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Laryngeal paresis as a presenting feature of idiopathic brachial plexopathy
Laryngeal paresis as a presenting feature of idiopathic brachial plexopathy GLYNES P. HYDE, RN, MSN, GREGORY N. POSTMA, MD, and JAMES B. CARESS, MD, Winston-Salem, North Carolina
Idiopathic brachial plexopathy (IBP) is a syndrome of shoulder pain and weakness usually seen by neurologists and orthopedic surgeons. IBP has a number of pseudonyms, including neuralgic amyotrophy, ParsonageTurner syndrome, and idiopathic brachial neuritis. The initial symptom typically seen with IBP is sudden, sharp, and throbbing pain in the shoulder girdle, followed by weakness in the scapular and proximal arm muscles. Sensory loss is usually not prominent. The pain usually subsides in 24 hours to 3 weeks, and the weakness and atrophy are recognized as the pain resolves. Weakness is maximal within 2 to 3 weeks of the onset of symptoms and is often accompanied by muscle wasting and scapular winging. Slow resolution of the weakness follows in nearly all patients, but recovery may be incomplete. Rarely, other nerves outside the brachial plexus have been involved in IBP. Vocal fold dysfunction is an extremely rare feature of IBP.
recurrent laryngeal nerve paresis. A neurologic examination found atrophy of the left deltoid and infraspinatus muscles. There was prominent right scapular winging. Using the Medical Research Council scale, deltoid strength on the right was 5– and left was 4, biceps were 5 bilaterally, and triceps on the right were 4+ and left was 5–. Infraspinatus was 3+ on the left and 5 on the right. Wrist extensors, interossei of the hands, and her lower extremities were full strength. Deep tendon reflexes and sensory examination were unremarkable. An EMG of the left upper extremity revealed a neurogenic lesion of the left upper trunk muscles in a patchy pattern with some ongoing denervation. The clinical and electrophysiologic pattern of the lesions conformed to a left upper trunk brachial plexopathy and a right long thoracic nerve lesion. The bilateral vocal fold paresis indicated additional lesions of the vagus or recurrent laryngeal nerves. Four months later, she had regained normal laryngeal function. There was also improvement in upper extremity strength.
CASE REPORT
DISCUSSION
A 24-year-old white woman was evaluated for hoarseness. She was in good health after the cesarean delivery of her first child; at 3-days postpartum, she developed bilateral proximal muscle weakness. Breathy speech developed several days later. Her dysphonia and vocal fatigue became so severe that she was unable to perform her duties as a schoolteacher. She noted difficulty drying her hair due to shoulder weakness, but she had no problems with fine dexterity. Her pain resolved with partial recovery of strength in her limbs, but because of persistent dysphonia, she presented to the otolaryngology clinic. Laryngeal examination demonstrated weakness of both vocal folds, resulting in incomplete glottal closure on phonation. Laryngeal electromyography (EMG) performed 2 months after onset showed early reinnervation of both thyroarytenoid muscles, consistent with a resolving bilateral
Although uncommon, IBP usually has a striking clinical presentation familiar to most neurologists. The shoulder pain that heralds the weakness is so characteristic that the diagnosis is easily made, unless there is a departure from the typical pattern of weakness. Most evidence suggests that the lesions involved in IBP are axonal in nature, rather than due to demyelination.1,2 The pattern of involvement favors a regional mononeuropathy multiplex, as it does not strictly respect the elements of the brachial plexus. IBP appears to have an autoimmune cause because many cases (including this one) have followed immunologic triggers, including surgery, viral syndromes, and vaccinations.2 Postpartum IBP has been described and has a similar clinical picture. Recovery in postpartum IBP is excellent, with the majority of women regaining 90% to 100% of function within 8 months.1 The diagnosis of IBP may be obscured when an isolated mononeuropathy occurs or when there are coexistent cranial neuropathies. Cases of apparent IBP with neurologic involvement apart from the brachial plexus and cranial nerves have been reported, including cases of isolated phrenic nerve dysfunction. Diaphragmatic involvement has been reported in isolation or with subtle evidence of a wider plexus lesion. These patients presented with shortness of breath as their chief complaint and initially presented
From the Department of Neurology (Ms Hyde and Dr Caress) and The Center for Voice Disorders (Dr Postma), Wake Forest University School of Medicine. Reprint requests: Dr James B. Caress, Department of Neurology, Wake Forest University School of Medicine, Medical Center Blvd, Winston-Salem, NC 27157; e-mail, [email protected]. Otolaryngol Head Neck Surg 2001;124:575-6. Copyright © 2001 by the American Academy of Otolaryngology– Head and Neck Surgery Foundation, Inc. 0194-5998/2001/$35.00 + 0 23/4/115167 doi:10.1067/mhn.2001.115167
