Article
Stereotactic radiosurgery for hypothalamic hamartomas
Stereotaktische Radiochirurgie bei Hypothalamushamartomen
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Published: | May 4, 2005 |
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Outline
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Objective
Hypothalamic hamartomas are nonneoplastic lesions often characterised by central precocious puberty and gelastic epilepsy. Due to the delicate location surgery is often unsuccessful and associated with considerable risks. In the presented series, Gamma Knife radiosurgery was applied. Low doses of radiosurgery (10-20 Gy) have proved effective for the suppression of epileptogenic activity and minimise the risk of complication.
Methods
Four cases (aged between 5-13 years) who presented with medically intractable gelastic epilepsy and increasing secondary generalisation, abnormal behaviour and precocious puberty (3 cases) are reported. Hypothalamic hamartomas sized 11-17 mm had been diagnosed by MR imaging. Radiosurgical treatment was performed in general anaesthesia with margin doses of 12-14 Gy to the 50-90% isodoses covering volumes of 600-2300 ccm.
Results
After follow-up periods of 24 to 78 months, a continuing decrease both in seizure frequency and intensity was noted (outcome according to Engel: II a (3 cases) and III a (1 case)). All patients are socially reintegrated. MR imaging did not reveal significant changes concerning the size of the lesions.
Conclusions
Gamma Knife radiosurgery can be an effective and safe alternative treatment modality for HH capable of achieving good seizure control and improving behavioural disorders in selected cases. To date, the number of treated cases is still too small to draw final conclusions. Radiosurgery is still in search of an optimised radiation dose, localisation, and target volume.