Clinical features and pathophysiological basis of sensory neuronopathies (ganglionopathies)

Details

Serval ID
serval:BIB_B634813F1C44
Type
Article: article from journal or magazin.
Publication sub-type
Review (review): journal as complete as possible of one specific subject, written based on exhaustive analyses from published work.
Collection
Publications
Institution
Title
Clinical features and pathophysiological basis of sensory neuronopathies (ganglionopathies)
Journal
Muscle and Nerve
Author(s)
Kuntzer  T., Antoine  J. C., Steck  A. J.
ISSN
0148-639X (Print)
Publication state
Published
Issued date
09/2004
Peer-reviewed
Oui
Volume
30
Number
3
Pages
255-68
Notes
Journal Article Review --- Old month value: Sep
Abstract
Sensory ganglionopathies have a frequent association with neoplastic disorders (paraneoplastic subacute sensory neuronopathy, or SSN) or dysimmune disorders (Sjogren's syndrome, SS; Miller Fisher syndrome; and Bickerstaff's brainstem encephalitis, BBE), with drugs, such as cisplatin or pyridoxine, and with inherited disorders with degeneration of dorsal root ganglion cells. Unsteady gait and pseudoathetoid movements of the hand are the distinctive signs encountered in these disorders. The chronic disorders are characterized by non-length-dependent abnormalities of sensory nerve action potentials (SNAPs) and differ from other sensory neuropathies in showing a global, rather than distal, decrease in SNAP amplitudes. This review focuses on recent advances in defining the mechanisms involved in sensory ganglionopathies. Specific topics include a summary of their clinical features, pathological findings, and immunopathology. In SSN, early diagnosis by the detection of anti-Hu antibodies and early treatment of the cancer gives the best chance of stabilizing the disorder. In SS sensory ganglionitis, response to treatment has been disappointing, but immunomodulating treatments are emerging. The immunological profile common to BBE and Fisher syndrome supports a common pathogenesis. In toxic sensory neuronopathy, no treatment is available. The differential diagnosis involves separating sensory ganglionopathies from other ataxic polyneuropathies, such as infectious neuropathies, sensory neuropathies with various autoantibodies, and the neuropathies seen in celiac disease.
Keywords
Animals Ganglia, Spinal/*physiopathology Humans Neurons, Afferent/*pathology Paraneoplastic Polyneuropathy/diagnosis/physiopathology Peripheral Nervous System Diseases/diagnosis/physiopathology Polyneuropathies/*diagnosis/*physiopathology
Pubmed
Web of science
Create date
25/01/2008 12:43
Last modification date
20/08/2019 15:24
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