AIM To study the surgical procedures and results on seizures, in 18 patients with refractory epilepsy due to hypothalamic hamartoma. PATIENTS AND METHODS Eighteen patients aged from 9 months to 32 years underwent surgery between 1997 and 2002. The mean age at seizure onset was 15.5 months. Seventeen patients had gelastic seizures, 14 had partial seizures, two had infantile spasms, 10 had toni...

Hypothalamic hamartomas are congenital malformations: consisting of disorganized, mature, neuronal elements in proportions, similar to that of normal tissue. The clinical presentation may include any combination of the following: asymptomatic, precocious puberty, behavioral disturbances and various types of seizures. The seizure pattern usually consists of laughing (gelastic seizures) beginning...

In a recent review article on gelastic seizures and hypothalamic hamartoma, Tellez-Zenteno and colleagues 1 used the term, " status gelasticus " mostly correctly but referenced completely incorrectly the origins of the definition of this rare but becoming increasingly known phenomenon. This mistake followed upon an unusual oversight of quoting another author who had simply used the term and mos...

Hamartomas are composed of cells native to an organ but abnormal in number, arrangement or maturity. In the tuberous sclerosis complex (TSC), hamartomas develop in multiple organs because of mutations in TSC1 or TSC2. Here we show that TSC2-null fibroblast-like cells grown from human TSC skin hamartomas induced normal human keratinocytes to form hair follicles and stimulated hamartomatous chang...

Introduction Central precocious puberty (CPP) presenting at a very young age is likely to have an underlying pathology. One of the pathologies is hypothalamic hamartoma (HH), a non-neoplastic tumour-like lesion located at the floor of the third ventricle, near the tuber cinereum. Two young children with CPP due to HH without gelastic seizures or mental retardation were successfully managed and ...