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Pubertal abnormalitiesPrecocious and delayed
Published in Joseph S. Sanfilippo, Eduardo Lara-Torre, Veronica Gomez-Lobo, Sanfilippo's Textbook of Pediatric and Adolescent GynecologySecond Edition, 2019
Central precocity is characterized by growth acceleration, bone age advancement, thelarche, and adrenarche occurring at earlier than expected ages, with pubertal levels of LH and FSH. Menarche may also occur early. Hypothalamic hamartomas (HHs) may be found, particularly now that magnetic resonance imaging (MRI) is more advanced, though idiopathic causes remain frequent in girls. Among patients with CNS causes, the benign HH is most common. HH may be accompanied by seizures, laughing spells, and other neurologic findings. Concurrent follow-up with a neurosurgeon should be recommended. Genetic or syndromic causes may cause central precocity, as can trauma or surgery, all by interrupting the feedback inhibition of GnRH secretion. Obesity may advance bone age in girls and lead to central pubertal activation. Given that a third of U.S. children are overweight or have obesity, elevated BMI is a frequent cause of referral for early pubertal change. Additionally, one must consider early puberty to have a genetic cause in a girl with obesity if she has had other family members with precocious puberty.
Test Paper 3
Published in Teck Yew Chin, Susan Cheng Shelmerdine, Akash Ganguly, Chinedum Anosike, Get Through, 2017
Teck Yew Chin, Susan Cheng Shelmerdine, Akash Ganguly, Chinedum Anosike
Hypothalamic hamartomas are developmental malformations consisting of tumour-like masses located in the tuber cinereum of the hypothalamus. Most patients present in the first or second decade of life, with boys being more commonly affected than girls. These lesions have been divided into parahypothalamic hamartomas and intrahypothalamic hamartomas. Parahypothalamic hamartomas are pedunculated masses attached to the floor of the hypothalamus. These lesions seem more likely to be associated with precocious puberty than with gelastic seizures. Intrahypothalamic hamartomas are sessile masses with a broad attachment to the hypothalamus. They lie within the hypothalamus and may distort the contour of the third ventricle. In addition, they seem to be associated more often with gelastic seizures than with precocious puberty. At MR imaging, they are seen as well-defined pedunculated or sessile lesions at the tuber cinereum and are isointense or mildly hypointense on T1-weighted images and iso- to hyperintense on T2-weighted images, with no contrast enhancement or calcification. The absence of any long-term change in the size, shape or signal intensity of the lesion strongly supports the diagnosis of hypothalamic hamartoma.
Central nervous system
Published in Dave Maudgil, Anthony Watkinson, The Essential Guide to the New FRCR Part 2A and Radiology Boards, 2017
Dave Maudgil, Anthony Watkinson
Hypothalamic hamartoma is a congenital malformation associated with gelastic seizures (uncontrollable attacks of laughing). It is composed of a collection of heterotopic normal neuronal tissue located at or close to the mamillary bodies and is only rarely found within the hypothalamus itself. On CT and MRI it is isointense with brain tissue and does not enhance with intravenous contrast.
Laser thermal ablation in epilepsy
Published in Expert Review of Neurotherapeutics, 2019
Sanjeet S. Grewal, William O. Tatum
Hypothalamic Hamartomas (HH) is rare developmental hamartomatous non-neoplastic lesions composed of normal neurons located in or around the hypothalamus [63]. Patients with HH typically present with gelastic seizures that often start in childhood and may be associated with developmental delay [63,64]. Open surgery for drug-resistant seizures associated with a HH has previously shown significant morbidity and moderate efficacy may result [63,65–67]. This is explainable due to the close proximity of the hamartoma to the hypothalamus which incorporates essential functional neuronal connections, as well as other adjacent eloquent structures including the mammillary bodies.
Neonate, Infant, Childhood, and Adolescent Epilepsy Syndromes
Published in The Neurodiagnostic Journal, 2023
Hypothalamic hamartomas are very rare, congenital, non-neoplastic lesions, which are characteristically associated with gelastic (laughing episodes without mirth) or, less commonly, dacrystic (crying) seizures that typically begin in infancy or early childhood (Specchio et al. 2022).