両側声帯外転障害を呈した脊髄小脳変性症例　比較的急速に悪化した喉頭所見の詳細

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  • Bilateral vocal cord abductor impairment in a patient with spino-cerebellar degeneration with a detailed report on relatively acute laryngeal symptoms.
  • ―比較的急速に悪化した喉頭所見の詳細―

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A 57-year-old male patient with spino-cerebellar degeneration and slowly progressive cerebellar ataxia, pyramidal tract signs, extrapyramidal tract signs and autonomic failure of 9 years duration finally developed a severe disturbance of bilateral vocal cord abduction (Gerhardt syndrome). He had had no vocal cord palsy until 2 years earlier. He showed incomplete vocal cord abductor palsy on admission later, although he had no dyspnea or stridor. Twenty one days later, his vocal cords were completely fixed in the midline, producing a low-pitched murmuring stridor with every exhalation. The vocal cords moved laterally only slightly on inhalation. Eleven days later, the cartil agenous portions of the vocal cords were completely fixed in the midline on both inhalation and exhalation. The membranous portions were pulled strongly medially and inferiorly, producing a relatively high-pitched hissing stridor on every inhalation, and were blown passively laterally on exhalation. This means a complete loss of the “normal” respiratory pattern of adduction-abduction of the vocal cords.<br>Although arterial blood gas analysis indicated deterioration of respiratory function, he did not complain of dyspnea. The patient underwent tracheostomy the next day. His general condition and blood gas analysis are now stationary, and he still shows complete abduction impairment of the vocal cords. Every patient with a degenerative disease of the central nervous system should be referred to otol aryngologists for laryngeal examination even if he does not yet complain of dyspnea or stridor. Otolaryngologists should then examine for abduction or adduction impairment of the vocal cords using a fiberscope, since indirect laryngoscopy may lead to an inaccurate or incomplete study.

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