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继发性发作性睡病作为小儿视路胶质瘤病例病情恶化的体征

Secondary Narcolepsy as Worsening Sign in a Pediatric Case of Optic Pathway Glioma.

作者信息

Laus Beatrice, Caroleo Anna Maria, Colafati Giovanna Stefania, Carai Andrea, Moavero Romina, Ferilli Michela Ada Noris, Valeriani Massimiliano, Mastronuzzi Angela, Cacchione Antonella

机构信息

Hispalense Institute of Pediatrics, 41014 Seville, Spain.

Department of Onco-Hematology, Cell Therapy, Gene Therapy, and Hemopoietic Transplant, Bambino Gesù Children's Hospital (IRCCS), Piazza Sant'Onofrio 4, 00165 Rome, Italy.

出版信息

Children (Basel). 2022 Sep 23;9(10):1455. doi: 10.3390/children9101455.

Abstract

Narcolepsy, a neurologic disorder that leads to excessive daytime sleepiness, may represent a rare consequence of neoplastic lesions involving the sellar/parasellar and hypothalamic regions, the anatomical areas responsible for wakefulness. Optic pathway gliomas represent the most common neoplasm of these regions and present an excellent overall survival, while long-term neurologic impairments, such as visual loss, endocrinopathies, or sleep disorders, are the principal causes of morbidity. In this case report, we describe a non-NF1 patient suffering from a very extensive optical pathway glioma, who several years after the diagnosis in a radiological condition of stable disease, presented with severe narcolepsy, a rare complication, that led to the death of the patient.

摘要

发作性睡病是一种导致日间过度嗜睡的神经系统疾病,可能是累及鞍区/鞍旁及下丘脑区域(负责清醒的解剖区域)的肿瘤性病变的罕见后果。视神经通路胶质瘤是这些区域最常见的肿瘤,总体生存率良好,而长期神经功能障碍,如视力丧失、内分泌疾病或睡眠障碍,是发病的主要原因。在本病例报告中,我们描述了一名非神经纤维瘤病1型(NF1)患者,患有非常广泛的视神经通路胶质瘤,在诊断为疾病稳定的放射学状态数年之后,出现了严重的发作性睡病,这是一种罕见的并发症,最终导致患者死亡。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7c65/9600478/cd5d308b0e3e/children-09-01455-g001.jpg

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