Friday, March 12, 2021

Lupine Publishers| Epileptic Syndromes Related to the Hypothalamic Hamartomas

 

Lupine Publishers Pediatrics and Neonatology 

Introduction

Gelastic epilepsy, progressive cognitive decline, behavioral disturbances, and precocious puberty, can be the symptoms of the hypothalamic hamartomas [1-3]. Gelastic seizures or the pressure to laugh phenomenon, are the hallmark of the hypothalamic hamartoma’s epilepsy. These seizures usually begin in the neonatal period and will develop to seizures types which would be catastrophic and refractory in their nature. Recurrent brief seizures which would be initiated by grimacing or laugher which is not emotional in nature, are the characteristics of the gelastic fits [4-6]. Although gelastic seizures are the main seizure types which are related to the hypothalamic hamartomas, but there are some other types of seizures which are related to such hamartomas and they usually would be more problematic and disabling in nature in comparison with gelastic seizures. Drop attacks and falling seizures, partial complex seizures, atypical absences, generalized tonic-clonic seizures and tonic seizures with or without secondary generalization, are the examples of other seizure types which can be occurred in the patients with hypothalamic hamartomas.


In the adult patient’s group with hypothalamic hamartomas, the severity of the seizures would be less than ones in the pediatric patient’s group. Cognitive and behavioral problems are also less common in the adult patient’s group [7]. These findings suggest that hypothalamic hamartomas related seizures, are more severe in their nature in the padiatric patients’ group.

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