Dysimmune small fiber neuropathies

Oaklander, Anne Louise

doi:10.1016/b978-0-12-814572-2.00010-8

Cited by 4 publications

(6 citation statements)

References 108 publications

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“…Plus, almost all patients here were female. Females comprised 69% in one case series of neuropathy incident to COVID-19, 9 they comprise the large majority of SFN patients, 19 and of patients with most systemic and organ-specific immune syndromes. Similarly, POTS and multiple types of peripheral neuropathy are well-described after other vaccinations 20 or illnesses, including brachial plexitis.…”

Section: Discussionmentioning

confidence: 99%

Neuropathic symptoms with SARS-CoV-2 vaccination

Safavi

Gustafson

Lehky

et al. 2022

Preprint

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Background and ObjectivesVarious peripheral neuropathies, particularly those with sensory and autonomic dysfunction may occur during or shortly after acute COVID-19 illnesses. These appear most likely to reflect immune dysregulation. If similar manifestations can occur with the vaccination remains unknown.ResultsIn an observational study, we studied 23 patients (92% female; median age 40years) reporting new neuropathic symptoms beginning within 1 month after SARS-CoV-2 vaccination. 100% reported sensory symptoms comprising severe face and/or limb paresthesias, and 61% had orthostasis, heat intolerance and palpitations. Autonomic testing in 12 identified seven with reduced distal sweat production and six with positional orthostatic tachycardia syndrome. Among 16 with lower-leg skin biopsies, 31% had diagnostic/subthreshold epidermal neurite densities (≤5%), 13% were borderline (5.01-10%) and 19% showed abnormal axonal swelling. Biopsies from randomly selected five patients that were evaluated for immune complexes showed deposition of complement C4d in endothelial cells. Electrodiagnostic test results were normal in 94% (16/17). Together, 52% (12/23) of patients had objective evidence of small-fiber peripheral neuropathy. 58% patients (7/12) treated with oral corticosteroids had complete or near-complete improvement after two weeks as compared to 9% (1/11) of patients who did not receive immunotherapy having full recovery at 12 weeks. At 5-9 months post-symptom onset, 3 non-recovering patients received intravenous immunoglobulin with symptom resolution within two weeks.ConclusionsThis observational study suggests that a variety of neuropathic symptoms may manifest after SARS-CoV-2 vaccinations and in some patients might be an immune-mediated process.

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Section: Discussionmentioning

confidence: 99%

Neuropathic symptoms with SARS-CoV-2 vaccination

Safavi

Gustafson

Lehky

et al. 2022

Preprint

Self Cite

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“…Although knowledge is incomplete, most of the clinical studies, and a few small studies using patient sera to passively induce small fibre neuropathy into animals,27 increasingly suggest that a substantial portion of initially idiopathic small fibre neuropathy may involve dysimmunity and inflammation, implying potential roles for immunomodulatory therapies 28. Trisulfated heparin disaccharide (TS-HDS) antibodies have been reported in as many as 37% of patients with idiopathic small fibre neuropathy,29 antiplexin D1 antibodies in 12.7% of patients30 and antibodies against the fibroblast growth factor receptor 3 (FGFR3) in up to 15% 31.…”

Section: Clinical Featuresmentioning

confidence: 99%

Advances in diagnosis and management of distal sensory polyneuropathies

Silsby

Feldman

Dortch

et al. 2023

J Neurol Neurosurg Psychiatry

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Distal sensory polyneuropathy (DSP) is characterised by length-dependent, sensory-predominant symptoms and signs, including potentially disabling symmetric chronic pain, tingling and poor balance. Some patients also have or develop dysautonomia or motor involvement depending on whether large myelinated or small fibres are predominantly affected. Although highly prevalent, diagnosis and management can be challenging. While classic diabetes and toxic causes are well-recognised, there are increasingly diverse associations, including with dysimmune, rheumatological and neurodegenerative conditions. Approximately half of cases are initially considered idiopathic despite thorough evaluation, but often, the causes emerge later as new symptoms develop or testing advances, for instance with genetic approaches. Improving and standardising DSP metrics, as already accomplished for motor neuropathies, would permit in-clinic longitudinal tracking of natural history and treatment responses. Standardising phenotyping could advance research and facilitate trials of potential therapies, which lag so far. This review updates on recent advances and summarises current evidence for specific treatments.

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“…Suspected cases can be confirmed with ophthalmologic evaluation and lip biopsy of salivary glands. On the other hand, paraneoplastic painful ganglionitis is most often associated with anti-Hu amphiphysin and anti-CV2 autoantibodies, for which the lung is the most common site of a malignant condition, followed by hematological and gastrointestinal tumors [45]. As there is a narrow therapeutic window to intervene in NLD-SFN before there are irreversible deficits, NLD-SFN requires prompt recognition for early disease-targeted intervention [46].…”

Section: Clinical Presentationmentioning

confidence: 99%

Biomarkers in small fiber neuropathy

Chan

Ong

et al. 2022

Explor Neuroprot Ther

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Small fiber neuropathy (SFN) is a relatively common, but largely understudied neurological syndrome which has affected the lives of many globally. The common symptoms of SFN include pain, dysesthesia, and autonomic dysfunction, which are caused by damage to small nerve fibers. Due to its heterogeneous nature, SFN causes a multitude of symptoms which makes the disease and its subtypes difficult to diagnose. Furthermore, as the pathophysiology of SFN remains largely enigmatic, no cause is found in around 50% of the cases and these are classified as idiopathic SFN (iSFN). The difficult task of diagnosing SFN, and the even more elusive feat of hunting for the underlying etiology, demands accurate, precise, preferably noninvasive, and affordable tools, or a combination of them. Accordingly, appropriate biomarkers for SFN are needed to stratify patients and develop cause-centered treatments in addition to symptomatic treatments. As peripheral axons grow and repair, identifying underlying causes of SFN and intervening early may spur axonal regeneration in young patients, which can greatly improve their symptoms and improve quality of life. This narrative review aims to objectively highlight functional, histological, and molecular biomarkers to aid clinicians in discerning the diagnostic tests they should use to diagnose, confirm and determine the etiology of SFN. The strengths and limitations of each potential biomarker will be discussed. Clearer diagnostic criteria, guidelines, and work-up for SFN are required for clinicians to better identify the disease in patients presenting with non-specific symptoms.

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Dysimmune small fiber neuropathies

Cited by 4 publications

References 108 publications

Neuropathic symptoms with SARS-CoV-2 vaccination

Neuropathic symptoms with SARS-CoV-2 vaccination

Advances in diagnosis and management of distal sensory polyneuropathies

Biomarkers in small fiber neuropathy

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