hypothalamic hamartoma
Pronunciation: hy-poh-thah-LAM-ik ha-mar-TOH-mah
A benign brain lesion in the hypothalamus that commonly causes gelastic seizures and precocious puberty in children.
Full Definition
A hypothalamic hamartoma is a benign, non-neoplastic brain lesion consisting of normal hypothalamic tissue in an abnormal location or configuration. These lesions are strongly associated with gelastic (laughing) seizures, which often begin in infancy and may initially be the only seizure type. Many patients also develop precocious puberty due to the lesion's effect on hypothalamic function controlling hormonal regulation. Over time, patients may develop additional seizure types, cognitive decline, and behavioral problems. Treatment options include surgical resection, stereotactic radiosurgery, or laser ablation, with the choice depending on lesion size, location, and patient factors.
Usage
Usage note: Benign lesion, not a tumor. Strongly associated with gelastic seizures and precocious puberty.
In Context
- "MRI revealed a hypothalamic hamartoma consistent with the patient's gelastic seizures and early puberty." — Radiology report
- "Laser ablation of the hypothalamic hamartoma resulted in seizure freedom at one-year follow-up." — Outcome study