Oki J, Miyamoto A, Takahashi S, Itoh J, Sakata Y, Okuno A
Department of Pediatrics, Asahikawa Medical College, Japan.
Brain Dev. 1998 Apr;20(3):186-9. doi: 10.1016/s0387-7604(98)00017-5.
A 13-year-old mentally retarded boy suffered from repeated vomiting attacks since infancy. Each episode lasted 2 to 10 days, and was precipitated by respiratory infection, exercise or stress. During an attack he became irritated, agitated and amnesic, but did not have headaches or seizures. Associated findings were transient elevation of serum creatine kinase (CK) (331-3381 IU/l), and of plasma ACTH and cortisol. The raised CK level was the result of muscle hypertonicity. Ictal EEGs showed delta activity in the front-temporal areas, and inter-ictal IMP-SPECT revealed hypoperfusion in both temporal regions. Unlike the periodic ACTH-ADH discharge syndrome, neither hypertension nor depression developed. These attacks were diagnosed as a migraine equivalent and were suppressed with phenytoin. From the EEG and SPECT findings, we concluded that the vomiting and behavioural changes were related to the paroxysmal vascular abnormality in the temporal regions, but it was not easy to make the distinction between migraine and focal epilepsy. Before a diagnosis of the periodic ACTH-ADH discharge syndrome is made, the possibility of migraine equivalent should be considered.
一名13岁的智障男孩自婴儿期起就反复出现呕吐发作。每次发作持续2至10天,由呼吸道感染、运动或压力诱发。发作期间,他会变得烦躁、激动且失忆,但没有头痛或癫痫发作。相关检查结果包括血清肌酸激酶(CK)短暂升高(331 - 3381 IU/l),以及血浆促肾上腺皮质激素(ACTH)和皮质醇升高。CK水平升高是肌肉张力亢进的结果。发作期脑电图显示额颞区有δ活动,发作间期单光子发射计算机断层扫描(IMP - SPECT)显示双侧颞叶灌注不足。与周期性促肾上腺皮质激素 - 抗利尿激素分泌综合征不同,既没有出现高血压也没有抑郁。这些发作被诊断为偏头痛等位发作,并通过苯妥英得到控制。根据脑电图和SPECT检查结果,我们得出结论,呕吐和行为改变与颞叶阵发性血管异常有关,但偏头痛和局灶性癫痫之间的区分并不容易。在诊断周期性促肾上腺皮质激素 - 抗利尿激素分泌综合征之前,应考虑偏头痛等位发作的可能性。