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一名同时具有抗Hu和抗NMDAR抗体阳性的患者出现共济失调-帕金森综合征。

Ataxia-Parkinsonism in a Patient With Double-Positive Anti-Hu and Anti-NMDAR Antibodies.

作者信息

Cunha Carolina, Faustino Pedro, Carvalho Inês, Morgadinho Ana, Cunha Rosário, Reis-Carneiro Diogo

机构信息

Neurology Department, Coimbra University Hospital Centre, Coimbra, Portugal.

Clinical Pathology Department, Coimbra University Hospital Centre, Coimbra, Portugal.

出版信息

Neurohospitalist. 2025 May 19:19418744251342295. doi: 10.1177/19418744251342295.

Abstract

Movement disorders may be a core feature of autoimmune brain disorders, including paraneoplastic neurological syndromes. A 73-year-old white male presents with memory impairment, gait instability, dysphagia, and severe dysarthria progressing over 1.5 years. He recently developed behavior and sleep disturbances. His cognitive assessment showed time disorientation, short-term memory impairment with preserved retrieval, and pseudobulbar affect. The remainder of the neurological exam showed seborrhea, facio-cervical dystonia, a right positive palmomental reflex, fragmented pursuit eye movements, dysarthria, minor right pyramidal signs, bilateral asymmetric limb ataxia and symmetric akinetic parkinsonism. He was wheelchair-bound, capable of only a few short steps with help. MRI depicted generalized cortico-subcortical atrophy with temporal predominance. He was positive for antibodies anti-Hu in serum (1:10 000) and CSF (1:100), as antibodies anti-NMDAR in serum (1:320) and CSF (1:10). The patient had no clinical improvement after a therapeutical trial with 1 g intravenous methylprednisolone. Additional workup for occult neoplasia was positive for prostatic adenocarcinoma. He remains stable 2.5 years after disease onset.

摘要

运动障碍可能是自身免疫性脑部疾病的核心特征,包括副肿瘤性神经系统综合征。一名73岁白人男性,出现记忆力减退、步态不稳、吞咽困难和严重构音障碍,病程长达1.5年。他最近出现了行为和睡眠障碍。其认知评估显示时间定向障碍、短期记忆受损但回忆能力保留,以及假性延髓麻痹。神经系统检查的其余部分显示有皮脂溢、面颈部肌张力障碍、右侧掌颏反射阳性、眼球追踪运动破碎、构音障碍、轻微的右侧锥体束征、双侧不对称肢体共济失调和对称性运动不能性帕金森综合征。他只能坐在轮椅上,在他人帮助下才能走短短几步。MRI显示广泛性皮质 - 皮质下萎缩,以颞叶为主。血清(1:10 000)和脑脊液(1:100)中抗Hu抗体呈阳性,血清(1:320)和脑脊液(1:10)中抗NMDAR抗体呈阳性。在接受1克静脉注射甲泼尼龙的治疗试验后,患者病情无改善。对隐匿性肿瘤的进一步检查发现前列腺腺癌呈阳性。疾病发作2.5年后,他的病情保持稳定。

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