TY - JOUR
T1 - Nigro-spino-dentatal degeneration with nuclear ophthalmoplegia. A unique and partially treatable clinico-pathological entity
AU - Woods, Bryan T.
AU - Schaumburg, Herbert H.
N1 - Funding Information:
This investigation was supported (in part) by Training Grants (No. NSO-5075, 5T1-5393) and Special Fellowship (1F 1-2183) Awards from the National Institute of Neurological Diseases and Stroke and in part by the Massachusetts General Hospital Parkinson's Fund.
PY - 1972/10
Y1 - 1972/10
N2 - A family with a dominant form of neuronal degeneration, marked clinically by ataxia, hyperreflexia, distal motor weakness, extrapyramidal rigidity, bulbar signs, and ophthalmoplegia has been followed for some years. Recent post-mortem study of 1 member revealed degeneration of the anterior horn cells, spino-cerebellar tracts, pons, dentate nucleus, substantia nigra, and oculomotor nuclei. This pattern of involvement appears to be a unique variant of neuronal degeneration, sharing features of both spino-cerebellar and extra-pyramidal system degenerations. Furthermore, the extrapyramidal signs of 3 affected family members have responded to a combination of dopaminergic and central anti-cholinergic medications; levodopa, amantidine hydrochloride, and trihexyphenidyl hydrochloride.
AB - A family with a dominant form of neuronal degeneration, marked clinically by ataxia, hyperreflexia, distal motor weakness, extrapyramidal rigidity, bulbar signs, and ophthalmoplegia has been followed for some years. Recent post-mortem study of 1 member revealed degeneration of the anterior horn cells, spino-cerebellar tracts, pons, dentate nucleus, substantia nigra, and oculomotor nuclei. This pattern of involvement appears to be a unique variant of neuronal degeneration, sharing features of both spino-cerebellar and extra-pyramidal system degenerations. Furthermore, the extrapyramidal signs of 3 affected family members have responded to a combination of dopaminergic and central anti-cholinergic medications; levodopa, amantidine hydrochloride, and trihexyphenidyl hydrochloride.
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U2 - 10.1016/0022-510X(72)90137-2
DO - 10.1016/0022-510X(72)90137-2
M3 - Article
C2 - 5053922
AN - SCOPUS:0015412724
SN - 0022-510X
VL - 17
SP - 149
EP - 166
JO - Journal of the Neurological Sciences
JF - Journal of the Neurological Sciences
IS - 2
ER -