Lo spettro e la storia naturale della sindrome epilessia gelastica-amartoma ipotalamico

Translated title of the contribution: The spectrum and the natural history of gelastic seizures-hypothalamic hamartoma syndrome

S. Striano, P. Striano, C. Sarappa, P. Boccella, C. Nocerino, L. Bilo, R. Meo, P. Ruosi, A. Elefante, F. Tortora

Research output: Contribution to journalArticle

Abstract

Background. Hypothalamic Hamartoma (HH) has been recognized as etiologic factor of a catastrophic infantile epileptic encephalopathy, whose hallmark are early-onset gelastic seizures (GS), associated with precocious puberty (PP). Recently, different clinical features have been related to GS-HH. Purpose. To delineate clinical spectrum and evolutive characteristics of the GS-HH syndrome. Patients and Methods. Patients with HH, observed since 1986, for whom at least one ictal VEEG or EEG recording of GS was available. Results. 6 subjects (4 M, 2 F), with sessile HH ranging 0.8-1.7 cm in diameter. Clinical onset: GS (2 months-20 years); evolution towards a secondary generalized epilepsy (SGE) in 1, drug-resistant (DR) partial seizures (usually complex), 2-13 years after GS onset. No patients had PP. Severe cognitive impairment in SGE patient, mild cognitive defect in other two. Patients with HH less than 1 cm in diameter did not showed neuropsychological or behavioural disturbances; DR was present in all cases. Surgical removal of HH markedly improved the clinical evolution in 2 pts. Conclusions. GS-HH syndrome may show a different spectrum of severity and course. Catastrophic evolution, and DR can be reversible after surgical (or by gamma-knife) ablation of HH.

Translated title of the contributionThe spectrum and the natural history of gelastic seizures-hypothalamic hamartoma syndrome
Original languageItalian
Pages (from-to)55-56
Number of pages2
JournalBollettino - Lega Italiana contro l'Epilessia
Issue number121-122
Publication statusPublished - Jul 2003

ASJC Scopus subject areas

  • Clinical Neurology

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    Striano, S., Striano, P., Sarappa, C., Boccella, P., Nocerino, C., Bilo, L., Meo, R., Ruosi, P., Elefante, A., & Tortora, F. (2003). Lo spettro e la storia naturale della sindrome epilessia gelastica-amartoma ipotalamico. Bollettino - Lega Italiana contro l'Epilessia, (121-122), 55-56.