,* Chicago, Ill. Episodes certain suggested toward severity
M.D., and
of loss of consoiozlsness following cough are an ?lncommon complication of chronic respiratory diseases. An illustrative case is p-resented, and some pathophysiologic mechanisms nre reviewed. Treatment mzlst be dire&d the basic zlnderlying respiratory disorder as well as the reduction in the of cough.
S
yncope following episodic coughing is a syndrome first described as “laryngeal vertigo” by Charcotl in 1876. Following this description many names have been applied to the syndrome, including posttussive syncope,2 tussive syncope,3 cough syndrome,4 and cough syncope.5 A review of the eases described provides a composite clinical picture. Patients with cough syncope are most often moderately obese, middle-aged men. In one series of 290 reported cases, 97 per cent were male.5 They have generally been described as hearty, robust, large-chested, outgoing individuals with gross appetites for alcohol, tobacco, and food.6 The majority of cases described have a chronic cough frequently associated with bronchial asthma, emphysema, or chronic bronchitis. Although it is a very rare cause of death, cough syncope is a medically and socially disabling disorder. An illustrative case, observed over a period of almost 4 years, is as follows. CASE
REPORT
A 41-year-old Puerto Rican man was evaluated by the Allergy Service in December, 1964. He gave a history of episodes of cough and wheezing since childhood but had never had a serious attack of asthma until March, 1964. At that time chronic respiratory symptoms became significantly more severe with the onset of daily wheezing, shortness of breath, and the first episodes of unconsciousness following cough. A typical attack followed severe coughing. The patient’s face would become plethoric, then cyanotic, and he would From the Allergy-Immunology Section, Department of Medical School. Supported in part by the Ernest 5. Bazley Asthmatic Memorial Hospital, Chicago, Ill. Received for publication April 13, 1970. “Ernest S. B’azley Professor of Allergy and Immunology.
Medicine, Research
Northwestern Fund,
University Chicago
Wesley
360
Aaronson,
Rovner,
and Patterson
J. Allerg. December, 1970
fall to the floor while exhibiting clonic spasms or tonic movements. At this time he was apneic. During these episodes he was unable to speak. Within a minute of the apneic period and the ensuing fall, normal breathing would resume along with return to full consciousness. The patient had no recall of the events. These syncopal attacks occurred from 12 to 20 times daily from March until December, 1964, when the patient was first seen by the Allergy Service. Although mild chronic asthma was persistently present at all times, as evidenced by expiratory wheezes and rales on auscultation of the chest, there was no increase in asthma prior to the syncopal attacks, nor did the syncope seem to occur more frequently during episodes of acute asthma. Because of these syncopal seizures the patient was completely unable to work. Therapy prior to being seen by the Allergy Service had consisted of bronchodilators, phenobarbital, diphenhydramine, and rectal aminophylline. None of these had resulted in control of asthma or the syncope. There was no past history of a head injury or an established diagnosis of a convulsive disorder. He had been a heavy smoker for a long period of time and continues to smoke cigarettes at the present time, although the number used daily can not be determined. Physical examination revealed an obese, moderately dyspneic, Puerto Rican man. Blood pressure was 130/84, pulse 92 per minute and regular, and the respiratory rate was 28 per minute. Inspection of the thorax showed a marked increase in the anteroposterior diameter. Auscultation of the lungs demonstrated expiratory prolongation with inspiratory and expiratory wheezing. No abnormal findings were noted on the remainder of the physical examination. Observation and the following studies were accomplished during hospitalization in order to evaluate the syncope. The hemoglobin was 17.7 Cm. per cent, hematocrit 52 per cent, and white blood cell count 7,400 per cubic millimeter, with 55 per cent neutrophils, 2 per cent monocytes, 13 per cent eosinophils, and 30 per cent lymphocytes. The VDRL and Reiter’s complement fixation tests were nonreactive. The fasting blood sugar was 77 mg. per cent, calcium 10.6 mg. per cent, phosphorous 2.53 mg. per cent, cholesterol 270 mg. per cent, and blood urea nitrogen 13 mg. per cent. Lumbar puncture revealed cerebrospinal fluid pressure on opening of 180 mm. H,O and 120 mm. H,O on closing with normal dynamics. Cerebrospinal fluid examination showed sugar 93 mg. per cent, protein 33.8 mg. per cent, 0 red blood cells, 2 white blood cells, negative wet stain for cryptococcus, and negative culture for bacteria and fungi. Chest x-ray revealed increased radiolucency consistent with hyperaeration without other significant abnormality. Electrocardiogram was normal. Brain scan with the use of radioiodinated mercury revealed no abnormalities. The electroencephalogram, including a recording done during a syncopal episode, will be described below. Skin tests revealed significant positive reactions to house dust and mold antigens only. Bronchodilator therapy was continued, and corticosteroids were added because of the increasingly intractable asthma. Hyposensitization therapy for the aeroallergens was begun and continues to the present time. In the enusing 6 years there were frequent episodes of purulent bronchitis requiring antibiotic therapy. During the past 18 months there has been a marked diminution in the frequency of syncopal episodes, which now occur only 3 to 5 times weekly. The same degree of improvement of the asthma has not been noted, the response to therapy being very slow. He continues to have daily wheezing and frequent episodes of severe asthma.
