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We investigated clinical and neuropathologic heterogeneity and apolipoprotein E(apoE)genotype in 11 cases of neuropathologically diagnosed corticobasal degeneration(CBD).Although seven of the 11 patients presented with unilateral limb dysfunction,the remaining four patients had less typical presentation including memory loss,behavioral changes and difficulties with speech or gait.All 11 patients eventually developed extrapyramidal signs as well as cortical features most commonly apraxia.At autopsy,the brains of seven of the 11 patients exhibited predominant neuronal loss and gliosis of perirolandic cortex;degeneration of more rostral frontal cortex was observed in three of the four patients with atypical clinical presentations.All displayed ballooned neurons,tau- positive neuronal and glial inclusions,treads,and grains,and nigral degeneration.Six of the 11 cases manifested overlapping neuropathological features of one or more disorders,including Alzheimers disease AD, progressive supranuclear palsy(PSP),Parkinson's disease PD,and hippocampal sclerosis.Interestingly,these six patients all exhibited memory loss early in the course of their illness.The 11 CBD cases exhibited increased frequency(0.32)of the e4 allele of apoE,relative to control populations; the frequency remained elevated(0.25)even when the three cases with concomitant AD were excluded.Betaobservations indicate the CBD is pathologically and clinically heterogenous disorder with substantial overlap with other neurodegenerative disorders. |
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