Anti-Ri paraneoplastic neurological syndrome presenting with bilateral cranial nerve VI palsy and jaw dystonia-a distinctive syndrome within the anti-Ri spectrum? : Case report and literature review.
Elisabeth Freydl, Alexander Tinchon, Katrin Blauensteiner, Stefan Oberndorfer
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引用次数: 0
Abstract
Objective: Paraneoplastic neurological syndromes (PNS) are rare disorders associated with various onconeuronal antibodies. Anti-Ri antibodies (ANNA-2) are typically found in patients with opsoclonus myoclonus syndrome (OMS) and ataxia.
Case report: We present an anti-Ri antibody-positive 77-year-old woman with subacute progressive bilateral cranial nerve VI palsy, gait disturbance and jaw dystonia. MRI of the brain showed hyperintense signals on T2 bitemporal without contrast enhancement. Cerebrospinal fluid (CSF) examination exhibited mild pleocytosis of 13 cells/µl and positive oligoclonal bands. CSF was overall inconspicuous for a malignant or inflammatory etiology. Immunofluorescence analysis revealed anti-Ri antibodies in both serum and CSF. Subsequent diagnostic work up resulted in a newly diagnosed ductal carcinoma of the right breast. PNS in this case partially responded to the anti-tumor therapy.
Conclusion: This case shows similarities with recently published anti-Ri syndromes, which might form a distinct triad within the anti-Ri spectrum.
目的：副肿瘤性神经综合征（PNS）是与各种肿瘤细胞抗体相关的罕见疾病。抗 Ri 抗体（ANNA-2）通常见于患有肌阵挛综合征（OMS）和共济失调的患者：我们接诊了一名抗 Ri 抗体阳性的 77 岁女性患者，她患有亚急性进行性双侧颅神经 VI 麻痹、步态障碍和下颌肌张力障碍。脑部核磁共振成像显示 T2 位颞叶高强信号，无对比度增强。脑脊液（CSF）检查显示轻度多细胞，13个细胞/µl，寡克隆带阳性。CSF总体上看不出恶性或炎症病因。免疫荧光分析显示血清和脑脊液中均存在抗 Ri 抗体。随后的诊断结果是新确诊的右乳腺导管癌。该病例的 PNS 对抗肿瘤治疗有部分反应：本病例与最近发表的抗 Ri 综合征有相似之处，可能在抗 Ri 范围内形成一个独特的三联征。
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''From the microscope to clinical application!'', Scientists from all European countries make available their recent research results and practical experience through Wiener Medizinische Wochenschrift, the renowned English- and German-language forum. Both original articles and reviews on a broad spectrum of clinical and preclinical medicine are presented within the successful framework of thematic issues compiled by guest editors. Selected cutting-edge topics, such as dementia, geriatric oncology, Helicobacter pylori and phytomedicine make the journal a mandatory source of information.
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