Catalogo Articoli (Spogli Riviste)

OPAC HELP

Titolo:
OCULOMOTOR ABNORMALITIES IN DYSSYNERGIA-CEREBELLARIS-MYOCLONICA
Autore:
WIEST G; MUELLER C; WESSELY P; STEINHOFF N; TRATTNIG S; DEECKE L;
Indirizzi:
UNIV VIENNA,DEPT NEUROL,WAEHRINGER GUERTEL 18 VIENNA AUSTRIA UNIV VIENNA,MRI INST VIENNA AUSTRIA
Titolo Testata:
Acta oto-laryngologica
, , anno: 1995,
parte:, 2 supplemento:, 520
pagine: 392 - 394
SICI:
0001-6489(1995):<392:OAID>2.0.ZU;2-9
Fonte:
ISI
Lingua:
ENG
Soggetto:
EPILEPSY;
Keywords:
DYSSYNERGIA CEREBELLARIS MYCOLONICA; OCULOMOTOR; EYE MOVEMENTS; RAMSAY-HUNT SYNDROME;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
8
Recensione:
Indirizzi per estratti:
Citazione:
G. Wiest et al., "OCULOMOTOR ABNORMALITIES IN DYSSYNERGIA-CEREBELLARIS-MYOCLONICA", Acta oto-laryngologica, 1995, pp. 392-394

Abstract

In 1921 Ramsay-Hunt first described the syndrome of dyssynergia cerebellaris myoclonica (DCM), characterized by the clinical triad of action myoclonus, progressive ataxia and epilepsy with cognitive impairment, subsequently also refered to as the ''Ramsay-Hunt syndrome''. The cause of the symptoms of this rare degenerative syndrome (incidence: 500,000) is the impairment of a regulatory mechanism between nucleus dentatus, nucleus ruber and the bulbar olive. We present two sisters, aged29 and 30 years, who were investigated for oculomotor abnormalities. The patients were diagnosed as having DCM according to clinical symptomatology, which was confirmed by neurophysiological and radiological findings. In both cases saccadic velocity was markedly reduced, whereassaccadic latency showed a significant increase. In addition, smooth pursuit eye-movements were abnormal and presented reduced gain. These findings suggest that pontine areas and the vestibulocerebellum also seem to be affected in DCM.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 25/11/20 alle ore 18:35:07