背景:神经淋巴瘤病(NL)是一种罕见的疾病,定义为淋巴瘤侵入周围神经,神经根,或者神经丛,包括颅神经.还没有明确的治疗方案为这种病理定义。
方法:一名40多岁的女性因颅内肿瘤活检确诊为原发性中枢神经系统淋巴瘤,并接受了化疗和放疗。在她抱怨躯干和四肢疼痛后,磁共振成像和[18F]氟脱氧葡萄糖(FDG)正电子发射断层扫描(PET)在初步诊断后25个月发现了多个病变的神经神经节,丛,和从颈椎到骶脊髓的周围神经。脑脊液细胞学检查显示脊髓腔中存在非典型淋巴细胞和淋巴瘤。基于这些发现,NL被诊断。鞘内抗肿瘤方案暂时减少FDG的异常摄取,但病变复发了.额外的大剂量甲氨蝶呤治疗后,先前确定的病变中的FDG积累消失。然而,周围神经性疼痛和截瘫仍然存在。患者在最初诊断为NL后9个月死亡。
结论:作者报道了一例原发性中枢神经系统淋巴瘤后的NL病例。在这种情况下,FDG-PET被证明对诊断有用,大剂量甲氨蝶呤治疗暂时有效.https://thejns.org/doi/suppl/10.3171/CASE24107。
BACKGROUND: Neurolymphomatosis (NL) is a rare disease defined as an invasion of lymphoma into peripheral nerves, nerve roots, or nerve plexuses, including the cranial nerves. No clear treatment protocols have yet been defined for this pathology.
METHODS: A woman in her 40s had a primary central nervous system lymphoma diagnosed from an intracranial tumor biopsy and underwent chemotherapy and radiation therapy. After she complained of pain in the trunk and extremities, magnetic resonance imaging and [18F]fluorodeoxyglucose (FDG) positron emission tomography (PET) performed 25 months after initial diagnosis revealed multiple lesions in the nerve ganglia, plexuses, and peripheral nerves from the cervical to the sacral spinal cord. Cerebrospinal fluid
cytology revealed atypical lymphocytes and lymphoma dissemination in the spinal cavity. Based on these findings, NL was diagnosed. An intrathecal antineoplastic regimen temporarily reduced abnormal uptake of FDG, but the lesion recurred. After additional high-dose methotrexate therapy, FDG accumulation in the previously identified lesions disappeared. However, peripheral neuropathic pain and paraplegia remained. The patient died 9 months after the initial diagnosis of NL.
CONCLUSIONS: The authors reported a
case of NL following primary central nervous system lymphoma. In this
case, FDG-PET proved useful for diagnosis, and high-dose methotrexate therapy was temporarily effective. https://thejns.org/doi/suppl/10.3171/CASE24107.