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Hypothalamic Hamartomas: A Comprehensive Review of the Literature – Part 1: Neurobiological Features, Clinical Presentations and Advancements in Diagnostic Tools
Clinical Neurology and Neurosurgery ( IF 1.9 ) Pub Date : 2020-10-01 , DOI: 10.1016/j.clineuro.2020.106076
Safwan O Alomari 1 , Mohammed N El Houshiemy 1 , Shadi Bsat 1 , Charbel K Moussalem 1 , Mohammed Allouh 2 , Ibrahim A Omeis 1
Affiliation  

Hypothalamic hamartomas (HH) are rare, non-neoplastic heterotopic tissues which contains normal neurons and glia including oligodendrocytes and fibrillary astrocytes but in an abnormal distribution. They arise from the floor of the third ventricle, tuber cinereum, or mammillary bodies. Estimated incidence ranges from 1 in 50,000-1 in 1,000,000. Hypothalamic hamartomas are associated with different clinical presentations including various types of seizures, most characteristically; the gelastic seizures, precocious puberty, cognitive impairment, and behavioral changes. In this review, the authors discuss advancements in different diagnostic elements of hypothalamic hamartoma; including clinical features, EEG findings, and neuroimaging techniques. Moreover, different classifications described in the literature will be discussed.

中文翻译:

下丘脑错构瘤:文献综合回顾 - 第 1 部分:神经生物学特征、临床表现和诊断工具的进展

下丘脑错构瘤 (HH) 是罕见的非肿瘤异位组织,包含正常神经元和神经胶质,包括少突胶质细胞和纤维状星形胶质细胞,但分布异常。它们起源于第三脑室底、灰质结节或乳头体。估计的发病率为五万分之一到百万分之一。下丘脑错构瘤与不同的临床表现有关,包括各种类型的癫痫发作,最典型的是;凝胶性癫痫发作、性早熟、认知障碍和行为改变。在这篇综述中,作者讨论了下丘脑错构瘤不同诊断要素的进展;包括临床特征、脑电图结果和神经影像学技术。此外,将讨论文献中描述的不同分类。
更新日期:2020-10-01
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