We report of a woman aged 52 years born to consanguineous parents and seeking treatment for progressive dementia and delusion. Neurologic examination revealed dementia and emotional instability, indifference, and confabulation. There was also mild spasticity of the bilateral lower limbs. MRI revealed diffuse white matter hyperintensity on T2-weighted images accompanied by hypointense areas on fluid-attenuated inversion recovery images. A homozygous missense mutation was identified in EIF2B5.

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A primary Burkitt-type lymphoma of the central nervous system (CNS) is presented. The right temporoparietal tumor in a woman of 55 was diagnosed histologically as a Burkitt-type lymphoma. Primary Burkitt-type lymphoma of the CNS is extremely rare and has been reported previously only four times. This is the first case reported in Eastern Asia.