Asanome Asuka, Kano Kohei, Takahashi Kae, Saito Tsukasa, Sawada Jun, Katayama Takayuki
Division of Neurology, First Department of Internal Medicine, Asahikawa Medical University.
Asahikawa Rehabilitation Hospital.
Rinsho Shinkeigaku. 2018 Feb 28;58(2):93-99. doi: 10.5692/clinicalneurol.cn-001080. Epub 2018 Jan 31.
A 58-year-old female was admitted to our hospital because of recurrent multiple cranial neuropathy (right facial palsy followed by involvement of the left trigeminal, facial, acoustic, pharyngeal, and vagal nerves and the right abducens nerve). Brain MRI showed gadolinium enhancement of the right abducens, bilateral facial/acoustic, and left pharyngeal/vagal nerves, and F-Fluorodeoxyglucose (FDG)-positron emission tomography revealed abnormal FDG uptake in the right facial, acoustic, pharyngeal, and vagal nerves and the left cervical lymph nodes. Blood and biochemical analyses did not show any abnormalities, including in the patient's lactate dehydrogenase and soluble interleukin-2 receptor (sIL2R) levels. A cerebrospinal fluid (CSF) examination showed gradual increases in the patient's cell counts and protein, β2-microglobulin, and sIL2R levels, but no malignant cells were detected. A thorough investigation involving repeated CSF examinations, whole-body computed tomography, bone marrow aspiration, random skin biopsies, and cervical lymph node aspiration biopsy examinations did not result in any definitive conclusions. Steroid therapy was ineffective, and the patient developed deafness in her left ear. Therefore, we performed a biopsy examination of the left acoustic nerve, which resulted in the patient being diagnosed with diffuse large B-cell lymphoma. High-dose MTX following the intrathecal administration of MTX, cytarabine, and prednisolone partially improved her symptoms, but she died after several episodes of clinical recurrence. Acoustic nerve biopsy may help diagnose neurolymphomatosis in carefully selected cases.
一名58岁女性因复发性多发性颅神经病变(右侧面神经麻痹,随后左侧三叉神经、面神经、听神经、咽神经和迷走神经以及右侧展神经受累)入住我院。脑部MRI显示右侧展神经、双侧面神经/听神经以及左侧咽神经/迷走神经有钆增强,氟脱氧葡萄糖(FDG)正电子发射断层扫描显示右侧面神经、听神经、咽神经和迷走神经以及左侧颈部淋巴结有异常FDG摄取。血液和生化分析未显示任何异常,包括患者的乳酸脱氢酶和可溶性白细胞介素-2受体(sIL2R)水平。脑脊液(CSF)检查显示患者的细胞计数、蛋白质、β2-微球蛋白和sIL2R水平逐渐升高,但未检测到恶性细胞。包括重复脑脊液检查、全身计算机断层扫描、骨髓穿刺、随机皮肤活检和颈部淋巴结穿刺活检检查在内的全面调查未得出任何明确结论。类固醇治疗无效,患者左耳失聪。因此,我们对左侧听神经进行了活检检查,结果该患者被诊断为弥漫性大B细胞淋巴瘤。鞘内注射甲氨蝶呤、阿糖胞苷和泼尼松龙后给予高剂量甲氨蝶呤部分改善了她的症状,但她在几次临床复发后死亡。在精心挑选的病例中,听神经活检可能有助于诊断神经淋巴瘤病。