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首页> 外文期刊>The Internet Journal of Neurology >Neurological manifestations in a patient with SLE complicated by TTP
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Neurological manifestations in a patient with SLE complicated by TTP

机译：SLE合并TTP的神经系统表现

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Neurological involvement in Systemic Lupus Erythematosus (SLE) is common, occurring in up to 14 to 75% of patients in different series.1 We describe a case of active SLE complicated by thrombotic thrombocytopenic purpura (TTP) in a 21-year-old woman and discuss the neurological and radiological findings. The significance of early diagnosis of SLE complicated by TTP and its aggressive treatment with plasma exchange and concomitant cyclophosphamide therapy is emphasized. Case Report A 21-year-old African American woman was admitted to our hospital with complaints of bilateral lower extremity edema and reduced urine output for the past 2 weeks. She had been diagnosed with SLE one year previously and was maintained on 20 mg of oral prednisolone daily. She was found to have a blood urea nitrogen (BUN) of 63 mg/dL and a serum creatinine of 2.5 mg/dL. A renal biopsy was scheduled to document the pathologic nature of her lupus nephritis. While awaiting the renal biopsy, the patient had two focal seizures starting from the right hand with rapid secondary generalization. Neurological examination immediately after the ictal event revealed a conscious woman with post-ictal confusion. She followed simple commands and had right-sided focal weakness with a right pronator drift. Power was 4/5 Medical Research Council (MRC) grade in the right arm and 5/5 MRC grade in the other limbs. Deep tendon reflexes were 3+ in the knees and ankles with well-sustained bilateral ankle clonus and bilateral extensor plantar response. Following the seizure the patient's condition decompensated necessitating transfer to the medical intensive care unit, where she was intubated for airway protection.Laboratory evaluation revealed a WBC count of 1800/ mm3 (cubic millimeter), hemoglobin=9.7g/dl, hematocrit of 28.1 % and platelet count of 18000/ mm3. Reticulocyte count was 7.6% and peripheral smear showed abundant schistocytes cells along with some tear drop cells (1%). LDH was 1249 IU/L, serum haptoglobin was less than 8 (normal 43-212 Units) and ANA titer was positive at 1: 80. Massive proteinuria, hematuria and urinary casts were present. MRI of the brain revealed multiple areas of T2 hyperintensity in the right and left cerebellar cortex and subcortical areas as well as the right and left frontal, parietal, temporal and occipital cortex and sub cortical areas. Similar findings were noted in the right and left lentiform nuclei, anterior and posterior limbs of the internal capsule with prominent gyral and irregular sub-cortical enhancement after intravenous gadolinium suggestive of sub-acute infarcts and vasculitis (Fig. 1a-d). Normal enhancement of the cortical veins and venous sinuses was visualized on MRV. MRA of the circle of Willis was normal.
机译：系统性红斑狼疮（SLE）的神经系统疾病很常见，在不同系列的患者中发生率高达14％至75％。1我们描述了一例活动性SLE并发血栓性血小板减少性紫癜（TTP）的21岁女性。并讨论神经和放射学发现。强调了早期诊断为SLE并发TTP的重要性，并积极地进行血浆置换和环磷酰胺治疗。病例报告一名21岁的非洲裔美国妇女在过去2周内因双侧下肢浮肿和尿量减少而入院。一年前，她被诊断出患有SLE，每天口服20 mg泼尼松龙。发现她的血液尿素氮（BUN）为63 mg / dL，血清肌酐为2.5 mg / dL。计划进行肾脏活检以记录其狼疮性肾炎的病理性质。在等待肾脏活检时，患者从右手开始有两次局灶性发作，继发快速继发性泛化。发作后立即进行神经系统检查，发现一名意识障碍的妇女患有发作后混乱。她遵循简单的命令，右侧偏侧无力，右旋前屈。右臂的力量为4/5，而其他四肢的力量为5/5。膝和踝关节的深部肌腱反射为3+，伴有双侧踝支气管持续良好和双侧伸肌足底反应。癫痫发作后患者病情失代偿，必须转移至重症监护病房，在该病房插管进行气道保护。实验室评估显示白细胞计数为1800 / mm3（立方毫米），血红蛋白= 9.7g / dl，血细胞比容为28.1％血小板计数为18000 / mm3。网织红细胞计数为7.6％，外周涂片显示丰富的血吸虫细胞以及一些泪滴细胞（占1％）。 LDH为1249 IU / L，血清触珠蛋白低于8（正常43-212单位），ANA滴度在1:80呈阳性。存在大量蛋白尿，血尿和尿管结扎。脑部MRI显示右小脑皮层和左小脑皮层和皮层下区域以及额叶，右额叶，顶叶，颞叶和枕叶皮层以及皮层下区域有多个T2高信号区域。静脉注射ado后，内囊的右，左半形核，前囊和后肢出现类似的发现，具有明显的回旋和不规则的皮质下增强，提示亚急性梗塞和血管炎（图1a-d）。在MRV上可以看到皮层静脉和静脉窦的正常增强。威利斯环的MRA正常。

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