Electroencephalogram An encephalographic tracing was obtained during a syncopal episode induced by coughing (Fig. 1). The initial control period was normal. Following the onset of the syncopal episode there was an initial slight slowing and decreased amplitude of background rhythm with generalized tonic (Fig. 1, arrow 1) and a few clonic movements (Fig. 1, arrow 3) of the extremities. No obvious EEG epileptiform activity was noted during the episode, but toward the end of the event rhythmical high amplitude slowing at 3 to 4 per second was seen over the posterior head regions (Fig. 1, arrow 9). The heart rate was seen to increase from 70
Volume Number
46 6
:ync
:op
362
Aaronson,
Rouner,
and Patterson
J. Allerg. December, 1970
to 120 per minute. Although muscle artifact obscured portions of the recording, it was apparent that electrographically there was no evidence of associated epileptiform activity such as . . spllung, spike and wave phenomena, or focal onset. The tracing was compatible with a severe syncopal episode.
COMMENT
The differential diagnosis of cough syncope does not appear to be a difficult problem. The occurrence of cough preceding every episode of loss of consciousness distinguishes this syndrome from other types of syncope. Although the clinical picture might suggest epilepsy, a number of factors tend to eliminate this diagnosis. Epilepsy in general has its onset before the age of 25, while the onset of cough syncope usually occurs at middle age. In addition, the total duration of each episode is usually only 30 to 60 seconds, and no postsyncopal residual are present. No aura preceding cough syncope has been described nor is it associated with fecal or urinary incontinence, as may be the case with epilepsy. The electroencephalogram in patients with cough syncope may vary. It has been reported to be normal, or it may show slight depression of activity. As was the case with our patient, it has also been reported as showing high amplitude slow waves seen in a rhythmical fashion at 2 to 4 per second. These variable findings depend on the suddenness and degree of anoxia or severity of the rapidly increased intracranial pressure. The more severe clinical cases have been accompanied by tonic or tonic-clonic activity clinically resembling the onset of a grand ma1 seizure, as demonstrated by our patient. Unless the patient was an epileptic with an abnormal resting electroencephalographic pattern, the tracing would reveal no evidence of epileptiform activity before, during, or after the clinical manifestations. PATHOLOGIC
PHYSIOLOGY
The mechanism of the syncopal episode following cough has been only partly explained. McIntosh and associates7 measured intracavitary (intrathoracic and intracranial) and intravascular pressures during cough. Intracranial pressures are reflected by the cerebrospinal fluid pressure. At the onset of cough an elevation in both the intracavitary and intravascular pressure occurs, the elevation of the intravascular pressure being significantly less than the increase in the intracavitary pressure. The elevated intrathoracic pressure obstructs venous return to the heart resulting in decreased cardiac output and consequently in intravascular pressure. At the end of the cough a further decrease in intravascular pressure occurs. Because of the relative increase in intracranial pressure around arteries and veins, blood may be mechanically squeezed from the intracranial vaaculature. This combined with the decrease in cardiac output may result in cerebral anoxia and resulting syncope. It is at the end of the cough paroxysm, when the arterial pressures are lowest and the extravascular pressures are greatest, that maximal cerebral anoxia occurs and syncope ensues. Kerr and Eich8 in 1961 suggested that another mechanism might more reasonably explain the underlying pathophysiology leading to the syncopal episode. In a study of patients who had loss of consciousness following voluntary cough, the
Volume Number
46 6
Cough
synoope
363
