The diagnosis and management of hypothalamic hamartomas in children

Abstract

Hypothalamic hamartoma (HH) is a rare developmental malformation often characterized by gelastic seizures. Recent advances in treating HH have led to dramatic improvements. However, clinical protocol of HH is poorly understood. Since 2002, department Pediatric Neurosurgery of Xinhua Hospital has maintained a multidisciplinary clinical program to evaluate and treat children with HH. This program has provided the opportunity to investigate the management of HH. In this review, we summarize the clinical progress and propose a clear management principle for different HH patients.

Minimally invasive procedures for hypothalamic hamartoma–related epilepsy: a systematic review and meta-analysis

“…HHs are associated with several seizures, including gelastic, complex partial, tonic-clonic, and secondarily generalized seizures. 6 Among the seizure types, gelastic attacks are the most common and associate explicitly with HH; these seizures are defined by inappropriate laughing and are often resistant to antiepileptic agents. 1 Gelastic and dacrystic (associated with a crying sound and less common than gelastic) seizures 7 are the primary seizure types in patients with an HH, and their onset usually occurs in the 1st year of life.…”