575
576
Otolaryngology– Head and Neck Surgery May 2001
HYDE et al
to pulmonologists. Similarly, patients with hoarseness and few other neurologic abnormalities may present to an otolaryngologist. Vocal fold dysfunction has been reported on 3 occasions but never confirmed with electrodiagnostic testing. In 1975, Bradley et al3 described a patient with severe shoulder pain and hoarseness along with dysphagia. Weakness was in a predominantly upper trunk pattern, and both vocal folds were involved. Vocal dysfunction resolved in 3 months. Sanders et al4 described an individual who suffered flu-like symptoms, followed by bilateral shoulder pain and a hoarse voice. Clinical and electrophysiologic testing revealed abnormalities consistent with left upper trunk IBP. Indirect laryngoscopy demonstrated left vocal fold paralysis. The symptoms resolved in 6 months. Pierre et al5 reported a patient with right shoulder pain, hoarseness, and dysphagia. Involvement of the right side of the tongue, sternocleidomastoid, trapezius, and upper trunk innervated shoulder girdle muscles was apparent on examination, and laryngoscopy disclosed a paralyzed right vocal fold. Recovery was nearly complete at 12 months. It is of interest that our case and all 3 previously reported cases demonstrated upper trunk and more proximal involvement in conjunction with vocal fold
dysfunction. Although IBP can be a multifocal disorder, the consistency of this association suggests the origin of dysphonia may be the vagus nerve that is closer in proximity to the upper trunk rather than the recurrent laryngeal branch. This case highlights the importance of recognizing dysphonia as a possible presentation of IBP. In patients with apparent idiopathic laryngeal paresis or paralysis, we recommend that physicians search for a history of preceding or concomitant shoulder pain and briefly examine shoulder abduction, external rotation, and scapular movements in an attempt to exclude an IBP variant. Unless aspiration is present or vocal function is critical, surgical procedures should be delayed, as good recovery is expected. REFERENCES 1. Lederman RJ, Wilbourn AJ. Postpartum neuralgic amyotrophy. Neurology 1996;47:1213-9. 2. England JD. The variations of neuralgic amyotrophy. Muscle Nerve 1999;22:435-6. 3. Bradley WG, Madrid R, Thrush DC, et al. Recurrent brachial plexus neuropathy. Brain 1975;98:381-98. 4. Sanders EACM, Van den Neste VMH, Hoogenraad TU. Brachial plexus neuritis and recurrent laryngeal nerve palsy. J Neurol 1988;235:323-5. 5. Pierre PA, Laterre CE, Van Den Bergh PY. Neuralgic amyotrophy with involvement of cranial nerves IX, X, XI, and XII. Muscle Nerve 1990;13:704-7.
Idiopathic brachial plexopathy (IBP) is a syndrome of shoulder pain and weakness usually seen by neurologists and orthopedic surgeons. IBP has a number of pseudonyms, including neuralgic amyotrophy, ParsonageTurner syndrome, and idiopathic brachial neuritis. The initial symptom typically seen with IBP is sudden, sharp, and throbbing pain in the shoulder girdle, followed by weakness in the scapular and proximal arm muscles. Sensory loss is usually not prominent. The pain usually subsides in 24 hours to 3 weeks, and the weakness and atrophy are recognized as the pain resolves. Weakness is maximal within 2 to 3 weeks of the onset of symptoms and is often accompanied by muscle wasting and scapular winging. Slow resolution of the weakness follows in nearly all patients, but recovery may be incomplete. Rarely, other nerves outside the brachial plexus have been involved in IBP. Vocal fold dysfunction is an extremely rare feature of IBP.
recurrent laryngeal nerve paresis. A neurologic examination found atrophy of the left deltoid and infraspinatus muscles. There was prominent right scapular winging. Using the Medical Research Council scale, deltoid strength on the right was 5– and left was 4, biceps were 5 bilaterally, and triceps on the right were 4+ and left was 5–. Infraspinatus was 3+ on the left and 5 on the right. Wrist extensors, interossei of the hands, and her lower extremities were full strength. Deep tendon reflexes and sensory examination were unremarkable. An EMG of the left upper extremity revealed a neurogenic lesion of the left upper trunk muscles in a patchy pattern with some ongoing denervation. The clinical and electrophysiologic pattern of the lesions conformed to a left upper trunk brachial plexopathy and a right long thoracic nerve lesion. The bilateral vocal fold paresis indicated additional lesions of the vagus or recurrent laryngeal nerves. Four months later, she had regained normal laryngeal function. There was also improvement in upper extremity strength.
