Globular Glial Tauopathy Presenting as Semantic Variant Primary Progressive Aphasia.

Report of aCase |Theclinical, neuropsychometric, and imaging featuresof this casepreviouslywere reported in2008.2Briefly, awoman in her 60swas referred to the Behavioral Neurology Clinic formemory loss, characterizedbydifficulty rememberingnames.Longitudinal evaluations revealedprogressiveanomia with loss of word knowledge, prosopagnosia, and surface dyslexia. Her last completed neuropsychometric evaluationoccurred at age 71 years (Figure 1). In her early 70s, her husband presented evidence of impaired object knowledge; for example, she frequentlywould use the incorrect silverware (eg, forkwith soup) andwasnoted tohaveused toothpaste instead of hand lotion. At age73years,disinhibitionbecamemoreprominent.Her judgmentcontinuedtodeteriorateasshewould inappropriately pickuphotobjectswithherhands. Inhermid-70s, shereturned for follow-upand itwasnoted thatdespiteher continueddeterioration inmostcognitiveaspects, shehadexpandedherpainting artistry. Later in her mid-70s, she was placed in a nursing home following a right hemispheric infarct. In the last few monthsoflife,shedevelopedsignificantecholalia.Threemonths afterhavingastroke, shedied inhermid-70s.Neuropathologic evaluationrevealedthepathologicsubstratetobeaGGTandnot TDP-43.Gross findings includedsevere frontotemporalatrophy (temporal > frontal),with lefthemibrainweightof 515g.Histopathologic features are illustrated in Figure 2.