LAUSR

Adult‐onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) is a rare autosomal dominant neurodegenerative disease resulting from heterozygous mutations in the colony‐stimulating factor 1 receptor gene.[1] ALSP is a challenging diagnosis in the absence of pathological or genetic confirmation because of its varied clinical presentation and imaging features. We report a case of ALSP in a 54‐year‐old lady presenting with predominant language disturbance in the form of logopenic variant of primary progressive aphasia (LvPPA) with no similar family history. Magnetic resonance imaging (MRI) brain showed frontoparietal predominant periventricular and corpus callosum signal changes with diffusion restrictions suggestive of leukodystrophy where eventually genetic testing aided in the final diagnosis.