The spinocerebellar ataxias
The spinocerebellar ataxias
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DOI：
10.1097/00002826-200011000-00002
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发表时间：
2000-11-01
影响因子：
1
通讯作者：
Gilman, S
中科院分区：
文献类型：
--
作者：
Gilman, S
脊髓小脑共济失调（SCAs）是一种以神经元进行性变性和随后的丧失为特征的疾病，伴有反应性胶质增生，退化神经元的纤维变性，以及反映丢失神经元位置的临床症状。退行性改变影响特定的神经元群，而其他神经元群则保留下来，因此这些疾病可以被视为系统变性。SCAs由显性遗传的遗传传播疾病或散发发生的未知原因引起。这些疾病大多影响小脑及其通路，导致小脑功能的进行性恶化，表现为步态不稳定增加，肢体运动不协调，熟练动作（如书写）受损，以及明显的构音障碍。其他神经系统在某些se障碍中受到影响，特别是皮质脊髓通路、基底神经节和脑干和脊髓的自主神经核。
The spinocerebellar ataxias (SCAs) are diseases characterized by the progressive degeneration and subsequent loss of neurons accompanied by reactive gliosis, degeneration of fibers from the deteriorating neurons, and clinical symptoms reflecting the locations of the lost neurons. The degenerative changes affect specific neuronal groups while others remain preserved, and these diseases can therefore be viewed as system degenerations. The SCAs result from either genetically transmitted diseases with dominant inheritance or unknown causes with sporadic occurrence. Most of these disorders affect the cerebellum and its pathways, resulting in progressive deterioration of cerebellar function manifested by increasing unsteadiness of gait, incoordination of limb movements with impairment of skilled movements such as handwriting, and a distinctive dysarthria. Other neuronal systems are affected in some of the se disorders, notably the corticospinal pathway, basal ganglia, and autonomic nuclei of the brain stem and spinal cord.