CASE REPORT
DISCUSSION
A 24-year-old white woman was evaluated for hoarseness. She was in good health after the cesarean delivery of her first child; at 3-days postpartum, she developed bilateral proximal muscle weakness. Breathy speech developed several days later. Her dysphonia and vocal fatigue became so severe that she was unable to perform her duties as a schoolteacher. She noted difficulty drying her hair due to shoulder weakness, but she had no problems with fine dexterity. Her pain resolved with partial recovery of strength in her limbs, but because of persistent dysphonia, she presented to the otolaryngology clinic. Laryngeal examination demonstrated weakness of both vocal folds, resulting in incomplete glottal closure on phonation. Laryngeal electromyography (EMG) performed 2 months after onset showed early reinnervation of both thyroarytenoid muscles, consistent with a resolving bilateral
Although uncommon, IBP usually has a striking clinical presentation familiar to most neurologists. The shoulder pain that heralds the weakness is so characteristic that the diagnosis is easily made, unless there is a departure from the typical pattern of weakness. Most evidence suggests that the lesions involved in IBP are axonal in nature, rather than due to demyelination.1,2 The pattern of involvement favors a regional mononeuropathy multiplex, as it does not strictly respect the elements of the brachial plexus. IBP appears to have an autoimmune cause because many cases (including this one) have followed immunologic triggers, including surgery, viral syndromes, and vaccinations.2 Postpartum IBP has been described and has a similar clinical picture. Recovery in postpartum IBP is excellent, with the majority of women regaining 90% to 100% of function within 8 months.1 The diagnosis of IBP may be obscured when an isolated mononeuropathy occurs or when there are coexistent cranial neuropathies. Cases of apparent IBP with neurologic involvement apart from the brachial plexus and cranial nerves have been reported, including cases of isolated phrenic nerve dysfunction. Diaphragmatic involvement has been reported in isolation or with subtle evidence of a wider plexus lesion. These patients presented with shortness of breath as their chief complaint and initially presented
From the Department of Neurology (Ms Hyde and Dr Caress) and The Center for Voice Disorders (Dr Postma), Wake Forest University School of Medicine. Reprint requests: Dr James B. Caress, Department of Neurology, Wake Forest University School of Medicine, Medical Center Blvd, Winston-Salem, NC 27157; e-mail, [email protected]. Otolaryngol Head Neck Surg 2001;124:575-6. Copyright © 2001 by the American Academy of Otolaryngology– Head and Neck Surgery Foundation, Inc. 0194-5998/2001/$35.00 + 0 23/4/115167 doi:10.1067/mhn.2001.115167
575
576
Otolaryngology– Head and Neck Surgery May 2001
HYDE et al
to pulmonologists. Similarly, patients with hoarseness and few other neurologic abnormalities may present to an otolaryngologist. Vocal fold dysfunction has been reported on 3 occasions but never confirmed with electrodiagnostic testing. In 1975, Bradley et al3 described a patient with severe shoulder pain and hoarseness along with dysphagia. Weakness was in a predominantly upper trunk pattern, and both vocal folds were involved. Vocal dysfunction resolved in 3 months. Sanders et al4 described an individual who suffered flu-like symptoms, followed by bilateral shoulder pain and a hoarse voice. Clinical and electrophysiologic testing revealed abnormalities consistent with left upper trunk IBP. Indirect laryngoscopy demonstrated left vocal fold paralysis. The symptoms resolved in 6 months. Pierre et al5 reported a patient with right shoulder pain, hoarseness, and dysphagia. Involvement of the right side of the tongue, sternocleidomastoid, trapezius, and upper trunk innervated shoulder girdle muscles was apparent on examination, and laryngoscopy disclosed a paralyzed right vocal fold. Recovery was nearly complete at 12 months. It is of interest that our case and all 3 previously reported cases demonstrated upper trunk and more proximal involvement in conjunction with vocal fold
dysfunction. Although IBP can be a multifocal disorder, the consistency of this association suggests the origin of dysphonia may be the vagus nerve that is closer in proximity to the upper trunk rather than the recurrent laryngeal branch. This case highlights the importance of recognizing dysphonia as a possible presentation of IBP. In patients with apparent idiopathic laryngeal paresis or paralysis, we recommend that physicians search for a history of preceding or concomitant shoulder pain and briefly examine shoulder abduction, external rotation, and scapular movements in an attempt to exclude an IBP variant. Unless aspiration is present or vocal function is critical, surgical procedures should be delayed, as good recovery is expected. REFERENCES 1. Lederman RJ, Wilbourn AJ. Postpartum neuralgic amyotrophy. Neurology 1996;47:1213-9. 2. England JD. The variations of neuralgic amyotrophy. Muscle Nerve 1999;22:435-6. 3. Bradley WG, Madrid R, Thrush DC, et al. Recurrent brachial plexus neuropathy. Brain 1975;98:381-98. 4. Sanders EACM, Van den Neste VMH, Hoogenraad TU. Brachial plexus neuritis and recurrent laryngeal nerve palsy. J Neurol 1988;235:323-5. 5. Pierre PA, Laterre CE, Van Den Bergh PY. Neuralgic amyotrophy with involvement of cranial nerves IX, X, XI, and XII. Muscle Nerve 1990;13:704-7.