Gwathmey Kelly Graham
Department of Neurology, University of Virginia, P.O. Box 800394, Charlottesville, Virginia, 22908, USA.
Muscle Nerve. 2016 Jan;53(1):8-19. doi: 10.1002/mus.24943. Epub 2015 Nov 23.
The sensory neuronopathies (or ganglionopathies) are a small subcategory of neuropathies characterized by primary degeneration of the dorsal root ganglia and trigeminal ganglion sensory neurons, resulting in a distinctive clinical presentation. Patients typically have subacute onset of asymmetric, non-length-dependent sensory impairment and early ataxia. The etiologies of acquired sensory neuronopathies are rather limited. Early identification is imperative, as they may herald an underlying malignancy or an autoimmune condition such as Sjögren syndrome. This review addresses the various causes of acquired sensory neuronopathies, the recommended diagnostic approach, and treatment options. Finally, I will briefly discuss a select few hereditary and degenerative sensory neuronopathies, which, in contrast to the acquired disorders, are slowly progressive and are usually associated with additional neurological symptoms.
感觉神经元病(或神经节病)是神经病的一个小亚类,其特征是背根神经节和三叉神经节感觉神经元的原发性变性,导致独特的临床表现。患者通常亚急性起病,表现为不对称、非长度依赖性感觉障碍和早期共济失调。获得性感觉神经元病的病因相当有限。早期识别至关重要,因为它们可能预示着潜在的恶性肿瘤或自身免疫性疾病,如干燥综合征。本综述探讨了获得性感觉神经元病的各种病因、推荐的诊断方法和治疗选择。最后,我将简要讨论少数几种遗传性和退行性感觉神经元病,与获得性疾病不同,这些疾病进展缓慢,通常伴有其他神经症状。