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Medical management and antiepileptic drugs in hypothalamic hamartoma
Author(s) -
Helen Cross J.,
Spoudeas Helen
Publication year - 2017
Publication title -
epilepsia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.687
H-Index - 191
eISSN - 1528-1167
pISSN - 0013-9580
DOI - 10.1111/epi.13758
Subject(s) - hypothalamic hamartoma , gelastic seizure , medicine , epilepsy , precocious puberty , hamartoma , endocrine system , pediatrics , hypothalamic disease , psychiatry , pathology , hormone , hypogonadotropic hypogonadism
Summary Hypothalamic hamartoma may present with epilepsy, specifically gelastic or dacrystic seizures, or endocrine dysfunction, commonly precocious puberty. The epilepsy in many patients is drug resistant, and has a high association with progressive cognitive, learning and behavioral difficulty. Medical treatment of seizures remains problematic, with many resistant to drug treatment. Surgical resection, or disconnection of the hamartoma provides the optimal chance of seizure control but with a relatively high risk of endocrine dysfunction, the result of interference with the hypothalamic‐pituitary axis in many. Careful assessment and monitoring by specialist centers with discussion of optimal intervention is required for individual cases.