Clinical features of autosomal recessive juvenile Parkinson disease / 临床神经病学杂志

ABSTRACT

Objective To explore the clinical features of autosomal recessive juvenile Parkinson disease(AR-JP).Methods The clinical materials of 28 patients from 15 families with AR-JP were analyzed retrospectively.Results The onset of all the patients was insidious and the mean age was 26.1 years old.In 23 patients(82.1%),the symptoms began at one limb or one side and progressed bilaterally in a mean time of((4.7?)3.6) years.Bradykinesia(100%),rigidity(100%),resting tremor (85.7%),postural instability(60.7%),hyperreflexia(53.6%),dystonia(32.1%) and diurnal fluctuations with sleep benefit(89.2%) were the cardinal symptoms.The mean improved Webster score was(11.2?)6.1.The mean maintenance dose of DOPA-preparation was((0.40?)0.28) g/d.The mean UPDRS motor score was(27.9?)10.3 before treatment and it decreased to(6.7?)5.4 after therapy(P