PMC7373778_01
Results of neuropsychological tests. The patient was examined thoroughly twice in 2017. Results are listed as z-scores in relation to normal results. The dashed line indicates the cut off for pathological scores adjusted for age. Trail Making B was also performed, but the patient was not able to finish the test. Furthermore, the patient was not able to complete the visual puzzles test in the follow up examination in 09/17. Tests included: *Wechsler Adult Intelligence Scale - Fourth Edition;  Wechsler Memory Scale - Fourth Edition and #Regensburger verbal fluency test.
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Overall, test results, especially the parietal dysfunction, pointed to an early neurodegenerative process consistent with a possible early-onset Alzheimer's disease (EOAD) or Lewy Body dementia (Figure 1).
Imaging biomarkers. (A-C) 18F-FDG-PET results. Three-dimensional surface projections (A, B) show distinct bilateral temporo-parietal and occipital hypometabolism. (A) Right hemisphere; lateral view. (B) Left hemisphere; lateral view. (C) Hypometabolism is relatively spared in the posterior cingulate gyrus compared to occipital areas (cingulate island sign); transverse view. (D) 18F-Florbetaben-PET did not show increased amyloid burden. (E, F) Dopamine transporter imaging with 123I-FP-CIT SPECT in transverse slices. While the initial scan was negative (E), follow up showed lower uptake within the right putamen (F).
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While MRI did not show any pathologies, 18F-FDG-PET showed bilateral hypometabolism of occipital, parietal and temporal lobe, as well as frontal lobes to a lesser extend with relative sparing of the posterior cingulate gyrus (cingulate island sign; Figures 2A-C). Amyloid PET with 18F-Florbetaben was negative (Figure 2D). However, it did not show a nigrostriatal deficit (binding ratios: right caudate nucleus 2.37; left caudate nucleus 2.39; right putamen 2.44; left putamen 2.39; Figure 2E). Due to the new core and supportive clinical features, 123I-FP-CIT SPECT was repeated. 123I-FP-CIT SPECT showed lower uptake mainly within the right putamen with lower binding ratios in both putamina and left caudate nucleus compared to 2017 (binding ratios: right caudate nucleus 2.42; left caudate nucleus 1.94; right putamen 1.83; left putamen 1.91; Figure 2F).
Timeline of symptoms, clinical findings, imaging biomarker, and treatment. Criteria for the clinical diagnosis of Dementia with Lewy bodies are indicated in red.
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Figure 3 summarizes symptoms, clinical findings, imaging results, and treatment.