electroencephalogram in 4 of 5 showed flattening of the alpha waves and rarely detected large slow waves. They postulated that severe cough results in a “cercbra1 concussion” with sudden depolarization of cerebral cells. This resulted in electroencephalographic patterns which they found were compatible with the immediate postconeussion electrooncephalographic changes described by Meyer and Denny-Brown in 1955. TREATMENT Whatever the actual mechanism of the syncope might be, treatment must be directed toward reduction of the severity or complete prevention of the cough. The usual measures directed toward control of the underlying respiratory disease must be instituted. Tracheobronchial irritants, such as smoking, should be eliminated. In view of the fact that it is the strenuousness of the coughing effort which may be the factor which determines whether or not a syncopal episode will occur, education of the patient with the objective of teaching him consciously to restrict the intensity of his coughing effort may reduce the frequency of episodes. REFERENCES
1. Charcot,
J. M.: Statement to the societe de biologie, Nov. 19, 1867, Gaz. Med. Paris 588, 1876. Description of “la grade attaque hysterique,” Progr. Med. ‘7: 17, 1876. Flindt, M.: Laryngeal vertigo, Lancet 1: 636, 1951. McCann, W. S.: Tussive syncope, Arch. Intern. Med. 84: 845, 1949. 4: Baker, C.: The cough syndrome, faintness and loss of consciousness from coughing so-called syndrome of laryngeal vertigo, Guy% Hosp. Rep. 98: 132, 1949. 5. Kerr, A., and Derbes, V. J.: The syndrome of cough syncope, Ann. Intern. Med.
5:
3”.
1240,
Engel, McIntosh, Heart 8. Kerr, Intern. ;:
the 39:
1953.
G. L.: Fainting, ed. 2, Springfield, Ill., 1962, Charles C Thomas, Publisher. H. D., Estes, E. H., and Warren, J. V.: Cough syncope. The mechanism, J. 52: 70, 1956. A., and Eich, R. H.: Cerebral concussion as a cause of cough syncope, Med. 108: 248, 1961.
Amer. Arch.
Cough syncope: Case presentation and review Doftald W. Aaronson, M.D., Richard N. Rower, Roy Patterson, M.D.,* Chicago, Ill. Episodes certain suggested toward severity
M.D., and
of loss of consoiozlsness following cough are an ?lncommon complication of chronic respiratory diseases. An illustrative case is p-resented, and some pathophysiologic mechanisms nre reviewed. Treatment mzlst be dire&d the basic zlnderlying respiratory disorder as well as the reduction in the of cough.
S
yncope following episodic coughing is a syndrome first described as “laryngeal vertigo” by Charcotl in 1876. Following this description many names have been applied to the syndrome, including posttussive syncope,2 tussive syncope,3 cough syndrome,4 and cough syncope.5 A review of the eases described provides a composite clinical picture. Patients with cough syncope are most often moderately obese, middle-aged men. In one series of 290 reported cases, 97 per cent were male.5 They have generally been described as hearty, robust, large-chested, outgoing individuals with gross appetites for alcohol, tobacco, and food.6 The majority of cases described have a chronic cough frequently associated with bronchial asthma, emphysema, or chronic bronchitis. Although it is a very rare cause of death, cough syncope is a medically and socially disabling disorder. An illustrative case, observed over a period of almost 4 years, is as follows. CASE
REPORT
A 41-year-old Puerto Rican man was evaluated by the Allergy Service in December, 1964. He gave a history of episodes of cough and wheezing since childhood but had never had a serious attack of asthma until March, 1964. At that time chronic respiratory symptoms became significantly more severe with the onset of daily wheezing, shortness of breath, and the first episodes of unconsciousness following cough. A typical attack followed severe coughing. The patient’s face would become plethoric, then cyanotic, and he would From the Allergy-Immunology Section, Department of Medical School. Supported in part by the Ernest 5. Bazley Asthmatic Memorial Hospital, Chicago, Ill. Received for publication April 13, 1970. “Ernest S. B’azley Professor of Allergy and Immunology.
