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Hallervorden-spatz disease—Late infantile and adult types, report of two cases

Hallervorden-spatz disease—Late infantile and adult types, report of two cases,10.1007/BF00690505,Acta Neuropathologica,B. Rozdilsky,J. N. Cumings,A.

Hallervorden-spatz disease—Late infantile and adult types, report of two cases   (Citations: 12)
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Two cases of Hallervorden-Spatz disease are reported, one of whom was a late infantile variety and the other an adult variety who died at the end of the fourth decade of life. The clinical and neuropathological aspects were similar to previously reported cases. Both cases were characterized by a slowly progressive illness featured by dementia, seizures, dysarthria, rigidity, spasticity and athetoid and myoclonic movements. Neuropathological examination disclosed excessive pigmentation of the globus pallidus and red zone of substantia nigra associated with neuroaxonal dystrophy. The latter was more generalized in the first case and was confined to the pallidonigral area, cerebellum and lower medulla in the adult case.
Journal: Acta Neuropathologica - ACTA NEUROPATHOL , vol. 10, no. 1, pp. 1-16, 1968
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