Early Onset Cerebellar Ataxia with Retained Tendon Reflexes Developed in Brothers: Report of two cases.
- Author:
Jeong Mee PARK
1
;
Tae Il PARK
;
Roh Wook PARK
Author Information
1. Department of Rehabiliatation Medicine, Yonsei University, Wonju College of Medicine, Korea.
- Publication Type:Case Report
- Keywords:
Cerebellar ataxia;
Tendon reflexes;
Friedreich's ataxia
- MeSH:
Cerebellar Ataxia;
Dysarthria;
Friedreich Ataxia;
Humans;
Male;
Reflex, Stretch*;
Siblings*;
Spinocerebellar Degenerations*;
Tendons*;
Young Adult
- From:Journal of the Korean Academy of Rehabilitation Medicine
1997;21(2):461-
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Early onset cerebellar ataxia with retained tendon reflexes is distinctive clinical syndrome characterized by progressive cerebellar ataxia of unknown etiology with an onset within the first two decades. This disorder was distinguished from Friedreich's ataxia by the preservation of the deep tendon reflexes. There is 22-year-old male with 13 year history of slowly progressive cerebellar ataxia and dysarthria. His elder brother, also, has milder clinical manifestations, electrophysiological and radiological abnormalities. We experienced two cases of early onset cerebellar ataxia with retained tendon reflexes developed in brothers which was diagnosed by clinical manifestations, electrophysiologic, radiologic studies and report with brief review of related literatures.
