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PubMed1985

Multiple system atrophy with neuronal intranuclear hyaline inclusions. Report of a case and review of the literature.

A 31/2-year-old girl presented with frequent falls. She had an unsteady gait, delayed behavioural development absent tendon reflexes and in the legs decreased strength tone and equivocal plantar responses. She then developed ataxia, nystagmus, choreoathetosis, cranial nerve palsies, diminished strength and tone in the arms, sensory deficit in the limbs and autonomic nervous system dysfunction. She became progressively less responsive and succumbed at the age of 63/4 years. Examination of the central, peripheral and autonomic nervous system showed ubiquitous neuronal intranuclear hyaline inclusions and neuronal loss in several sites.

Amino Acids↗
PubMed1981

Laryngeal abductor paralysis in multiple system atrophy. A report on three necropsied cases, with observations on the laryngeal muscles and the nuclei ambigui.

Clinical and pathological findings are reported in three cases of Shy-Drager syndrome with laryngeal stridor severe enough to require tracheostomy. Indirect laryngoscopy in all three cases revealed a failure to abduct the vocal cords properly with a resulting narrowing of the glottic airway. Histological studies showed a marked atrophy of the posterior crico-arytenoid muscles and little atrophy of the other laryngeal muscles. The posterior crico-arytenoid muscles have the sole responsibility of abducting the vocal cords, so it is concluded that the patient's stridor was caused by the selective paralysis of these muscles. Histological studies of the posterior crico-arytenoid muscles were suggestive of denervation but despite this finding, no clear evidence of any motor cell loss in the nuclei ambigui was obtained. Other possible causes of the selective posterior crico-arytenoid atrophy are discussed.

Autonomic Nervous System Diseases↗