Hamartoma, derived from the Greek hamartia meaning “failure”, is a benign malformation composed of local native tissues with disorganized growth. Hamartomas are the most common benign tumors of the lung and the pediatric heart (cardiac rhabdomyomas), though they can be found throughout the body in most anatomical structures. Though typically benign ‘incidentalomas’, they can cause adjacent comp...

Hypothalamic hamartomas are often associated with early onset gelastic seizures, precocious puberty, behavioral problem and suboptimal response to antiepiletptic drugs. Until now, four surgical options have been reported to reduce seizure by >50%. Surgical excision have good seizure outcome but postoperative complications were not infrequent, whether by pterional or transcallosal interforniceal...

Pulmonary chondromatous (or chondroid) hamartomas are common benign tumours of the lung and may be either parenchymal or endobronchial. The latter are only rarely encountered and have a variation in their clinical presentation related to the endobronchial obstructive lesion, including atelectasis, pneumonia, hemoptysis. If not diagnosed early and treated properly, endobronchial hamartomas can c...

Objective: The epilepsy associated with the hypothalamic hamartomas constitutes a syndrome with peculiar seizures, usually refractory to medical therapy, mild cognitive delay, behavioural problems and multifocal spike activity in the scalp electroencephalogram (EEG). The cortical origin of spikes has been widely assumed but not specifically demonstrated. Methods: We present results of a source ...

Pathological laughter has been recognized as an epileptic manifestation since the dawn of modern neurology, and is attributed to Trousseau. The association of laughing (gelastic) seizures with hypothalamic hamartoma was clearly described nearly 50 years ago [1], although the probable first case, described as an ‘astrocytoma’ was published in 1938 [1, 2]. In 1967, Gascon and Lombroso analyzed th...

True precocious puberty occurs as a result of the premature release of luteinizing hormone-releasing hormone from the hypothalamus, which stimulates the secretion of the pituitary gonadotropins, which in turn stimulate the gonadal sex steroids. The differential diagnosis of true precocious puberty includes cerebral and idiopathic categories. This differentiation, which cannot be made endocrinol...