Ramsay hunt syndrome and coeliac disease: A new association?

Abstract

Two patients with the syndrome of Ramsay Hunt (dyssynergia cerebellaris myoclonica, DCM), associated with malabsorption due to adult coeliac disease, are reported. Both presented with progressive cerebellar ataxia, action myoclonus, and epilepsy. One had gastrointestinal symptoms (recurrent diarrhea and weight loss which responded satisfactorily to a gluten-free diet), but the other did not. In both patients, jejunal biopsy revealed subtotal villous atrophy; serum folate and vitamin E level were also reduced. Neither a gluten-free diet nor vitamin supplements improved the neurological picture. However, some symptomatic relief was afforded by treatment with clonazepam, sodium valproate, carbamazepine, and piracetam. It could be argued that the association between these two disorders is coincidental. However, since we have found this combination in 2 of 14 consecutive cases with DCM, a causal relationship seems likely, although the underlying mechanism remains unknown. Patients with the Ramsay Hunt syndrome should be investigated for malabsorption, and also undergo small intestinal biopsy.

Idiopathic cerebellar ataxia associated with celiac disease: lack of distinctive neurological features

“…2 Neurological complications occur in about 8%-10% of patients with the disease3 4 including peripheral neuropathy,5 6 progressive multifocal leucoencephalopathy,7 cerebellar ataxia,4 8 9 progressive myoclonic ataxia,10 11 dementia,12 and myopathy 13. These disorders have been generally described as associated with the classic celiac disease featuring weight loss and diarrhoea 4…”

Lafora Body-Like Inclusions in a Case of Progressive Myoclonic Ataxia Associated with Coeliac Disease

“…Detection of Lafora bodies in apocrine or eccrine sweat glands of the skin has been considered as a reliable test for diagnosis of Lafora bodies [2,6]. Lafora bodies are PAS-positive inclusions observed in the secretory acini of the apocrine glands and/or in the cells of the eccrine duct.…”

The Neuroimmunology of Gluten Intolerance

“…In 1986 Lu and colleagues published two cases with action myoclonus, ataxia and CD who in addition had epilepsy (Lu et al, 1986). The authors provided electrophysiological evidence for the cortical origin of the myoclonus.…”

“…Single and multiple case reports of patients with established CD who then developed neurological dysfunction continued to be published (Binder et al, 1967;Bundley, 1967;Morris et al, 1970;Coers et al, 1971;Kepes et al, 1975;Coers et al, 1971;Kepes et al, 1975;Finelli et al, 1980;Kinney et al, 1982;Ward and Murphy 1985;Lu et al, 1986;Kristoferitsch and Pointer, 1987;Kaplan et al, 1988;Tison et al, 1989;Hermaszewski et al, 1991;Colin et al, 1991;Dick et al, 1995;Bhatia et al, 1995;Muller et al, 1996) Key findings from these reports were that ataxia and/or neuropathy were the commonest manifestations always seen in the context of established CD and almost always attributed to nutritional deficiencies. Some reports reported improvement of the neurological problems with adherence to a GFD whilst others did not.…”