Post hoc analysis of the characteristics and treatment needs of patients with dementia with Lewy bodies (DLB) and their caregivers and their physicians' awareness of those treatment needs according to the duration after diagnosis of DLB.

OBJECTIVES: To investigate the differences in patient/caregiver characteristics, their treatment needs, and the attending physician's understanding of those treatment needs according to the duration after diagnosis of dementia with Lewy bodies (DLB). METHODS: This was a post hoc analysis of a multicenter, cross-sectional, questionnaire survey study. A total of 263 patient-caregiver pairs were reclassified into two groups according to the median duration after diagnosis of DLB as follows: short (<24 months; S-group) and long (≥24 months; L-group) post-DLB diagnosis duration. Treatment need was defined as the symptom domain that caused the patient or caregiver the most distress. Concordance rates between patient-physician and caregiver-physician were calculated for physicians' understanding of treatment needs. RESULTS: In this analysis, 126 pairs (32 physicians) and 137 pairs (34 physicians) were classified as the S- and L-groups, respectively. Patient and caregiver characteristics were broadly similar between groups (mean age for patients 78.7 ± 6.6 vs. 79.8 ± 6.7, for caregivers 64.7 ± 12.9 vs. 64.9 ± 12.8; number of male/female for patients 61/65 vs. 67/70, for caregivers 34/92 vs. 38/99), but the prevalence of parkinsonism (82.5% vs. 66.7%) and autonomic dysfunction (49.6% vs. 33.3%), severity of parkinsonism (MDS-UPDRS Part III total scores, 29.2 ± 22.6 vs. 18.0 ± 16.4; Part II total score, 14.6 ± 12.0 vs. 7.6 ± 7.9), and caregiver burden (J-ZBI_8 score, 9.1 ± 6.7 vs. 7.5 ± 5.8) were higher in the L-group than the S-group. Regarding treatment needs, the invalid answer rates for patients were 34.9% and 46.8%, and those for caregivers were 28.6% and 34.9% in the S- and L groups, respectively. Patients' treatment needs did not significantly differ (p = 0.056), but S-group patients were more likely to select cognitive impairment (p = 0.045) as their treatment need, whereas L-group patients were more likely to select parkinsonism (p = 0.003). Caregivers' treatment needs significantly differed (p = 0.032) between groups. S-group caregivers were more likely to select cognitive impairment (p = 0.001), whereas L-group caregivers were more likely to select other symptom domains such as parkinsonism (S-group vs. L-group: 10.3% vs. 16.7%), psychiatric symptoms (20.6% vs. 24.6%), sleep-related disorder (4.0% vs. 7.1%), and autonomic dysfunction (4.8% vs. 9.5%). Concordance rates between patient-physician and caregiver-physician were low in both groups. CONCLUSIONS: There were some differences in characteristics according to the duration after diagnosis of DLB. Cognitive dysfunction may be a particular concern for patients and caregivers soon after diagnosis of DLB. Treatment needs of patients and caregivers for parkinsonism, psychiatric symptoms, sleep-related disorder, or autonomic dysfunction were different according to the duration after diagnosis of DLB. Physicians' perception of patients'/caregivers' treatment needs was poor regardless of the duration after diagnosis of DLB. CLINICAL TRIAL REGISTRATION: UMIN Clinical Trials Registry (UMIN000041844).

Factors Associated with Quality of Life in Patients with Dementia with Lewy Bodies: Additional Analysis of a Cross-Sectional Study.

