Department of Neurology, King's College Hospital NHS Foundation Trust, Denmark Hill, London, UK.
Department of Neurology, Hospital Universitario Miguel Servet, Zaragoza, Spain.
J Peripher Nerv Syst. 2021 Mar;26(1):66-74. doi: 10.1111/jns.12433. Epub 2021 Jan 31.
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.
感觉神经元病是背根神经节的异质性疾病。描述了包括治疗反应和预后在内的单中心系列的临床和实验室特征。他们回顾性纳入了符合 Camdessanché 等人（2009 年）感觉神经元病标准的 54 例患者。根据可能的病因对患者进行分类，并分析其人口统计学、临床、神经生理学、组织学和脊髓 MRI 特征。用改良 Rankin 量表（mRS）评估结局，并评估治疗反应。大约有 54 名患者被纳入（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。一半的患者起病缓慢，在看神经科医生之前超过 12 个月。根据可能的病因（意味着免疫异常的非特异性实验室证据），18 名患者（33%）为炎症性、8 名（15%）为副肿瘤性、7 名（13%）为自身免疫性和 6 名（11%）为特发性。约 31 名患者接受了免疫治疗，其中 11 名（35%）得到改善或稳定。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%）。在最终随访（中位 24 个月）时，67%的患者 mRS≥3，46%的患者 mRS≥4，包括 15%的患者死亡。逻辑回归显示，全面反射消失和假性手足徐动症与较差的预后相关，而单变量分析显示，运动障碍和脑脊液蛋白升高与较差的预后相关。感觉神经元病导致严重残疾，尤其是在广泛反射消失和假性手足徐动症患者中。在那些没有明显病因的患者中，大多数有免疫失调的证据。一些患者对免疫治疗有阳性反应，但很少能显著改善残疾程度。
