Cauda equina syndrome with beta thalassemia: a case report.

IF 0.7 Q4 CLINICAL NEUROLOGY Spinal Cord Series and Cases Pub Date : 2024-09-27 DOI:10.1038/s41394-024-00681-y
Ahmed Almoallem, Saud Alhamad, Nayef Bin Dajim, Elamir Bachar Harfouch, Shahid Bashir
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Abstract

Introduction: Cauda equina syndrome (CES) related to beta thalassemia with extramedullary hematopoiesis is a rarely reported and challenging clinical presentation. A thorough literature review revealed only a limited number of documented cases, each demonstrating a variety of treatment modalities with divergent outcomes.

Case presentation: In this case, a 29-year-old male with beta thalassemia, undergoing frequent blood transfusions, and with a history of splenectomy, presented with 2 days of worsening in his lower back pain, extending to both lower limbs, numbness, and urinary incontinence. Following the ASIA ISNCSCI scoring system for physical assessment, there was a significant decrease in anal tone and perianal sensation, suggesting possible sacral nerve roots involvement, but no other upper or lower extremities sensory or motor deficits were detected. Provided with the patient history of frequent blood transfusion and Thalassemia for which hematology referral was promoted. Spinal MRI revealed extramedullary hematopoiesis, disc protrusion, and cauda equina compression.

Discussion: Spine surgery, including decompression and laminectomy, resulted in improved back pain and lower limb symptoms during the one-year follow-up. However, persistent sensory impairment and neurogenic bladder necessitated ongoing urological management. The absence of clear guidelines for the management of such cases underscores the need for further data collection and comprehensive outcome reviews.

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地中海贫血马尾综合征:病例报告。
导言:与髓外造血的β地中海贫血相关的马尾综合征(CES)是一种极少报道且具有挑战性的临床表现。通过全面查阅文献,仅发现了数量有限的记录病例,每个病例都展示了多种治疗方法,但结果却各不相同:在本病例中,一名 29 岁的男性患者患有乙型地中海贫血症,经常接受输血,并有脾脏切除术史,2 天后出现下背部疼痛加剧,并延伸至双下肢、麻木和尿失禁。按照 ASIA ISNCSCI 评分系统进行体格评估后发现,患者的肛门张力和肛周感觉明显下降,这表明他的骶神经根可能受累,但没有发现其他上肢或下肢感觉或运动障碍。根据患者频繁输血和地中海贫血的病史,血液科建议转诊。脊柱磁共振成像显示髓外造血、椎间盘突出和马尾受压:讨论:包括减压和椎板切除术在内的脊柱手术使患者在一年的随访期间背痛和下肢症状得到了改善。然而,持续的感觉障碍和神经源性膀胱需要持续的泌尿科治疗。对于此类病例的管理缺乏明确的指导原则,因此需要进一步收集数据并进行全面的结果审查。
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来源期刊
Spinal Cord Series and Cases
Spinal Cord Series and Cases Medicine-Neurology (clinical)
CiteScore
2.20
自引率
8.30%
发文量
92
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