Other Heredodegenerative Diseases


Dentato-rubro-pallido-luysian atrophy (DRPLA) is a rare familial disorder comprising degeneration of the cerebellar efferent system and of the pallido-luysian tracts. Ataxic choreoathetoid, pseudo-Huntington, and myoclonic epilepsy forms of this syndrome have been described. The disorder begins between 7 and 20 years of age.


Intellectual Disability Dentate Nucleus Myotonic Dystrophy Rett Syndrome Spinocerebellar Ataxia 
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15 Other Heredodegenerative Diseases

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    Lowe JS, Leigh N (2002) Disorders of movement and system degenerations. In: Graham DI, Lantos PL (eds) Greenfields’ neuropathology, 7th edn, Vol 1. Arnold, London, pp 361–370.Google Scholar
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    Rett A (1977) Cerebral atrophy associated with hyperammonemia. In: Vinken PW, Bruyn GW (eds) Handbook of clinical neurology, Vol 29. North Holland, Amsterdam, pp 205–225.Google Scholar
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    Ronnet GV, Leopold D, Cai X, Hoffbuhr KC, Moses L, Hoffman EP, Naidu S (2003) Olfactory biopsies demonstrate a defect in neuronal development in Rett syndrome. Ann Neurol 54:206–218.CrossRefGoogle Scholar
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    Esiri MM, Crow TJ (2002) Neuropathology of psychiatric disorders In: Graham DI, Lantos PL (eds) Greenfields’ neuropathology, 7th edn, Vol 1. Arnold, London, pp 455–457.Google Scholar
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    Endo A, Motonaga K, Arahata K, Yamada T, Takashima S (2000) Developmental expression of myotonic dystrophy protein kinase in brain and its relevance to clinical phenotype. Acta Neuropathol (Berl) 100:513–520.PubMedCrossRefGoogle Scholar
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