Report of three cases of dopa-responsive dystonia in one family
- VernacularTitle:多巴敏感性肌张力障碍一家三例报告
- Author:
Sheqing ZHANG
;
Huimin ZHENG
;
Suju DING
;
Laihui TU
;
- Publication Type:Journal Article
- Keywords:
dystonia,dopa responsive;
levodopa
- From:
Academic Journal of Second Military Medical University
1981;0(03):-
- CountryChina
- Language:Chinese
-
Abstract:
Objective: To study the clinical characteristics of dopa responsive dystonia (DRD),providing data for earlier diagnosis and treatment. Methods: Three cases of DRD patients from the same family were studied through their clinical data, laboratory investigations and therapeutic effects. Results: Two males and one female were the same generation. The age of onset remembered was about 10 years. The symptoms were insidiously progressive. Clinical features were extrapyramidal presentations such as fast words speed, limbs stiffness and involuntary movements. All 3 patients had leadtube muscle rigidity, exaggerated tendon reflexes; Two patients had feet deformity. Babinski signs were absent.CT, MRI, serum muscular enzymes and EMG, etc . were normal. Madopa,0.125 0.375 g, daily was effective with the longest treatment period for nearly 30 years and no obvious side effects found. Conclusion: The incidence of DRD is rare with specific presentations, early diagnosis and treatment may achieve better results.
