Abstract

CEMELI-CANO, Mercedes et al. Hypothalamyc hamartomas: different ways of clinical debut. Cases report. Arch. argent. pediatr. [online]. 2015, vol.113, n.6, pp.e323-e326. ISSN 0325-0075.  http://dx.doi.org/10.5546/aap.2015.e323.

Hypothalamic hamartomas are benign tumors of gray substance composed by hyperplasic neurons. They are usually asymptomatic small masses with extensions into the third ventricular cavity. In some instances they can cause cognitive behavioral alterations, seizures and/or central precocious puberty depending on the location. Here we present two cases of central precocious puberty due to hypothalamic hamartomas at 2^8/12 and 7 years of age. The younger patient also presents gelastic seizures, typically associated with hypothalamic hamartomas. After the clinical and radiological findings, they started treatment with GnRH analogues and a regression of the puberty signs without progression in the hamartomas size was observed.

Keywords : Hypothalamic hamartomas; Central precocious puberty; Gelastic epilepsy.

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