UCPPS - Prostatitis, Interstitial Cystitis Forum

Lancet. 1998 Nov 14;352(9140):1582-5.

Clinical, radiological, neurophysiological, and neuropathological characteristics of gluten ataxia.

Hadjivassiliou M, Grunewald RA, Chattopadhyay AK, Davies-Jones GA, Gibson A, Jarratt JA, Kandler RH, Lobo A, Powell T, Smith CM.
Department of Neurology, Royal Hallamshire Hospital, Sheffield, UK.

BACKGROUND: Ataxia [inability to coordinate voluntary muscle movements; unsteady movements and staggering gait] is the commonest neurological manifestation of coeliac disease. Some individuals with genetic susceptibility to the disease have serological evidence of gluten sensitivity without overt gastrointestinal symptoms or evidence of small-bowel inflammation. The sole manifestation of disease in such patients may be ataxia. We describe the clinical, radiological, and neurophysiological features of this disorder.

METHODS: Patients with ataxia attending the neurology outpatient clinics at the Royal Hallamshire Hospital, Sheffield, UK, were screened for gluten sensitivity as shown by the titre of antibody to gliadin. Those with other causes of ataxia were excluded. We carried out clinical, neurophysiological, neuroradiological, and, in two cases, neuropathological examinations.

FINDINGS: 28 patients with gluten ataxia were identified. All had gait ataxia and most had limb ataxia. Those with more severe gait ataxia had longer disease duration. No patient had tremor or other extrapyramidal features. 19 patients showed some form of peripheral neuropathy on neurophysiological examination. 16 patients had no gastrointestinal symptoms. Distal duodenal biopsy showed lymphocytic infiltration in two patients, and changes compatible with coeliac disease in 11. Six patients had evidence of cerebellar atrophy on magnetic-resonance imaging. Necropsy was done on two patients who died; there was lymphocytic infiltration of the cerebellum, damage to the posterior columns of the spinal cord, and sparse infiltration of the peripheral nerves.

INTERPRETATION: Gluten sensitivity is an important cause of apparently idiopathic ataxia and may be progressive. The ataxia is a result of immunological damage to the cerebellum, to the posterior columns of the spinal cord, and to peripheral nerves. We propose the term gluten ataxia to describe this disorder.

PMID: 9843103

1. I found that gluten had a major effect on my symptoms
2. Gluten is a nerve poison for celiacs. UCPPS is highly nerve-mediated, hence the connection.
3. An amazing 1% of the world's population has celiac disease, but only 10-15% of that 1% know it. The rest are undiagnosed. You could be in that 1%!

[Chronic inflammatory neuromyopathies in adults treated for gluten-sensitive enteropathy. A report on three cases with microvascular nerve and muscle lesions (author's transl)]

[Article in French]

Buge A, Escourolle R, Rancurel G, Hauw JJ, Denvil D, Bernier JJ, Rambaud J, Modigliani R.

Neuromyopathies developed in three patients with gluten-sensitive enteropathy, a long time after they had been cured of their digestive disease by following a gluten-free diet. These cases differed radically from typical deficiency neuropathies by the presence of microvascular inflammatory lesions in nerves and muscles. The semiological findings were similar in all 3 cases, and were distinguished by the association of signs evoking lesions of the largest myelinated nerves fibers to the posterior rami with lesions in the muscles. Corticotherapy improved the condition but did not affect its chronic course. Nerve and muscle biopsies revealed the presence of segmentary microrascularitis, mainly lymphohistiocytic. The probable mechanism of these histological changes is alterations in the circulating immune-complexes, usually found in gluten-sensitive enteropathy, producing various types of associated disorders. Some of these immune-complexes would not be related straight to digestive intolerance to gluten, but would persist during the gluten-free diet period, and could be responsible for the micro-angiitis.

Publication Types:

* Case Reports

PMID: 44001 [PubMed - indexed for MEDLINE]

Ann Med Interne (Paris). 1976 Oct;127(10):721-9.

[Nondeficiency chronic polyneuropathies in celiac disease in adults (2 cases with inflammatory neuromuscular vascularitis)]

[Article in French]

Bernier JJ, Buge A, Rambaud JC, Rancurel G, Hauw JJ, Modigliani R, Denvil D.

The neurological and muscular complications seen in coeliac disease in adults are usually attributed to deficiency secondary to malabsorption. Amongst them, however, there exists a very rare cateogory, described by Cooke et al. (1966) taking the form of a chronic myeloneuropathy which cannot be explained in terms of the malabsorption syndrome. Our two cases of gluten intolerance enteropathy, confirmed by biopsy before and after diet, fell into this group of polyneuropathies. The patients, both women, suffered from an essentially sensory ataxic polyneuropathy with accessory motor component with pyramidal and posterior column signs. CSF findings showed a meningeal inflammatory reaction in one of the two cases. These neurological signs, appearing paradoxically during a digestive disease cured by diet, evolve chronically but become stabilised with corticosteroid therapy. Any vitamin deficiency may be excluded in the aetiology of these problems. Neuropathological study of neuromuscular biopsies in very fine serial sections confirmed the mild peripheral nervous involvement but revealed identical inflammatory lesions in the nerve and muscle which were remarkable by virtue of their very highly segmentally selective micro-vasculitis appearance. In these two cases, general, clinical and biological arguments, as well as the type of histological lesion, make it possible to exclude monoclonal gammapathies, malignant haemopathies, amyloidosis and the major collagen diseases. This micro-vasculitis, having transient forms with P.A.N. is no less distinctive, and may be integrated into the provisional group of "allergic angeitis", related to physiopathology of circulating immune complexes and very fashionable in theories as to the mechanism of gluten-sensitive enteropathies. The exact nature of the link between the latter and these types of polyneuropathy remains unknown.

Publication Types: * Case Reports


PMID: 1008365 [PubMed - indexed for MEDLINE]