Asadollahzadeh Elnaz, Shahmaei Vahid, Ebadi Zahra, Johari Mohammad-Sadegh, Rezaeimanesh Nasim, Naser Moghadasi Abdorreza
Multiple Sclerosis Research Center, Neuroscience Institute Tehran University of Medical Sciences Tehran Iran.
MAHAK Hematology Oncology Research Center (MAHAK-HORC), MAHAK Hospital Shahid Beheshti University of Medical Sciences Tehran Iran.
Clin Case Rep. 2024 Oct 31;12(11):e9546. doi: 10.1002/ccr3.9546. eCollection 2024 Nov.
This case highlights a rare form of solitary sclerosis with a relapsing pattern, contrasting with the typical single attack and nonrelapsing nature of the disease. Despite the lack of new lesions on MRI, This case demonstrates that, despite the rarity of this variant, a relapsing form of solitary sclerosis can occur and should be considered a differential diagnosis.
Progressive solitary sclerosis is characterized by isolated demyelinating damage to the central nervous system in the spinal cord and brainstem, leading to progressive motor impairment. We describe the case of a 49-year-old woman who suffered several recurrent attacks of right hemiparesis over time. The patient initially responded well to methylprednisolone pulse therapy without maintenance therapy. However, subsequent episodes resulted in mild residual symptoms and the progression of her condition. Clinical examination revealed normal cranial nerve function, decreased sensation in the right limbs, and abnormal signal findings on MRI of the cervical spine. Laboratory tests, vasculitis screening, cerebrospinal fluid (CSF) analysis, and brain/spinal cord angiography were all within normal limits. Based on these findings and the patient's clinical presentation, a diagnosis of progressive solitary sclerosis with relapsing attacks was made. Rituximab treatment was initiated with administration of a first dose of 1000 mg, followed by a second dose 6 months later.
该病例突出了一种罕见的具有复发模式的孤立性硬化症，与该疾病典型的单次发作且无复发的性质形成对比。尽管磁共振成像（MRI）上未发现新病灶，但该病例表明，尽管这种变异型罕见，但复发性孤立性硬化症仍可能发生，应将其视为鉴别诊断之一。
进行性孤立性硬化症的特征是脊髓和脑干的中枢神经系统出现孤立性脱髓鞘损伤，导致进行性运动障碍。我们描述了一名49岁女性的病例，她随着时间的推移出现了几次右侧偏瘫的复发发作。患者最初对甲泼尼龙冲击疗法反应良好，无需维持治疗。然而，随后的发作导致了轻度残留症状和病情进展。临床检查显示颅神经功能正常、右侧肢体感觉减退以及颈椎MRI出现异常信号。实验室检查、血管炎筛查、脑脊液（CSF）分析以及脑/脊髓血管造影均在正常范围内。基于这些发现和患者的临床表现，诊断为伴有复发发作的进行性孤立性硬化症。开始使用利妥昔单抗治疗，首剂给予1000毫克，6个月后给予第二剂。
