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eRAM

encyclopedia of Rare Disease Annotation for Precision Medicine




Disease hypothalamic hamartoma
Symptom C0036572|seizures
Sentences 12
PubMedID- 24791078 other surgically remediable epilepsy syndromes include focal epilepsies due to discrete resectable structural lesions, epilepsies due to diffuse hemispheric disturbances, such as hemimegencephaly, rasmussen's encephalitis, sturge-weber syndrome, and large porencephalic cysts, and gelastic seizures with hypothalamic hamartomas, because seizures originate within this alien tissue.
PubMedID- 24808722 hypothalamic hamartomas typically present with focal seizures, which may have a gelastic semiology.
PubMedID- 21269296 Purpose: human hypothalamic hamartomas (hhs) are associated with gelastic seizures, intrinsically epileptogenic, and notoriously refractory to medical therapy.
PubMedID- 21377333 seizures associated with hypothalamic hamartoma (hh) are notoriously intractable to medical therapy, and while surgical resection affords most affected patients with complete or near seizure-freedom, there remains a need to identify alternative treatments.
PubMedID- 25495642 Mechanisms of intrinsic epileptogenesis in human gelastic seizures with hypothalamic hamartoma.
PubMedID- PMC4001227 Other surgically remediable syndromes include neocortical epilepsies due to structural lesions that can be easily resected, diffuse hemispheric disturbances such as rasmussen's encephalitis, hemimegencephaly, sturge-weber, and large porencephalic cysts, that can be treated by hemispherectomy or hemispherotomy, and gelastic seizures with hypothalamic hamartoma, which is the source of the ictal events.
PubMedID- 21212454 Gelastic seizures are typically associated with hypothalamic hamartoma.
PubMedID- 20887368 Ictogenesis and symptomatogenesis of gelastic seizures in hypothalamic hamartomas: an ictal spect study.
PubMedID- 24328885 Gelastic seizures associated with hypothalamic hamartomas (hhs) are a clinicoradiologic syndrome presenting with a variety of symptoms, including pharmacoresistant epilepsy with multiple seizure types, electroencephalography (eeg) abnormalities, precocious puberty, behavioral disturbances, and progressive cognitive deterioration.
PubMedID- 20677592 hypothalamic hamartoma presenting with gelastic seizures, generalized convulsions, and ictal psychosis.
PubMedID- 22595332 Psychogenic gelastic seizures in a patient with hypothalamic hamartoma.
PubMedID- 22823743 Gelastic seizures due to hypothalamic hamartoma: rapid resolution after endoscopic tumor disconnection.

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