抗浦肯野细胞2型抗体相关自身免疫性小脑炎一例

A Case Report of Anti-PCA-2-Positive Autoimmune Cerebellitis

摘要: 病史摘要：患者，女性，69岁，因“头晕、呕吐伴行走不稳3小时”入院，发病后未行特殊处理。既往有“高血压病、糖尿病、胸闷”病史。症状体征：神志清，精神不振，言语流利，智能正常。双侧瞳孔等大等圆，对光反射灵敏，双眼各方向活动自如，眼球震颤(−)，双侧鼻唇沟对称，伸舌居中，四肢肌力肌张力正常，无感觉障碍，双侧指鼻试验欠稳准，闭目难立征(+)，容易前后倾倒，腱反射(++)，病理征(−)。颈软，双侧克氏征(−)。诊断方法：脑干 + 小脑薄层MRI扫描未见异常。自身免疫性小脑共济失调抗体谱筛查提示抗浦肯野细胞2型抗体(PCA-2)血清和脑脊液均为阳性，滴度分别为1:10和1:30，患者诊断为抗PCA-2抗体相关自身免疫性小脑炎。治疗方法：患者入院后给予对症治疗，考虑存在糖尿病，未给予激素冲击及丙球治疗，给予丁螺环酮10 mg口服，每天3次，患者头晕及走路不稳逐渐改善，给予加用甲泼尼龙片每天20 mg口服，每周减1片，同时给予雷贝拉唑、氯化钾及维D钙片等药物辅助治疗。临床转归：患者接受激素治疗后头晕、走路不稳症状迅速改善，出院后病情进一步好转，嘱其随访异常肿瘤标志物的变化。

Abstract: Patient Histories of Summary: The 69-year-old female patient was admitted to the hospital due to dizziness, vomiting and unstable walking for 3 hours, and had a history of hypertension, diabetes and chest tightness without special treatment after onset. Symptoms Signs: Conscious, languid, Verbal fluency, normal mental state, bilateral pupil big circle, sensitive to pupillary light reflex, eyes in each direction activities freely, nystagmus (−), nasolabial groove on both sides is symmetrical, tongue in the middle and normal muscle force and muscle strength of four limbs, no disorders of sensation, double side finger-to-nose test no steady, Romberg sign (+), easy to dump before and after, tendon reflex (++), pathologic sign (−). Soft neck, Kernig signs (−). Diagnostic Method: Thin-layer MRI scans of brainstem and cerebellum showed no abnormalities. Autoimmune cere-bellar ataxia antibody spectrum screening showed positive anti-purkinje cell type 2 antibody (PCA-2) in serum and cerebrospinal fluid with titers of 1:10 and 1:30, respectively, and the patient was diagnosed with anti-PCA-2-positive autoimmune cerebellitis. Therapy: Patients after admission to give symptomatic treatment, considering existing diabetes, not given hormone and gamma globulin treatment, give buspirone 10 mg orally, three times a day, patients with dizziness and walking instability gradually improve, to give every day with a prednisolone 20 mg orally, minus 1 piece per week, at the same time give rabeprazole, potassium chloride and vitamin D, calcium tablet drugs such as adjuvant therapy. Clinical Outcome: The patient’s symptoms of dizziness and unsteady walking improved rapidly after hormone therapy, and his condition improved further after discharge. Follow-up of abnormal tumor markers was ordered.