Authors: P. Ševčík 1; H. Vacovská 1; V. Rohan 1; P. Lavička 2; Z. Ambler 1; J. Polívka 1; Z. Hess 3 Authors‘ workplace: Neurologická klinika LF UK a FN Plzeň 1; Neurochirurgické oddělení LF UK a FN Plzeň 2; II. interní klinika LF UK a FN Plzeň 3 Published in: Cesk Slov Neurol N 2007; 70/103(6): 702-706 Category: Case Report
Práce byla podpořena výzkumným záměrem MŠM 0021620816
Hypothalamic hamartoma can cause catastrophic and extremely drug-resistant epilepsy with epileptic encephalopathy. The first epileptic seizure is typically gelastic, and is followed by other types of seizures. Diagnosing small hypothalamic hamartoma with the use of magnetic resonance imaging of the brain may be difficult. Neurosurgical treatment, including radiosurgery, is complicated due to the location, and involves the risk of side effects. However, removal of hypothalamic hamartoma has a very positive effect on epileptic seizures and prevents the development of encephalopathy. We report a case of a patient with hypothalamic hamartoma successfully treated with stereotactic radiosurgery.
Key words: hypothalamic hamartoma – epilepsy – drug-resistance – gelastic seizures – secondary epileptogenesis – epileptic encephalopathy – Leksell Gamma Knife
1. Freeman JL, Coleman LT, Wellard RM, Kean MJ, Rosenfeld JV, Jackson GD et al. MR imaging and spectroscopic study of epileptogenic hypothalamic hamartomas: analysis of 72 cases. AJNR Am J Neuroradiol 2004; 25 : 450–462.
2. Valdueza JM, Cristante L, Dammann O, Bentele K, Vortmeyer A, Saeger W et al. Hypothalamic hamartomas: with special reference to gelastic epilepsy and surgery. Neurosurgery. 1994; 34 : 949–958.
3. Berkovic SF, Andermann F, Melanson D, Ethier RE, Feindel W, Gloor P et al: Hypothalamic hamartomas and ictal laughter: Evolution of a characteristic epileptic syndrome and diagnostic value of magnetic resonance imaging. Ann Neurol 1988; 23 : 429–439.
4. Ng YT, Rekate HL, Prenger EC, Chung SS, Feiz-Erfan I, Wang NC et al. Transcallosal resection of hypothalamic hamartoma for intractable epilepsy. Epilepsia 2006; 47 : 1192–1202.
5. Vojtěch Z, Kalina M. Stereotaktická radiochirurgie. In: Brázdil M, Hadač J, Marusič P (eds). Farmakorezistentní epilepsie. 1st. ed, Praha: Triton 2004 : 245–251.
6. Regis J, Hayashi M, Eupierre LP, Villeneuve N, Bartolomei F, Brue T et al. Gamma knife surgery for epilepsy related to hypothalamic hamartomas. Acta Neurochir Suppl 2004; 91 : 33–50.
7. Berkovic SF, Arzimanoglou A, Kuzniecky R, Harvey AS, Palmini A, Andermann F. Hypothalamic Hamartoma and Seizures: A Treatable Epileptic Encephalopathy. Epilepsia 2003; 44 : 969–973.
8. Kreuzberg B, Kastner J, Ferda J. The contribution of MRI to the diagnosis of diffuse meningeal lesions. Neuroradiology 2004; 46 : 198–204.
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