Clinical Presentation and Management of Hypothalamic Hamartomas: A Systematic Review of the Literature

Abstract

Hypothalamic hamartomas (HHs) are composed of abnormally distributed but cytologically normal cellular elements. This disease typically manifests in infancy with gelastic seizures characterized by outbursts of mechanical laughter (mirthless laughter), altered or retained consciousness, refractory to antiepileptic, and later progresses to behavioral and cognitive disturbances and secondary seizure types. A systematic review of the literature on the clinical presentation and management of HHs. A literature search was carried out in electronic databases such as PubMed, COCHRANE, SCOPUS, ScienceDirect, which included the studies reported on “hypothalamic,” “hamartoma,” and “epilepsy,” as well as the outcome with full texts in English. Letters, case reports, reviews, preclinical studies, conference proceedings, protocols, and nonhuman studies were excluded. Duplicates were removed in EndNote X7, and titles and abstracts of all listed articles were scanned. Data analysis of all included studies indicates that smaller lesions result in better outcomes and stereotactic laser ablative procedures have the highest Engel class I outcome percentage. Delalande type II lesions are much more common (n=201) followed by type III (n = 182), IV (n = 71), and I (n = 50). Stereotactic laser ablation is safe for large lesions. HHs can be treated safely when they are small. Therefore, early identification and intervention play a major role. This also prevents the seizure progression, leading to morbidity. SLA has fewer complications and increased seizure-free survival compared to other modalities of treatment.