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Clinical Pathologic Conference |

A Young Man With Progressive Language Difficulty and Early-Onset Dementia

Hugo Botha, MB, ChB1; Bradley F. Boeve, MD1; Lyell K. Jones, MD1; Joseph E. Parisi, MD1,2; James P. Klaas, MD1
[+] Author Affiliations
1Department of Neurology, Mayo Clinic, Rochester, Minnesota
2Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota
JAMA Neurol. 2016;73(5):595-599. doi:10.1001/jamaneurol.2016.0246.
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A man in his late 40s presented with cognitive decline characterized by gradually increasing difficulty expressing his thoughts and ideas. His family noted word-finding difficulty, especially with the names of people, but no problems with his memory for recent events. Initial workup findings were unremarkable, but during the course of the next decade left anterior temporal atrophy was noted on magnetic resonance imaging and the patient developed increasing reasoning difficulty, apathy, and disinhibition. Several degenerative causes were considered. The patient died 22 years after symptom onset, and the final diagnosis was confirmed at autopsy.

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Figure 1.
Magnetic Resonance Images

A and B, T2-weighted fluid-attenuated inversion recovery (FLAIR) imaging (A) and T1-weighted noncontrast sequences (B) 6 years after symptom onset show marked left anterior temporal atrophy. C and D, T2-weighted FLAIR imaging (C) and T1-weighted noncontrast sequences (D) 10 years after symptom onset show further left temporal atrophy, with knife-edge gyral atrophy.

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Figure 2.
Pathological Findings

A, Left lateral view of the brain showing marked circumscribed atrophy of the frontal and anterior temporal lobes with less severe involvement of the parietal lobe (arrowheads). B, Coronal sections demonstrate marked thinning of the frontal and anterior temporal neocortex with flattening of the caudate head (left image, arrowhead). The amygdala and entorhinal cortex are severely atrophic (middle image, arrowhead), while the hippocampus is relatively preserved (right image). C, The anterior superior temporal cortex shows severe pancortical neuronal loss and gliosis or status spongiosis (hematoxylin-eosin, original magnification ×100). D, Surviving superior temporal neurons contain distinctive, well-demarcated, slightly basophilic, rounded inclusions (arrowheads) characteristic of Pick bodies (hematoxylin-eosin, original magnification ×600). E-H, Pick bodies are consistently present in the dentate fascia of the hippocampus, showing intense staining with silver (arrowheads) (Bielschowsky silver stain) (E), striking immunoreactivity to tau (arrowheads) (AT8 antibody) (F), and selective tau staining with immunoreactivity to 3-repeat tau (arrowheads) (G) but not to 4-repeat tau (H) (original magnification ×600).

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