Beck Goichi, Yamashita Rika, Kawai Makiko, Yamamura Ryohei, Okuno Tatsusada, Matsui Misa, Toyooka Keiko, Morii Eiichi, Mochizuki Hideki, Murayama Shigeo
Department of Neurology, Osaka University Graduate School of Medicine, Suita, Japan.
Department of Pathology, Osaka University Graduate School of Medicine, Suita, Japan.
Front Immunol. 2024 Dec 5;15:1477615. doi: 10.3389/fimmu.2024.1477615. eCollection 2024.
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated disease that mainly affects the peripheral nerves and nerve roots and typically presents with distal dominant motor and sensory disturbances as clinical symptoms. Central nervous system (CNS) demyelination with inflammation occurs infrequently in patients with CIDP. Here, we present a unique autopsy report of CIDP causing severe demyelination along the entire spinal cord. A Japanese woman exhibited progressive muscle weakness, muscle atrophy, sensory disturbances, and tremors in her upper and lower extremities, which began in her 60s. A nerve conduction study revealed a marked prolongation of distal latencies and very low or no compound muscle action potential amplitudes, and cerebrospinal fluid protein levels were prominently elevated. Following the diagnosis of CIDP, a combination of methylprednisolone pulse therapy, high-dose intravenous immunoglobulin therapy, and plasma exchange mildly improved her symptoms. The patient died of septic shock at the age of 74 years. Neuropathological examination revealed demyelinating lesions with inflammation in the peripheral regions of the anterior, lateral, and posterior funiculi along the entire spinal cord (from the cervical to the sacral cord), and axons and neurons were well preserved in these lesions. The nerve roots in the cervical and lumbar plexuses, cauda equina, sciatic nerve, and sural nerve showed prominent swelling and edema with infiltration of inflammatory cells. Many onion bulbs were visible in the fascicles of the sciatic and sural nerves. Our results suggest that demyelination with inflammation can occur in the CNS and peripheral nervous system in CIDP, especially in patients with specific conditions, such as severe intrathecal inflammation.
慢性炎症性脱髓鞘性多发性神经根神经病（CIDP）是一种免疫介导的疾病，主要影响周围神经和神经根，临床症状通常表现为以远端为主的运动和感觉障碍。CIDP患者很少发生伴有炎症的中枢神经系统（CNS）脱髓鞘。在此，我们报告一例独特的CIDP尸检病例，该病例导致整个脊髓严重脱髓鞘。一名日本女性在60多岁时开始出现进行性肌肉无力、肌肉萎缩、感觉障碍以及上下肢震颤。神经传导研究显示远端潜伏期显著延长，复合肌肉动作电位幅度极低或无，脑脊液蛋白水平显著升高。诊断为CIDP后，甲基强的松龙冲击疗法、大剂量静脉注射免疫球蛋白疗法和血浆置换联合应用使她的症状稍有改善。该患者74岁时死于感染性休克。神经病理学检查发现，沿整个脊髓（从颈髓到骶髓）的前索、外侧索和后索周边区域存在伴有炎症的脱髓鞘病变，这些病变中的轴突和神经元保存良好。颈丛和腰丛神经根、马尾、坐骨神经和腓肠神经显示出明显肿胀和水肿，伴有炎症细胞浸润。在坐骨神经和腓肠神经束中可见许多洋葱球样结构。我们的结果表明，CIDP患者的中枢神经系统和周围神经系统均可发生伴有炎症的脱髓鞘，尤其是在存在严重鞘内炎症等特定情况下的患者。
