Résumé
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.
Résumé
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.