Servicio de Neurologia, Hospital Universitario Virgen del Rocío, Seville, Spain.
Muscle Nerve. 2013 Jul;48(1):145-50. doi: 10.1002/mus.23772. Epub 2013 Jun 6.
Sensory ganglionopathies are uncommon but potentially very disabling. They have heterogeneous etiologies including autoimmune, paraneoplastic, toxic, and inflammatory although many remain idiopathic despite intensive investigation. Asymmetric sensory loss is relatively common at the onset, but with time, symptoms usually spread to involve all limbs symmetrically.
We report 6 patients with a persistent strikingly asymmetrical sensory ganglionopathy with acute or subacute onset and slow progression.
Peripheral nerve biopsies in 5 patients showed axonal loss without significant inflammation; a dorsal root ganglion biopsy in 1 patient showed neuronal loss and inflammatory infiltrate. Four patients received immunomodulatory treatment, but overall the response to treatment was poor.
Asymmetrical sensory ganglionopathies may have an inflammatory basis. Immunomodulatory therapy may be considered early in the disease course, although in this series there was a limited response to treatment.
感觉神经元病并不常见，但可能会导致严重残疾。其病因多样，包括自身免疫、副肿瘤、中毒和炎症等，但许多病因仍不明确，尽管进行了深入的调查。在发病初期，通常会出现不对称性感觉丧失，但随着时间的推移，症状通常会扩散至四肢对称受累。
我们报告了 6 例以急性或亚急性起病、进展缓慢为特征的持续性显著不对称感觉神经元病。
5 例患者的周围神经活检显示轴索丢失而无明显炎症；1 例患者的背根神经节活检显示神经元丢失和炎症浸润。4 例患者接受了免疫调节治疗，但总体治疗反应不佳。
不对称性感觉神经元病可能具有炎症基础。尽管在本系列中，治疗反应有限，但在疾病早期可能考虑免疫调节治疗。
