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We report a 46-year-old patient with Lhermitte-Duclos disease (LDD) who underwent a successful surgery but died of other causes four years later. The autopsy revealed Lhermitte-Duclos disease asymmetrically affecting both cerebellar hemispheres. The subcortical white matter of both cerebral hemispheres contained several foci of grey matter heterotopia. Only heterotopic neurons contained tau-positive neurofibrillary tangles (NFT), displaying characteristic ultrastructural features of paireddoi:10.5114/fn.2016.60434 fatcat:37itvhgzjfextpzargvbmaa5de