Small fibre neuropathy: Diagnostic approach and therapeutic issues, and its association with primary Sjögren's syndrome.
Summary of "Small fibre neuropathy: Diagnostic approach and therapeutic issues, and its association with primary Sjögren's syndrome."
This article reviews the diagnostic issues and the therapeutic management of small fibre neuropathy (SFN), and a detailed literature analysis of its association with primary Sjögren's syndrome (pSS). A diagnosis of SFN should be raised in the presence of diffuse neuropathic painful manifestations (burning sensation, paresthesia, pricking, allodynia or hyperesthesia) and neurovegetative signs. The neurological examination and the electroneuromyogram are usually normal. The diagnosis of SFN can be confirmed by the evidence of decreased intra-epidermal nerve fibre density after a skin punch biopsy or the presence of abnormal nonconventional neurophysiological tests exploring the A-delta and C small nerve fibres (laser-evoked potentials, quantitative sensory tests, cutaneous sympathic reflex, autonomic function tests). The association of SFN and pSS has been scarcely evaluated, probably because of its lack of awareness and the low availability of the required diagnostic procedures. According to our literature review, pSS may be present in 9 to 30% of patients with SFN. Conversely, a pure SFN is present in 3 to 9% of patients with pSS where it may represent 25 to 35% of pSS-associated peripheral neuropathies. The treatment of SFN is mainly symptomatic and based on antalgic neuropsychotropic drugs and conventional analgesics. Corticosteroids and immunosuppressive drugs are usually unsuccessful. The effectiveness of intravenous immunoglobulins is only supported by a few case reports. SFN deserves to be separately evaluated among pSS-associated peripheral neuropathies. This requires a better availability of the appropriate diagnostic procedures, the investigation of underlying immunopathological mechanisms and the assessment of the new treatments recently proposed in pSS, mainly rituximab.
Service de médecine interne, hôpital Pitié-Salpêtrière, AP-HP, université Paris-6, 47-83, boulevard de l'Hôpital, 75013 Paris, France.
This article was published in the following journal.
Name: La Revue de medecine interne / fondee ... par la Societe nationale francaise de medecine interne
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/20851508
- DOI: http://dx.doi.org/10.1016/j.revmed.2010.07.022
Medical and Biotech [MESH] Definitions
Peripheral, autonomic, and cranial nerve disorders that are associated with DIABETES MELLITUS. These conditions usually result from diabetic microvascular injury involving small blood vessels that supply nerves (VASA NERVORUM). Relatively common conditions which may be associated with diabetic neuropathy include third nerve palsy (see OCULOMOTOR NERVE DISEASES); MONONEUROPATHY; mononeuropathy multiplex; diabetic amyotrophy; a painful POLYNEUROPATHY; autonomic neuropathy; and thoracoabdominal neuropathy. (From Adams et al., Principles of Neurology, 6th ed, p1325)
Optic Neuropathy, Ischemic
Ischemic injury to the OPTIC NERVE which usually affects the OPTIC DISK (optic neuropathy, anterior ischemic) and less frequently the retrobulbar portion of the nerve (optic neuropathy, posterior ischemic). The injury results from occlusion of arterial blood supply which may result from TEMPORAL ARTERITIS; ATHEROSCLEROSIS; COLLAGEN DISEASES; EMBOLISM; DIABETES MELLITUS; and other conditions. The disease primarily occurs in the sixth decade or later and presents with the sudden onset of painless and usually severe monocular visual loss. Anterior ischemic optic neuropathy also features optic disk edema with microhemorrhages. The optic disk appears normal in posterior ischemic optic neuropathy. (Glaser, Neuro-Ophthalmology, 2nd ed, p135)
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