Background: No method for the clinical diagnosis of MM2-type sporadic Creutzfeldt-Jakob disease (sCJD) has been established except for pathologic examination. Objective: To identify a reliable marker for the clinical diagnosis of MM2-type sCJD. Methods: CSF, EEG, and neuroimaging studies were performed in eight patients with MM2-type sCJD confirmed by neuropathologic, genetic, and western blot analyses. Results: The eight cases were pathologically classified into the cortical (n = 2), thalamic (n = 5), and combined (corticothalamic) (n = 1) forms. The cortical form was characterized by late-onset, slowly progressive dementia, cortical hyperintensity signals on diffusion-weighted imaging (DWI) of brain, and elevated levels of CSF 14-3-3 protein. The thalamic form showed various neurologic manifestations including dementia, ataxia, and pyramidal and extrapyramidal signs with onset at various ages and relatively long disease duration. Characteristic EEG and MRI abnormalities were almost absent. However, all four patients examined with cerebral blood flow (CBF) study using SPECT showed reduction of the CBF in the thalamus as well as the cerebral cortex. The combined form had features of both the cortical and the thalamic forms, showing cortical hyperintensity signals on DWI and hypometabolism of the thalamus on [¹⁸F]2-fluoro-2-deoxy-d-glucose PET. Conclusion: For the clinical diagnosis of MM2-type sporadic Creutzfeldt-Jakob disease, cortical hyperintensity signals on diffusion-weighted MRI are useful for the cortical form and thalamic hypoperfusion or hypometabolism on cerebral blood flow SPECT or [¹⁸F]2-fluoro-2-deoxy-d-glucose PET for the thalamic form.

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To investigate the improvement of prognostication of active bone metastatic burden by discriminating bone metastases from degenerative changes in hot foci, using skeletal standardized uptake values (SUVs) by quantitative bone single photon emission tomography/computed tomography (SPECT/CT) in patients with prostate cancer. We investigated 170 patients with prostate cancer who underwent skeletal quantitative SPECT/CT using 99mTc-methylene-diphosphonate (MDP), through conjugate gradient reconstruction with tissue zoning, attenuation, and scatter corrections applied, called as CGZAS reconstruction, in a retrospective cohort study. The maximum, peak, and average SUVs (SUVmax, SUVpeak, and SUVave, respectively) were obtained for visually normal thoracic (T; n = 100) and lumbar (L; n = 140) vertebral bodies as controls, as well as for bone metastases (n = 126) and degenerative changes (n = 114) as hot foci. They were also correlated with age, body-weight, height, biochemistry data, and extent of disease (EOD). Discrimination accuracy of the SUVs for bone metastases in hot foci was evaluated by a patient-based and lesion-based receiver-operator characteristic curve (ROC) analysis. The skeletal SUVmax was 7.58 ± 2.42 for T, 8.12 ± 12.24 for L, 16.73 ± 6.74 for degenerative changes, and 40.90 ± 33.46 for bone metastases. The SUVs of the bone metastasis group were significantly (p < 0.001) greater than of the other three groups. With disease extent, serum alkaline phosphatase and prostate specific antigen were increased, while SUVs for bone metastases were decreased in EOD grade 4. In ROC analyses for bone metastases by skeletal SUVs demonstrating the diagnostic accuracy of skeletal SUVs for discriminating bone metastasis from degenerative changes in hot foci, area under curves were 0.840, 0.817, and 0.845 in patient-based mode, and 0.932, 0.920, and 0.930 in lesion-based mode. The skeletal SUVs by 99mTc-MDP SPECT/CT for active bone metastases were greater than those for degenerative changes in patients with prostate cancer, with a feasible discrimination accuracy in the hot foci. Therefore, skeletal SUVs, especially SUVmax, in quantitative bone SPECT/CT may be helpful indices for the prognostication of bone metastatic burden, improving discrimination of active bone osteoblastic metastases in patients with prostate cancer from frequently coexisting degenerative changes.

In Alzheimer's disease (AD), regional cerebral blood flow (rCBF) in the posterior cingulate gyrus and precuneus has been reported to decrease even at a very early stage. We performed a multicentre SPECT study to evaluate the discrimination ability of an easy Z-score imaging system (eZIS) by detecting an rCBF decrease in this area with a common normal database between very early AD patients at the stage of mild cognitive impairment and age-matched healthy volunteers. Brain perfusion SPECT images of 40 Alzheimer's disease patients and 40 healthy volunteers were acquired from four gamma camera systems in different institutions. Systematic differences of SPECT images between different gamma cameras were corrected using conversion maps calculated from the SPECT images of the same brain phantom. Ten observers with various degrees of expertise graded eZIS results for receiver operating characteristic (ROC) curves. ROC curves for a positive Z-score in the volume of interest (VOI) of the posterior cingulate gyrus and precuneus were also analysed. An area under the ROC curve value (AZ) for ten observers showed the highest value of 0.866 on average with the smallest standard deviation of 0.027 in the condition of the lower threshold of a Z-score map of 2 without superimposition of VOI. Automated analysis of a Z-score in the VOI showed an AZ value of 0.895. Since the degree of expertise of the observers with respect to reading eZIS did not influence the performance and an eZIS can use a common normal database by converting site-specific SPECT data to the core data, the eZIS was considered to be very useful for diagnosing early AD in routine studies in many institutions.