Gelastic seizures and hypothalamic hamartoma: a case report and review of literature.

Yıl 2011, Cilt: 3 Sayı: 6, 1 - 5, 11.01.2012

Arif Khan , Jenna Deemıng , Nahin Hussaın

Öz

Gelastic (laughing) epilepsy is characterized by recurrent unprovoked episodes of stereotyped, brief and mirthless laughter. Hypothalamic hamartoma is most often the cause of gelastic epilepsy. Hypothalamic gelastic epilepsy presenting as a progressive epileptic disease is well known, but isolated without multiple seizure types, precocious puberty, cognitive decline and behavioural problems is not usually reported. We
report a case of refractory gelastic seizures associated with indolent hypothalamic hamartoma and polysyndactyly with a follow-up of 18 years. Our case highlights the possibility of underdiagnosed hypothalamic hamartomas and unrecognised gelastic epilepsy in paediatric
population.

Anahtar Kelimeler

gelastic epilepsy, hypothalamic hamartoma, polydactaly, seizure disorder

Kaynakça

• Weissenberger AA, Dell ML, Liow K, et al., Aggression and psychiatric comorbidity in children with hypothalamic hamartomas and their unaffected sibling. J Am Acad Child Adolesc Psychiatry 2011; 40: 696-703.
• Brandberg G, Raininko R, Eeg-Olofsson O. Hypothalamic hamartoma with gelastic seizures in Swedish children and adolescents. Eur J Paediatr Neurol 2004; 8: 35-44.
• Kerringan JF, Ng YT, Chung S, Rekate HL. The hypothalamic hamartoma: a model of subcortical epileptogenesis and encephalopathy. Semin Pediatr Neurol. 2005; 12: 119-31.
• Goran Brandberg, Orvar Eeg-Olofsson. Gelastic seizures. ILAE website. Date of submission: April 19, 2001. Accessed 13 Sept 2006. http://www.ilae- epilepsy.org/ctf/gelastic.html.
• Schaefer GB, Olney AH. Hypothalamic dysfunction with polydactyly and hypoplastic nails. Semin Pediatr Neurol. 1999; 6: 238-242.
• Clarren SK, Alvord EC, Hall JG. Congenital hypothalamic hamartoblastoma, imperforate anus, and postaxial polydactyly – a new syndrome? Part II. Neuropathological considerations. Am J Med Genet. 1980; 7: 75-83
• Berkovic SF, Andermann F, Melanson D, et al. Hypothalamic evolution of a characteristic epileptic syndrome and diagnostic value of magnetic resonance imaging. Ann Neurol. 1988; 23: 429-439. and ictal laughter:
• N. Mullati, R. Selway and L. Nashef et al., The clinical spectrum of epilepsy and adults with hypothalamic hamartoma. Epilepsia 2003; 44: 1310-9.
• Murphy JV, Wheless JW, Schmoll CM. Left vagal nerve stimulation in six patients with hypothalamic hamartomas. Pediatr Neurol. 2000; 23: 167-8.