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Neuroimaging evidence of brain abnormalities in mastocytosis

机译:肥大细胞增多症脑异常的神经影像学证据

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摘要

Mastocytosis is a rare disease in which chronic symptoms are related to mast cell accumulation and activation. Patients can display depression–anxiety-like symptoms and cognitive impairment. The pathophysiology of these symptoms may be associated with tissular mast cell infiltration, mast cell mediator release or both. The objective of this study is to perform morphological or functional brain analyses in mastocytosis to identify brain changes associated with this mast cell disorder. We performed a prospective and monocentric comparative study to evaluate the link between subjective psycho-cognitive complaints, psychiatric evaluation and objective medical data using magnetic resonance imaging with morphological and perfusion sequences (arterial spin-labeled perfusion) in 39 patients with mastocytosis compared with 33 healthy controls. In the test cohort of 39 mastocytosis patients with psycho-cognitive complaints, we found that 49% of them had morphological brain abnormalities, mainly abnormal punctuated white matter abnormalities (WMA). WMA were equally frequent in cutaneous mastocytosis patients and indolent forms of systemic mastocytosis patients (42% and 41% of patients with WMA, respectively). Patients with WMA showed increased perfusion in the putamen compared with patients without WMA and with healthy controls. Putamen perfusion was also negatively correlated with depression subscores. This study demonstrates, for we believe the first time, a high prevalence of morphological and functional abnormalities in the brains of mastocytosis patients with neuropsychiatric complaints. Further studies are required to determine the mechanism underpinning this association and to ascertain its specificity.
机译:肥大细胞增多症是一种罕见的疾病,其慢性症状与肥大细胞的积累和激活有关。患者可以表现出抑郁-焦虑样症状和认知障碍。这些症状的病理生理可能与组织性肥大细胞浸润,肥大细胞介质释放或两者有关。这项研究的目的是在肥大细胞增多症中进行形态学或功能性大脑分析,以鉴定与此肥大细胞疾病相关的大脑变化。我们进行了一项前瞻性和单中心的比较研究,使用磁共振成像技术结合形态学和灌注序列(动脉自旋标记灌注)对39例肥大细胞增多症患者进行了前瞻性和单中心对比研究,以评估其主观心理认知障碍,精神病学评估和客观医学数据之间的联系,而33例健康控件。在39例患有心理认知障碍的肥大细胞增多症患者的测试队列中,我们发现其中49%的人患有形态学上的脑部异常,主要是斑状白质异常(WMA)。 WMA在皮肤肥大细胞增多症患者和惰性形式的全身性肥大细胞增多症患者中同样频繁(分别占WMA患者的42%和41%)。与没有WMA和健康对照的患者相比,WMA患者的核壳灌注增加。壳聚糖灌流与抑郁评分也呈负相关。这项研究表明,对于我们认为这是第一次,患有神经精神病的肥大细胞增多症患者的大脑中形态和功能异常的患病率很高。需要进一步的研究来确定支撑这种关联的机制并确定其特异性。

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