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ISSN: 2637-4722

Progressing Aspects in Pediatrics and Neonatology

Mini Review(ISSN: 2637-4722)

Epileptic Syndromes Related to the Hypothalamic Hamartomas Volume 2 - Issue 5

Behzad Saberi*

  • Medical Research, Esfahan, Iran

Received: June 02, 2020   Published: July 29, 2020

Corresponding author: Behzad Saberi, Medical Research, Esfahan, Iran

DOI: 10.32474/PAPN.2020.02.000147

Abstract PDF


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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