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马尾综合征合并β地中海贫血:一例报告

Cauda equina syndrome with beta thalassemia: a case report.

作者信息

Almoallem Ahmed, Alhamad Saud, Bin Dajim Nayef, Harfouch Elamir Bachar, Bashir Shahid

机构信息

Spine Department, Neuroscience Center, King Fahad Specialist Hospital- Dammam. 6830 Ammar Bin Thabet St, Al Muraikabat, Dammam, 32253-3202, Saudi Arabia.

Neuroscience Center, King Fahad Specialist Hospital- Dammam. 6830 Ammar Bin Thabet St, Al Muraikabat, Dammam, 32253-3202, Saudi Arabia.

出版信息

Spinal Cord Ser Cases. 2024 Sep 27;10(1):68. doi: 10.1038/s41394-024-00681-y.

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.

摘要

引言

与β地中海贫血合并髓外造血相关的马尾综合征(CES)是一种罕见且具有挑战性的临床表现。全面的文献回顾显示,仅有少数病例记录,每个病例都展示了多种治疗方式及不同的治疗结果。

病例介绍

在本病例中,一名29岁男性,患有β地中海贫血,频繁输血,有脾切除术史,出现下背部疼痛加重2天,疼痛延伸至双下肢,伴有麻木和尿失禁。按照ASIA ISNCSCI身体评估评分系统,肛门张力和肛周感觉显著下降,提示可能存在骶神经根受累,但未检测到其他上肢或下肢的感觉或运动功能障碍。鉴于患者有频繁输血和地中海贫血病史,建议血液科会诊。脊柱磁共振成像显示髓外造血、椎间盘突出和马尾神经受压。

讨论

脊柱手术,包括减压和椎板切除术,在一年的随访期间使背痛和下肢症状得到改善。然而,持续的感觉障碍和神经源性膀胱需要持续的泌尿外科治疗。缺乏针对此类病例管理的明确指南凸显了进一步收集数据和全面评估治疗结果的必要性。

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Cauda equina syndrome with beta thalassemia: a case report.马尾综合征合并β地中海贫血:一例报告
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