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At age 58 a woman was investigated for symptomatic anaemia and found to be iron and folate deficient. She had no abdominal symptoms. Findings on upper gastrointestinal endoscopy were normal but distal duodenal biopsies showed subtotal villous atrophy, inflammatory infiltration of the lamina propria and an increase in intraepithelial lymphocytes. Coeliac disease was diagnosed and she was started on a gluten-free diet. She gained weight and ceased to be anaemic; however, repeat biopsies of her duodenal mucosa showed no improvement in the villous atrophy. Compliance with a gluten-free diet was confirmed by dietitians' assessments. She subsequently became hypothyroid with a 1 in 25 600 antithyroid antibody titre, and was treated with thyroxine.
11 years later she complained of diarrhoea, ankle oedema and 7 kg weight loss. Endoscopic duodenal biopsies again showed features consistent with untreated coeliac disease, and again a dietitian confirmed adherence to a strict gluten-free diet. Her haemoglobin was 12.6 g/dL, mean corpuscular volume 82 fL, albumin 33 g/L (normal 35-50), antiendomysial antibody positive, vitamin B12 normal and red cell folate low at 145 ng/mL (normal >185). A barium follow-through showed a rather featureless jejunal mucosa but no obstructive lesion of the small bowel; nothing abnormal was seen on an ultrasound scan of the abdomen. In view of her worsening symptoms despite a gluten-free diet, she was started on prednisolone, initially 30 mg daily, and over the next year her clinical condition improved, with resolution of her diarrhoea and a weight gain of 5 kg. At the time of discontinuation of prednisolone a year later she developed involuntary writhing movements of her left limbs. On examination she had choreo-athetoid movements involving the left limbs and persistent dysarthria. There was no associated weakness and sensation was normal. At that time she was taking digoxin, aspirin, ferrous sulphate and thyroxine; she had never received any neuroleptic medication. Magnetic resonance scanning of the brain and brainstem was normal, with no evidence of an infarct and no lesion in the basal ganglia. Genetic testing for Huntington's disease—a search for the hallmark abnormal CAG repeats on chromosome 4—was negative. No underlying neurological disease to explain her chorea was found.
Her chorea continued and seven months later she developed acute abdominal pain. At emergency laparotomy she was found to have a perforated jejunal tumour, which was resected. It proved to be a non-Hodgkin's T-cell lymphoma. She subsequently had intravenous chemotherapy with doxorubicin, cyclophosphamide and vincristine, but deteriorated three months postoperatively and died.
Apart from the lack of response to a gluten-free diet this patient had typical coeliac disease, and subsequently developed the recognized complication of T-cell lymphoma of the jejunum. There is an argument, however, that patients who do not respond histologically to a gluten-free diet do not strictly have coeliac disease. Enteropathy-associated T-cell lymphoma is most likely to develop in coeliac patients who either do not adhere to3 or do not respond to4,5 a gluten-free diet. The prognosis for this tumour is poor.
Various different neurological abnormalities have been recognized in coeliac disease1,2. The most common are ataxia, neuropathy and epilepsy. We have found no previous report of chorea. Our patient had no associated neurological disease, no history of taking drugs with extrapyramidal effects and no genetic markers for Huntington's chorea. Although her chorea may have been a neurological association of her refractory coeliac disease, an alternative explanation is that it was a paraneoplastic complication of her impending T-cell lymphoma. Chorea has been described as a paraneoplastic phenomenon in patients with non-Hodgkin's lymphoma6, but to our knowledge this is the first case where it has been associated with lymphoma arising from a background of coeliac disease. The finding of chorea in association with coeliac disease should prompt a search for possible underlying intestinal T-cell lymphoma.