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Two Cases of Precocious Puberty Associated with Hypothalamic Hamartoma

机译：下丘脑血肿伴性早熟两例

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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.
机译：下丘脑错构瘤（HH）是根据磁共振成像（MRI）和组织学检查结果诊断的先天性畸形;它通常与中枢性性早熟（CPP），地核性癫痫发作，行为异常和智力低下有关。在本文中，我们报告了回顾性的假设，即症状与治疗以及HH的治疗之间存在关联，并描述了2例与CPP相关的HH病例。两种病例均在椎间盘水箱内有无蒂块，在MRI上延伸至下丘脑（分别为1.2？〜1.5 cm和2.0？〜2.5 cm）。第一例患有顽固性癫痫发作，而第二例没有癫痫发作。在这两名患者中，在第一种情况下通过γ刀和外科手术部分切除了错构瘤，在第二种情况下通过手术切除了错构瘤，并开具了促性腺激素释放激素（GnRH）类似物。一例显示智商（IQ）得分和癫痫发作均改善，另一例显示IQ和异常行为改善。很难确定任何拓扑/症状关系。手术和GnRH类似物治疗可以缓解癫痫，行为异常和与HH相关的智力低下。

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