Multiple System Atrophy

Multiple system atrophy (MSA), a sporadic neurodegenerative disease of adults, combines the clinical and pathologic features of three conditions, namely, olivo-pontocerebellar atrophy (OPCA), striatonigral degeneration (SND), and dysautonomia of the Shy-Drager syndrome (SDS). Occurring together, they form a clini-copathologic entity that is defined by the presence of distinct oligodendrocytic and neuronal cytoplasmic inclusions.

FIGURE 5.34

Adult-onset hereditary spastic spinocerebellar ataxia. A 43-year-old man developed rapidly progressing visual impairment, cerebellar ataxia, and weakness in all extremities. By age 44, he was blind and had pale, sharply outlined discs. Deep tendon reflexes were brisk, and Hoffmann and Babinski signs were present on both sides. All extremities were weak and spastic. Cerebel-lar ataxia and intention tremor were present in the upper extremities. Sensation was normal. His condition steadily deteriorated and, at age 49, he died. His mother and two sisters, aged 51 and 37 years, respectively, suffered from a similar illness. A and B. The optic nerves are partially demyelinated (Weil stain). C. The cerebellar cortex and white matter are diffusely atrophic. D. The corticospinal tracts and the spinocerebellar tracts are degenerated in the spinal cord (Weil stain).

FIGURE 5.34

Adult-onset hereditary spastic spinocerebellar ataxia. A 43-year-old man developed rapidly progressing visual impairment, cerebellar ataxia, and weakness in all extremities. By age 44, he was blind and had pale, sharply outlined discs. Deep tendon reflexes were brisk, and Hoffmann and Babinski signs were present on both sides. All extremities were weak and spastic. Cerebel-lar ataxia and intention tremor were present in the upper extremities. Sensation was normal. His condition steadily deteriorated and, at age 49, he died. His mother and two sisters, aged 51 and 37 years, respectively, suffered from a similar illness. A and B. The optic nerves are partially demyelinated (Weil stain). C. The cerebellar cortex and white matter are diffusely atrophic. D. The corticospinal tracts and the spinocerebellar tracts are degenerated in the spinal cord (Weil stain).

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