[Juvenile parkinsonism with symmetrical hypoperfusion in the cerebellum--a case report].
We report a 24-year-old female presenting levodopa-responsive juvenile parkinsonism with symmetrical hypoperfusion in the cerebellum. At the age of 21, she noticed difficulty in brushing her teeth and writing with the right hand. She developed resting tremor in the right hand. These symptoms were dramatically relieved by levodopa. One year prior to the admission, she noticed dystonia and drug-induced motor fluctuations and her symptoms became worse. Neurological examinations disclosed resting and postural tremor in both hands and the right leg. Bradykinesia and cogwheel rigidity were noted on the right side. Deep tendon reflexes were slightly increased on the right side, while Babinski sign was negative. Slight lateropulsion was observed without retropulsion. Sensory, autonomic and cerebellar disturbances were not observed. No abnormalities were found in parkins gene or in the genes of spinocerebellar ataxia (SCA) 1,2,3,6,7,8 and alpha-synuclein. Cranial CT scan and brain MRI were normal, but technetium-99m ethyl cysteinate dimer (ECD) single photon emission computed tomography (SPECT) showed symmetrical hypoperfusion in the cerebellum. Other 5 patients presenting juvenile parkinsonism and 10 aged-matched normal controls in our hospital did not show hypoperfusion in the cerebellum on ECD SPECT. Cerebellar blood flow has not been measured in the previously reported cases of juvenile parkinsonism. These results suggested that etiopathogenesis in this patient was different from that in previously reported cases.
Yoshioka
M
M
Shigeto
H
H
Oya
Y
Y
Ogawa
M
M
Kawai
M
M
D000328:Adult; D002531:Cerebellum / Q000098:blood supply*; D002560:Cerebrovascular Circulation; D003545:Cysteine / Q000031:analogs & derivatives*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009140:Musculoskeletal Diseases / Q000209:etiology; D015609:Organotechnetium Compounds; D020734:Parkinsonian Disorders / Q000503:physiopathology*; D011187:Posture; D019275:Radiopharmaceuticals; D012018:Reflex; D015899:Tomography, Emission-Computed, Single-Photon
D015609:Organotechnetium Compounds; D019275:Radiopharmaceuticals; C059659:technetium Tc 99m bicisate; D003545:Cysteine