Neurology, Aster MIMS, Calicut, India
Hemato-Oncology, Meitra Hospital, Kozhikode, Kerala, India.
BMJ Case Rep. 2022 Apr 21;15(4):e249089. doi: 10.1136/bcr-2022-249089.
We report a unique case of rhombencephalomyelitis with unclear aetiology, diagnosed with Hodgkin's lymphoma (HL) on follow-up.A woman in her 50s was presented with gait difficulty, dysarthria, left Horner's syndrome and left trigeminal sensory loss. MRI of the brain revealed an enhanced T2 and fluid attenuated inversion recovery hyperintense lesion at the cervico-medullary junction, suggesting rhombencephalomyelitis. The initial differential diagnosis included demyelination, other inflammatory conditions like sarcoidosis, Behcet's disease and paraneoplastic syndrome. Cerebrospinal fluid analysis, serum myelin oligodendrocyte glycoprotein, neuromyelitis optica antibodies, vasculitis work-up, onconeural antibody panel and whole-body fluorodeoxyglucose-positron emission tomography scan were all unrevealing, and she was kept under close follow-up. After 18 months, she developed a prolonged fever and was diagnosed with HL. Rhombencephalomyelitis as a HL-associated paraneoplastic syndrome has not been reported in the literature. This case report highlights the importance of serial screening for occult malignancy in patients with suspected paraneoplastic syndrome.
我们报告了一例病因不明的菱形脑炎,在随访中诊断为霍奇金淋巴瘤(HL)。一位 50 多岁的女性出现步态困难、构音障碍、左侧霍纳氏综合征和左侧三叉神经感觉丧失。脑部 MRI 显示颈髓交界处增强 T2 和液体衰减反转恢复高信号病变,提示菱形脑炎。最初的鉴别诊断包括脱髓鞘、其他炎症性疾病,如结节病、贝赫切特病和副肿瘤综合征。脑脊液分析、血清髓鞘少突胶质细胞糖蛋白、视神经脊髓炎抗体、血管炎检查、癌相关抗体谱和全身氟脱氧葡萄糖正电子发射断层扫描均无结果,因此对她进行了密切随访。18 个月后,她出现长期发热,被诊断为 HL。菱形脑炎作为 HL 相关的副肿瘤综合征在文献中尚未报道。本病例报告强调了对疑似副肿瘤综合征患者进行隐匿性恶性肿瘤连续筛查的重要性。