Hereditary neuropathy and vocal cord paralysis in a man with childhood diphtheria.
We present the case of a 37-year-old Afghani man with a history of childhood diphtheria, who was diagnosed with bilateral vocal cord paralysis at age 15 years. At about this time he developed progressive muscular wasting and distally predominant weakness, and subsequently developed respiratory insufficiency, necessitating nocturnal ventilatory support. His examination suggested a distal symmetric sensorimotor neuropathy, and his brother was similarly affected, although to a lesser degree. Electromyography (EMG) and nerve conduction studies revealed this process to be purely axonal. A diagnosis of possible hereditary motor and sensory neuropathy (HMSN) type IIc, hereditary axonal polyneuropathy with vocal cord paralysis, is proposed, although the question of early diphtheritic involvement of the vocal cords and peripheral nerves is also considered.
Reichler
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BD
Department of Neurology, Mount Sinai School of Medicine, One Gustave Levy Place, Box 1052, New York, New York 10029, USA. breichler@smtplink.mssm.edu
Scelsa
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SN
Simpson
D M
DM
D000328:Adult; D004165:Diphtheria / Q000150:complications / Q000473:pathology*; D004576:Electromyography; D006801:Humans; D008297:Male; D018908:Muscle Weakness / Q000473:pathology; D009133:Muscular Atrophy / Q000473:pathology; D009431:Neural Conduction / Q000502:physiology; D009468:Neuromuscular Diseases / Q000150:complications / Q000235:genetics* / Q000473:pathology*; D009475:Neurons, Afferent / Q000502:physiology; D012129:Respiratory Function Tests; D014826:Vocal Cord Paralysis / Q000150:complications / Q000235:genetics* / Q000473:pathology*