Tsai C H, Lu C S
Department of Neurology, Chang Gung Memorial Hospital Taipei, Taiwan, R.O.C.
J Formos Med Assoc. 1991 Oct;90(10):964-9.
We describe the clinical features of parkinsonism in 25 patients whose age of onset was under 40 years. Among them, 17 patients, whose age of onset was after their 21st birthday, were classified as young onset Parkinson's disease (YOPD), and the remaining 8 whose age of onset was before their 21st birthday were classified as juvenile parkinsonism (JP). Rigidity and akinesia were revealed in all 25 patients. Resting tremor was observed in only 5 patients; 3 in the YOPD group and 2 in the JP group. There were 8 of the 25 patients (32%) who experienced an aching sensation in the leg before or at the onset of the parkinsonian features. In 6 of these 8 cases, the sensory symptoms were on the same side where the clinical manifestations of parkinsonism later developed. In the JP group, 2 patients had right foot dystonia, which improved with levodopa. Diurnal fluctuations in parkinsonian symptoms were found in 9 of the 25 cases. The familial incidence of parkinsonism was higher in the JP group. The parkinsonian disabilities in all 25 cases responded dramatically to levodopa therapy. Unfortunately, 10 cases, 5 in the YOPD group and 5 in the JP group, developed dyskinesia. The longer they took levodopa, the greater the chance of developing dyskinesia. The cumulative percentage of dyskinesia was 100% in the YOPD group and 83% in the JP group by the seventh and fourth year of treatment, respectively. A positive correlation was found between the prevalence of dyskinesia and the duration of treatment in both groups.
我们描述了25例发病年龄在40岁以下的帕金森综合征患者的临床特征。其中，17例发病年龄在21岁之后的患者被归类为青年型帕金森病（YOPD），其余8例发病年龄在21岁之前的患者被归类为青少年帕金森综合征（JP）。所有25例患者均出现强直和运动不能。仅5例患者出现静止性震颤；YOPD组3例，JP组2例。25例患者中有8例（32%）在帕金森综合征特征出现之前或同时出现腿部疼痛感觉。在这8例中的6例中，感觉症状出现在帕金森综合征临床表现后来出现的同一侧。在JP组中，2例患者出现右脚肌张力障碍，左旋多巴治疗后有所改善。25例中有9例出现帕金森综合征症状的日波动。JP组帕金森综合征的家族发病率更高。所有25例患者的帕金森综合征残疾对左旋多巴治疗反应显著。不幸的是，10例患者出现运动障碍，YOPD组和JP组各5例。服用左旋多巴时间越长，发生运动障碍的几率越大。到治疗的第七年和第四年，YOPD组和JP组运动障碍的累积发生率分别为100%和83%。两组中运动障碍的患病率与治疗持续时间之间均呈正相关。
