hypothalamic hamartoma (hh) is a rare intracranial lesion that usually presents with classic triad of central precocious puberty, gelastic epilepsy, and developmental delay. herein, a 14-year old boy is presented in whom the diagnosis of hh was made by magnetic resonance imaging. while he did not have any complain of precocious puberty, he surprisingly suffered from delay in puberty. the defini...

BACKGROUND AND PURPOSE Reports of MR imaging in hypothalamic hamartomas associated with epilepsy are few, and the number of patients studied is small. We aimed to detail the relationship of hypothalamic hamartomas to surrounding structures, to determine the frequency and nature of associated abnormalities, and to gain insight into mechanisms of epileptogenesis. METHODS We systematically exami...

Manifestations of non-syndromic hypothalamic hamartomas are different from those of the syndromic hamartomas, and the syndromic ones are rare. In spite of the rarity of the syndromic hamartomas, they can be a powerful biological tool to help us understand the pathophysiology of these heritable disorders. Several disorders that include hypothalamic hamartomas are reviewed here and the current un...

BACKGROUND/AIMS Hypothalamic hamartomas are the most common identifiable cause of central precocious puberty (CPP). Hamartoma characteristics proposed to be associated with CPP include specific anatomic features and expression of molecules such as gonadotropin-releasing hormone (GnRH), transforming growth factor alpha (TGFalpha), and GRM1A, which encodes the type 1 metabotropic glutamate recept...