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. 2020 Jan 2:2020:8796519.
doi: 10.1155/2020/8796519. eCollection 2020.

Small Fiber Neuropathy: Clinicopathological Correlations

Endre Pál  1   2 , Krisztina Fülöp  2 , Péter Tóth  1 , Gabriella Deli  1 , Zoltán Pfund  1 , József Janszky  1 , Sámuel Komoly  1
Affiliations

Small Fiber Neuropathy: Clinicopathological Correlations

Endre Pál et al. Behav Neurol. .

Abstract

Small fiber neuropathy develops due to the selective damage of the thin fibers of peripheral nerves. Many common diseases can cause this condition, including diabetes, infections, autoimmune and endocrine disorders, but it can occur due to genetic alterations, as well. Eighty-five skin biopsy-proven small-fiber neuropathy cases were analyzed. Forty-one (48%) cases were idiopathic; among secondary types, hypothyreosis (9.4%), diabetes mellitus (7%), cryoglobulinemia (7%), monoclonal gammopathy with unproved significance (4.7%), Sjögren's disease (3%), and paraneoplastic neuropathy (3%) were the most common causes. Two-thirds (68%) of the patients were female, and the secondary type started 8 years later than the idiopathic one. In a vast majority of the cases (85%), the distribution followed a length-dependent pattern. Intraepidermal fiber density was comparable in idiopathic and secondary forms. Of note, we found significantly more severe pathology in men and in diabetes. Weak correlation was found between patient-reported measures and pathology, as well as with neuropathic pain-related scores. Our study confirmed the significance of small fiber damage-caused neuropathic symptoms in many clinical conditions, the gender differences in clinical settings, and pathological alterations, as well as the presence of severe small fiber pathology in diabetes mellitus, one of the most common causes of peripheral neuropathy.

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Conflict of interest statement

The authors report no conflict of interest.

Figures

Figure 1
Figure 1
Intraepidermal nerve fiber density (IENFD) in idiopathic (iSFN) and secondary SFN (sSFN). No significant differences were found between the two cohorts.
Figure 2
Figure 2
Relationship of the IENFD and age. Intraepidermal nerve fiber density (IENFD) showed negative correlation with the age of the investigated subjects.
Figure 3
Figure 3
Correlations of intraepidermal, subepidermal, and autonomic fiber densities. Although, subepidermal nerve fiber density (SENFD) and autonomic nerve fiber density (ANFD) were assessed semiquantitatively, the amount of these fibers was comparable to intraepidermal nerve fiber density (IENFD). Asterisks mark significant differences from grade 0.
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