Abstract
OBJECTIVE: To compare the demographic conditions and disease associations of NLD-SFSN and LD-SFSN (length-dependent small-fiber sensory neuropathies).
BACKGROUND: Inflammatory status of some NLD-SFSN has been suggested in previous reports.
DESIGN/METHODS: We studied 59 patients having SFSN on the criteria of neuropathic pain with normal muscle strength, absence of sensory ataxia, normal nerve conduction study, and reduced intra-epidermal nerve fiber density (IENFD) quantified by skin biopsies performed at distal leg (DL) and at proximal tight (PT). Diagnosis of NLD-SFSN was assumed in 17 patients on reduced IENFD at PT but not at DL. The others patients were classified as LD-SFSN on decreased IENFD only at DL. Features of dysimmune, metabolic or infectious diseases, including analysis of biopsy of minor salivary gland, were screened in all the patients.
RESULTS: Patients with NLD-SFSN were younger than patients with LD-SFSN (51 y-o vs. 59 y-o, p=0.029) and were more often female (15/17 vs. 30/42, p=0.17). Onset of sensory symptoms was acute in 8/17 patients (47%) with NLD-SFSN and in 1/42 patients (2%) with LD-SFNP (p<0.01). IENFD was similar at PT in both NLD-SFSN and LD-SFSN patients (5.6 fibers/mm vs. 7.5, p=0.1), and was lower at DL in LD-SFSN (7.7 fibers/mm vs. 1.9, p<0.0001). Association with another disease was identified in 60% of the population. Immune mediated condition was diagnosed in 9/17 (53%) NLD-SFSN (cryoglobulinemia, positive lip biopsy, albumino-cytological dissociation at CSF analysis) and in 10/42 (24%) LD-SFSN (p=0.035). Good efficiency on pain intensity has been observed in 2 NLD-SFN patients after intravenous immunoglobulins infusions. Other conditions associated with NLD-SFSN were bariatric surgery (1) and metabolic syndromes (1).
CONCLUSIONS: NLD-SFSN occurs more commonly in younger female, onset is more likely acute and development appears to be more frequently associated with an immune mediated condition. This study confirms that NLD-SFSN is often associated with an inflammatory process.
Disclosure: Dr. Laffon has nothing to disclose. Dr. Delmont has nothing to disclose. Dr. Alchaar has nothing to disclose. Dr. Lantéri Minet has nothing to disclose. Dr. Jeandel has nothing to disclose. Dr. Butori has nothing to disclose. Dr. Desnuelle has nothing to disclose.
Monday, March 18 2013, 2:00 pm-6:30 pm
- Copyright © 2013 by AAN Enterprises, Inc.
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