Fever, unilateral convulsion status, disturbance of consciousness / 中华实用儿科临床杂志

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.

Consensus definition and development of new-onset refractory status epilepticus and the related syndromes / 中华实用儿科临床杂志

New-onset refractory status epilepticus(NORSE)is a clinical syndrome,not a specific diagnosis of diseases in patients with new onset of refractive status epilepticus without active epilepsy or other preexisting relevant neurological disorders and is not associated with a clearly acute or active structrue,toxic or metabolic cause. Febrile in-fection-related epilepsy syndrome(FIRES)is a subcategory of NORSE,applicable for all ages,occurring in a prior fe-brile infection starting between 24 hours and 2 weeks before onset of refractory status epilepticus( RSE)with fever or not. Proposed consensus definitions are also applicable for infantile hemiconvulsion - hemiplegia and epilepsy syn-drome,RSE,super-RSE,prolonged RSE and prolonged super-RSE. These consensus definitions will promote much more effective communication and multicenter research,and ultimately improve recognition and treatment of these condi-tions.

Consensus definition and development of new-onset refractory status epilepticus and the related syndromes / 中华实用儿科临床杂志

New-onset refractory status epilepticus(NORSE) is a clinical syndrome, not a specific diagnosis of diseases in patients with new onset of refractive status epilepticus without active epilepsy or other preexisting relevant neurological disorders and is not associated with a clearly acute or active structrue, toxic or metabolic cause.Febrile infection-related epilepsy syndrome(FIRES) is a subcategory of NORSE, applicable for all ages, occurring in a prior febrile infection starting between 24 hours and 2 weeks before onset of refractory status epilepticus(RSE) with fever or not.Proposed consensus definitions are also applicable for infantile hemiconvulsion-hemiplegia and epilepsy syndrome, RSE, super-RSE, prolonged RSE and prolonged super-RSE.These consensus definitions will promote much more effective communication and multicenter research, and ultimately improve recognition and treatment of these conditions.

Fever, unilateral convulsion status, disturbance of consciousness / 中华实用儿科临床杂志

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.

Hemiconvulsion-hemiplegia-epilepsy Syndrome Sparing Paracentral Lobule:Early Magnetic Resonance Imaging Findings and Neuroradiological Follow-up / 대한소아신경학회지

HHE syndrome is characterized by hemiconvulsive seizure, hemiplegia and epilepsy occurring in sequence. Recently, cytotoxic edematous swelling of one hemisphere was presented as the pathogenesis because high signal intensity was shown in the diffusion image but no remarkable findings were noticed in the FLAIR image and T2WI in two cases presented by Freeman etc. The clinical course and the radiologic findings in our patient was the same as the above description other than sparing paracentral lobule. This reinforces the assumption that the brain damage in HHE syndrome is not induced by vascular hypoxic damage but by selectively cytotoxic damage through immunologic pathogenesis. Therefore, diffusion image is needed for the diagnosis of HHE syndrome in a febrile convulsive child. Also, early adequate treatment is required such as immunosuppressive treatment with high dose steroid and intravenous immunoglobulin. Furthermore, a proper measure to reduce cerebral edema is essential for the prevention of neurologic sequalae and deterioration to epilepsy.

Clinical Characteristics and Diagnosis of Hemiconvulsion-Hemiplegia-Epilepsy Syndrome / 实用儿科临床杂志

Objective To summarize the clinical characteristics and diagnosis of hemiconvulsion-hemiplegia-epilepsy(HHE) syndrome.Methods The clinical data of 5 children with HHE syndrome were analyzed.Cranial magnetic resonance imaging MRI and /or computerized tomography(CT) and electroencephalogram(EEG) were done in all patients.Results The age of onset was less than 4 years in 4 of 5 patients and initial seizures were associated with fever in 3 cases.The longest duration of convulsive seizures lasted more than 2 hours in all cases,followed by the appearance of hemiplegia involving the side of the body where the convulsions were localized.Subsequent epilepsy developed within 2 years of the hemiplegia in all 5 cases,with 4 cases simple partial seizure and 1 case psychomotor seizures.All cases had mental retardation.Cranial MRI showed left hippocampal sclerosis in 4 patients and CT brain scan showed right cerebral hemiatrophy in 1 case.The EEG showed abnormality in all patients.Epileptiform discharges were found in 4 cases,and the damage hemisphere showed slowing and low voltage of background activity in 1 case.All cases received mainly carbamazepine therapy,and the seizures were satisfactorily controlled or partly controlled.Conclusions HHE syndrome is recognized as hemiplegia-epilepsy syndrome after prolonged hemiconvulsion.The hippocampal sclerosis may be the consequence of repeated seizures,but not the cause.Better understanding of the syndrome,early and adequate treatment of prolonged seizures,especially of febrile convulsion,will reduce the incidence of HHE syndrome.