両側声帯固定を呈したＳｈｙ‐Ｄｒａｇｅｒ症候群例

Abstract

Shy-Drager syndrome has been labeled one type of multiple system atrophy. We report a patient with bilateral vocal cord abductor dysfunction and Shy-Drager syndrome.The patient was a 79-year-old male suffering from dysuria. In January 2003, he was referred with a complaint of nocturnal stridor lasting two years. Fiberscopic examination of the larynx revealed insufficient abduction of the bilateral vocal cords with a slight aperture. No definitive diagnosis regarding the nocturnal stridor was established at that time. Six months later he complained of deterioration of the nocturnal stridor. While he did not have any other symptoms of respiration, the laryngeal examination showed his bilateral vocal cords lying in the midline position. Three months later, languor and dizziness occurred after every meal. When he was hospitalized for examination, he experienced faintness after meals. His condition progressed from bad to worse. He began suffering from aspiration pneumonia, requiring artificial respiration with intubation. The next day, he was given a tracheostomy to prevent laryngeal obstruction. After his recovery from pneumonia, orthostatic hypotension and hypotension after meals were discovered. T2-weighted magnetic resonance images showed high-intensity areas in the pons, an abnormal sign indicating multiple system atrophy. The diagnosis of Shy-Drager syndrome was made from these clinical observations. We reviewed 13 reports mentioning the surgical treatment for bilateral abductor dysfunction with Shy-Drager syndrome. It is necessary in patients with bilateral abductor dysfunction to restore the airway in order to prevent nocturnal sudden death. 33 cases have been treated by tracheostomy (29 cases), vocal cord lateralization (3 cases), and selective resection of the adductor branch from the recurrent laryngeal nerve (1 case).It is important for physicians treating patients with bilateral abductor dysfunction to formulate an examination plan concerning nervous system degeneration diseases and to consider the indication for an operation to maintain the airway.