Lauria Giuseppe, Pareyson Davide, Sghirlanzoni Angelo
Department of Clinical Neurosciences, National Neurological Institute 'Carlo Besta', Milan, Italy.
Eur Neurol. 2003;50(3):146-52. doi: 10.1159/000073055.
We examined 29 patients with chronic progressive ganglionopathy of different etiology. Neurophysiological abnormalities were dominated by a widespread decrease in sensory nerve action potential amplitudes, which involved both upper and lower limb nerves, even in patients with asymmetrical or patchy clinical presentation. This impairment of sensory nerve conduction, reflecting a nonlenght-dependent pattern of peripheral axon degeneration, should be considered the hallmark of ganglionopathies. The evidence of central sensory pathway impairment, which allows to localize the pathology to the dorsal root ganglion neurons, could be better confirmed by cervical magnetic resonance imaging, which showed a diffuse hyperintensity in the posterior columns in all patients, than by somatosensory evoked potentials, which were undetectable in most of the patients. Few patients showed an impairment of individual motor nerves, which appeared more severe in paraneoplastic associated ganglionopathies. Neurophysiological abnormalities did not appear significantly changed at the 4-year follow-up. We emphasize that distinct abnormalities allow to differentiate ganglionopathies from axonal sensory neuropathies on routine neurophysiological examination.
我们检查了29例不同病因的慢性进行性神经节病患者。神经生理学异常主要表现为感觉神经动作电位幅度广泛降低，累及上肢和下肢神经，即使是临床表现不对称或呈斑片状的患者也是如此。这种感觉神经传导障碍反映了外周轴突变性的非长度依赖性模式，应被视为神经节病的标志。与体感诱发电位相比，颈椎磁共振成像能更好地证实中枢感觉通路受损的证据，该证据可将病变定位到背根神经节神经元，颈椎磁共振成像显示所有患者后索均有弥漫性高信号，而体感诱发电位在大多数患者中无法检测到。少数患者出现个别运动神经受损，在副肿瘤相关性神经节病中似乎更严重。在4年的随访中，神经生理学异常未见明显变化。我们强调，在常规神经生理学检查中，明显的异常有助于将神经节病与轴索性感觉神经病区分开来。
