MULTIPLE SYSTEM ATROPHY WITH NEURONAL INTRANUCLEAR HYALINE INCLUSIONS - REPORT OF A CASE AND REVIEW OF THE LITERATURE
MULTIPLE SYSTEM ATROPHY WITH NEURONAL INTRANUCLEAR HYALINE INCLUSIONS - REPORT OF A CASE AND REVIEW OF THE LITERATURE
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DOI：
10.1016/0022-510x(85)90022-x
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发表时间：
1985-01-01
影响因子：
4.4
通讯作者：
BERRY, KE
中科院分区：
文献类型：
--
作者：
PATEL, H;NORMAN, MG;BERRY, KE
一名31岁半的女孩经常出现福尔斯。她步态不稳，行为发育延迟，腱反射缺失，腿部力量减弱，足底反应不明确。然后，她出现共济失调、眼球震颤、舞蹈手足徐动症、颅神经麻痹、手臂力量和张力减弱、四肢感觉缺陷和自主神经系统功能障碍。她逐渐变得反应迟钝，并在63/4岁时死亡。中枢、外周和自主神经系统检查显示多个部位神经元核内透明包涵体和神经元丢失。
A 31/2-yr old girl presented with frequent falls. She had an unsteady gait, delayed behavioral 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 yr. Examination of the central, peripheral and autonomic nervous system showed ubiquitous neuronal intranuclear hyaline inclusions and neuronal loss in several sites.