Ramsay hunt syndrome and coeliac disease: A new association?

Authors

  • Dr. Chin-Song Lu,

    1. University Department of Neurology, Institute of Psychiatry, King's College Hospital Medical School, London, England
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    • Department of Neurology, Chang Gung Memorial Hospital, 199 Tung Hwa North Rd, Taipei, Taiwan, R.O.C.

  • Philip D. Thompson,

    1. University Department of Neurology, Institute of Psychiatry, King's College Hospital Medical School, London, England
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  • Niall P. Quinn,

    1. University Department of Neurology, Institute of Psychiatry, King's College Hospital Medical School, London, England
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  • J. D. Parkes,

    1. University Department of Neurology, Institute of Psychiatry, King's College Hospital Medical School, London, England
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  • Professor C. David Marsden

    Corresponding author
    1. University Department of Neurology, Institute of Psychiatry, King's College Hospital Medical School, London, England
    • Department of Neurology, Institute of Psychiatry, DeCrespigny Park, London, SE5 8AF, U.K.
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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 glutenfree 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.

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