Sporadic Creutzfeldt-Jakob disease presenting as progressive nonfluent aphasia with speech apraxia.
Progressive non-fluent aphasia (PNFA) is typically associated with pathological changes consistent with frontotemporal lobar degeneration. A 65-year-old male presented with effortful speech, markedly impaired naming and features of speech apraxia, consistent with PNFA. Perceptuospatial function, calculation and executive function were intact. Brain SPECT showed left perisylvian hypoperfusion. He deteriorated profoundly over the subsequent eight months, with appearances on diffusion-weighted magnetic resonance imaging typical of sporadic Creutzfeldt-Jakob disease, which was confirmed pathologically at postmortem examination. While the presence of PNFA with speech apraxia is thought to predict underlying tauopathy, sporadic Creutzfeldt-Jakob disease may mimic this presentation and present in a highly circumscribed form not previously described.
*Cerebral Function Unit §Department of Neuroradiology, Greater Manchester Neurosciences Centre, Salford †Mental Health and Neurodegeneration Research Group, School of Community-Based Medicine ‡School of Cancer and Enabling Sciences, University of Manchester, Manchester ∥Department of Neurology, Royal Preston Hospital, Preston ¶National CJD Research and Surveillance Unit, Western General Hospital, Edinburgh, UK.
Christopher
C
Kobylecki
Jennifer C
JC
Thompson
Matthew
M
Jones
Samantha J
SJ
Mills
Sandip
S
Shaunak
James W
JW
Ironside
Julie S
JS
Snowden
Anna M
AM
Richardson
D000368:Aged; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis* / Q000523:psychology*; D003937:Diagnosis, Differential; D006801:Humans; D008297:Male; D057178:Primary Progressive Nonfluent Aphasia / Q000175:diagnosis* / Q000523:psychology*