Hemiatrophy, juvenile-onset exertional alternating leg paresis, hypotonia, and hemidystonia and adult-onset hemiparkinsonism: the spectrum of hemiparkinsonism-hemiatrophy syndrome.
A 45-year-old woman with a history of probable perinatal craniocerebral trauma resulting in mild asymptomatic right hemiatrophy developed right leg weakness and hypotonia alternating with dystonia only after prolonged exertion at age 12. At age 27, she developed right-sided parkinsonism. Exertional paresis and dystonia and parkinsonism responded completely to levodopa; however, she developed a progressive reduction in the duration of action of levodopa over the first 4 years of treatment. Investigations including computed tomography, magnetic resonance imaging, [18F]fluorodopa, and [18F]fluorodeoxyglucose positron emission tomography scans suggested a static lesion involving the left substantia nigra. This unusual exertion-induced weakness and hypotonia alternating with hypertonia and dystonia has not been reported previously. The role of dopamine deficiency in dystonia and the role of levodopa in the development of fluctuations in Parkinson's disease are discussed. Review of the literature, including this patient, emphasizes the heterogeneity of the syndrome of hemiparkinsonism-hemiatrophy.
Lang
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Morton and Gloria Shulman Movement Disorders Centre, Toronto Hospital, Ontario, Canada.
D000978:Antiparkinson Agents / Q000627:therapeutic use; D001786:Blood Glucose / Q000378:metabolism*; D002230:Carbidopa / Q000627:therapeutic use; D003287:Contrast Media; D003847:Deoxyglucose / Q000031:analogs & derivatives; D004295:Dihydroxyphenylalanine / Q000031:analogs & derivatives; D004292:Dominance, Cerebral / Q000187:drug effects / Q000502:physiology; D004338:Drug Combinations; D005260:Female; D019788:Fluorodeoxyglucose F18; D007839:Functional Laterality / Q000187:drug effects / Q000502:physiology*; D006429:Hemiplegia / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000503:physiopathology; D006801:Humans; D007980:Levodopa / Q000627:therapeutic use; D008875:Middle Aged; D009123:Muscle Hypotonia / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000503:physiopathology; D009133:Muscular Atrophy / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000503:physiopathology; D009460:Neurologic Examination / Q000187:drug effects; D010302:Parkinson Disease, Secondary / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000503:physiopathology; D005082:Physical Exertion / Q000187:drug effects / Q000502:physiology*; D013378:Substantia Nigra / Q000000981:diagnostic imaging* / Q000187:drug effects / Q000503:physiopathology; D014055:Tomography, Emission-Computed
D000978:Antiparkinson Agents; D001786:Blood Glucose; D003287:Contrast Media; D004338:Drug Combinations; C009265:carbidopa, levodopa drug combination; D019788:Fluorodeoxyglucose F18; C029112:5-fluorodopa; D007980:Levodopa; D004295:Dihydroxyphenylalanine; D003847:Deoxyglucose; D002230:Carbidopa