Hypothalamic hamartomas and ictal laughter: Evolution of a characteristic epileptic syndrome and diagnostic value of magnetic resonance imaging
- 1 May 1988
- journal article
- research article
- Published by Wiley in Annals of Neurology
- Vol. 23 (5), 429-439
- https://doi.org/10.1002/ana.410230502
Abstract
Detailed study of 4 patients and review of the literature allowed us to delineate further the epileptic syndrome associated with hypothalamic hamartomas, which characteristically begins in infancy with laughing seizures. Because early childhood psychomotor development is usually normal, the condition appears benign and may not even be recognized. The episodes of laughter are brief, frequent, and mechanical in nature. These features distinguish it from other forms of epileptic laughter, particularly that which occurs in temporal lobe epilepsy. Subsequently, the seizures become longer, other seizure types appear, and between the ages of 4 and 10 years, the clinical and electroencephalographic features of secondary generalized epilepsy develop. Cognitive deterioration occurs and severe behavior problems are frequent. Prognosis for seizure control and social adjustment is poor. Cortical abnormality occurs in association with the hypothalamic hamartoma. The lesions are best detected by magnetic resonance imaging but may be difficult to identify by computed tomographic scanning.Keywords
This publication has 41 references indexed in Scilit:
- The NIH experience with precocious puberty: Diagnostic subgroups and response to short-term luteinizing hormone releasing hormone analogue therapyThe Journal of Pediatrics, 1986
- Gelastic Epilepsy and True Precocious Puberty due to Hypothalamic HamartomaDevelopmental Medicine and Child Neurology, 1984
- How many cases of true precocious puberty in girls are idiopathic?The Journal of Pediatrics, 1983
- Hypothalamic HamartomaJournal of Computer Assisted Tomography, 1981
- Epileptic laughter with precocious puberty.Archives of Disease in Childhood, 1978
- Hypothalamic HamartomaNew England Journal of Medicine, 1977
- Gelastic Epilepsy A Review and Report of Five CasesEpilepsia, 1971
- Epileptic (Gelastic) LaughterEpilepsia, 1971
- DISORDERS OF LAUGHTER DUE TO BRAIN LESIONSBrain, 1956
- Hamartoma of the hypothalamus: Report of two cases with associated developmental defectsThe Journal of Pediatrics, 1953