Is ataxic gait the predominant presenting manifestation of Creutzfeldt-Jakob disease? Experience of 14 Chinese cases from Taiwan.
Creutzfeldt-Jakob disease (CJD) is caused by an unusual prion protein. Rare CJD cases have been reported in Chinese individuals. This report describes the clinical manifestations of 14 Chinese individuals with clinically definite CJD from the National Taiwan University Hospital during the period 1976-1995. It is the largest case series of Chinese CJD up to now. All these patients fulfil the clinical definite diagnosis of CJD proposed by Brown et al. (1986), including rapidly evolving dementia, myoclonus, periodic electroencephalographic (EEG) activity (0.5-2 Hz) and death within 12 months. The clinical characteristics of the present series, including age at onset, sex ratio, duration, initial symptoms, neurological signs, EEG abnormalities, and neuroimaging studies were similar to those reported in other countries. However, there is a high incidence of initial ataxic gait as the presentation in our patients. Eight (57%) out of 14 patients initially had gait ataxia alone or in association with dementia. CJD should be considered in the differential provisional diagnosis of any middle-aged patient with a progressive ataxic syndrome.
Liou
H H
HH
Department of Neurology, National Taiwan University Hospital, Taipei, ROC.
Jeng
J S
JS
Chang
Y C
YC
Chen
R C
RC
Yip
P K
PK
D000368:Aged; D001259:Ataxia / Q000208:ethnology / Q000473:pathology*; D001921:Brain / Q000473:pathology; D002681:China; D007562:Creutzfeldt-Jakob Syndrome / Q000208:ethnology / Q000473:pathology*; D005260:Female; D005684:Gait; D006801:Humans; D008297:Male; D008875:Middle Aged; D013624:Taiwan / Q000208:ethnology