scholarly journals Idiopathic distal sensory polyneuropathy

Neurology ◽  
2020 ◽  
Vol 95 (22) ◽  
pp. 1005-1014 ◽  
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.

scholarly journals Small-fiber neuropathy and celiac disease: A narrative review

2021 ◽  
Author(s):  
Gustavo Figueiredo da Silva ◽  
Giulia Murillo Wollmann ◽  
Luana Schlindwein Imhof ◽  
Marina Steingräber Pereira ◽  
Matheus Fellipe Nascimento de Souza ◽  
...  
Keyword(s):  
Celiac Disease ◽  
Clinical Management ◽  
Sensory Neuropathy ◽  
Review Literature ◽  
Small Fiber Neuropathy ◽  
Narrative Review ◽  
Small Fiber ◽  
Slow Progression ◽  
Large Fiber ◽  
Inflammatory Autoimmune Disease

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.

scholarly journals Small and large fiber sensory polyneuropathy in type 2 diabetes: Influence of diagnostic criteria on neuropathy subtypes

2020 ◽  
Author(s):  
Mustapha Itani ◽  
Sandra Sif Gylfadottir ◽  
Thomas Krøigård ◽  
Alexander Gramm Kristensen ◽  
Diana Hedevang Christensen ◽  
...  
Keyword(s):  
Type 2 Diabetes ◽  
Diagnostic Criteria ◽  
Large Fiber ◽  
Sensory Polyneuropathy

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.

Diagnostic Criteria for Small Fiber Neuropathy

2017 ◽  
Vol 18 (3) ◽  
pp. 125-131 ◽  
Author(s):  
Derrick Blackmore ◽  
Zaeem A. Siddiqi
Keyword(s):  
Diagnostic Criteria ◽  
Small Fiber Neuropathy ◽  
Small Fiber

Syncope in pulmonary embolism: a retrospective cohort study

2020 ◽  
pp. postgradmedj-2020-138677
Author(s):  
Craig Richmond ◽  
Hannah Jolly ◽  
Chris Isles
Keyword(s):  
Pulmonary Embolism ◽  
Cohort Study ◽  
Retrospective Cohort Study ◽  
Retrospective Cohort ◽  
District General Hospital ◽  
Clinical Feature ◽  
Pulmonary Angiogram ◽  
Ct Pulmonary Angiogram ◽  
Age Range ◽  
Symptoms And Signs

ObjectiveTo determine the prevalence of syncope or collapse in pulmonary embolism (PE).MethodsA retrospective cohort study was conducted. We examined the frequency with which syncope or collapse (presyncope) occurred alone or with other symptoms and signs in an unselected series of 224 patients presenting to a district general hospital with PE between September 2012 and March 2016. Confirmation of PE was by CT pulmonary angiogram in each case.ResultsOur cohort of 224 patients comprised 97 men and 127 women, average age 66 years with age range of 21–94 years. Syncope or collapse was one of several symptoms and signs that led to a diagnosis of PE in 22 patients (9.8%) but was never the sole presenting feature. In descending order, these other clinical features were hypoxaemia (17 patients), dyspnoea (12), chest pain (9), tachycardia (7) and tachypnoea (7). ECG abnormalities reported to occur more commonly in PE were found in 13/17 patients for whom ECGs were available. Patients with PE presenting with syncope or collapse were judged to have a large clot load in 15/22 (68%) cases.ConclusionSyncope was a frequent presenting symptom in our study of 224 consecutive patients with PE but was never the sole clinical feature. It would be difficult to justify routine testing for PE in patients presenting only with syncope or collapse.

scholarly journals Long-Time Course of Idiopathic Small Fiber Neuropathy

2018 ◽  
Vol 79 (3-4) ◽  
pp. 161-165 ◽  
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.

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.

Sign in / Sign up

Export Citation Format

Share Document