Background: Quality of life (QOL) and treatment needs of patients with dementia with Lewy bodies (DLB) and their caregivers are important factors to consider when developing treatment strategies. Objective: To investigate factors associated with QOL in patients with DLB, and to examine factors associated with activities of daily living (ADL) if ADL was associated with QOL. Methods: We previously conducted a questionnaire survey study to investigate the treatment needs of patients with DLB and their caregivers. This pre-specified additional analysis evaluated the Physical Component Score (PCS) and Mental Component Score (MCS) of the Short Form-8 for QOL, and the Movement Disorder Society-Unified Parkinson's Disease Rating Scale (MDS-UPDRS) Part II total score for ADL. Results: In total, 231 patient- caregiver pairs and 38 physicians were included. Multivariable analysis of QOL showed that the MDS-UPDRS Part II total score (standard regression coefficient [ß], - 0.432) was associated with the PCS, and presence of depression (ß, - 0.330) was associated with the MCS. The severity of postural instability/gait disorder (PIGD) (ß, 0.337) and rigidity (ß, 0.266), presence of hallucinations (ß, 0.165), male sex (ß, 0.157), and use of "short stay" or "small-scale, multifunctional home care" (ß, 0.156) were associated with worsened ADL. Conclusions: In patients with DLB, QOL was negatively impacted by severity of ADL disability and depression, and ADL was negatively impacted by severity of PIGD and rigidity, hallucinations, male sex, and use of "short stay" or "small-scale, multifunctional home care."

Differences in the treatment needs of patients with dementia with Lewy bodies and their caregivers and differences in their physicians' awareness of those treatment needs according to the clinical department visited by the patients: a subanalysis of an observational survey study.

BACKGROUND: We investigated whether the treatment needs of patients with dementia with Lewy bodies (DLB) and their caregivers, along with their attending physicians' perception of those treatment needs, differ according to the clinical department visited by the patients. METHODS: This was a subanalysis of a multicenter, cross-sectional, observational survey study. Data from the main study were classified according to the clinical department visited by the patient: psychiatric group (P-group), geriatric internal medicine group (G-group), and neurology group (N-group). The treatment needs of patients and caregivers were defined as "the symptom that causes them the most distress", and the frequency of each answer was tabulated. RESULTS: This subanalysis included 134, 65, and 49 patient-caregiver pairs in the P-, G-, and N-groups, respectively. Statistically significant differences in patient background characteristics such as patient age; initial symptom domains; use of cholinesterase inhibitors, levodopa, antipsychotics, and Yokukansan; and total scores of the Mini-Mental State Examination, Neuropsychiatric Inventory-12, and Movement Disorder Society-Unified Parkinson's Disease Rating Scale Parts II and III were shown among the three subgroups. While there were no differences in patients' treatment needs among the subgroups, residual analysis showed that in the N-group, parkinsonism was more of a problem than other symptom domains (p = 0.001). There were significant differences in caregivers' treatment needs among the three subgroups (p < 0.001). The patient-physician concordance rates for the symptom domains that caused patients the most distress were: P-group, 42.9% (kappa coefficient [κ] = 0.264); G-group, 33.3% (κ = 0.135), and N-group, 67.6% (κ = 0.484). The caregiver-physician concordance rates for the symptom domains that caused the caregivers the most distress were: P-group, 54.8% (κ = 0.351), G-group, 50.0% (κ = 0.244), and N-group, 47.4% (κ = 0.170). CONCLUSION: This subanalysis revealed differences in the treatment needs of patients with DLB and their caregivers according to the clinical department they attended. There might be a lack of awareness of those treatment needs by the attending physicians, regardless of their specialty. TRIAL REGISTRATION: UMIN Clinical Trials Registry UMIN000041844.

Questionnaire survey of satisfaction with medication for five symptom domains of dementia with Lewy bodies among patients, their caregivers, and their attending physicians.

BACKGROUND: The real-world status of satisfaction with medication for dementia with Lewy bodies (DLB) has not been elucidated. We assessed the satisfaction of patients with DLB, their caregivers, and their attending physicians (trios) with medication according to the clinical symptom domains of DLB. METHODS: This was a subanalysis of a cross-sectional, questionnaire-based, survey study of trios. The subanalysis set comprised analysis populations for cognitive impairment, parkinsonism, psychiatric symptoms, sleep-related disorders, and autonomic dysfunction (orthostatic hypotension, constipation, and dysuria). These analysis populations included trios of patients who had any symptom domain and took medication for each symptom domain, and for which all trio data on satisfaction with medication for the symptom domain were available. The degrees of satisfaction with medication were classified as 'satisfied', 'neutral', or 'dissatisfied'. RESULTS: The analysis set for this study included 110 trios for cognitive impairment, 62 for parkinsonism, 47 for psychiatric symptoms, 29 for sleep-related disorders, none for orthostatic hypotension, 11 for constipation, and seven for dysuria. There were no statistically significant differences in the degree of satisfaction with medication for symptom domains other than parkinsonism and dysuria between patients-caregivers, patients-physicians, and caregivers-physicians. Regarding satisfaction with medication for parkinsonism, significantly more physicians than patients answered 'satisfied' (75.8% vs. 51.6%), and significantly more patients than physicians answered 'neutral' (35.5% vs. 14.5%) (P = 0.013). Regarding satisfaction with medication for dysuria, significantly more caregivers than physicians answered 'satisfied' (100% vs. 28.6%, P = 0.038). CONCLUSIONS: Satisfaction with medication for symptom domains other than parkinsonism and dysuria was similar among trios. Our results suggest that physicians should pay more attention to patients' satisfaction with medication for parkinsonism, and to caregivers' satisfaction with medication for dysuria to help prevent undermedication.

