[Stereotactic radiosurgery in treatment of patients with hypothalamic hamartomas]

Hypothalamic hamartoma (HH) is malformation of the brain manifesting by paroxysmal disorders (gelastic seizures) and endocrine disorders (mostly precocious sexual development). Surgical resection of HH is associated with the risk of severe neurological and neuroendocrine complications. Alternative ways of treatment are being investigasted. Stereotactic radiosurgery is one of the leading techniques in treatment of pharmacoresistant seizures due to high effectiveness and low complication rate. Since 2005 till 2009 7 patients with HH were treated in Burdenko Moscow Neurosurgical Institute. Age of patients varied between 9 months and 20 years 9 mean - 8.5 years). In all cases the disease manifested by gelastic seizures with frequency differing between 2-3 per months and 17 per day. All patients received long-term combined anticonvulsant treatment (using valproates, carbamazepine, lamotrigine, oxcarbazepine, topiramate) with temporary and insufficient effect or without any effect. In 4 patients frequency of seizures increased during anticonvulsant therapy. I patient had been operated (partial resection of HH) with temporary effect which consisted of decrease in frequency of seizures for 8 months; later frequency of paroxysms increased. Main indications to radiosurgical treatment included progressive course of disease, low effectiveness of anticonvulsant therapy and high risk of direct surgical treatment. 6 patients were treated using linear accelerator "Novalis" and 1 using "Cyber-knife". Treatment was performed under pharmacological sedation (midazolam, ketamine). Marginal dose was 11-18 Gy (mean 15.7 Gy). Volume of hamartomas was 0.3-3 cm3. Follow-up period varied between 3 and 50 months. Decreased frequency of seizures was observed in 100% of cases. Stereotactic radiosurgery is an effective and safe method of treatment for HH and may be considered as treatment of choice for pharmacoresistant seizures caused by HH.