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Case Reports
. 2015 Mar 19;372(12):1151-62.
doi: 10.1056/NEJMcpc1409839.

Case records of the Massachusetts General Hospital. Case 9-2015. A 31-year-old man with personality changes and progressive neurologic decline

Bruce L Miller, Bradford C Dickerson, Diane E Lucente, Mykol Larvie, Matthew P Frosch
Case Reports

Case records of the Massachusetts General Hospital. Case 9-2015. A 31-year-old man with personality changes and progressive neurologic decline

Bruce L Miller et al. N Engl J Med. .
No abstract available

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Figures

Figure 1
Figure 1. Neuroimaging Studies
Neuroimaging studies were obtained 3 years before this evaluation. An axial T2-weighted image of the brain shows diffuse loss of parenchymal volume (Panel A). A coronal T2-weighted fluid-attenuated inversion recovery image shows severe focal atrophy in the left temporal lobe, with a small area of hyperintensity in the underlying white matter (Panel B, arrow). A sagittal T1-weighted image shows mild atrophy in the frontal lobes bilaterally (Panel C). Further neuroimaging studies were obtained during this evaluation. An axial T2-weighted image (Panel D), a coronal T1-weighted image (Panel E), and a sagittal T1-weighted image (Panel F) show marked progression of atrophy, which is now severe and has a knifelike appearance in the temporal and frontal lobes and insular cortexes. There is relative but not absolute sparing of the parietal lobes (Panels C and F, arrowheads).
Figure 2
Figure 2. Gross Neuropathological Photographs at Autopsy
A medial aspect of the brain after fixation (Panel A) reveals moderate atrophy of the frontal and temporal lobes. A coronal section anterior to the head of the caudate (Panel B) reveals thinning of the cerebral cortex and ventricular enlargement. A coronal section obtained at the midbody of the amygdala (Panel C) reveals thinning of the cerebral cortex at the frontal and temporal lobes, with sparing of the superior temporal gyrus (arrow).
Figure 3
Figure 3. Microscopic Neuropathological Images at Autopsy
A combination of Luxol fast blue and hematoxylin and eosin staining of the frontal lobe shows attenuation of the neuropil, neuronal loss, and reactive gliosis (Panel A). A Pick body is evident in a cortical neuron (Panel B, arrow). Impregnation with the use of a modified Bielschowsky silver method shows a Pick body (Panel C, arrow). Immunohistochemical staining with phosphospecific antibodies against tau was performed; staining for AT8 (specific for phosphorylation at S199, S202, and T205) (Panel D) and staining for PHF1 (specific for phosphorylation at S396 and S404) (Panel E) show tau-containing inclusions. A combination of Luxol fast blue and hematoxylin and eosin staining shows neuronal loss and extracellular neuromelanin in the substantia nigra pars compacta (Panel F). Immunohistochemical staining for PHF1 shows cytoplasmic inclusions and neuropil threads in the substantia nigra that contain phosphorylated tau (Panel G).
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