Curr Neurol Neurosci Rep (2017) 17:79 DOI 10.1007/s11910-017-0784-4 NERVE AND MUSCLE (L H WEIMER, SECTION EDITOR) 1 1 Allison Crowell & Kelly G. Gwathmey Springer Science+Business Media, LLC 2017 Abstract Introduction Purpose of Review The sensory neuronopathies are sensory- predominant polyneuropathies that result from damage to the Sensory neuronopathies (SN) are a subset of peripheral neu- dorsal root and trigeminal sensory ganglia. This review ex- ropathies that result from destruction of the dorsal root gan- plores the various causes of acquired sensory neuronopathies, glion (DRG) and trigeminal ganglion sensory neurons. They the approach to diagnosis, and treatment. go by many names in the literature, including dorsal root Recent Findings Diagnostic criteria have recently been pub- ganglionopathies and even ataxic neuropathies. These disor- lished and validated to allow differentiation of sensory ders can be broadly classified into acquired, or inherited and neuronopathies from other polyneuropathies. On the basis of degenerative SN. An in-depth discussion of the inherited and serial electrodiagnostic studies, the treatment window for the degenerative disorders, which include Friedreich ataxia, acquired sensory neuronopathies has been identified as ap- POLG mutations, cerebellar ataxia, neuropathy, and vestibular proximately 8 months. If treatment is initiated within 2 months areflexia syndrome (CANVAS),
Current Neurology and Neuroscience Reports – Springer Journals
Published: Aug 23, 2017
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