Small-fiber neuropathy and celiac disease: A narrative review

Background: Neurological manifestations of celiac disease (CD) have a prevalence of 10% to 22% among patients. Of this group, neuropathy is present in up to 23%, with small fiber neuropathy (SFN) being the most described, with a predominance of painful symptoms and appendicular paresthesia. Objectives: Review literature to describe the clinical management of SFN in CD. Design and setting: Narrative review. Methods: Non-systematic review on Pubmed and Scielo database. Results: CD is a chronic inflammatory autoimmune disease that can generate extraintestinal manifestations as SFN. Small fiber neuropathy is a painful focal sensory neuropathy of slow progression, with distal predominance, symmetrical or not, beginning in adulthood and, sometimes, followed by autonomic dysfunction. Electroneuromyography studies (ENMG) suggest greater involvement of myelinated Adelta and C myelinated thin fibers, which is a precursor of sensory ganglionopathy in the dorsal root and can progress to large fiber neuropathy. The ENMG of SFN is usually normal because it is generally not demyelinating. To confirm the diagnosis, a skin biopsy that evaluates the fiber’s intra-epidermal density is indicated. Another exam is the quantitative test of the sudomotor reflex, capable of evaluating autonomic function. Finally, treatment should be directed to the underlying cause, optimization of the treatment of CD, and the management of symptoms, such as pain. Conclusions: The SFN, despite being an uncommon manifestation of CD, is possibly underdiagnosed due to the lack of studies evaluating this manifestation in celiac patients. Therefore, further studies are needed in order to instigate early diagnosis and adequate clinical management.

Download Full-text

Long-Time Course of Idiopathic Small Fiber Neuropathy

European Neurology ◽

10.1159/000487717 ◽

2018 ◽

Vol 79 (3-4) ◽

pp. 161-165 ◽

Cited By ~ 5

Author(s):

Pia Flossdorf ◽

Walter F. Haupt ◽

Anna Brunn ◽

Martina Deckert ◽

Gereon R. Fink ◽

...

Keyword(s):

Quality Of Life ◽

Time Course ◽

Benign Disease ◽

Small Fiber Neuropathy ◽

Disease Course ◽

Peripheral Neuropathies ◽

Small Fiber ◽

Long Time ◽

Large Fiber

Background: Small fiber neuropathy (SFN) is a challenging subtype of peripheral neuropathies. Once the diagnosis has been established, there is an uncertainty how SFN may progress, whether larger fibers will become involved over time, whether quality of life may be compromised, or whether repeated diagnostic workup in patients with unknown underlying cause may increase the yield of treatable causes of SFN. Methods: We evaluated 16 patients with documented long-time course of idiopathic SFN. Results: Clinical and electrophysiological course remained stable in 75% of the patients, while 25% SFN-patients developed large fiber neuropathies. Conclusions: Our data suggest that SFN represents a benign disease course in the majority of patients without severely limiting the quality of life.

Download Full-text

Accuracy of Ipswich Touch Test (IpTT) to detect small fiber neuropathy and large fiber neuropathy as a risk factor of diabetic foot ulcers in public health centers

Enfermería Clínica ◽

10.1016/j.enfcli.2019.07.108 ◽

2020 ◽

Vol 30 ◽

pp. 308-312

Author(s):

Ita Sulistiani Basir ◽

Yuliana Syam ◽

Saldy Yusuf ◽

Serlina Sandi

Keyword(s):

Public Health ◽

Risk Factor ◽

Diabetic Foot ◽

Small Fiber Neuropathy ◽

Health Centers ◽

Foot Ulcers ◽

Diabetic Foot Ulcers ◽

Small Fiber ◽

Large Fiber ◽

Large Fiber Neuropathy

Download Full-text

A 50-Year-Old Woman with Burning Feet

10.1093/med/9780190491901.003.0020 ◽

2016 ◽

Author(s):

Jeffrey A. Cohen ◽

Justin J. Mowchun ◽

Victoria H. Lawson ◽

Nathaniel M. Robbins

Keyword(s):

Autonomic Neuropathy ◽

Nerve Conduction Studies ◽

Small Fiber Neuropathy ◽

Rational Approach ◽

Burning Pain ◽

Unmyelinated Fibers ◽

Small Fiber ◽

Large Fiber ◽

Diagnostic Modalities ◽

Atypical Presentations

Small-fiber neuropathy typically presents with burning pain or with widespread brief stabbing pains, by atypical presentations including asymmetric sensory symptoms are common. Nerve conduction studies are usually normal, as this disorder test only interrogates large fiber function; in small-fiber neuropathy the pathology is restricted to smaller unmyelinated fibers. Autonomic neuropathy can accompany the painful peripheral neuropathy but can be difficult to recognize since the symptoms can be protean. In this chapter, clinical characteristics of small-fiber and autonomic neuropathy are discussed. Various diagnostic modalities are described, including the benefits and pitfalls of available options. The most common conditions causing small-fiber and autonomic neuropathy are reviewed. The controversy surrounding impaired glucose tolerance as an etiological factor is dicusssed. We discuss the available medications and outline a rational approach to treatment.

Download Full-text

Idiopathic distal sensory polyneuropathy

Neurology ◽

10.1212/wnl.0000000000010988 ◽

2020 ◽

Vol 95 (22) ◽

pp. 1005-1014 ◽

Cited By ~ 2

Author(s):

Roy Freeman ◽

Jennifer S. Gewandter ◽

Catharina G. Faber ◽

Christopher Gibbons ◽

Simon Haroutounian ◽

...

