Gamma knife surgery for hypothalamic hamartomas accompanied by medically intractable epilepsy and precocious puberty: experience in Mexico.

OBJECT: Hypothalamic hamartoma is a nonneoplastic malformative mass of neurons and glia in the region of the hypothalamus. Because of its location, open surgery is associated with high morbidity and mortality rates. Gamma knife surgery (GKS) may be an efficient and safe treatment approach, which produces little morbidity. The authors describe the results of GKS in three patients with hypothalamic hamartomas. METHODS: All patients were male, aged 3, 12, and 15 years. The lesions were classified according to the Valdueza scale: one was Type IIb and two were Type IIa. The patients presented with gelastic seizures (15-20 per day), generalized epilepsy, behavioral abnormalities, and alterations of the sleep cycle. Precocious puberty was present in one patient. The Type IIb tumor had a volume of 1.8 cm3, and the Type IIa tumors were 597 mm3 and 530.1 mm3. The lesions received 12.5 Gy, 14 Gy, and 15 Gy, respectively, to the 50% isodose line. The patients were followed for 30 to 50 months. After 3 months, all patients showed improvement of their sleep, behavior, and epilepsy. At the present time, these patients are receiving low-dose antiepileptic agents and have achieved adequate social development and school integration. CONCLUSIONS: Gamma knife surgery appears to be a good, safe, and effective option for the treatment of selected hypothalamic hamartomas. No morbidity or mortality was associated with these three cases.

Participación del nervio trigémino en el reflejo acústico humano / Role of the trigeminal nerve in the acoustic reflex in humans

Para investigar sobre la participación del nervio trigémino y del músculo tensor del tímpano en el reflejo acústico humano se planteó un estudio prospectivo en once pacientes portadores de neuralgia del trigémino y programados para microcompresión del Ganglio de Gasser con balón percutáneo. Todos los pacientes presentaron estudios audiológico normales, incluyendo umbrales tonales, BéKésy, logoaudiometría, potenciales evocados y timpanograma, así como integridad del nervio facial detectada por clínica y por electroneuronografía. Los once pacientes presentaban ausencia del reflejo acústico. Seis meses después de la compresión del Ganglio de Gasser, tres pacientes presentaron reflejo acústico normal y en el resto permaneció ausente. Los ocho pacientes que no presentaron reflejo cursaron con paresia de músculos masticadores. Nuestros resultados parecen indicar que para la integridad del reflejo acústico es necesario tanto el sistema facial como el trigeminal. El reflejo acústico puede ser una prueba útil en el estudio de la neuralgia de trigémino