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[Involvements of the peripheral nervous system and primary Gougerot-Sjögren syndrome]
1Service des maladies du système nerveux et du muscle, hôpital Civil, hôpitaux universitaires de Strasbourg, France.
Abstract:
Peripheral neuropathy in primary Sjögren's syndrome is common although often unrecognized in mild forms of this affection. They are characterized by a predominently sensory deficiency and can be divided in several entities, of which the most important are: axonal distal symmetric polyneuropathy, either of sensory or sensory-motor presentation, which are supposed to be consecutive to ischemia induced by the vasculitis of the vasa nervorum; sensory neuronopathy mimicking the paraneoplastic syndromes from a clinical and electrophysiological point of view which are supposed to be linked to neuronal degeneration secondary to a lymphocytic infiltration of the dorsal root and ganglia; trigeminal sensory neuropathy, either alone or associated with one of the previously cited forms. The prescribed courses of etiological treatment consisting of corticosteroids and immunosuppressive agents are only effective in some acute forms. Tracking down systematically these peripheral neuropathies in patients affected by Sjögren's syndrome is the best way to recognize them. Alternatively, patients with unexplained neuropathies should be evaluated for the presence of Sjögren's syndrome.