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Multiple system atrophy with retinal degeneration in a young child.
Neuropediatrics 1987; 18(2):91-5N

Abstract

A 4-year-old girl with multiple system degeneration and retinal degeneration was presented. There was onset of an ataxic gait at two years and rapid progression of retinal degeneration, myoclonus and cranial nerve palsy. Neuropathological examination revealed severe degeneration of the cerebellar cortex and the pathways of auditory and deep sensation, as well as degeneration of the cerebellar efferent fibers, the striatonigral system, the cerebellar afferent fiber system and lower motor neurons. Cases of young children with spinocerebellar degeneration have been reported in several families of olivopontocerebellar atrophy (OPCA), but degenerative changes in our case were more widespread than those in OPCA cases. The multiple system lesions in the central nervous system and retina of this child are different from those of any other previously reported cases.

Authors

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Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

3474543

Citation

Nishimura, M, et al. "Multiple System Atrophy With Retinal Degeneration in a Young Child." Neuropediatrics, vol. 18, no. 2, 1987, pp. 91-5.
Nishimura M, Mito T, Takashima S, et al. Multiple system atrophy with retinal degeneration in a young child. Neuropediatrics. 1987;18(2):91-5.
Nishimura, M., Mito, T., Takashima, S., Kawahara, H., Tanaka, J., Nakamura, H., & Kisa, T. (1987). Multiple system atrophy with retinal degeneration in a young child. Neuropediatrics, 18(2), pp. 91-5.
Nishimura M, et al. Multiple System Atrophy With Retinal Degeneration in a Young Child. Neuropediatrics. 1987;18(2):91-5. PubMed PMID: 3474543.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Multiple system atrophy with retinal degeneration in a young child. AU - Nishimura,M, AU - Mito,T, AU - Takashima,S, AU - Kawahara,H, AU - Tanaka,J, AU - Nakamura,H, AU - Kisa,T, PY - 1987/5/1/pubmed PY - 1987/5/1/medline PY - 1987/5/1/entrez SP - 91 EP - 5 JF - Neuropediatrics JO - Neuropediatrics VL - 18 IS - 2 N2 - A 4-year-old girl with multiple system degeneration and retinal degeneration was presented. There was onset of an ataxic gait at two years and rapid progression of retinal degeneration, myoclonus and cranial nerve palsy. Neuropathological examination revealed severe degeneration of the cerebellar cortex and the pathways of auditory and deep sensation, as well as degeneration of the cerebellar efferent fibers, the striatonigral system, the cerebellar afferent fiber system and lower motor neurons. Cases of young children with spinocerebellar degeneration have been reported in several families of olivopontocerebellar atrophy (OPCA), but degenerative changes in our case were more widespread than those in OPCA cases. The multiple system lesions in the central nervous system and retina of this child are different from those of any other previously reported cases. SN - 0174-304X UR - https://www.unboundmedicine.com/medline/citation/3474543/Multiple_system_atrophy_with_retinal_degeneration_in_a_young_child_ L2 - http://www.thieme-connect.com/DOI/DOI?10.1055/s-2008-1052460 DB - PRIME DP - Unbound Medicine ER -