Hidden chamber discovery in the underground Hellenistic necropolis of Neapolis by muography.

We report in this paper the muography of an archaeological site located in the highly populated "Sanità" district in the center of Naples, ten meters below the current street level. Several detectors capable of detecting muons - high energy charged particles produced by cosmic rays in the upper layers of atmosphere - were installed underground at the depth of 18 m, to measure the muon flux over several weeks. By measuring the differential flux with our detectors in a wide angular range, we have produced a radiographic image of the upper layers. Despite the architectural complexity of the site, we have clearly observed the known structures as well as a few unknown ones. One of the observed new structures is compatible with the existence of a hidden, currently inaccessible, burial chamber.

Precise characterization of a corridor-shaped structure in Khufu's Pyramid by observation of cosmic-ray muons.

Khufu's Pyramid is one of the largest archaeological monument all over the world, which still holds many mysteries. In 2016 and 2017, the ScanPyramids team reported on several discoveries of previously unknown voids by cosmic-ray muon radiography that is a non-destructive technique ideal for the investigation of large-scale structures. Among these discoveries, a corridor-shaped structure has been observed behind the so-called Chevron zone on the North face, with a length of at least 5 meters. A dedicated study of this structure was thus necessary to better understand its function in relation with the enigmatic architectural role of this Chevron. Here we report on new measurements of excellent sensitivity obtained with nuclear emulsion films from Nagoya University and gaseous detectors from CEA, revealing a structure of about 9 m length with a transverse section of about 2.0 m by 2.0 m.

Oral environment and cerebral blood flow in patients with neurodegenerative dementia: comparison of Alzheimer type dementia and dementia with Lewy bodies.

BACKGROUND: The number of patients with dementia is increasing worldwide. Modifying risk factors may significantly reduce the prevalence and cost of dementia. The number of remaining teeth, occlusal force, denture use, and periodontal disease status are associated with the risk of developing dementia. The oral environment may be a risk factor for dementia. This study aimed to investigate the association between the oral environment and cerebral blood flow in patients with mild cognitive impairment and dementia (either Alzheimer type dementia (ATD) or dementia with Lewy bodies (DLB)). We aimed to identify differences in the oral environment according to the underlying neurodegenerative disease. METHODS: The participants were 25 patients with ATD and 25 patients with DLB who visited the Department of Dementia and Geriatric Medicine, Kanagawa Dental University Hospital. Patients were diagnosed with ATD and DLB by well-trained specialists certified by the Japanese Dementia Society and categorised as Level 3 or 4 by the Functional Assessment Staging of Alzheimer Disease scale. The correlation between oral environment (number of teeth, periodontal tissue, and oral function) and cerebral blood flow in each brain area calculated by single photon emission computed tomography was examined. RESULTS: The DLB group showed a significant correlation between masticatory performance and cerebral blood flow in some areas, while no such correlation was observed in the ATD group. Significant correlations were found between periodontal pocket depth and bleeding on probing and cerebral blood flow in some areas in both groups, although the correlation was considerable in the ATD group. CONCLUSIONS: The results of this study suggest that the association between changes in the oral environment and cerebral blood flow may differ among patients with ATD and DLB, depending on the underlying causative disease.

