Abstract | OBJECTIVE: METHODS: In a retrospective analysis, 40 cases of cryptogenic SFN in a university neuropathy clinic were identified. Of these, TS-HDS and FGFR-3 cases were identified, and clinical features and treatment responses were analyzed. RESULTS: In this cohort, 95% were women, and 55% had either TS-HDS or FGFR-3 antibodies (77% of these had TS-HDS). Of the seropositive group, 41% had a nonlength dependent epidermal nerve fiber density on skin punch biopsy (OR = 1.80). In the seropositive group, 82% had neuropathic pain as their primary symptom (OR = 1.73). Also 32% of seropositive patients reported widespread pain (OR = 1.63). 63% of seropositive cases presented acutely (OR = 11.0). In the seropositive group, 23% had an initial erroneous diagnosis (OR = 1.47). Eight seropositive patients improved on intravenous immunoglobulin treatment, with a 42% reduction in pain scores (P = 0.02), a 44% reduction in the Utah Neuropathy Score, and improved epidermal nerve fiber density post-treatment. CONCLUSIONS:
TS-HDS and FGFR-3 antibodies may be present in a high proportion of cryptogenic SFN cases with acute onset, nonlength dependent pathology, and primary neuropathic and widespread pain. They are often misdiagnosed as other conditions including fibromyalgia. These cases may be responsive to immune treatment, especially with intravenous immunoglobulin.
|
Authors | Lawrence A Zeidman, Konrad Kubicki |
Journal | Journal of clinical neuromuscular disease
(J Clin Neuromuscul Dis)
Vol. 22
Issue 4
Pg. 192-199
(Jun 01 2021)
ISSN: 1537-1611 [Electronic] United States |
PMID | 34019003
(Publication Type: Journal Article)
|
Copyright | Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved. |
Chemical References |
- Antibodies
- Biomarkers
- Disaccharides
- heparin disaccharide
- Heparin
- Receptor, Fibroblast Growth Factor, Type 3
|
Topics |
- Adult
- Antibodies
(blood)
- Biomarkers
(blood)
- Biopsy
- Cohort Studies
- Disaccharides
(blood)
- Female
- Heparin
(analogs & derivatives, blood)
- Humans
- Male
- Middle Aged
- Neuralgia
(diagnosis)
- Receptor, Fibroblast Growth Factor, Type 3
(blood)
- Retrospective Studies
- Small Fiber Neuropathy
(diagnosis)
- Young Adult
|