Lees A J, Robertson M, Trimble M R, Murray N M
J Neurol Neurosurg Psychiatry. 1984 Jan;47(1):1-8. doi: 10.1136/jnnp.47.1.1.
The clinical features of 53 British-born patients with Gilles de la Tourette syndrome are described. The mean age at onset of body tics was seven years and for vocalisations 11 years. Coprolalia was present in 39%, copropraxia in 21%, echolalia in 46% and echopraxia in 21%. Complicated antics and mannerisms were also common, often involving the compulsive touching of objects or self-injurious behaviour. Forty-six per cent of cases had a family history of tics in a single close relative and in two individuals a further member of the family had Gilles de la Tourette syndrome. Focal dystonia was present in four patients who had never received neuroleptics drugs and chorea was seen in two other untreated patients. In three patients acoustic startle consistently induced brief eye blink followed by a whole body jerk or jump. Rapid repetitive movements of the hands increased the frequency and severity of tics in 13 patients, but the performance of mental arithmetic under time pressure had a much more unpredictable effect. Electroencephalographic abnormalities occurred in eight (13%) but no definite CT brain scan abnormalities were detected. The incidence of left handedness did not differ from that in the general population and no evidence to suggest organic impairment was found on neuropsychological testing. This study provides no support for the notion that Gilles de la Tourette syndrome is a degenerative disorder of the central nervous system but provides some evidence for heterogeneity.
本文描述了53名在英国出生的患有抽动秽语综合征患者的临床特征。身体抽动开始的平均年龄为7岁,发声抽动开始的平均年龄为11岁。秽语症患者占39%,秽亵行为患者占21%,模仿言语患者占46%,模仿动作患者占21%。复杂的滑稽动作和怪癖也很常见,常涉及强迫性触摸物体或自伤行为。46%的病例在一位近亲中有抽动症家族史,有两位患者的家族中还有其他成员患有抽动秽语综合征。4名从未服用过抗精神病药物的患者出现局灶性肌张力障碍,另外2名未接受治疗的患者出现舞蹈症。3名患者受到听觉惊吓后持续出现短暂的眨眼,随后是全身抽搐或跳动。手部快速重复运动会增加13名患者抽动的频率和严重程度,但在时间压力下进行心算产生的影响则更难以预测。8名患者(13%)脑电图异常,但脑部CT扫描未发现明确异常。左撇子的发生率与普通人群无异,神经心理学测试未发现有器质性损害的证据。这项研究不支持抽动秽语综合征是中枢神经系统退行性疾病的观点,但为其异质性提供了一些证据。