Medicine, Research
Northwestern Fund,
University Chicago
Wesley
360
Aaronson,
Rovner,
and Patterson
J. Allerg. December, 1970
fall to the floor while exhibiting clonic spasms or tonic movements. At this time he was apneic. During these episodes he was unable to speak. Within a minute of the apneic period and the ensuing fall, normal breathing would resume along with return to full consciousness. The patient had no recall of the events. These syncopal attacks occurred from 12 to 20 times daily from March until December, 1964, when the patient was first seen by the Allergy Service. Although mild chronic asthma was persistently present at all times, as evidenced by expiratory wheezes and rales on auscultation of the chest, there was no increase in asthma prior to the syncopal attacks, nor did the syncope seem to occur more frequently during episodes of acute asthma. Because of these syncopal seizures the patient was completely unable to work. Therapy prior to being seen by the Allergy Service had consisted of bronchodilators, phenobarbital, diphenhydramine, and rectal aminophylline. None of these had resulted in control of asthma or the syncope. There was no past history of a head injury or an established diagnosis of a convulsive disorder. He had been a heavy smoker for a long period of time and continues to smoke cigarettes at the present time, although the number used daily can not be determined. Physical examination revealed an obese, moderately dyspneic, Puerto Rican man. Blood pressure was 130/84, pulse 92 per minute and regular, and the respiratory rate was 28 per minute. Inspection of the thorax showed a marked increase in the anteroposterior diameter. Auscultation of the lungs demonstrated expiratory prolongation with inspiratory and expiratory wheezing. No abnormal findings were noted on the remainder of the physical examination. Observation and the following studies were accomplished during hospitalization in order to evaluate the syncope. The hemoglobin was 17.7 Cm. per cent, hematocrit 52 per cent, and white blood cell count 7,400 per cubic millimeter, with 55 per cent neutrophils, 2 per cent monocytes, 13 per cent eosinophils, and 30 per cent lymphocytes. The VDRL and Reiter’s complement fixation tests were nonreactive. The fasting blood sugar was 77 mg. per cent, calcium 10.6 mg. per cent, phosphorous 2.53 mg. per cent, cholesterol 270 mg. per cent, and blood urea nitrogen 13 mg. per cent. Lumbar puncture revealed cerebrospinal fluid pressure on opening of 180 mm. H,O and 120 mm. H,O on closing with normal dynamics. Cerebrospinal fluid examination showed sugar 93 mg. per cent, protein 33.8 mg. per cent, 0 red blood cells, 2 white blood cells, negative wet stain for cryptococcus, and negative culture for bacteria and fungi. Chest x-ray revealed increased radiolucency consistent with hyperaeration without other significant abnormality. Electrocardiogram was normal. Brain scan with the use of radioiodinated mercury revealed no abnormalities. The electroencephalogram, including a recording done during a syncopal episode, will be described below. Skin tests revealed significant positive reactions to house dust and mold antigens only. Bronchodilator therapy was continued, and corticosteroids were added because of the increasingly intractable asthma. Hyposensitization therapy for the aeroallergens was begun and continues to the present time. In the enusing 6 years there were frequent episodes of purulent bronchitis requiring antibiotic therapy. During the past 18 months there has been a marked diminution in the frequency of syncopal episodes, which now occur only 3 to 5 times weekly. The same degree of improvement of the asthma has not been noted, the response to therapy being very slow. He continues to have daily wheezing and frequent episodes of severe asthma.
Electroencephalogram An encephalographic tracing was obtained during a syncopal episode induced by coughing (Fig. 1). The initial control period was normal. Following the onset of the syncopal episode there was an initial slight slowing and decreased amplitude of background rhythm with generalized tonic (Fig. 1, arrow 1) and a few clonic movements (Fig. 1, arrow 3) of the extremities. No obvious EEG epileptiform activity was noted during the episode, but toward the end of the event rhythmical high amplitude slowing at 3 to 4 per second was seen over the posterior head regions (Fig. 1, arrow 9). The heart rate was seen to increase from 70
Volume Number
46 6
:ync
:op
362
Aaronson,
Rouner,
and Patterson
J. Allerg. December, 1970
to 120 per minute. Although muscle artifact obscured portions of the recording, it was apparent that electrographically there was no evidence of associated epileptiform activity such as . . spllung, spike and wave phenomena, or focal onset. The tracing was compatible with a severe syncopal episode.
