Beatty Cynthia, Bowler Rachael A, Farooq Osman, Dudeck Lindsay, Ramasamy Deepa, Yeh E Ann, Zivadinov Robert, Weinstock-Guttman Bianca, Parrish Joy B
Division of Pediatric Neurology, Department of Neurology, University at Buffalo, Women & Children's Hospital of Buffalo, Buffalo, New York.
Department of Neurology, University at Buffalo, Buffalo, New York.
Pediatr Neurol. 2016 Apr;57:64-73. doi: 10.1016/j.pediatrneurol.2016.01.003. Epub 2016 Jan 7.
Acute disseminated encephalomyelitis (ADEM) is a demyelinating disorder that is usually self-limited. Recent studies have suggested ongoing neurological deficits and neurocognitive impairment in these patients. Little information on the correlation of clinical and neuroimaging markers in ADEM is available. We examined potential clinical factors (e.g., age of onset, acute symptom duration, magnetic resonance imaging [MRI] lesions) and their relation to neurocognitive and psychosocial outcomes.
This is a retrospective chart review of consecutive pediatric patients diagnosed with ADEM between 2006 and 2012. Patients were evaluated with standard neurological assessment, MRI of the brain, and neuropsychological evaluation.
Twenty-three patients with ADEM with average age at neuropsychological assessment of 10.1 years (±3.50) were included. Five (22.7%) patients were impaired on three or more neurocognitive measures. Psychosocial problems were reported in 20%-40% of patients. Earlier age of onset was correlated with poorer sustained attention and psychosocial problems, whereas acute symptom duration and Expanded Disability Status Scale were not. MRI outcomes were correlated with psychosocial outcomes but not neuropsychological findings.
Our findings suggest lingering cognitive and psychosocial deficits in children with a history of ADEM. Clinical features and MRI findings correlated more strongly with psychosocial outcomes than cognitive functioning. Further studies are needed to confirm relationships and other possible contributing factors to lingering deficits.
急性播散性脑脊髓炎(ADEM)是一种通常为自限性的脱髓鞘疾病。近期研究表明这些患者存在持续的神经功能缺损和神经认知障碍。关于ADEM临床和神经影像学标志物之间相关性的信息较少。我们研究了潜在的临床因素(如发病年龄、急性症状持续时间、磁共振成像[MRI]病变)及其与神经认知和社会心理结局的关系。
这是一项对2006年至2012年间连续诊断为ADEM的儿科患者的回顾性病历审查。患者接受了标准的神经学评估、脑部MRI和神经心理学评估。
纳入了23例ADEM患者,神经心理学评估时的平均年龄为10.1岁(±3.50)。5例(22.7%)患者在三项或更多神经认知测量中存在受损。20%-40%的患者报告有社会心理问题。发病年龄较早与持续注意力较差和社会心理问题相关,而急性症状持续时间和扩展残疾状态量表则无关。MRI结果与社会心理结局相关,但与神经心理学发现无关。
我们的研究结果表明,有ADEM病史的儿童存在持续的认知和社会心理缺陷。临床特征和MRI发现与社会心理结局的相关性比与认知功能的相关性更强。需要进一步研究来证实这些关系以及导致持续缺陷的其他可能因素。