Mesial temporal lobe epilepsy with hippocampal sclerosis

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Mesial temporal lobe epilepsy with hippocampal sclerosis

Synonyms: HS-MTLE | Hippocampal sclerosis-related mesial temporal lobe epilepsy | MTLE-HS

Mesial temporal lobe epilepsy with hippocampal sclerosis is a rare epilepsy syndrome defined by seizures originating in limbic areas of the mesial temporal lobe particularly in the hippocampus amygdala and in the parahippocampal gyrus and its connections and hippocampal sclerosis usually unilateral or assymetric. It is frequently associated with an initial precipitating event such as febrile seizures hypoxia intracranial infection or head trauma most often occurring in the first five years of life followed by a latent period without seizures. Typical seizures consist of a characteristic aura that is frequently a rising epigastric sensation associated with emotional disturbances illusions and autonomic symptoms (widened pupils palpitations) progressive impairment of consciousness oro-alimentary automatisms (lip smacking chewing licking tooth grinding) behavioral arrest head deviation dystonic postures hand and verbal automatisms. Seizures are followed by postictal dysfunction. Initially seizures are easily controlled with antiepileptic drugs later they frequently become refractory and associated with progressive behavioral changes and memory deficits.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.

Reference: Access aggregated data from Orphanet at Orphadata.

Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http: // Data version September 2023.

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Advocacy Organizations

Pediatric Epilepsy Research Consortium

The Pediatric Epilepsy Research Consortium (PERC) is a group of US pediatric epilepsy centers, researchers and professionals in field of epilepsy whose goal is to provide a network and infrastructure to facilitate collegial, collaborative practice-changing research that will provide answers needed to improve the care of children with epilepsy.

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