PMC5662557_01
Diagnostic results. The cerebral magnetic resonance imaging demonstrated juxtacortical white matter lesions; the electroencephalography showed intermittent slowing.
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F1
The cMRI showed multiple lesions in deep and peripheral white matter whithout diffusion restriction or contrast enhancement, while intermittent slow activity was detected in the EEG (Figure 1). [18F]fluorodeoxyglucose positron emission tomography (FDG-PET) showed mild-to-moderate medial and superior dorsolateral frontal hypometabolism, which did not allow a clear distinction between early-stage frontotemporal lobar degeneration and secondary non-specific changes (e.g., due to reduced consciousness/apathy, atrophy).
The course of the patient's memory dysfunction using the CERAD (z-values). TMT A/B, trailmaking tests A/B; t1, before plasmapharesis; t2, 1 month after plasmapheresis; t3, nearly 6 months after plasmapheresis.
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Neuropsychological test batteries for dementia, following the Consortium to Establish a Registry for Alzheimer's Disease (CERAD), showed deficits in verbal fluency, word list memory, constructional praxis, and trailmaking A/B (Figure 2). The improvement was neuropsychologically documented (Figure 2). One month after plasmapheresis, the patient improved in almost all the initially impaired categories of the CERAD testing battery (Figure 2); however, the BADS only improved slightly and remained significantly below average (sum score only increased from 6 to 8). Nearly 6 months after undergoing plasmapheresis, the CERAD (Figure 2) and BADS (sum score of 8 points) findings were still stable.
FDG-PET showing mild-to-moderate medial and superior dorsolateral frontal hypometabolism before and normalization after plasmapheresis. The upper and lower row images show the transaxial fluorodeoxyglucose positron emission tomography (FDG-PET) images and the 3D surface projections of the regions with decreased FDG uptake (color-coded Z-score and compared with age-matched healthy controls, respectively).
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The follow-up FDG-PET showed normalization in July 2017 (i.e., the frontal hypometabolism was no longer detectable; Figure 3).