Central precocious puberty due to hypothalamic hamartoma in a 7-month-old infant girl
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Gelastic seizure
Girl
Central precocious puberty
Hypothalamic disease
Hamartoma
Hypothalamic hamartomas (HHs) are benign lesions that are often associated with central precocious puberty and may present with gelastic seizures. Treatment modalities for HH include medical therapy with long-term gonadotropin-releasing hormone analogs or resection. The authors report the case of a 7-year-old girl who was diagnosed with an HH due to precocious puberty and was treated medically with a gonadotropin-releasing hormone analog for 3 years. Despite normalization of her plasma levels of luteinizing hormone, follicle-stimulating hormone, and estradiol and arrest of her precocious puberty, the patient developed progressive weight gain associated with extreme hyperphagia and morbid obesity by the age of 10 years. Her compulsive eating patterns were refractory to counseling and other interventions attempted by her parents and physicians. After resection of the HH, her hyperphagia resolved and her weight stabilized. To the authors' knowledge, this is the first report describing resection of an HH for the purpose of treating hyperphagia and obesity.
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We described the clinical presentation,image changes,pathologic manifestation,and hormone data before and after surgical excision in a 2 years-old boy with typical central precocious puberty (CPP) resulted from hypothalamic hamartoma(HH).With literature review,the epidemiologic and clinical features,hormone profile,diagnosis,and therapeutic options of CPP induced by HH were discussed to update the general understandings for the management of this disease.
Key words:
Hypothalamic disease; Hamartoma; Central precocious puberty
Hamartoma
Central precocious puberty
Presentation (obstetrics)
Hypothalamic disease
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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 definite diagnosis of HH can only be made by appropriate imaging, in a case with atypical feature of delay in puberty and in the absence of gelastic epilepsy. To our best knowledge, this is the first case of HH who is presented with delay in puberty as of first manifestation.
Gelastic seizure
Hamartoma
Central precocious puberty
Delayed puberty
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Girl
Gelastic seizure
Hypothalamic disease
Hamartoma
Central precocious puberty
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Citations (1)
Hamartoma
Gelastic seizure
Hypothalamic disease
Central precocious puberty
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Gelastic seizure
Girl
Central precocious puberty
Hypothalamic disease
Hamartoma
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Citations (14)
Hypothalamic hamartoma is an uncommon cause of central precocious puberty and sometimes of gelastic epilepsy and delayed development. The purpose of this report is to describe a case of central precocious puberty in an 18-month-old girl who was referred to our department for further investigation of vaginal bleeding that had appeared during the 8th month of life. The patient's puberty was compatible with Tanner stage 3, her psychomotor development was normal, and epilepsy was not observed. Diagnosis was based on abnormal hormonal workup findings and discovery of a hypothalamic hamartoma on brain magnetic resonance imaging. Neurosurgery was not performed. Administration of long-acting gonadotropin releasing hormone analog to slow puberty led to clinical improvement. Hypothalamic hamartoma must always be suspected in case of central precocious puberty. Although magnetic resonance imaging is the most reliable modality, it is not always available nor affordable in low-income countries. Neurosurgery is not indicated in patients without epileptic seizure. Gonadotropin releasing hormone agonists are usually indicated to slow down puberty and improve the adult height. However, the cost is prohibitive in developing countries.
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Hamartoma
Hypothalamic disease
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Hypothalamic hamartoma is a rare congenital nonneoplastic heterotopia consisting of neurons, glial cells and fiber bundles. Clinically, most patients with hypothalamic hamartomas present with precocious puberty and/or gelastic epilepsy. We report an interesting case of hypothalamic hamartoma causing precocious puberty in a young male. The lesion was excised totally through frontotemporal craniotomy and transSylvian approach without any added morbidity.
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Hamartoma
Central precocious puberty
Heterotopia
Hypothalamic disease
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Hypothalamic hamartoma (HH) is a congenital malformation diagnosed based on magnetic resonance imaging (MRI) and histological findings; it is often associated with central precocious puberty (CPP), gelastic seizures, abnormal behavior and mental retardation. In the present paper, we report our retrospective hypothesis that there is a relationship between symptoms and therapy, as well as the treatment for HH, and describe two cases of HH associated with CPP. Both cases had sessile masses located in the interpeduncular cistern, with extension to the hypothalamus on MRI (1.2 ~ 1.5 cm and 2.0 ~ 2.5 cm, respectively). The first case had intractable seizures, while the second had no seizures with paroxysmal discharge. In both patients, the hamartomas were partially removed, by γ-knife and surgical operation in the first case and surgically in the second, and a gonadotropin releasing hormone (GnRH) analogue was prescribed. One case showed improvement of both intelligence quotient (IQ) score and seizures, and the other showed improvements in IQ and abnormal behavior. It was difficult to determine any topology/symptom relationships. Surgery and GnRH analogue treatment can alleviate seizures, abnormal behavior and mental retardation associated with HH.
Central precocious puberty
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