Treatment needs of dementia with Lewy bodies according to patients, caregivers, and physicians: a cross-sectional, observational, questionnaire-based study in Japan.

BACKGROUND: Understanding the treatment needs of patients with dementia with Lewy bodies (DLB) is essential to develop treatment strategies. We examined the treatment needs of patients with DLB and their caregivers and the extent to which the attending physicians understand these treatment needs. METHODS: This was a cross-sectional, observational study conducted using questionnaires for patients, caregivers, and physicians. The study participants included patients, their caregivers, and their attending physicians who were experts in DLB. Fifty-two symptoms that are frequent and clinically important in DLB were pre-selected and classified into seven symptom domains. Treatment needs of patients and caregivers were defined as "symptom that causes them most distress," and the frequency of each answer was tabulated. To assess the physician's understanding of the treatment needs of patients and caregivers, patient-physician and caregiver-physician concordance rates for each answer regarding treatment needs were calculated according to symptom domains. RESULTS: In total, 263 pairs of patients-caregivers and 38 physicians were surveyed. The mean age of patients was 79.3 years, and their mean total score on the Mini-Mental State Examination was 20.9. Thirty-five and 38 symptoms were selected as symptoms causing patients and caregivers most distress, respectively. Memory impairment was most frequently selected for the treatment needs of patients, followed by constipation and bradykinesia. Memory impairment was also most frequently selected by caregivers, followed by visual hallucinations. For the symptom domain that causes patients or caregivers most distress, only about half of the patient-physician pairs (46.9%) and caregiver-physician pairs (50.8%) were matched. Logistic regression analysis identified that concordance rates for treatment needs between patient-physician and caregiver-physician were lower when autonomic dysfunction and sleep-related disorders were selected as the symptom domains that cause most distress. CONCLUSION: There was considerable variability in the treatment needs of patients with DLB and their caregivers. Attending physicians had difficulty understanding the top treatment needs of their patients and caregivers, despite their expertise in DLB, because of various clinical manifestations. Attending physicians should pay more attention to autonomic dysfunction and sleep-related disorders in the treatment of DLB. TRIAL REGISTRATION: UMIN Clinical Trials Registry, UMIN000041844. Registered on 23 September 2020.

A Preliminary Trial in the Efficacy of Yokukansankachimpihange on REM Sleep Behavior Disorder in Dementia With Lewy Bodies.

Background: Clonazepam (CNZP) is effective in ~90% of patients with rapid eye movement sleep behavior disorder (RBD) but has risks of oversedation, muscular relaxation, and adverse effects on cognitive function when used to treat RBD associated with dementia with Lewy bodies (DLB). Yokukansankachimpihange (YKSCH), a traditional herbal medicine, decreases sleep latency and increases sleep stage 2, like benzodiazepines (BZPs), but does not cause adverse events such as oversedation, muscular relaxation, and adverse effects on cognitive function. Given these pharmacological properties, YKSCH was studied as a potential alternative to CNZP. Methods: Of patients who were diagnosed with DLB according to the criteria for the clinical diagnosis of DLB established by the Consortium on Dementia with Lewy Bodies (CDLB) in 2017, 13 consecutive patients with the cutoff score (5 points) or more in a REM sleep behavior disorder screening questionnaire and polysomnographic evidence of REM without atonia were observed using the Neuropsychiatric Inventory (NPI) night-time behavior disturbance, visual analog scale (VAS) frequency, and VAS severity as the co-primary endpoints. Data from 11 patients who completed the study were statistically analyzed. Results: Statistically significant improvements were observed in the NPI night-time behavior disturbance, VAS frequency, and VAS severity. No notable adverse events were reported. Conclusion: The results indicated that YKSCH, which does not cause oversedation, muscular relaxation, or adverse effects on cognitive function, may provide a new therapeutic option for RBD associated with DLB as an alternative to CNZP.

The effect of masticatory behaviour on generalized attention in heathy volunteers.

