The vermis. There was good correlation between the clinical severity of

The vermis. There was good correlation between the clinical severity of the ataxia and the severity of the cerebellar atrophy. By contrast those patients who did not adopt a gluten free diet continued to progress both clinically and radiologically. Most of the patients who showed limited recovery had radiological evidence of cerebellar atrophy. These results suggest that strict-gluten free diet may improve or stabilize GA A small, uncontrolled study looked at the effect of intravenous immunoglobulins (IVIg) in the treatment of 4 patients with GA free of enteropathy [13] (Additional file 1: Table S1). IVIg improved CAs in 3 of the 4 patients. However, these responders still showed CAs that interfered with their daily lives. Finally, the effect of intravenous methylprednisolone (mPSL) in GA has D(+)-Galactosamine (hydrochloride) not been reported so far.Prognostic factorslack of response correlates with poor adherence to gluten-free diet [15, 16]. Other reasons include the presence of small amounts of gluten in most gluten-free products. In patients with high sensitivity to gluten, a minute amount of gluten is enough to cause cerebellar damage [15, 16]. In those patients who continue to be positive for anti-gliadin Ab despite strict adherence, wheat-free diet should be considered first before embarking PubMed ID:https://www.ncbi.nlm.nih.gov/pubmed/24145079 on immunosuppressive therapy [16].Immunotherapy for gluten-free resistant patientsTrials were reported for patients who failed to respond to gluten-free diet (though hypersensitivity to a small amount of gluten in gluten-free products could not be ruled out) [14, 17] (Additional file 1: Table S1). The MRI of these studied patients showed evident atrophy of the cerebellar vermis. Souayah et al. [14] reported three patients who were treated with IVIg whose CAs and neuropathic pain (small fiber neuropathy syndrome) were resistant to strict gluten-free diet. All three patients responded to the IVIg induction therapy. The effectiveness of such therapy was confirmed by worsening of CAs upon discontinuation of IVIg in two patients. Accordingly, these patients were treated with a maintenance dose of IVIg, which produced stabilization of clinical symptoms. Consistent with these results, Nanri et al. [17] reported a transient response to IVIg in two patients with GA. These results suggest that continuous immunosuppression is necessary in such patients. Thus, maintenance therapy with IVIg or immunosuppressants (mycophenolate, cyclosporin, cyclophosphamide, and mycophenolate mofetil) should be considered for longterm follow-up of patients who fail to respond to glutenfree diet provided that the patient is negative for all serological gluten-related antibodies [16].Immunotherapies for GA subtypesDespite adherence to gluten-free diet, few patients show gradual progression of CAs [14]. In general, such patients have cerebellar atrophy before diagnosis and started on the diet long after the onset of CAs. However, such cases are rare and by far the commonest cause ofSome patients with gluten-related disorders present with myoclonus ataxia and refractory coeliac disease [18]. The myoclonus is cortical in origin and sometimes widespread. This entity is rare and 9 patients were reported among more than 600 patients with neurological manifestations of gluten-related disorders [18]. In spite of strict gluten-free diet, five of these 9 patients presented with persistent symptoms of malabsorption and evidence of on-going enteropathy on biopsies, and two patients developed enteropathy-associated lym.

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