Srichawla Bahadar S, Bose Abigail, Kipkorir Vincent
Department of Neurology, University of Massachusetts Chan Medical School, Worcester, MA, USA.
University of Nairobi, Nairobi, Kenya.
SAGE Open Med Case Rep. 2023 Dec 30;12:2050313X231221466. doi: 10.1177/2050313X231221466. eCollection 2024.
Neurolymphomatosis occurs due to the infiltration of a nerve by malignant cells. Cranial neurolymphomatosis is a rare disease process associated with non-solid tumors (i.e., lymphoma, leukemia, etc.). Cranial neurolymphomatosis presents with single or multifocal neuropathy. Primary cranial neurolymphomatosis is defined as the initial presenting symptom leading to a new diagnosis of cancer. Secondary cranial neurolymphomatosis is defined as cancer progression with spread to a cranial nerve. While cranial neurolymphomatosis is a recognized cause of cranial nerve neuropathies, a myriad of other malignancies can also lead to similar clinical manifestations. This case series elucidates not only the classical presentations associated with cranial neurolymphomatosis but also introduces other oncologic entities that may compromise cranial nerve functions. A descriptive case series is presented on six patients with malignancy-related cranial neuropathy who came to a tertiary-care center from 2018 to 2022. 5/6 (83.3%) of patients presented with primary cranial neuropathy. Diffuse large B-cell lymphoma was the most prevalent malignancy observed in 3/6 (50.0%) cases. Other malignancies observed include non-Hodgkin lymphoma, monoclonal B-cell lymphocytosis, and peripheral T-cell lymphoma. The most affected cranial nerve was the trigeminal nerve in 4/6 (66.6%) individuals. Multiple cranial neuropathies were seen in 2/6 (33.3%) of patients. The most common neuroradiographic finding was a lesion to Meckel's cave. Other cranial nerves affected include the optic, facial, and vestibulocochlear nerves. Diagnostic modalities utilized included magnetic resonance imaging and F-fluoro-2-D-glucose positron emission tomography-computerized tomography. Cerebrospinal fluid analysis for flow cytometry may also have diagnostic value in patients with increased disease burden. Treatment was guided according to individual malignancy and 2/6 (33.3%) patients achieved complete remission, 2/6 (33.3%) died within 1 year, and 1/6 (16.6%) were referred to hospice. Cranial neuropathy may be the first symptom of a neoplastic process; thus, prompt recognition and treatment may improve morbidity and mortality.
神经淋巴瘤是由于恶性细胞浸润神经所致。颅内神经淋巴瘤是一种罕见的疾病过程,与非实体肿瘤(如淋巴瘤、白血病等)相关。颅内神经淋巴瘤表现为单发性或多灶性神经病变。原发性颅内神经淋巴瘤被定义为导致新的癌症诊断的初始症状。继发性颅内神经淋巴瘤被定义为癌症进展并扩散至颅神经。虽然颅内神经淋巴瘤是颅神经病变的一个公认原因,但许多其他恶性肿瘤也可导致类似的临床表现。本病例系列不仅阐明了与颅内神经淋巴瘤相关的典型表现,还介绍了其他可能损害颅神经功能的肿瘤实体。本文呈现了一个描述性病例系列,涉及2018年至2022年期间到一家三级医疗中心就诊的6例与恶性肿瘤相关的颅神经病变患者。5/6(83.3%)的患者表现为原发性颅神经病变。弥漫性大B细胞淋巴瘤是在3/6(50.0%)的病例中观察到的最常见恶性肿瘤。观察到的其他恶性肿瘤包括非霍奇金淋巴瘤、单克隆B细胞淋巴细胞增多症和外周T细胞淋巴瘤。4/6(66.6%)的个体中受影响最严重的颅神经是三叉神经。2/6(33.3%)的患者出现多发性颅神经病变。最常见的神经影像学表现是 Meckel腔病变。其他受影响的颅神经包括视神经、面神经和前庭蜗神经。所采用的诊断方法包括磁共振成像和F-氟-2-D-葡萄糖正电子发射断层扫描-计算机断层扫描。对于疾病负担增加的患者,脑脊液流式细胞术分析也可能具有诊断价值。治疗根据个体恶性肿瘤情况进行指导,2/6(33.3%)的患者实现完全缓解,2/6(33.3%)的患者在1年内死亡,1/6(16.6%)的患者被转诊至临终关怀机构。颅神经病变可能是肿瘤性疾病的首发症状;因此,及时识别和治疗可能改善发病率和死亡率。