The benign spectrum of hypothalamic hamartomas: Infrequent epilepsy and normal cognition in patients presenting with central precocious puberty

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The benign spectrum of hypothalamic hamartomas: Infrequent epilepsy and normal cognition in patients presenting with central precocious puberty

PURPOSE Hypothalamic hamartoma (HH) is the main structural cause of central precocious puberty (CPP). HH is frequently associated with cognitive impairment and epileptic encephalopathies. Disease severity in case series from neurology services may be biased towards more neurologically impaired patients. AIM To perform a prospective cognitive evaluation in patients with HH presenting with CPP ...

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Gene expression profiling of hypothalamic hamartomas: a search for genes associated with central precocious puberty.

BACKGROUND Hypothalamic hamartomas (HHs) are congenital lesions composed of neurons and astroglia. Frequently, HHs cause central precocious puberty (CPP) and/or gelastic seizures. Because HHs might express genes similar to those required for the initiation of normal puberty, we used cDNA arrays to compare the gene expression profile of an HH associated with CPP with three HHs not accompanied by...

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Central precocious puberty secondary to hypothalamic hamartoma

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 ...

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Whether all patients with Central precocious puberty should be treated?

Extended Abstract   Precocious puberty is the appearance of secondary sexual characteristics before 8 years old in girls and 9 years old in boys. Precocious puberty is divided into 3 groups of central, peripheral and normal variants. Central precocious puberty is accompanied by activation of hypothalamic-pituitary-gonadal axis which causes increase in secretion of GnRH, and in turn increase i...

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Central precocious puberty due to hypothalamic hamartomas correlates with anatomic features but not with expression of GnRH, TGFalpha, or KISS1.

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...

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ژورنال

عنوان ژورنال: Seizure

سال: 2013

ISSN: 1059-1311

DOI: 10.1016/j.seizure.2012.09.013