scholarly journals Small Fiber Neuropathy: Clinicopathological Correlations

2020 ◽  
Vol 2020 ◽  
pp. 1-7 ◽  
Author(s):  
Endre Pál ◽  
Krisztina Fülöp ◽  
Péter Tóth ◽  
Gabriella Deli ◽  
Zoltán Pfund ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Genetic Alterations ◽  
Small Fiber Neuropathy ◽  
Endocrine Disorders ◽  
Fiber Density ◽  
Fiber Damage ◽  
Small Fiber ◽  
Patient Reported ◽  
Common Causes ◽  
Patient Reported Measures

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.

scholarly journals Corneal Confocal Microscopy in the Diagnosis of Small Fiber Neuropathy: Faster, Easier, and More Efficient Than Skin Biopsy?

2021 ◽  
Vol 29 (1) ◽  
pp. 1-8
Author(s):  
Mariia V. Lukashenko ◽  
Natalia Y. Gavrilova ◽  
Anna V. Bregovskaya ◽  
Lidiia A. Soprun ◽  
Leonid P. Churilov ◽  
...  
Keyword(s):  
Confocal Microscopy ◽  
Skin Biopsy ◽  
Nerve Fiber ◽  
Small Fiber Neuropathy ◽  
Fiber Damage ◽  
Advantages And Disadvantages ◽  
Small Fiber ◽  
Corneal Confocal Microscopy ◽  
Common Causes ◽  
Small Nerve

Chronic pain may affect 30–50% of the world’s population and an important cause is small fiber neuropathy (SFN). Recent research suggests that autoimmune diseases may be one of the most common causes of small nerve fiber damage. There is low awareness of SFN among patients and clinicians and it is difficult to diagnose as routine electrophysiological methods only detect large fiber abnormalities, and specialized small fiber tests, like skin biopsy and quantitative sensory testing, are not routinely available. Corneal confocal microscopy (CCM) is a rapid, non-invasive, reproducible method for quantifying small nerve fiber degeneration and regeneration, and could be an important tool for diagnosing SFN. This review considers the advantages and disadvantages of CCM and highlights the evolution of this technique from a research tool to a diagnostic test for small fiber damage, which can be a valuable contribution to the study and management of autoimmune disease.

scholarly journals Diagnosing small fiber neuropathy in clinical practice: a deep phenotyping study

2021 ◽  
Vol 14 ◽  
pp. 175628642110043
Author(s):  
Nadine Egenolf ◽  
Caren Meyer zu Altenschildesche ◽  
Luisa Kreß ◽  
Katja Eggermann ◽  
Barbara Namer ◽  
...  
Keyword(s):  
Neurological Examination ◽  
Punch Biopsy ◽  
Small Fiber Neuropathy ◽  
Fiber Density ◽  
Axon Reflex ◽  
Small Fiber ◽  
Corneal Confocal Microscopy ◽  
Intraepidermal Nerve Fiber Density ◽  
Number Of Patients ◽  
Skin Punch Biopsy

Background and aims: Small fiber neuropathy (SFN) is increasingly suspected in patients with pain of uncertain origin, and making the diagnosis remains a challenge lacking a diagnostic gold standard. Methods: In this case–control study, we prospectively recruited 86 patients with a medical history and clinical phenotype suggestive of SFN. Patients underwent neurological examination, quantitative sensory testing (QST), and distal and proximal skin punch biopsy, and were tested for pain-associated gene loci. Fifty-five of these patients additionally underwent pain-related evoked potentials (PREP), corneal confocal microscopy (CCM), and a quantitative sudomotor axon reflex test (QSART). Results: Abnormal distal intraepidermal nerve fiber density (IENFD) (60/86, 70%) and neurological examination (53/86, 62%) most frequently reflected small fiber disease. Adding CCM and/or PREP further increased the number of patients with small fiber impairment to 47/55 (85%). Genetic testing revealed potentially pathogenic gene variants in 14/86 (16%) index patients. QST, QSART, and proximal IENFD were of lower impact. Conclusion: We propose to diagnose SFN primarily based on the results of neurological examination and distal IENFD, with more detailed phenotyping in specialized centers.

Case 51: Small Fiber Neuropathy and Transient Orthostatic Hypotension in Diabetes

2019 ◽  
pp. 283-286
Author(s):  
Peter Novak
Keyword(s):  
Diabetes Mellitus ◽  
Orthostatic Hypotension ◽  
Autonomic Failure ◽  
Small Fiber Neuropathy ◽  
Autonomic Testing ◽  
Small Fiber

This patient is an example of a typical autonomic failure associated with diabetes mellitus. Autonomic testing showed generalized autonomic failure with orthostatic hypotension and severe small fiber neuropathy affecting both sensory and autonomic fibers.

Small Fiber Neuropathy

2019 ◽  
Vol 39 (05) ◽  
pp. 570-577 ◽  
Author(s):  
Lan Zhou
Keyword(s):  
Skin Biopsy ◽  
Lifestyle Modification ◽  
Specific Treatment ◽  
Small Fiber Neuropathy ◽  
Fiber Density ◽  
Small Fiber ◽  
Intraepidermal Nerve Fiber Density ◽  
Associated Conditions ◽  
Density Evaluation

AbstractSmall fiber neuropathy (SFN) is common, and can be associated with many medical conditions. The majority of the patients with SFN suffer from painful paresthesia which can negatively impact their quality of life. Skin biopsy with intraepidermal nerve fiber density evaluation is the gold standard diagnostic test. Autonomic function testing is useful when autonomic symptoms are present. Screening for associated conditions should be done in every patient, even when a known underlying associated condition is present, before the neuropathy evaluation. Etiology-specific treatment, lifestyle modification, and pain control are the key elements of SFN management. This article will review the clinical presentation, skin biopsy procedure, utility of diagnostic tests, associated conditions, management, and prognosis of SFN.

