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Vacuolar leucoencephalopathy and pulvinar sign in association with coeliac disease
  1. A M Ryan1,
  2. J Ryan1,
  3. M Wan-Ahmed1,
  4. O Hardiman2,
  5. M A Farrell3,
  6. B McNamara4,
  7. B J Sweeney5
  1. 1Department of Neurology, Cork University Hospital, Cork, Ireland
  2. 2Department of Neurology, National Institute for Neurology and Neurosurgery, Beaumont Hospital, Dublin, Ireland
  3. 3Department of Neuropathology, National Institute for Neurology and Neurosurgery, Beaumont Hospital, Dublin, Ireland
  4. 4Department of Neurophysiology, Cork University Hospital, Cork, Ireland
  5. 5Department of Neurology, Cork University Hospital, Cork, Ireland
  1. Correspondence to:
 B J Sweeney
 Department of Neurology, Cork University Hospital, Cork, Ireland; bsweeney{at}

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Several neurological disorders have been described in association with coeliac disease, including epilepsy, myoclonus, ataxia, myelopathy and peripheral neuropathy. Disorders of white matter have been reported but are rare.1 We report the clinical, radiological and pathological findings of a man with coeliac disease, progressive neurological decline and spongiform white matter degeneration.


A 55-year-old man presented with anxiety, headache and left upper limb jerking. Coeliac disease had been diagnosed 6 months previously after small bowel biopsy showing positive tissue transglutaminase antibodies. There was no medical history. The patient was not taking any drugs and there was no history of substance misuse. His daughter also has coeliac disease but is neurologically normal. Magnetic resonance imaging (MRI) of the brain and electroencephalogram (EEG) were normal and the patient was started on oral anticonvulsants. He re-presented 2 months later, with increasing anxiety, tremulousness, ataxia, confusion and focal onset seizures becoming secondarily generalised. On examination, he was agitated and was tremulous at rest. He looked about vaguely but was capable of following a 1-step command. Extraocular movements were full, with no nystagmus. Reflexes and plantar responses were normal. He had ataxia and dysmetria on finger-nose-finger testing and a broad-based gait. Sensation was intact. He deteriorated and developed tonic–clonic seizures, requiring intensive care unit admission. Repeat MRI of the brain showed diffuse abnormality of the white matter. Brain biopsy …

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  • Competing interests: None.

    Informed consent was obtained for publication of the patient’s details described in this report.