Occurrence of Parkinson's syndrome in type I Gaucher disease.
Gaucher disease, the most prevalent glycolipid storage disorder, is classically subdivided into types according to the presence or absence of neurological involvement. Type I has hitherto been considered non-neuronopathic. We present six cases and a review of the literature of Parkinsonian symptoms in type I Gaucher disease patients. The hallmark of this atypical Parkinsonian syndrome is a relatively severe clinical course with early appearance of neurological signs in the 4th to 6th decade of life, aggressive progression of the signs and refractoriness to conventional anti-Parkinson therapy. We discuss the implications of these findings in the light of enzyme replacement therapy for Gaucher disease.
Neudorfer
O
O
Gaucher Clinic, Department of Medicine, Shaare-Zedek Medical Center, Jerusalem, Israel.
Giladi
N
N
Elstein
D
D
Abrahamov
A
A
Turezkite
T
T
Aghai
E
E
Reches
A
A
Bembi
B
B
Zimran
A
A
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000978:Antiparkinson Agents / Q000627:therapeutic use; D004351:Drug Resistance; D005260:Female; D005776:Gaucher Disease / Q000150:complications* / Q000208:ethnology; D006801:Humans; D007585:Jews; D008297:Male; D008875:Middle Aged; D010300:Parkinson Disease / Q000150:complications* / Q000188:drug therapy
D000978:Antiparkinson Agents