In vivo confocal microscopy of corneal small fiber damage in diabetes mellitus

2010 ◽  
Vol 248 (9) ◽  
pp. 1307-1312 ◽  
Author(s):  
Elisabeth M. Messmer ◽  
Christoph Schmid-Tannwald ◽  
Daniel Zapp ◽  
Anselm Kampik
Keyword(s):  
Diabetes Mellitus ◽  
Confocal Microscopy ◽  
In Vivo Confocal Microscopy ◽  
Fiber Damage ◽  
Small Fiber

scholarly journals Small Fiber Neuropathy in Pulmonary Sarcoidosis and Tuberculosis: Clinical and Histological Correlates

2020 ◽  
Author(s):  
Anna Starshinova ◽  
Natalia Basantsova ◽  
Yulia Zinchenko ◽  
Valeria Shapkina ◽  
Anna Malkova ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Neurological Complication ◽  
Unknown Origin ◽  
Pulmonary Sarcoidosis ◽  
Nerve Fibers ◽  
Small Fiber Neuropathy ◽  
Fiber Density ◽  
Tuberculosis Patients ◽  
Small Fiber ◽  
Intraepidermal Nerve Fiber Density

Abstract Sarcoidosis (SC) is the granulomatous disease of an unknown origin, where the differential diagnosis with tuberculosis (TB) is challenging and vital for patients’ prognosis. The common neurological complication in SC is a small fiber neuropathy (SFN), that is considered to be the result of the chronic inflammation, and remains significantly understudied. There is no reliable data, whether such complication is observed in TB patients, where the systemic inflammation is also described. Aim: To identify the clinical and histological correlates of the small fiber neuropathy in sarcoidosis and tuberculosis patients.Materials and methods. A study was performed in 2018 – 2019 years and included 71 patients with pulmonary sarcoidosis (n=25), pulmonary tuberculosis (n=21), and healthy subjects (n=25). For the clinical verification of the SFN, the “Small fiber neuropathy screening list” (SFN-SL) was used. A punch biopsy of the skin was performed followed by the enzyme immunoassay analysis with PGP 9.5 antibodies.Results of the study: Up to 60% of sarcoidosis patients and 19% tuberculosis patients report the presence of at least one complaint, which may be associated with SFN. The most frequent complaints included dysfunctions of the cardiovascular, musculoskeletal system and gastrointestinal tract. A negative, statistically significant correlation between the intraepidermal nerve fiber density (IEND) and SFN-SL score was revealed in both groups (Spearman coefficient, r = -0.3508, p = 0.0102, and r = -0.7382, p = 0.0064, respectively). Wherein, the density of small nerve fibers in the patients with pulmonary sarcoidosis was lower, compared to the patients with tuberculosis (Mann-Whitney test, p = 0.0047). Conclusion: In patients with pulmonary sarcoidosis, small fiber neuropathy may develop as a result of systemic immune-mediated inflammation. The most common symptoms of this complication were dysautonomia and mild sensory dysfunction. Wherein, in tuberculosis patients clinical and histological symptoms of the small fiber neuropathy were subsequently less prominent, which may represent the difference between the autoimmune and bacterial inflammation. The validated questionnaires and histologic verification of the diagnosis help to establish the severity of neuropathy of small fibers, to determine the prognosis, to plan the treatment strategн, and also may allude the possibility for the additional criteria of differential diagnosis between two diseases.

Case 62: Small Fiber Neuropathy with Symptomatic Improvement on Immune Globulins

2019 ◽  
pp. 328-331
Author(s):  
Peter Novak
Keyword(s):  
Symptomatic Improvement ◽  
Small Fiber Neuropathy ◽  
Fiber Density ◽  
Autonomic Testing ◽  
Small Fiber ◽  
Epidermal Nerve Fiber Density ◽  
Immune Globulins ◽  
Sensory Fibers ◽  
Nerve Fiber Density ◽  
Epidermal Nerve Fiber

Autonomic testing revealed length-dependent small fiber neuropathy affecting sensory fibers. Repeated titer of acetylcholine ganglionic antibody was negative, but repeated epidermal nerve fiber density testing showed progressive deterioration. Symptoms improved with intravenous immune globulin therapy.

scholarly journals Presence of Decreased Intraepidermal Nerve Fiber Density Consistent with Small Fiber Neuropathy in Patients with Central Post-Stroke Pain

Pain Medicine ◽  
2016 ◽  
Vol 17 (8) ◽  
pp. 1569-1571 ◽  
Author(s):  
Yefim Cavalier ◽  
Phillip J. Albrecht ◽  
Colum Amory ◽  
Gary L. Bernardini ◽  
Charles E. Argoff
Keyword(s):  
Nerve Fiber ◽  
Small Fiber Neuropathy ◽  
Fiber Density ◽  
Post Stroke ◽  
Small Fiber ◽  
Intraepidermal Nerve Fiber Density ◽  
Nerve Fiber Density ◽  
Intraepidermal Nerve Fiber
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