Freiha Joumana, Grand Effie, Marshall Ben, Arunchalam Ramamurthy, Pinto Ashwin, Osman Chinar
Department of Neurology, Wessex Neurological Centre, University Hospital Southampton, Southampton, UK.
Department of Haematology, Salisbury NHS Foundation Trust, Salisbury, UK.
BMC Neurol. 2025 Jan 11;25(1):16. doi: 10.1186/s12883-025-04020-y.
Sarcoid-like reaction is an immunological reaction that can affect lymph nodes and organs but does not meet the diagnostic criteria for systemic sarcoidosis. Anti-CD20 auto-antibodies have been reported to be responsible for such reactions. There are several reported associations between Chronic lymphocytic leukaemia (CLL), Amyotrophic lateral sclerosis (ALS) and Sarcoid-like reactions (SLR). We report a case of ALS developing in a patient with treated CLL and drug related SLR one day after exposure to Venetoclax and Rituximab. A 60-year-old male presented with lower limb rash, left leg weakness followed by bulbar symptoms which progressed over 12-months. Workup demonstrated a Cerebrospinal fluid (CSF) pleocytosis and inguinal lymphadenopathy. Skin and inguinal lymph node biopsies showed non-necrotising granulomata. Electromyography met diagnostic criteria for ALS. He was treated for presumed neurosarcoidosis mimicking ALS. Despite prednisolone and infliximab treatment, the motor symptoms rapidly progressed; Hence, we made a clinical diagnosis of ALS. We discuss the diagnostic and treatment challenges of this case.
结节病样反应是一种免疫反应，可累及淋巴结和器官，但不符合系统性结节病的诊断标准。据报道，抗CD20自身抗体是此类反应的病因。慢性淋巴细胞白血病（CLL）、肌萎缩侧索硬化症（ALS）与结节病样反应（SLR）之间有多种关联报道。我们报告一例在接受治疗的CLL患者中发生的ALS病例，该患者在接触维奈托克和利妥昔单抗一天后出现与药物相关的SLR。一名60岁男性出现下肢皮疹、左腿无力，随后出现延髓症状，并在12个月内逐渐进展。检查显示脑脊液（CSF）细胞增多和腹股沟淋巴结病。皮肤和腹股沟淋巴结活检显示非坏死性肉芽肿。肌电图符合ALS的诊断标准。他因疑似模仿ALS的神经结节病而接受治疗。尽管使用了泼尼松龙和英夫利昔单抗治疗，但运动症状迅速进展；因此，我们做出了ALS的临床诊断。我们讨论了该病例的诊断和治疗挑战。
