Abe M, Arai M, Maehara K, Arikawa E, Arahata K
Department of Neuropsychiatry, Juntendo University School of Medicine, Tokyo, Japan.
No To Shinkei. 1990 Nov;42(11):1061-6.
A 23-year-old male patient with Becker muscular dystrophy (BMD) who showed schizophrenic symptoms was reported. He tumbled easily and was poor at running since age at 8 years. He had difficulty in climbing stairs and was idle away all day long since age at 21 years. Although his premorbid personality was not schizoid, he showed auditory hallucinations and delusions without any psychogenetic moment at the age of 23. At first, he seemed to be schizophrenic, but after the treatment with antipsychotics, he always had an insight into his disease and exhibited natural emotional communication. He showed no autism and character changes. According to the Wechsler Adult Intelligence Scale (WAIS), intellectual impairment was notified (total IQ58). Neurological examinations revealed weakness and atrophy of muscles in the proximal part of his lower extremities, and pseudohypertrophy of calves. In the serum enzyme, serum creatine kinase (CK) level was elevated (700 U/L). Abnormal Q waves appeared in the leads, II, III, aVF, V5, V6 on the electrocardiogram (ECG), and the finding of the echocardiography suggested dilated cardiomyopathy. The electroencephalogram (EEG) revealed the basic rhythm of 9-10 Hz with 0 activities of 6-7 Hz which were predominant in frontparietal and central leads. The electromyogram (EMG) revealed a myopathic pattern with low voltage and short duration. A muscle biopsy from right biceps brachii disclosed the abnormal immunofluorescent staining pattern of dystrophin which is consistent with BMD patient, i.e., "patchy," discontinuous and faint immunoreaction at surface membrane of the fiber. Both molecular weight (380 kd: n = 400) and amount (30%; n = 100) of dystrophin were reduced.(ABSTRACT TRUNCATED AT 250 WORDS)
报告了一名患有贝克型肌营养不良(BMD)且出现精神分裂症症状的23岁男性患者。他自8岁起就容易摔倒且跑步能力差。21岁起爬楼梯困难,整日无所事事。尽管他病前性格并非分裂样,但在23岁时出现了幻听和妄想,且无任何心理诱发因素。起初,他似乎患有精神分裂症,但在使用抗精神病药物治疗后,他始终能洞察自己的病情,并表现出自然的情感交流。他没有自闭症和性格改变。根据韦氏成人智力量表(WAIS),发现存在智力损害(总智商58)。神经系统检查显示其下肢近端肌肉无力和萎缩,小腿假性肥大。血清酶方面,血清肌酸激酶(CK)水平升高(700 U/L)。心电图(ECG)的II、III、aVF、V5、V6导联出现异常Q波,超声心动图检查结果提示扩张型心肌病。脑电图(EEG)显示基本节律为9 - 10 Hz,6 - 7 Hz的θ活动在前额顶叶和中央导联占主导。肌电图(EMG)显示为低电压、短时限的肌病模式。右侧肱二头肌肌肉活检显示肌营养不良蛋白免疫荧光染色模式异常,与BMD患者一致,即纤维表面膜处呈“斑片状”、不连续且微弱的免疫反应。肌营养不良蛋白的分子量(380 kd:n = 400)和含量(30%;n = 100)均降低。(摘要截断于250字)