Selig Megan, Lee Gloria, Lebowitz Brian, Franceschi Dinko, Absar Nicole
Department of Neuropsychiatry Stony Brook University Renaissance School of Medicine, Stony Brook, NY, USA.
Neurocognitive Clinic Mass General Brigham-Martha's Vineyard Hospital, Martha's Vineyard, MA, USA.
Case Rep Psychiatry. 2024 Aug 19;2024:2863662. doi: 10.1155/2024/2863662. eCollection 2024.
The differential diagnosis of a patient with cognitive, behavioral, and motor symptoms is broad. There is much overlap between neurocognitive disorders due to frontotemporal dementia and other subcortical dementia. A less known diagnosis, cerebellar cognitive affective syndrome (CCAS), should also be considered. . A 29-year-old female presented with ataxia and left-sided weakness. CSF showed oligoclonal bands, and MRI showed multiple white matter lesions with some atrophy. She was diagnosed with multiple sclerosis (MS). At age 35, she developed frontal lobe symptoms and executive dysfunction; she was diagnosed with MS with bipolar disorder. Neuropsychological evaluation at that time showed significant deficits in multiple cognitive domains. Subsequent MRI showed progressive frontotemporal atrophy, and FDG-PET uncovered hypometabolism in the frontotemporal lobes and cerebellum. At age 38, her behavior worsened with aggression, and she was started on olanzapine. She responded well with decreased agitation and improved motivation and attention. Compared with previous scans, most recent MRI and FDG-PET showed interval increase in cerebellar atrophy with increase in hypometabolism in the cerebellum, respectively.
Based on cerebellar, affective, and subcortical cognitive examination findings, our diagnosis is probable CCAS. The cerebellum should be considered as a possible etiology of frontal subcortical cognitive impairment.
患有认知、行为和运动症状的患者的鉴别诊断范围广泛。额颞叶痴呆所致的神经认知障碍与其他皮质下痴呆之间存在很多重叠。还应考虑一种鲜为人知的诊断，即小脑认知情感综合征（CCAS）。一名29岁女性出现共济失调和左侧肢体无力。脑脊液显示寡克隆带，磁共振成像（MRI）显示多个白质病变并伴有一些萎缩。她被诊断为多发性硬化症（MS）。35岁时，她出现额叶症状和执行功能障碍；她被诊断为患有双相情感障碍的MS。当时的神经心理学评估显示多个认知领域存在显著缺陷。随后的MRI显示额颞叶进行性萎缩，氟代脱氧葡萄糖正电子发射断层扫描（FDG-PET）显示额颞叶和小脑代谢减低。38岁时，她的行为因攻击性而恶化，开始使用奥氮平治疗。她的激越症状减轻，动机和注意力得到改善，治疗反应良好。与之前的扫描相比，最近的MRI和FDG-PET分别显示小脑萎缩加重以及小脑代谢减低增加。
根据小脑、情感和皮质下认知检查结果，我们的诊断可能为CCAS。小脑应被视为额叶皮质下认知障碍的一种可能病因。
