title: Neurophysiological diagnosis of acquired sensory ganglionopathies.
abstract: 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.
authors.affiliation: Department of Clinical Neurosciences, National Neurological Institute 'Carlo Besta', Milan, Italy. g_lauria@libero.it
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Lauria
authors.forename: Davide
authors.initials: D
authors.lastname: Pareyson
authors.forename: Angelo
authors.initials: A
authors.lastname: Sghirlanzoni
mesh_terms: D000200:Action Potentials; D000328:Adult; D000368:Aged; D001369:Axons / Q000473:pathology; D003937:Diagnosis, Differential; D005073:Evoked Potentials, Somatosensory; D005260:Female; D005724:Ganglia / Q000503:physiopathology*; D005727:Ganglia, Spinal / Q000503:physiopathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000473:pathology; D009410:Nerve Degeneration / Q000503:physiopathology; D009431:Neural Conduction; D009475:Neurons, Afferent / Q000473:pathology; D010523:Peripheral Nervous System Diseases / Q000175:diagnosis* / Q000503:physiopathology*