发热,偏侧惊厥持续状态,意识障碍

Fever, unilateral convulsion status, disturbance of consciousness

患儿为6岁女童,主因"4 h内发作性抽搐4次"入院。入院时患儿主要表现为偏侧惊厥持续状态、发热、意识障碍,意识好转后查体发现其惊厥侧肢体偏瘫。入院时初步考虑病毒性脑炎。但入院后查脑脊液常规、生化未见异常,脑脊液免疫抗体及病原高通量测序未见异常,排除中枢神经系统感染。结合颅脑磁共振成像显示右侧大脑半球广泛水肿,考虑特发性偏侧惊厥-偏瘫综合征(IHHS)。调整抗癫痫药物为苯巴比妥后,惊厥发作明显减少。但间隔1个月后出现难治性癫痫,最终诊断特发性偏侧惊厥-偏瘫-癫痫综合征(IHHES)。临床表现为发热、偏侧惊厥持续状态、意识障碍的患儿,诊断需结合脑脊液检查、影像学特点等考虑,IHHS后期可能发展为IHHES。

There was a 6-years-old girl who was hospitalized for " four episodes of convulsions within four hours". On admission, the main manifestations of the patient were unilateral convulsion status, fever and disturbance of consciousness.After improvement of consciousness, physical examination revealed hemiplegia on the convulsive side.Viral encephalitis was considered at admission.However, there were no abnormalities in routine and biochemical examinations of cerebrospinal fluid(CSF), and there were no abnormalities in immune antibodies and pathogen high-throughput sequencing of CSF, which excluded central nervous system infection.According to the craniocerebral magnetic resonance imaging, extensive edema in the right cerebral hemisphere was demonstrated.Diagnosis was considered to be idiopathic hemiconvulsion hemiplegia syndrome(IHHS). The antiepileptic drug was adjusted as phenobarbital, and the seizures were reduced.But one month later, intractable epilepsy occurred, and the final diagnosis was idiopathic hemiconvulsion-hemiplegia-epilepsy syndrome(IHHES). The clinical manifestations were fever, unilateral convulsion status, and disturbance of consciousness.The diagnosis should be combined with CSF examination and imaging characteristics and other considerations.IHHS may develop to IHHES in the later stage.