Keyword(s):

Diagnostic Criteria ◽

Sensory Nerve ◽

Sensory Neuropathy ◽

Clinical Feature ◽

Small Fiber ◽

Intraepidermal Nerve Fiber Density ◽

Large Fiber ◽

Symptoms And Signs ◽

Sensory Polyneuropathy ◽

Standardized Diagnostic

ObjectiveTo present standardized diagnostic criteria for idiopathic distal sensory polyneuropathy (iDSP) and its subtypes: idiopathic mixed fiber sensory neuropathy (iMFN), idiopathic small fiber sensory neuropathy (iSFN), and idiopathic large fiber sensory neuropathy (iLFN) for use in research.MethodsThe Analgesic, Anesthetic, and Addiction Clinical Trial Translations, Innovations, Opportunities and Networks (ACTTION) public-private partnership with the Food and Drug Administration convened a meeting to develop consensus diagnostic criteria for iMFN, iSFN, and iLFN. After background presentations, a collaborative, iterative approach was used to develop expert consensus for new criteria.ResultsAn iDSP diagnosis requires at least 1 small fiber (SF) or large fiber (LF) symptom, at least 1 SF or LF sign, abnormalities in sensory nerve conduction studies (NCS) or distal intraepidermal nerve fiber density (IENFD), and exclusion of known etiologies. An iMFN diagnosis requires that at least 1 of the above clinical features is SF and 1 clinical feature is LF with abnormalities in sensory NCS or IENFD. Diagnostic criteria for iSFN require at least 1 SF symptom and at least 1 SF sign with abnormal IENFD, normal sensory NCS, and the absence of LF symptoms and signs. Diagnostic criteria for iLFN require at least 1 LF symptom and at least 1 LF sign with normal IENFD, abnormal sensory NCS, and absence of SF symptoms and signs.ConclusionAdoption of these standardized diagnostic criteria will advance research and clinical trials and spur development of novel therapies for iDSPs.

Download Full-text

Small Fiber Neuropathy Incidence, Prevalence, Longitudinal Impairments, and Disability

Neurology ◽

10.1212/wnl.0000000000012894 ◽

2021 ◽

pp. 10.1212/WNL.0000000000012894

Author(s):

Stephen A Johnson ◽

Kamal Shouman ◽

Shahar Shelly ◽

Paola Sandroni ◽

Sarah E Berini ◽

...

Keyword(s):

Lewy Body ◽

Population Based ◽

Small Fiber Neuropathy ◽

Myocardial Infarctions ◽

Small Fiber ◽

Impairment Score ◽

Targeted Testing ◽

Large Fiber ◽

Al Amyloid ◽

Incidence Prevalence

Background and Objectives:There is limited population-based data on small fiber neuropathy (SFN). We wished to determine SFN incidence, prevalence, comorbidities, longitudinal impairments, and disabilities.Methods:Test-confirmed patients with SFN in Olmsted, Minnesota and adjacent counties were compared 3:1 to matched controls (January 1st, 1998-December 31st, 2017).Results:Ninety-four patients with SFN were identified, incidence 1.3/100,000/year increasing over the study period, prevalence 13.3/100,000. Average follow-up was 6.1 years (0.7-43 years), mean onset age 54 years (range 14–83). Female (67%), obesity (BMI mean 30.4 versus 28.5), insomnia (86% versus 54%), analgesic-opioid prescriptions (72% versus 46%), hypertriglyceridemia (180 mg/dl mean versus 147 mg/dl) and diabetes mellitus (DM) (51% versus 22%, p<0.001) were more common (OR 3.8-9.0, all p<0.03). Patients with SFN did not self-identify as disabled with median mRS of 1.0 (range 0-6) versus controls 0.0 (0-6), p=0.04. Higher Charlson comorbidities (median 6, range 3-9) occurred versus controls (median 3, range 1-9) p<0.001. Myocardial infarctions occurred in 46% versus 27% of controls (p<0.0001). Classifications included: idiopathic (70%); DM (15%); Sjögrens (2%); AL-amyloid (1%); transthyretin-amyloid (1%); Fabry (1%); lupus (1%); post viral (1%); Lewy body (1%) and multifactorial (5%). Foot ulcers occurred in 17, with 71% having DM. Large fiber neuropathy developed in 36%, on average 5.3 years (range 0.2-14.3 years) from SFN onset. Median onset Composite Autonomic Severity Score (CASS) was 3, change/year 0.08 (range 0-2.0). Median Neuropathy Impairment Score (NIS) was 2 at onset (range 0 to 8), change/year +1.0 (range -7.9 to +23.3). NIS and CASS change >+1 point/year occurred in only AL-amyloid, hereditary transthyretin-amyloid, Fabry, uncontrolled DM, and Lewy body. Death from symptom onset was higher in patients with SFN 19% versus controls 12%, p<0.001, 50% secondary to DM complications.Discussion:Isolated SFN is uncommon but increasing in incidence. Most patients do not develop major neurological impairments and disability but have multiple comorbidities, including cardiovascular ischemic events, and increased mortality from SFN onsets. Development of large fiber involvements and DM are common over time. Targeted testing facilitates interventional therapies for DM but also rheumatologic and rare genetic forms.

Download Full-text