一位年轻女士急性小脑共济失调的罕见原因:罕见的红斑狼疮路径

R. Gomes
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摘要

神经精神表现常见于系统性红斑狼疮(SLE)患者。特别是,已知神经系统受累在抗心磷脂抗体和狼疮抗凝剂阳性的患者中更常见。然而,小脑性共济失调很少被报道,特别是作为神经精神系统性红斑狼疮(NPSLE)的第一个临床表现。脑血管梗死或缺血、血管源性水肿和抗体介导的脑血管病或血管化过程被认为是SLE相关急性小脑性共济失调的可能病因。我们报告一位38岁的糖尿病女性的临床和放射学特征,她发展为快速进展的小脑综合征作为SLE的第一个征兆;没有发现其他原因可以解释她的小脑性共济失调。该患者的脑部影像显示明显的小脑萎缩。患者最初脉搏静脉注射甲基强的松龙,后来口服强的松龙和口服霉酚酸酯。治疗开始后,她的神经症状没有进一步恶化。病因不明的小脑综合征的出现与可能的全身自身免疫功能障碍相关,应在临床实践中考虑到适当的诊断检查,以便提供有效的治疗方案。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
An Unwonted Cause of Acute Cerebellar Ataxia in a Young Lady: A Rare Path to Lupus
Neuropsychiatric manifestations are commonly observed in systemic lupus erythematosus (SLE) patients. In particular, neurological involvement is known to be more common in patients with positive anticardiolipin antibodies and lupus anticoagulants. Nevertheless, cerebellar ataxia has rarely been reported, especially as the first clinical manifestation of neuropsychiatric systemic lupus erythematosus (NPSLE). Cerebral vascular infarction or ischemia, vasogenic oedema and antibody-mediated cerebral vasculopathy or vasculitic process have been supposed as possible aetiologies of acute cerebellar ataxia related to SLE. We report the clinical and radiological features of a 38 years old diabetic lady who developed a rapidly progressive cerebellar syndrome as first sign of SLE; no other cause explaining her cerebellar ataxia was found. Imaging of brain in this patient revealed prominent cerebellar atrophy. She was treated with initial pulse intravenous methylprednisolone later oral prednisolone and oral mycophenolate mofetil. There was no further progression of her neurological signs after the initiation of therapy. The appearance of a cerebellar syndrome with unknown aetiology with associated features of possible systemic autoimmune dysfunction, should be taken into account in clinical practice for appropriate diagnostic workup in order to provide effective therapeutic options.
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