論文

We report a patient with logopenic variant primary progressive aphasia (lv-PPA) who was diagnosed as having non-Alzheimer's disease (AD) tauopathy after multiple biophysical/biological examinations, including amyloid and 18F-florzolotau tau positron emission tomography (PET), had been performed. A woman in her late 60s who had previously been diagnosed as having AD was referred to us for a further, detailed examination. She had been unaware of any symptoms at the time of AD diagnosis, but she subsequently became gradually aware of a speech impairment. She talked nearly completely and fluently, although she occasionally exhibited word-finding difficulty and made phonological errors during naming, word fluency testing, and sentence repetition; these findings met the criteria for the diagnosis of lv-PPA, which is known to be observed more commonly in AD than in other proteinopathies. Magnetic resonance imaging, single photon emission computed tomography, and plasma phosphorylated tau and plasma neurofilament light chain measurements showed an AD-like pattern. However, both 11C-Pittsburgh compound-B and 18F-florbetaben amyloid PET showed negative results, whereas 18F-florzolotau tau PET yielded positive results, with radio signals predominantly in the left superior temporal gyrus, middle temporal gyrus, supramarginal gyrus, and frontal operculum. Whole-genome sequencing revealed no known dominantly inherited mutations in AD or frontotemporal lobar degeneration genes, including the genes encoding amyloid precursor protein, microtubule-associated protein tau, presenilin 1 and 2. To the best of our knowledge, this patient was a rare case of lv-PPA who was diagnosed as having non-AD tauopathy based on the results of multiple examinations, including whole-genome sequencing, plasma measurement, and amyloid and 18F-florzolotau tau PET. This case underscores the clinicopathologically heterogeneous nature of this syndrome.

Background: In early-stage amnestic mild cognitive impairment (aMCI), differences in the neuropsychological characteristics of each individual are subtle. We investigated differences in neuropsychological performance between aMCI patients with and without hypoperfusion in the medial parietal regions (MP). We further compared patients with hypoperfusion in the left and right lateral parietal regions. Methods: We examined 165 aMCI patients (mean age: 76.8 ± 5.5 years; 87 women) who had undergone neuropsychological measurement and single-photon emission computed tomography. We classified participants into two subgroups with and without hypoperfusion: MP hypoperfusion (+) and MP hypoperfusion (−); classification was based on Z-scores (calculated by three-dimensional stereotactic surface projection technique) of three regions of interest in the parietal lobes (i.e. MP regions including posterior cingulate cortex and precuneus and left and right inferior parietal lobules (lateral parietal regions)). The MP hypoperfusion (−) group was classified into left lateral parietal hypoperfusion (+) and right lateral parietal hypoperfusion (+) subgroups. We performed either univariate or multivariate ancova to compare neuropsychological scores for continuous variables between groups and examined dichotomous variables using χ2 tests. Results: In the overall aMCI sample, scores on logical memory delayed recall in the MP hypoperfusion (+) group were significantly lower than those in the MP hypoperfusion (−) group. Total scores on Rey-Osterrieth Complex Figure Test delayed recall were also marginally lower in the MP hypoperfusion (+) group than in the MP hypoperfusion (−) group. Comparisons of neuropsychological test scores between the left and right lateral parietal hypoperfusion (+) groups revealed no significant differences. Conclusions: The present findings suggest that MP hypoperfusion (+) is associated with more robust memory deficits than MP hypoperfusion (−). Combining neuropsychological tests and single-photon emission computed tomography findings may be useful for early detection of cognitive decline in aMCI.

© 2018 The Authors. Psychiatry and Clinical Neurosciences © 2018 Japanese Society of Psychiatry and Neurology Aim: While emotional processing is implicated in various psychiatric illnesses, its differences among diagnoses are unclear. We compared associative learning of social values in patients with depression and schizophrenia by measuring skin conductance response to interpersonal stimuli. Methods: We included 20 female outpatients each with depression and schizophrenia. They underwent Pavlovian conditioning experiments in response to a classical aversive sound, and an interpersonal stimulus that was designed to cause aversive social conditioning with actors’ faces coupled with negative verbal messages. Multiple regression analysis was performed to examine the associations between the degree of conditioned response and the clinical characteristics of the participants. Results: Conditioned responses during the acquisition phase in both conditions were higher in depression compared to schizophrenia. Patients with depression successfully showed fear conditioning in both conditions, and they exhibited slower extinction in the interpersonal condition. The conditioned response during the extinction phase showed a positive association with Emotion Regulation Questionnaire Expressive Suppression score, and a negative association with the Emotion Regulation Questionnaire Cognitive Reappraisal score and the use of antidepressants. Patients with schizophrenia did not become conditioned in either of the conditions. The Positive and Negative Syndrome Scale Negative Syndrome score was negatively associated with the degree of conditioned response during the acquisition phase in the interpersonal condition. Conclusion: Female patients with schizophrenia, especially those who prominently demonstrated negative symptoms, suggested their intrinsic impairments in the associative learning of social context. Antidepressants and adaptive emotional regulation strategy may enhance the extinction learning of aversive social conditioning in depression.