Department of Neurology, University of São Paulo, São Paulo, Brazil.
Department of Hematology, University of São Paulo, São Paulo, Brazil.
Spinal Cord Ser Cases. 2021 Sep 25;7(1):85. doi: 10.1038/s41394-021-00449-8.
Although neurological complications are well recognized in patients with sickle cell disease, myelopathy has been rarely described, with few reported cases of compressive and ischemic myelopathy. We present the first case report of longitudinally extensive myelitis (LETM) in SCD and review the differential diagnosis of myelopathy in these patients.
We report the case of a 29-year-old African-Brazilian man with SCD, who experienced a subacute flaccid paraparesis, with T2 sensory level and urinary retention. Cerebrospinal fluid analysis showed a lymphocytic pleocytosis and increased protein levels. MRI disclosed a longitudinally extensive spinal cord lesion, with a high T2/STIR signal extending from C2 to T12. We searched Medline/PubMed, Embase, Scopus, and Google Scholar databases for myelopathy in SCD patients.
Spinal cord compression by vertebral fractures, extramedullary hematopoietic tissue, and Salmonella epidural abscess have been reported in SCD. We found only three case reports of spinal cord infarction, which is unexpectedly infrequent compared to the prevalence of cerebral infarction in SCD. We found only one case report of varicella-zoster myelitis and no previous report of LETM in SCD patients. Specific and time-sensitive causes of myelopathy should be considered in SCD patients. In addition to compression and ischemia, LETM is a possible mechanism of spinal cord involvement in SCD patients.
尽管镰状细胞病患者的神经系统并发症已得到广泛认识，但很少有脊髓病变的报道，仅有少数压迫性和缺血性脊髓病变的病例报告。我们报告了首例镰状细胞病中长节段横贯性脊髓炎（LETM）的病例，并回顾了这些患者中脊髓病变的鉴别诊断。
我们报告了一例 29 岁的非洲裔巴西男性镰状细胞病患者，他经历了亚急性弛缓性截瘫，伴有 T2 感觉平面和尿潴留。脑脊液分析显示淋巴细胞增多和蛋白水平升高。MRI 显示脊髓长节段病变，T2/STIR 信号高，从 C2 延伸至 T12。我们在 Medline/PubMed、Embase、Scopus 和 Google Scholar 数据库中搜索了镰状细胞病患者的脊髓病变。
镰状细胞病中曾报道过椎体骨折、髓外造血组织和沙门氏菌硬膜外脓肿引起的脊髓压迫。我们仅发现了三例脊髓梗死的病例报告，与镰状细胞病中脑梗死的发生率相比，这一发生率出乎意料地低。我们仅发现一例带状疱疹性脊髓炎的病例报告，而镰状细胞病患者中从未有过 LETM 的报告。镰状细胞病患者应考虑到脊髓病变的特异性和时效性病因。除了压迫和缺血外，LETM 也是镰状细胞病患者脊髓受累的一种可能机制。
