Hypothalamic hamartoma is a potentially complex entity with diverse clinical manifestations. We report a case of gelastic seizures associated with a hypothalamic hamartoma, which followed a benign course. A 31-year-old woman with episodes of laughter was referred for diagnostic evaluation. Her initial MRI and EEG were reported as normal. However, her episodes of laughter were typical of gelasti...

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...

Hypothalamic hamartomas are often associated to a progressive epileptic encephalopathy. Non-invasive data indicates that destruction or isolation of the hamartoma may stop seizures. We present data on patients who underwent stereotactic radiofrequency with or without endoscopy. Epilepsy improved in 60% of patients with minimal morbidity. This approach was less successful in large lesions. These...

Whether the intrinsic epileptogenicity of hypothalamic hamartomas (HH) is responsible for the entire clinical spectrum of epileptic, neuropsychological and behavioural disorders associated with HH, remains an open issue, in as much as morphologically similar HH can be associated with dramatically different seizure types and cognitive outcomes. The aim of this study was to investigate brain gluc...

Hypothalamic hamartomas (HH) are developmental malformations that are associated with gelastic seizures, other types of seizures, cognitive decline, and symptoms related to hypothalamic dysfunction. Although aggressive behavior is frequently described, data on the neuropsychiatric profile are limited. In this article, five patients with HH are described who displayed a wide variety of psychiatr...

Hypothalamic hamartomas present with isolated fits of ictal laughter (gelastic epilepsy) or a combination of gelastic and other types of seizures. Many of these patients also suffer from cognitive decline, neuropsychiatric comorbidities and precocious puberty. Although there is a large body of anecdotal evidence about hypothalamic hamartomas and gelastic seizures, many questions still remain to...

PURPOSE Hypothalamic hamartoma (HH) related epilepsy presents with gelastic seizures (GS), other seizure types and cognitive deterioration. Although seizure origin in GS has been well established, non-GS are poorly characterized. Their relationship with the HH and cognitive deterioration remains poorly understood. We analyzed seizure type, spread pattern in non-GS and their relationship with th...