Findings that shed new light on the possible pathogenesis of a disease or an adverse effect
Vacuolar leucoencephalopathy and pulvinar sign in association with coeliac disease
1 Department of Neurology, Cork University Hospital, Cork, Ireland
2 Department of Neurology, National Institute for Neurology and Neurosurgery, Beaumont Hospital, Dublin, Ireland
3 Department of Neuropathology, National Institute for Neurology and Neurosurgery, Beaumont Hospital, Dublin, Ireland
4 Department of Neurophysiology, Cork University Hospital, Cork, Ireland
Correspondence to:
bsweeney{at}eircom.net
Several neurological disorders have been associated with coeliac disease, including epilepsy, ataxia and neuropathy. Here we report a rare case of white matter disease in a 55-year-old man with coeliac disease. He presented with anxiety, headache and left upper limb jerking. He subsequently developed epilepsy and brain MRI revealed diffuse white matter abnormality. He died 6 months after presentation due to status epilepticus and sepsis. Brain biopsy demonstrated vacuolar leucoencephalopathy with no evidence of vCJD. An extensive clinical screen excluded infectious, inflammatory and para-neoplastic causes for this condition. Coeliac disease may be causally associated with vacuolar leucoencephalopathy in this case.
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