COMMENT
The differential diagnosis of cough syncope does not appear to be a difficult problem. The occurrence of cough preceding every episode of loss of consciousness distinguishes this syndrome from other types of syncope. Although the clinical picture might suggest epilepsy, a number of factors tend to eliminate this diagnosis. Epilepsy in general has its onset before the age of 25, while the onset of cough syncope usually occurs at middle age. In addition, the total duration of each episode is usually only 30 to 60 seconds, and no postsyncopal residual are present. No aura preceding cough syncope has been described nor is it associated with fecal or urinary incontinence, as may be the case with epilepsy. The electroencephalogram in patients with cough syncope may vary. It has been reported to be normal, or it may show slight depression of activity. As was the case with our patient, it has also been reported as showing high amplitude slow waves seen in a rhythmical fashion at 2 to 4 per second. These variable findings depend on the suddenness and degree of anoxia or severity of the rapidly increased intracranial pressure. The more severe clinical cases have been accompanied by tonic or tonic-clonic activity clinically resembling the onset of a grand ma1 seizure, as demonstrated by our patient. Unless the patient was an epileptic with an abnormal resting electroencephalographic pattern, the tracing would reveal no evidence of epileptiform activity before, during, or after the clinical manifestations. PATHOLOGIC
PHYSIOLOGY
The mechanism of the syncopal episode following cough has been only partly explained. McIntosh and associates7 measured intracavitary (intrathoracic and intracranial) and intravascular pressures during cough. Intracranial pressures are reflected by the cerebrospinal fluid pressure. At the onset of cough an elevation in both the intracavitary and intravascular pressure occurs, the elevation of the intravascular pressure being significantly less than the increase in the intracavitary pressure. The elevated intrathoracic pressure obstructs venous return to the heart resulting in decreased cardiac output and consequently in intravascular pressure. At the end of the cough a further decrease in intravascular pressure occurs. Because of the relative increase in intracranial pressure around arteries and veins, blood may be mechanically squeezed from the intracranial vaaculature. This combined with the decrease in cardiac output may result in cerebral anoxia and resulting syncope. It is at the end of the cough paroxysm, when the arterial pressures are lowest and the extravascular pressures are greatest, that maximal cerebral anoxia occurs and syncope ensues. Kerr and Eich8 in 1961 suggested that another mechanism might more reasonably explain the underlying pathophysiology leading to the syncopal episode. In a study of patients who had loss of consciousness following voluntary cough, the
Volume Number
46 6
Cough
synoope
363
electroencephalogram in 4 of 5 showed flattening of the alpha waves and rarely detected large slow waves. They postulated that severe cough results in a “cercbra1 concussion” with sudden depolarization of cerebral cells. This resulted in electroencephalographic patterns which they found were compatible with the immediate postconeussion electrooncephalographic changes described by Meyer and Denny-Brown in 1955. TREATMENT Whatever the actual mechanism of the syncope might be, treatment must be directed toward reduction of the severity or complete prevention of the cough. The usual measures directed toward control of the underlying respiratory disease must be instituted. Tracheobronchial irritants, such as smoking, should be eliminated. In view of the fact that it is the strenuousness of the coughing effort which may be the factor which determines whether or not a syncopal episode will occur, education of the patient with the objective of teaching him consciously to restrict the intensity of his coughing effort may reduce the frequency of episodes. REFERENCES
1. Charcot,
J. M.: Statement to the societe de biologie, Nov. 19, 1867, Gaz. Med. Paris 588, 1876. Description of “la grade attaque hysterique,” Progr. Med. ‘7: 17, 1876. Flindt, M.: Laryngeal vertigo, Lancet 1: 636, 1951. McCann, W. S.: Tussive syncope, Arch. Intern. Med. 84: 845, 1949. 4: Baker, C.: The cough syndrome, faintness and loss of consciousness from coughing so-called syndrome of laryngeal vertigo, Guy% Hosp. Rep. 98: 132, 1949. 5. Kerr, A., and Derbes, V. J.: The syndrome of cough syncope, Ann. Intern. Med.
5:
3”.
1240,
Engel, McIntosh, Heart 8. Kerr, Intern. ;:
the 39:
1953.
G. L.: Fainting, ed. 2, Springfield, Ill., 1962, Charles C Thomas, Publisher. H. D., Estes, E. H., and Warren, J. V.: Cough syncope. The mechanism, J. 52: 70, 1956. A., and Eich, R. H.: Cerebral concussion as a cause of cough syncope, Med. 108: 248, 1961.
Amer. Arch.