Central precocious puberty (CPP) is caused by premature activation of the hypothalamic-gonadal axis, and must be treated adequately. In particular, CPP that occurs at a relatively young age or in boys is likely to be caused by an organic lesion. Hypothalamic hamartoma (HH) is the most common organic cause of CPP. The present case report describes an 11-month-old female infant who presented with...

neural tissue arising from the wall of the hypothalamus. Patients with HH can present with gelastic seizures, precocious puberty or both1. Seizures are usually progressive and accompanied by worsening behavioral problems and developmental delay1. Stereotactic radiosurgery (SRS) has been shown to be safe and effective, resulting in improvement in seizures and halting tumor progression. Gamma Kni...

The progresses of neuroimaging have allowed an earlier detection of hypothalamic hamartoma in children presenting with gelastic or dacrystic seizures. Associated symptoms can include other types of seizures, precocious puberty, and behavioral or cognitive deterioration. Combination of all these features is not constant and, when present, their evolution may be variable. When epilepsy proves int...

PURPOSE Peutz-Jeghers syndrome (PJS) is a hamartomatous polyposis disorder with a high cancer risk. Debate exists about the premalignant potential of hamartomas. Also, treatment options other than surveillance are not available. Therefore, molecular alterations in hamartomas and PJS carcinomas were studied. The objective was (a) to evaluate expression of cyclooxygenase (COX)-2 as target for che...

Introduction: Hamartomas is a neoplasms composed of mature tissue elements from the affected site with disproportion between their components. Although lingual hamartomas are traditionally infrequent in the head and neck, a significant number of case reports with this disorder in infancy are arising from the literature.   Case Report:</em...

Ictal crying is a rare type of epileptic seizure associated with hypothalamic hamartoma and with other lesions such as tumours, vascular malformations, hippocampal sclerosis, or cerebral infarction. We describe the case of an infant with gelastic, dacrystic and other types of seizures associated with a giant hypothalamic hamartoma, and present a video sequence of dacrystic seizures. Dacrystic e...

An emerging strength of stereotactic RFTC is its high precision brain areas where conventional resection would carry a nificant risk for persistent neurological deficits. This observation s been made before by other groups who applied stereotactic TC to hypothalamic hamartomas and periventricular nodular terotopia. The volume-of-destruction achieved by RFTC can be anticipated liably. Commercial...

PURPOSE To present a case of small hypothalamic hamartoma (HH) causing gelastic seizures and treated with stereotactic radiofrequency thermocoagulation. CASE REPORT A 22-year-old man presented with intractable gelastic seizures and focal seizures refractory to medical treatment. Magnetic resonance imaging showed a 6 mm x 6 mm x 7 mm sessile intraventricular HH. Under local anesthesia, four in...

1Neurology Unit and Laboratories, Anna Meyer Children’s University Hospital, Florence; 2Stella Maris Foundation, Calambrone, Pisa; 3Health Sciences Department, University of Florence, Anna Meyer Children’s University Hospital, Florence; 4Quantitative and Functional Neuroradiology Research Program at Anna Meyer Children’s and Careggi University Hospitals, University of Florence, Florence; 5Medic...

The hypothalamic and neighbouring sellar regions give rise to a wide spectrum of malignant and benign tumours of glial/glioneuronal, meningothelial, mesenchymal, epithelial and germ cell origin [1]. Some of them are rather hamartomas than true neoplasms or they are regarded as malformations. Among this omnium gatherum of entities osteolipoma is a rare but sometimes symptomatic finding. The oste...