Mesial temporal lobe epilepsy with hippocampal sclerosis

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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://www.orphadata.org. Data version July 2024

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Project CASK

VISION Project CASK believes in a world free of the life limitations and debilitating effects of CASK gene disorders. ​MISSION To accelerate breakthroughs in research to develop treatments and a cure for CASK gene disorders. ​VALUES Collaboration. Transparency. Urgency.

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