Department of Neurology, Kawasaki Municipal Tama Hospital: Kawasaki Shiritsu Tama Byoin, 1-30-37, Shukugawara Tama-ku Kawasaki-shi, Kawasaki, Kanagawa, Japan.
Department of Neurology, Shin Yurigaoka General Hospital: Shinyurigaoka Sogo Byoin, Kawasaki, Kanagawa, Japan.
BMC Neurol. 2022 May 2;22(1):165. doi: 10.1186/s12883-022-02684-4.
As a debilitating syndrome, paraneoplastic cerebellar degeneration (PCD) remains challenging to treat. Further, anti-Yo antibody (directed against human cerebellar degeneration-related protein 2) detection in patients with PCD is associated with unsatisfactory responses to existing therapies. Here, we present the case of a 60-year-old woman who developed PCD with anti-Yo antibodies and a submandibular gland tumor.
A 60-year-old woman presented with a 5-day history of unsteadiness of gait and inadequate coordination of her extremities, along with truncal instability. Although walking without aid was possible, dysmetria of all four limbs, trunk, and gait ataxia was observed. While routine biochemical and hematological examinations were normal, the patient's blood was positive for anti-Yo antibodies. When the neurological symptoms deteriorated despite administration of intravenous methylprednisolone, fluorodeoxyglucose-positron emission tomography (FDG-PET) and computed tomography (CT) images with contrast enhancement were performed, which showed a tumor in the left submaxillary gland. She underwent total left submandibular gland resection, including the tumor; histological and immunohistochemical results revealed a salivary duct carcinoma. She was administered intravenous methylprednisolone, followed by 10 plasma exchange sessions, intravenous immunoglobulins, and cyclophosphamide therapy. Following treatment, her symptoms were not alleviated, even after the reduction of anti-Yo titers.
Although tumor detection was delayed, early tumor detection, diagnosis, and PCD treatment are essential because any delay can result in the progression of the disorder and irreversible neurological damage. Therefore, we recommend that the possibility of a salivary gland tumor should be considered, and whole-body dual-modality CT, including the head and neck, and FDG-PET should be performed at the earliest for patients with well-characterized paraneoplastic antibodies when conventional imaging fails to identify a tumor.
副肿瘤性小脑变性（PCD）是一种使人衰弱的综合征，其治疗仍然具有挑战性。此外，在 PCD 患者中检测到抗 Yo 抗体（针对人类小脑变性相关蛋白 2）与对现有治疗方法的反应不佳有关。在这里，我们报告了一例伴有抗 Yo 抗体和下颌下腺肿瘤的 60 岁女性 PCD 病例。
一名 60 岁女性因步态不稳和四肢协调不足，以及躯干不稳，出现 5 天病史。虽然可以在没有帮助的情况下行走，但观察到四肢、躯干和步态共济失调。虽然常规生化和血液检查正常，但患者血液呈抗 Yo 抗体阳性。尽管给予静脉甲基强的松龙治疗，但神经症状仍恶化，随后进行氟脱氧葡萄糖正电子发射断层扫描（FDG-PET）和增强对比计算机断层扫描（CT）检查，显示左下颌下腺有肿瘤。她接受了包括肿瘤在内的左下颌下腺全切除术；组织学和免疫组织化学结果显示为唾液腺癌。她接受了静脉甲基强的松龙、10 次血浆置换、静脉注射免疫球蛋白和环磷酰胺治疗。治疗后，她的症状没有缓解，即使抗 Yo 滴度降低后也没有缓解。
尽管肿瘤检测延迟，但早期肿瘤检测、诊断和 PCD 治疗至关重要，因为任何延迟都可能导致疾病进展和不可逆的神经损伤。因此，我们建议当常规影像学检查未能发现肿瘤时，对于具有明确副肿瘤抗体的患者，应考虑存在唾液腺癌的可能性，并尽早进行包括头颈部在内的全身双能 CT 和 FDG-PET 检查。
