Department of Clinical Immunology and Rheumatology, Kalinga Institute of Medical Sciences, KIIT University, Bhubaneswar, India.
Lupus. 2023 Jul;32(8):1001-1007. doi: 10.1177/09612033231181636. Epub 2023 Jun 2.
Neuropsychiatric manifestations in systemic lupus erythematosus (SLE) occur in about half of the patients; however, movement disorders like Parkinsonism are rare. We describe a case of SLE who presented solely with features of Parkinsonism.
50-year-old female presented with global slowing of movements and slowing of speech since 2 months. On examination, she had mask-like facies with a faint malar rash sparing the nasolabial folds, hard palate ulcer, cog-wheel rigidity, and proximal muscle weakness. Lab evaluation revealed lymphopenia, high ESR, elevated lactate dehydrogenase, creatinine phosphokinase, AST, and ALT levels. She had high anti-dsDNA levels with low complements. Urinalysis showed proteinuria and hematuria. ANA was positive at a titer of 1:320, and she had positive anti-ribosomal-P antibody. She had severe flare with a SLEDAI of 33. She was treated with pulse IV methylprednisolone followed by cyclophosphamide (NIH protocol). At 4 weeks follow-up, she had dramatic improvement in her Parkinsonian symptoms and her proximal muscle weakness.
The prevalence of movement disorders in cases of neuropsychiatric SLE is very low at 0.7%, with chorea being most frequent and Parkinsonism rare. The pathogenesis is multifactorial including anti-dopaminergic antibodies or associated anti-phospholipids causing microvascular thrombosis or vasculitis of the thalamostriatal arteries or disease activity itself. As in our case, immunosuppression and optimal treatment of active lupus reverts symptoms in most cases.
A high index of suspicion needs to be exercised in cases of SLE presenting with Parkinsonism as adequate immunosuppression translates to near-complete recovery.
系统性红斑狼疮(SLE)患者约有一半会出现神经精神表现;然而,像帕金森病这样的运动障碍却很少见。我们描述了一例仅以帕金森病特征表现的 SLE 病例。
一名 50 岁女性,因动作缓慢和言语缓慢 2 个月就诊。检查时,她有面具样面容,轻度蝶形红斑,鼻唇沟未累及,硬腭溃疡,齿轮样强直和近端肌无力。实验室检查发现淋巴细胞减少、血沉升高、乳酸脱氢酶、肌酸磷酸激酶、天冬氨酸转氨酶和丙氨酸转氨酶水平升高。她的抗双链 DNA 水平高,补体低。尿蛋白和血尿。ANA 阳性,滴度为 1:320,抗核糖体 P 抗体阳性。SLEDAI 评分为 33,疾病严重发作。她接受了静脉注射甲基强的松龙脉冲治疗,随后接受环磷酰胺(NIH 方案)治疗。在 4 周的随访中,她的帕金森病症状和近端肌无力有明显改善。
神经精神性 SLE 病例中运动障碍的患病率非常低,为 0.7%,其中舞蹈症最为常见,帕金森病罕见。发病机制是多因素的,包括抗多巴胺能抗体或相关抗磷脂导致微血管血栓形成或丘脑纹状体动脉的血管炎,或疾病本身的活动。就像我们的病例一样,免疫抑制和最佳的狼疮活动治疗在大多数情况下都能使症状得到缓解。
对于出现帕金森病的 SLE 患者,需要高度怀疑,因为充分的免疫抑制会导致近乎完全的恢复。