Centre de référence des mastocytoses, Hôpital Necker Enfants malades, Fondation Imagine Paris, Université Paris Descartes, Sorbonne, Paris Cité, Paris, France.
PLoS One. 2012;7(6):e39468. doi: 10.1371/journal.pone.0039468. Epub 2012 Jun 20.
Mastocytosis is a heterogeneous disease characterized by mast cells accumulation in one or more organs. We have reported that depression is frequent in mastocytosis, but although it was already described, little is known about the prevalence and features of cognitive impairment. Our objective was to describe the prevalence and features of cognitive impairment in a large cohort of patients with this rare disease (n = 57; mean age = 45) and to explore the relations between memory impairment and depression. Objective memory impairment was evaluated using the 3(rd) edition of the Clinical Memory scale of Wechsler. Depression symptoms were evaluated using the Hamilton Depression Rating Scale. Age and education levels were controlled for all patients. Patients with mastocytosis presented high levels of cognitive impairment (memory and/or attention) (n = 22; 38.6%). Cognitive impairment was moderate in 59% of the cases, concerned immediate auditory (41%) and working memory (73%) and was not associated to depression (p≥0.717). In conclusion, immediate auditory memory and attention impairment in mastocytosis are frequent, even in young individuals, and are not consecutive to depression. In mastocytosis, cognitive complaints call for complex neuropsychological assessment. Mild-moderate cognitive impairment and depression constitute two specific but somewhat independent syndromes in mastocytosis. These results suggest differential effects of mast-cell activity in the brain, on systems involved in emotionality and in cognition.
肥大细胞病是一种以一个或多个器官中肥大细胞积聚为特征的异质性疾病。我们已经报道过肥大细胞病患者常伴有抑郁，但尽管这种情况已经被描述过，但是对于认知障碍的患病率和特征知之甚少。我们的目的是描述在这一罕见疾病的大型患者队列中（n=57；平均年龄=45 岁）认知障碍的患病率和特征，并探讨记忆障碍与抑郁之间的关系。使用韦氏第三版临床记忆量表评估客观记忆障碍。使用汉密尔顿抑郁评定量表评估抑郁症状。对所有患者进行年龄和教育程度的控制。肥大细胞病患者表现出较高水平的认知障碍（记忆和/或注意力）（n=22；38.6%）。认知障碍的 59%为中度，涉及即时听觉（41%）和工作记忆（73%），与抑郁无关（p≥0.717）。总之，肥大细胞病中即时听觉记忆和注意力障碍很常见，即使是在年轻人中，也与抑郁无关。在肥大细胞病中，认知障碍需要进行复杂的神经心理学评估。轻度至中度认知障碍和抑郁在肥大细胞病中构成两种特定但有些独立的综合征。这些结果表明，肥大细胞活性对大脑中涉及情感和认知的系统有不同的影响。
