Background

Isolated primary neurolymphomatosis (NL) of cranial multineuritis is a very rare condition that refers to the lymphomatous invasion of cranial nerves only. There are sparse cases of isolated cranial nerves NL reported worldwide.

Case presentation

We present magnetic resonance imaging (MRI) and cerebrospinal fluid (CSF) findings of a 63-year-old female patient suffering from isolated neurolymphomatosis of cranial multineuritis with a wide constellation of syndromes including binocular diplopia, left facial paralysis and pain, syncope episodes, and progressive dysphagia. A contrasted MRI brain showed multiple cranial nerves enhancement. Extensive workup for infectious, autoimmune, neoplastic, paraneoplastic, or inflammatory etiologies had been unrevealing except CSF cytology revealed large atypical monotypic B cells that were suspicious for non-Hodgkin lymphoma on the third large volume tap. The decision of biopsy was deferred after the risks and benefits discussion. Following the four cycles of empiric methotrexate-based induction chemotherapy, the patient’s symptoms resolved, and a complete radiographic response was achieved without whole-brain radiation or autologous hematopoietic cell transplantation. In the latest follow-up, she is independent with her daily activities and remains in clinical and radiographic remission more than 3 years since initial chemotherapy.

Conclusion

Isolated NL of cranial nerves can present diagnostic and management pitfalls for the neurologist, neurosurgeons, and oncologists. Since current diagnostic modalities have modest sensitivity and a pathological diagnosis is often difficult, empiric treatment once other possibilities are ruled out can carry a good prognosis.

中文翻译：

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孤立性原发性神经淋巴瘤病合并颅内多发性神经炎：一例

背景

颅内多发性神经炎的孤立性原发性神经淋巴瘤病（NL）是一种非常罕见的病状，仅指颅神经的淋巴瘤性侵犯。全世界有稀疏的孤立性颅神经病例报道。

案例介绍

我们介绍了一名63岁的女性患者的磁共振成像（MRI）和脑脊液（CSF）发现，该患者患有孤立的颅内多发性神经炎性神经淋巴瘤病，伴有广泛的综合征，包括双眼复视，左面部瘫痪和疼痛，晕厥发作，和进行性吞咽困难。对比的MRI大脑显示出多处颅神经增强。传染性，自身免疫性，肿瘤性，副肿瘤性或炎性病因的广泛检查尚未公开，但脑脊液细胞学检查显示，在第三次大批量水龙头中可疑为非霍奇金淋巴瘤的大型非典型单型B细胞。风险和收益讨论后，活检的决定推迟了。在基于甲氨蝶呤的经验性诱导化疗四个周期后，患者的症状得到缓解，无需进行全脑放射或自体造血细胞移植，即可获得完整的放射线反应。在最新的随访中，她独立于日常活动，自初次化疗以来一直保持3年以上的临床和影像学缓解。

结论

孤立的颅神经NL可能会给神经科医生，神经外科医生和肿瘤科医生带来诊断和管理上的陷阱。由于当前的诊断方式灵敏度不高，并且病理诊断通常很困难，因此一旦排除其他可能性，经验性治疗可以带来良好的预后。