Colombo Daniele, Falasca Laura, Monardo Francesca, D'Ambrosio Mario, Di Napoli Arianna, Salerno Antonio, Del Nonno Franca, Comanducci Giovanna
Pathology Unit, National Institute for Infectious Diseases "Lazzaro Spallanzani," IRCCS, Rome, Italy.
Laboratory of Electron Microscopy, National Institute for Infectious Diseases "Lazzaro Spallanzani," IRCCS, Rome, Italy.
Neuropathology. 2025 Feb;45(1):76-82. doi: 10.1111/neup.13003. Epub 2024 Sep 23.
Guillain-Barré syndrome (GBS) is an acute disorder of the peripheral nervous system, causing flaccid paralysis, areflexia, and variable sensory involvement. Proximal as well distal muscles of the limbs can be involved, and in most severe and advanced cases progresses to respiratory failure and death. GBS is considered an autoimmune disease, and at the basis of the attack at the peripheral nervous system different mechanisms have been recognized, in particular viral infections or other immune stimulations. Cranial nerve involvement in patients with diffuse large B-cell lymphoma (DLBCL) and primary central nervous system lymphoma are rare conditions that could present with similar clinical features. Here we present a case of a 36-year-old man hospitalized for acute polyradiculoneuritis of the cranial nerves and lumbar roots that arose a 14 days after severe acute respiratory syndrome COVID-19 2 (Sars-CoV-2) vaccination. Most of the main criteria for the diagnosis of GBS were met, including clinical and electrophysiological criteria. Albuminocytologic dissociation and high protein level in cerebrospinal fluid were also found. Therefore, the patient was treated with a cycle of intravenous immunoglobulin (IVIG) with notable improvement of symptoms and gradual recovery of motility. A five months later, following SARS-CoV-2 infection, the patient presented with worsening of neurological symptoms and was readmitted to the hospital. He underwent instrumental tests again and was treated with repeated cycles of IVIG and then with a cycle of plasmapheresis without any improvement. In the following 10 days he developed very serious conditions; he was transferred to intensive care unit and deceased after 6 days. The cause of the neurological syndrome was determined only after autoptic analysis, which revealed the presence of primary peripheral nervous system (PNS) DLBCL. The reported case highlights that GBS-like presentation always requires a careful differential diagnosis, and physicians should also consider the possibility of an occult cancer.
吉兰 - 巴雷综合征（GBS）是一种急性周围神经系统疾病，可导致弛缓性麻痹、反射消失以及不同程度的感觉障碍。四肢近端和远端肌肉均可受累，在最严重和病情进展的病例中会发展为呼吸衰竭甚至死亡。GBS被认为是一种自身免疫性疾病，其对周围神经系统的攻击存在不同机制，尤其是病毒感染或其他免疫刺激。弥漫性大B细胞淋巴瘤（DLBCL）和原发性中枢神经系统淋巴瘤患者出现颅神经受累的情况较为罕见，可能表现出相似的临床特征。在此，我们报告一例36岁男性患者，因在严重急性呼吸综合征冠状病毒2（Sars-CoV-2）疫苗接种14天后出现颅神经和腰神经根急性多发性神经根炎而住院。该病例符合GBS诊断的大多数主要标准，包括临床和电生理标准。脑脊液中还发现了蛋白细胞分离和高蛋白水平。因此，患者接受了一个疗程的静脉注射免疫球蛋白（IVIG）治疗，症状明显改善，运动功能逐渐恢复。五个月后，该患者感染Sars-CoV-2后，神经症状恶化并再次入院。他再次接受了相关检查，并接受了重复疗程的IVIG治疗，随后进行了一个疗程的血浆置换，但均无改善。在接下来的10天里，他病情严重恶化；被转入重症监护病房，6天后死亡。仅在尸检分析后才确定了神经综合征的病因，结果显示存在原发性周围神经系统（PNS）DLBCL。该报告病例强调，类似GBS的表现始终需要仔细的鉴别诊断，医生还应考虑隐匿性癌症的可能性。
