RESUMO
Hemiconvulsion-Hemiplegia-Epilepsy (HHE) syndrome is a rare pediatric epilepsy syndrome characterized by prolonged focal febrile convulsive status epilepticus with unilateral hemispheric cerebral edema and followed by the subsequent development of hemiplegia, global atrophy of the affected hemisphere, and epilepsy. The pathophysiology of HHE syndrome remains poorly understood though is clearly multifactorial. Factors thus far implicated are hyperthermia, pro-inflammatory state, and cytotoxic edema from prolonged ictal activity. Prognosis is variable, from the resolution of hemiplegia and seizures to permanent hemiparesis and refractory epilepsy. We describe a 2-year-old boy who presented with super-refractory focal status epilepticus in the setting of acute Coronavirus Infectious Disease-2019 (COVID-19) and multisystem inflammatory syndrome in children (MIS-C). He had right-sided hemiplegia on neurological examination, and an MRI of the brain showed left cerebral hemispheric edema consistent with HHE syndrome. Our case represents the first report in the literature on HHE syndrome in the setting of acute COVID-19 and MIS-C.
RESUMO
Metabotropic Glutamate Receptor 1 (mGluR1) encephalitis is a rare encephalitis characterized by ataxia, neuropsychiatric symptoms, dysarthria and cognitive impairment. This disease process has been described in several adult patients and has been associated with paraneoplastic syndrome in Hodgkin's lymphoma and other cancers as well as parainfectious and underlying autoimmune etiologies. However, only two cases of anti-mGluR1 encephalitis in children have been reported in the literature. The underlying etiology of one case was not provided but post-infectious disease has been reported. Here, we report the first case of anti-mGluR1 encephalitis in a child with a presumed "idiopathic" basis.