AIM: As our society ages, the number of people living with dementia also steadily increases. Some work has focused on masticatory behaviour as a form of daily health care that could help prevent cognitive impairment and dementia. However, it is not yet clear how masticatory behaviour influences various cognitive functions. Therefore, the purpose of this study was to examine the effect of masticatory behaviour on the decline of generalized attention, an important cognitive function. METHODS: Participants were 35 healthy, dentulous individuals without stomatognathic abnormalities (24 men, 11 women; mean age: 56.8 ± 4.8 years). All participants completed three interventions: mastication, foot-stepping, and none (control). Pre- and post-intervention measures of generalized attention were measured by using neuropsychological tests to examine general attention; the results were then compared. Simultaneously, during the generalized attention task, the functional activity of the prefrontal cortex was observed on functional near-infrared spectroscopy. RESULTS: Response time of generalized attention improved in both the masticatory and foot-stepping interventions. There was a transient increase in oxyhaemoglobin activity in the right and left prefrontal cortices in the masticatory intervention. CONCLUSIONS: Masticatory behaviour may be involved in a partial improvement of generalized attention and may induce prefrontal cortex activity in middle-aged and older adults.

A survey of doctors on diagnosis and treatment of dementia with Lewy bodies: examination and treatment of behavioural and psychological symptoms.

BACKGROUND: Dementia with Lewy bodies (DLB) is a progressive form of dementia, accompanied by a range of behavioural and psychological symptoms. The aim of this study was to identify current clinical practice for the treatment of DLB in Japan. METHODS: We conducted a survey of medical doctors engaged in the management of dementia in Japan. Participants were divided into two groups: psychiatrists (Group P) and neurologists or neurosurgeons (Group NS). Doctors completed a questionnaire and we analysed their responses to compare the two groups with regard to diagnosis and treatment of DLB, and in particular the treatment of behavioural and psychological symptoms of dementia (BPSD). RESULTS: Responses suggested that Group P conducted biomarker examinations less frequently and decided on their own therapeutic strategies more frequently than did Group NS. Both groups most frequently selected hallucinations/delusions as the symptoms given highest treatment priority. More than 70% of respondents in both groups reported having difficulties in treating BPSD. Atypical antipsychotics were more frequently prescribed by Group P, but were also prescribed in 70% of patients in Group NS. A third of patients received atypical antipsychotics for more than 1 year. CONCLUSIONS: The responses to this survey highlighted the difficulties faced by clinicians managing patients with DLB and identified the need to effectively treat BPSD in such patients.

Fact-finding survey on diagnostic procedures and therapeutic interventions for parkinsonism accompanying dementia with Lewy bodies.

BACKGROUND: We performed a questionnaire survey of medical doctors engaged in the management of dementia to identify the actual status of treatment for dementia with Lewy bodies (DLB) in Japan. METHODS: Among participating medical doctors, we selected neurologists (Group N) and psychiatrists (Group P) because these physicians are usually involved in the management of DLB patients. The two groups were compared based on their diagnosis and treatment of DLB and in particular, parkinsonism. RESULTS: Neurological examinations and biomarker tests were less frequently performed by Group P than Group N. Antipsychotics and other psychotropics excluding anti-dementia drugs were significantly more frequently administered by Group P than Group N. The proportion of physicians who selected L-dopa as a first-line therapy for parkinsonism was significantly higher in Group N than in Group P. Despite these between-group differences, the following findings were common to the two groups: there was a discrepancy between the symptom that patients expressed the greatest desire to treat, and the awareness of physicians regarding the treatment of these symptoms; the initial agent was L-dopa; and physicians exercised caution against the occurrence of hallucinations, delusions, and other adverse drug reactions. CONCLUSIONS: The results of the present survey offer valuable insight for the formulation of future DLB therapeutic strategies.

Discovery of a big void in Khufu's Pyramid by observation of cosmic-ray muons.

