Correia Inês, Marques Inês B, Ferreira Rogério, Sousa Lívia
Department of Neurology, Centro Hospitalar e Universitário de Coimbra, Praceta Professor Mota Pinto, 3000-075 Coimbra, Portugal.
Department of Internal Medicine, Centro Hospitalar e Universitário de Coimbra, Praceta Professor Mota Pinto, 3000-075 Coimbra, Portugal.
Case Rep Med. 2016;2016:9183979. doi: 10.1155/2016/9183979. Epub 2016 Apr 5.
Autoimmune thyroid disease may occasionally associate with unspecific neurological symptoms, which are more commonly insidious, include cognitive or behavioural symptoms, and may associate with tremor, myoclonus, or ataxia. We report a 61-year-old female patient who presented with chronic headache, insidious mood, and cognitive disturbance which evolved in a few months to dementia associated with exuberant limb myoclonus. Diagnostic workup revealed high anti-thyroid peroxidase antibody titers and an inflammatory CSF profile, and it was negative for other possible etiologies. Treatment with steroids induced significant improvement. The diagnosis of encephalopathy associated with autoimmune thyroid disease is still controversial given the fact that the clinical presentation and diagnostic workup are unspecific, the pathophysiology is still undetermined, and the diagnosis is mostly of exclusion. No direct correlation is found between anti-thyroid antibody titers and clinical presentation, and it is currently speculated that other still unrecognized antibodies may be responsible for this clinical entity. It is extremely important to recognize this entity because it is potentially treatable with immunotherapies. It is also increasingly recognized that clinical improvement with first-line treatment with steroids may be absent or incomplete, and other immunotherapies as immunosuppressants, intravenous immunoglobulin, or plasma exchange must be attempted in the clinical suspicion of EEAT.
自身免疫性甲状腺疾病偶尔可能与非特异性神经症状相关,这些症状通常较为隐匿,包括认知或行为症状,还可能伴有震颤、肌阵挛或共济失调。我们报告了一名61岁女性患者,她出现慢性头痛、隐匿性情绪变化及认知障碍,数月内进展为伴有肢体肌阵挛的痴呆。诊断检查显示抗甲状腺过氧化物酶抗体滴度升高及脑脊液炎症指标异常,其他可能病因检查为阴性。使用类固醇治疗后症状显著改善。鉴于临床表现和诊断检查缺乏特异性、病理生理机制仍未明确且诊断主要依靠排除法,自身免疫性甲状腺疾病相关脑病的诊断仍存在争议。抗甲状腺抗体滴度与临床表现之间未发现直接关联,目前推测可能存在其他尚未被认识的抗体导致了这一临床病症。认识到这一病症极为重要,因为它可能通过免疫疗法进行治疗。人们也越来越认识到,使用类固醇一线治疗可能无法出现临床改善或改善不完全,对于临床怀疑自身免疫性甲状腺疾病相关脑病的情况,必须尝试其他免疫疗法,如免疫抑制剂、静脉注射免疫球蛋白或血浆置换。