Bruyn R P
Department of Neurology, Oudenrijn Hospital, Utrecht, The Netherlands.
Clin Neurol Neurosurg. 1992;94 Suppl:S16-8. doi: 10.1016/0303-8467(92)90010-z.
Hereditary spastic paraparesis or Strümpell's disease is a genetically determined neurodegenerative disorder in which the signs and symptoms are predominant in the legs. Inheritance is usually autosomal dominant and in a minority recessive. Neuropathological study reveals a degeneration of the corticospinal tract decreasing from lower lumbar to cervical level and of posterior columns increasing from lumbar to upper cervical level as well as degeneration of the spinocerebellar tracts in approximately 50%. The nature of this nucleo-distal central axonopathy and clinicopathological discrepancy for posterior columns, as well as the limits of the pathological process are poorly understood.
遗传性痉挛性截瘫或施特吕姆佩尔病是一种由基因决定的神经退行性疾病，其体征和症状主要出现在腿部。遗传方式通常为常染色体显性遗传，少数为隐性遗传。神经病理学研究显示，皮质脊髓束从下腰椎到颈椎水平逐渐退化，后索从腰椎到上颈椎水平逐渐加重，约50%的患者脊髓小脑束也发生退化。这种核远端中枢轴索性神经病的本质、后索的临床病理差异以及病理过程的限度尚不清楚。
