Sensory neuronopathies are heterogeneous disorders of dorsal root ganglia. The clinical and laboratory features in a single‐centre series, including response to treatment and outcome have been described. They retrospectively included 54 patients meeting Camdessanché et al (2009) criteria for sensory neuronopathy. The patients were classified according to their likely aetiology and analysed their demographic, clinical, neurophysiological, histological and spinal MRI features. The outcome with the modified Rankin Scale (mRS) was evaluated, and the response to treatment was assessed. About 54 patients were included (18 male; median age 54.5 years). The most common initial symptoms were hypoaesthesia, paraesthesia, ataxia and pain. Half of patients had a slow onset, greater than 12 months before seeing a neurologist. The aetiology as possibly inflammatory (meaning nonspecific laboratory evidence of immune abnormality) in 18 patients (33%), paraneoplastic 8 (15%), autoimmune 7 (13%) and idiopathic 6 (11%) was classified. About 31 patients received immune therapy of which 11 (35%) improved or stabilised. Corticosteroids were the most used treatment (24 patients) and cyclophosphamide had the highest response rate (3/6, 50%). At the final follow up (median 24 months) 67% had mRS ≥3 and 46% mRS ≥4, including 15% who died. Worse outcome was associated with generalised areflexia and pseudoathetosis by logistic regression, and with motor involvement and raised CSF protein by univariate analysis. Sensory neuronopathies caused severe disability, especially in patients with generalised areflexia and pseudoathetosis. Of those without an obvious cause, most had some evidence of dysimmunity. Some patients had a positive response to immunotherapy, but rarely enough to improve disability much.

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感觉神经元病：病例系列和文献综述

感觉神经元病是背根神经节的异质性疾病。已经描述了单中心系列的临床和实验室特征，包括对治疗的反应和结果。他们回顾性纳入了 54 名符合 Camdessanché 等人 (2009) 感觉神经元病标准的患者。根据可能的病因对患者进行分类，并分析了他们的人口统计学、临床、神经生理学、组织学和脊柱 MRI 特征。评估了改良 Rankin 量表 (mRS) 的结果，并评估了对治疗的反应。包括约 54 名患者（18 名男性；中位年龄 54.5 岁）。最常见的初始症状是感觉减退、感觉异常、共济失调和疼痛。半数患者起病缓慢，在就诊前超过 12 个月。18 名患者 (33%)、副肿瘤性 8 名 (15%)、自身免疫性 7 名 (13%) 和特发性 6 名 (11%) 的病因可能为炎症性（意味着免疫异常的非特异性实验室证据）。大约 31 名患者接受了免疫治疗，其中 11 名 (35%) 改善或稳定。皮质类固醇是最常用的治疗方法（24 名患者），环磷酰胺的反应率最高（3/6，50%）。在最后一次随访时（中位 24 个月），67% 的 mRS ≥3 和 46% 的 mRS ≥4，其中 15% 死亡。更糟糕的结果与通过逻辑回归分析的全身性反射消失和假手足徐动症有关，通过单变量分析与运动受累和脑脊液蛋白升高有关。感觉神经元病导致严重的残疾，特别是在全身性反射消失和假性手足徐动症的患者中。在没有明显原因的情况下，大多数人有一些免疫力低下的证据。一些患者对免疫疗法有积极反应，但很少足以改善残疾。