Sensory ganglionopathy due to gluten sensitivity

Hadjivassiliou, Marios; Rao, Dasappaiah Ganesh; Wharton, Stephen B.; Sanders, David S.; Grünewald, Richard A.; Davies-Jones, Aelwyn G. B.

doi:10.1212/wnl.0b013e3181f25ee0

Cited by 70 publications

(82 citation statements)

References 27 publications

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“…Among autoimmune ganglionitides, SS is best known, but rheumatoid arthritis, systemic lupus erythematosus, and autoimmune hepatitis are also implicated [8]. Celiac, another autoimmune condition associated with ganglionitis, is discussed in the section on polyneuropathy as most of the studies did not distinguish between ganglionopathy and distal axonopathy [12,13].…”

Section: Immune-mediated Painful Sensory Ganglionitis/neuronitismentioning

confidence: 99%

Immunotherapy Prospects for Painful Small-fiber Sensory Neuropathies and Ganglionopathies

Oaklander

2016

Neurotherapeutics

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The best-known peripheral neuropathies are those affecting the large, myelinated motor and sensory fibers. These have well-established immunological causes and therapies. Far less is known about the somatic and autonomic "small fibers"; the unmyelinated C-fibers, thinly myelinated A-deltas, and postganglionic sympathetics. The small fibers sense pain and itch, innervate internal organs and tissues, and modulate the inflammatory and immune responses. Symptoms of small-fiber neuropathy include chronic pain and itch, sensory impairment, edema, and skin color, temperature, and sweating changes. Small-fiber polyneuropathy (SFPN) also causes cardiovascular, gastrointestinal, and urological symptoms, the neurologic origin of which often remains unrecognized. Routine electrodiagnostic study does not detect SFPN, so skin biopsies immunolabeled to reveal axons are recommended for diagnostic confirmation. Preliminary evidence suggests that dysimmunity causes some cases of small-fiber neuropathy. Several autoimmune diseases, including Sjögren and celiac, are associated with painful small-fiber ganglionopathy and distal axonopathy, and some patients with "idiopathic" SFPN have evidence of organ-specific dysimmunity, including serological markers. Dysimmune SFPN first came into focus in children and teenagers as they lack other risk factors, for example diabetes or toxic exposures. In them, the rudimentary evidence suggests humoral rather than cellular mechanisms and complement consumption. Preliminary evidence supports efficacy of corticosteroids and immunoglobulins in carefully selected children and adult patients. This paper reviews the evidence of immune causality and the limited data regarding immunotherapy for small-fiberpredominant ganglionitis, regional neuropathy (complex regional pain syndrome), and distal SFPN. These demonstrate the need to develop case definitions and outcome metrics to improve diagnosis, enable prospective trials, and dissect the mechanisms of small-fiber neuropathy.

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Section: Immune-mediated Painful Sensory Ganglionitis/neuronitismentioning

confidence: 99%

Immunotherapy Prospects for Painful Small-fiber Sensory Neuropathies and Ganglionopathies

Oaklander

2016

Neurotherapeutics

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“…The commonest types are symmetrical sensorimotor axonal length dependent peripheral neuropathy and sensory ganglionopathy (Hadjivassiliou et al, 2010b). Other types of neuropathies have also been reported including asymmetrical neuropathy (Kelkar et al, 1996;Hadjivassiliou et al, 1997;Chin et al, 2006) small fibre neuropathy (Brannagan et al, 2005) and rarely pure motor neuropathy (Hadjivassiliou et al, 1997) or autonomic neuropathy (Gibbons and Freeman, 2005).…”

Section: Gluten Neuropathymentioning

confidence: 99%

“…In such cases, there is evidence of inflammatory infiltrates within the dorsal root ganglia. The disease progresses slowly if untreated but strict adherence to a gluten free diet may result in stabilisation or even improvement of the neuropathy irrespective of the presence of enteropathy (Hadjivassiliou et al, 2010b).…”

Section: Gluten Neuropathymentioning

confidence: 99%

The Neuroimmunology of Gluten Intolerance

Hadjivassiliou

Sanders

Aeschlimann

2016

Neuro-Immuno-Gastroenterology

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The term Gluten Related Disorders (GRD) denotes a spectrum of diverse immune mediated diseases triggered by the ingestion of gluten (protein found in wheat, barley and rye). Coeliac disease (CD) or gluten sensitive enteropathy is the most recognised and studied entity within GRD.Extraintestinal manifestations, are gaining recognition and are increasingly the subject of further studies as they may hold the key to unravelling the pathophysiology of GRD. Such manifestations include skin involvement in the form of dermatitis herpetiformis (DH) and neurological dysfunction (eg gluten ataxia and gluten neuropathy). Furthermore the recent concept of extraintestinal manifestations without enteropathy (termed Non-Coeliac Gluten Sensitivity-NCGS) has become accepted as part of the same spectrum. In this chapter we review the neurological manifestations in GRD, discuss recent advances in diagnosis, and possible pathophysiological mechanisms.

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“…The commonest type of gluten neuropathy is symmetrical sensorimotor axonal peripheral neuropathy [1]. Sensory ganglionopathy, an asymmetrical pure sensory neuropathy is the second commonest form of neuropathy seen in the context of gluten sensitivity [3]. This type of neuropathy exclusively affects the dorsal root ganglia and the co o est causes apart fro glute se sitivity i clude Sjogre 's sy dro e a d paraneoplastic neurological syndromes.…”

Section: Introductionmentioning

confidence: 99%