The Great Pyramid, or Khufu's Pyramid, was built on the Giza plateau in Egypt during the fourth dynasty by the pharaoh Khufu (Cheops), who reigned from 2509 bc to 2483 bc. Despite being one of the oldest and largest monuments on Earth, there is no consensus about how it was built. To understand its internal structure better, we imaged the pyramid using muons, which are by-products of cosmic rays that are only partially absorbed by stone. The resulting cosmic-ray muon radiography allows us to visualize the known and any unknown voids in the pyramid in a non-invasive way. Here we report the discovery of a large void (with a cross-section similar to that of the Grand Gallery and a minimum length of 30 metres) situated above the Grand Gallery. This constitutes the first major inner structure found in the Great Pyramid since the nineteenth century. The void, named ScanPyramids' Big Void, was first observed with nuclear emulsion films installed in the Queen's chamber, then confirmed with scintillator hodoscopes set up in the same chamber and finally re-confirmed with gas detectors outside the pyramid. This large void has therefore been detected with high confidence by three different muon detection technologies and three independent analyses. These results constitute a breakthrough for the understanding of the internal structure of Khufu's Pyramid. Although there is currently no information about the intended purpose of this void, these findings show how modern particle physics can shed new light on the world's archaeological heritage.

123I-metaiodobenzylguanidine myocardial scintigraphy with early images alone is useful for the differential diagnosis of dementia with Lewy bodies.

123I-metaiodobenzylguanidine cardiac scintigraphy (MIBG) is a useful imaging technique for the diagnosis of dementia with Lewy bodies (DLB). However, MIBG has a serious disadvantage in that it demands a long examination time. The objective of this study was to evaluate statistically the usefulness of the heart/mediastinum ratio (H/M) from the early phase of MIBG for the differential diagnosis of DLB. In total, 113 patients were examined, including 32 non-DLB (19 with Alzheimer's dementia) and 79 DLB patients. The mean early-H/M ratio was 2.83 in the non-DLB group and 1.95 in the DLB group. The mean delayed-H/M ratio was 3.0 in the non-DLB group and 1.76 in the DLB group. With a cutoff point of 2.27 on early images, the sensitivity, specificity, and diagnostic accuracy were 65%, 94%, and 73%, respectively, and the area under the curve was 0.82, indicating moderate accuracy. This analysis indicates that images from the early phase of MIBG alone are sufficient for the differential diagnosis of Alzheimer's disease and DLB.

Increased dosage of donepezil for the management of behavioural and psychological symptoms of dementia in dementia with Lewy bodies.

BACKGROUND: As with other types of dementia, the behavioral and psychological symptoms of dementia (BPSD) can make caregiving difficult for patients with dementia with Lewy bodies (DLB). We hypothesized that administration of donepezil at an increased dose of 10 mg/day might dose-dependently improve BPSD in DLB patients with relapse, after their symptoms had been controlled initially by donepezil therapy at the standard dose. METHODS: The present study was as an open-label trial. We enrolled 24 patients with DLB (diagnosed according to the Consortium on Dementia with Lewy Bodies Guideline-Revised) who experienced a relapse of BPSD despite treatment with donepezil at the standard dose (5 mg/day). The donepezil dose for these patients was increased to 10 mg/day, and we evaluated the efficacy and safety of this dose escalation strategy. RESULTS: The Neuropsychiatric Inventory (NPI) scores for BPSD showed statistically significant improvements as a result of the increased dosage, except those for anxiety and euphoria, disinhibition, irritability/lability. High-dose donepezil therapy caused gastrointestinal symptoms in 4 patients, but there were no life-threatening adverse events, such as arrhythmias, or no exacerbation of parkinsonian symptoms. CONCLUSIONS: We found that donepezil dose-dependently improved relapsing BPSD in these patients. Therefore, increasing the dosage of donepezil is a safe and effective treatment for patients with DLB who experience a relapse of BPSD.

Comparison of (123)I-MIBG myocardial scintigraphy, brain perfusion SPECT, and voxel-based MRI morphometry for distinguishing between dementia with Lewy bodies and Alzheimer's disease.

