Genidy Radwa, Abbas Aymen, Adi Abd Al Kareem, Abdullah Shazia, Nizam Ahmad
Internal Medicine, Sheikh Khalifa Medical City, Abu Dhabi, ARE.
Rheumatology, Sheikh Khalifa Medical City, Abu Dhabi, ARE.
Cureus. 2024 Nov 7;16(11):e73262. doi: 10.7759/cureus.73262. eCollection 2024 Nov.
Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT) is a rare condition that can present with multiple neurological and psychiatric manifestations. SREAT diagnosis poses a challenge due to the nature of its nonspecific symptomatology and its overlap with numerous autoimmune, metabolic, infectious, and neuropsychiatric disorders. It is associated with elevated anti-thyroid antibodies, occurs in correspondence with autoimmune thyroiditis, and shows great response to corticosteroid treatment. We present the case of a 27-year-old female patient with a complex medical history including systemic lupus erythematosus (SLE) and autoimmune thyroiditis. She presented to the hospital with bizarre behavior, psychosis, and confusion. Urine toxicology screen and septic workup were negative. Neuropsychiatric SLE (NPSLE) was high on the list of differential diagnosis but was excluded as the patient's previous lupus flares demonstrated an upward trend in anti-double-stranded deoxyribonucleic acid antibody (anti-dsDNA Ab) titers and low complement levels and leukocyte counts which were not present during this admission. Her cerebrospinal fluid (CSF) analysis was unremarkable except for higher-than-normal immunoglobulin G4 (IgG4) levels. Brain magnetic resonance imaging (MRI) was normal, and magnetic resonance angiography (MRA)/magnetic resonance venography (MRV) studies were unremarkable. Serum anti-thyroid antibodies were elevated which led to the consideration of Hashimoto encephalopathy (HE). SREAT diagnosis was made, and she made a remarkable recovery with the commencement of high-dose steroids slowly tapered over the course of weeks. Post-discharge outpatient visits showed back-to-baseline neurological and psychiatric status. It is important to note that both SREAT and NPSLE are rare diagnoses. They both overlap in many areas including their similar presentation, the lack of confirmatory tests, the diagnostic challenges, and their excellent response to steroids.
自身免疫性甲状腺炎相关的类固醇反应性脑病（SREAT）是一种罕见疾病，可出现多种神经和精神症状。由于其非特异性症状的性质以及与众多自身免疫性、代谢性、感染性和神经精神性疾病的重叠，SREAT的诊断具有挑战性。它与抗甲状腺抗体升高有关，与自身免疫性甲状腺炎同时发生，并且对皮质类固醇治疗反应良好。我们报告一例27岁女性患者，其病史复杂，包括系统性红斑狼疮（SLE）和自身免疫性甲状腺炎。她因怪异行为、精神病和意识模糊入院。尿液毒理学筛查和败血症检查均为阴性。神经精神性SLE（NPSLE）在鉴别诊断中可能性较大，但被排除，因为该患者先前的狼疮发作显示抗双链脱氧核糖核酸抗体（抗dsDNA Ab）滴度呈上升趋势，补体水平和白细胞计数较低，而此次入院时并未出现这些情况。她的脑脊液（CSF）分析除免疫球蛋白G4（IgG4）水平高于正常外无异常。脑磁共振成像（MRI）正常，磁共振血管造影（MRA）/磁共振静脉造影（MRV）检查无异常。血清抗甲状腺抗体升高，这导致考虑桥本脑病（HE）。诊断为SREAT，在开始使用大剂量类固醇并在数周内缓慢减量后，她有显著恢复。出院后的门诊随访显示神经和精神状态恢复到基线水平。需要注意的是，SREAT和NPSLE都是罕见的诊断。它们在许多方面都有重叠，包括相似的表现、缺乏确诊检查、诊断挑战以及对类固醇的良好反应。