OBJECTIVE: This study aimed to compare the diagnostic value of (123)I-metaiodobenzylguanidine (MIBG) myocardial scintigraphy, N-isopropyl-p[(123)I]iodoamphetamine (IMP) brain perfusion single-photon emission computed tomography (SPECT), and brain magnetic resonance imaging (MRI) voxel-based morphometry (VBM) for the differentiation of dementia with Lewy bodies (DLB) and Alzheimer's disease (AD). METHODS: Thirty-five and 34 patients with probable DLB and probable AD, respectively, were enrolled. All patients underwent (123)I-MIBG myocardial scintigraphy, (123)I-IMP brain perfusion SPECT, and brain MRI. For (123)I-MIBG imaging, we calculated early and delayed heart-to-mediastinum (H/M) uptake ratios. Three-dimensional stereotactic surface projections (3D-SSP) were used to analyze the results of (123)I-IMP SPECT. VBM with statistical parametric mapping 8 plus diffeomorphic anatomical registration using exponentiated Lie algebra (DARTEL) was used to analyze the brain MRI data. RESULTS: The area under the receiver operating characteristic curves (AUC) for discriminating DLB and AD was highest (0.882) for the delayed H/M ratio on (123)I-MIBG scintigraphy. AUC for z-score measurement in the occipital lobe was 0.818 and that for the extent of gray matter (GM) atrophy in the whole brain was 0.788. AUC for the combination of 3D-SSP and VBM analysis was 0.836. The respective sensitivities and specificities for distinguishing DLB from AD were 97.1 and 100 % for the delayed H/M ratio using (123)I-MIBG scintigraphy; 88.6 and 73.5 % for the occipital lobe z-score using 3D-SSP analysis; 85.7 and 64.7 % for the extent of whole brain GM atrophy using voxel-based MRI morphometry; and 91.4 and 76.5 % for the combination of 3D-SSP analysis and VBM. CONCLUSIONS: (123)I-MIBG myocardial scintigraphy was superior to brain perfusion SPECT and brain MRI using an advanced statistical technique to differentiate DLB and AD.

[Content validity and inter-rater reliability of the Cognitive Fluctuation Inventory].

OBJECTIVE: The Cognitive Fluctuation Inventory (CFI) was developed to evaluate cognitive fluctuation in patients with dementia with Lewy bodies (DLB). The objective of this study was to assess the content validity and inter-rater reliability of the CFI. SUBJECTS AND METHODS: Nine specialists in DLB treatment were invited to participate in the survey to assess the content validity of the CFI. They were asked to assess the relevance and comprehensibility of the question items. In the validation study, inter-rater reliability was assessed using the intraclass correlation coefficient (ICC). RESULTS: Regarding content validity, all nine specialists considered the main question and sub-questions to be relevant to cognitive fluctuation in patients with DLB. Eight out of nine specialists considered the CFI to be a comprehensive measure for detecting cognitive fluctuation in patients with DLB. In the analysis, which used data from 29 patients and their caregivers, the ICC of the CFI was 0.746, which suggests good inter-rater reliability. CONCLUSION: We found that the CFI showed good content validity and inter-rater reliability for evaluating cognitive fluctuation in patients with DLB.

Three presenile patients in which neuropsychological and neuroimaging examinations suggest possible progression to dementia with Lewy bodies.

We report three presenile patients who were initially suspected of having Alzheimer's disease (AD) or being in the prodromal stage of AD, regardless of visuoperceptual dysfunctions in daily living, because they lacked the core features and prodromal non-motor symptoms of dementia with Lewy bodies. Subsequently, progression to dementia with Lewy bodies was suspected based on neuropsychological and neuroimaging findings; additionally, one of the three patients suffered from visual hallucinations. Neuropsychological examinations such as subjective contours, cube copying and block design in the Wechsler Adult Intelligence Scale-III revealed visuoperceptual dysfunction in all three patients even when other cognitive functions were rather preserved. Brain magnetic resonance imaging revealed no significant brain atrophy, including in the parieto-occipital area and the hippocampus, while brain (18)F-fluorodeoxyglucose positron emission tomography demonstrated right dominant metabolic reductions in the occipital lobe, including the primary visual cortex, in all three patients. We suggest the possibility of progression to dementia with Lewy bodies, but not AD or posterior cortical atrophy. Regardless of the presence of core features and prodromal non-motor symptoms, this progression is suggested when there are difficulties only in higher-level visual processing such as subjective contours and block design in the Wechsler Adult Intelligence Scale-III, no significant atrophy of the parieto-occipital area and hippocampus on brain magnetic resonance imaging, and hypometabolism in the occipital lobe including the primary visual cortex on brain (18)F-fluorodeoxyglucose positron emission tomography.