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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). Key points: This multicenter, cross‐sectional observational study collected survey data from 263 patient–caregiver pairs and 66 physicians to investigate how the duration after diagnosis of dementia with Lewy bodies (DLB) influences clinical symptoms, prescribed medications, and caregiver burden, and how perception of treatment needs differs among patients, their caregivers, and their physicians.Presence of parkinsonism and autonomic dysfunction, severity of parkinsonism, and caregiver burden were all found to be higher in patients with more than 24 months duration.Although patient treatment needs did not differ significantly between patients by post‐diagnosis duration (<24 vs. ≥ 24 months), patients with short (<24 months) post‐diagnosis duration were more likely to select cognitive impairment as treatment need; while those with long (≥24 months) post‐diagnosis duration were more likely to select parkinsonism.Caregivers for patients with short post‐diagnosis duration were more likely to select cognitive impairment as the greatest treatment need, whereas caregivers for patients with long post‐diagnosis duration were more likely to select other symptom domains such as parkinsonism, psychiatric symptoms, sleep‐related disorder, and autonomic dysfunction as the greatest treatment need.Physicians' perception of patients' and caregivers' treatment needs was poor, regardless of the duration after diagnosis of DLB. [ABSTRACT FROM AUTHOR]

Objectives: Frontotemporal lobar degeneration (FTLD) is associated with accumulation of neurodegeneration‐related protein, such as tau, TAR DNA‐binding protein 43 (TDP‐43), or fused in sarcoma protein (FUS). There have been very few systematic studies of the early symptoms of clinical phenotypes: behavioral variant frontotemporal dementia (bvFTD), semantic variant primary progressive aphasia (svPPA). Clinical subtypes and the patterns of atrophy reflect protein‐accumulation patterns, but the relationship between early symptoms and pathological findings remains unclear. Methods: We retrospectively investigated the clinical records and examined the neuropathology of 39 bvFTD and 6 svPPA patients to identify symptoms appearing within 2 years of the first clinically apparent changes. Results: The bvFTD group consisted of 13 FTLD‐tau, 18 FTLD‐TDP, and 8 FTLD‐FUS, and the svPPA group consisted of 6 FTLD‐TDP. Age at death is significantly younger in FTLD‐FUS (52.8 ± 12.6; P = 0.0104 < 0.05). Over 50% of bvFTD patients show apathy or inertia, and distinct language features appear early in svPPA. Interestingly, bvFTD and svPPA frequently present additional symptoms, not included in the diagnostic criteria, such as physical signs, reticence, dazed condition, and delusions. Stereotyped behaviors, hyperorality and dietary changes are prominent in FTLD‐FUS, while linguistic deficits are greater in FTLD‐TDP. Conclusions: Specific symptoms tend to appear in the early stage of FTLD in each pathological background. They might reflect the morphological features and pathological progression, and should be helpful in the stratification of patients for future therapeutic trials based on the proteinopathies. [ABSTRACT FROM AUTHOR]

Objectives: Apathy is prevalent in patients with idiopathic normal pressure hydrocephalus (iNPH), a treatable disorder resulting from ventricular enlargement. We assessed the relationship between apathy and regional cerebral blood flow (rCBF) in patients with iNPH.Methods: Before lumbo-peritoneal shunt surgery (LPS), 56 iNPH patients were evaluated on apathy and dysphoria subscales of the Neuropsychiatric inventory (NPI), and were divided into two groups according to NPI apathy score: 15 without apathy (iNPH-APA) and 41 with apathy (iNPH+APA). Among iNPH+APA, 29 patients were evaluated for apathy and dysphoria 3 months after LPS, and were divided into two groups on the basis of the change in NPI apathy score: 13 with improvement (iNPH+ImpAPA) and 16 without improvement in apathy (iNPH-ImpAPA). N-isopropyl-p-iodoamphetamine single photon emission computed tomography using the autoradiography method was performed before and after LPS, and rCBF was calculated in 22 regions of interest in the frontal cortex, basal ganglia, and limbic system.Results: In iNPH+APA, rCBF in the right caudate nuclei before LPS was significantly lower than that in iNPH-APA (P = 0.004; two-sample t test). Between iNPH-ImpAPA and iNPH+ImpAPA, a significant group-by-shunt interaction was observed for rCBF in only the right caudate nuclei (F1, 28  = 11.75, P = 0.002; two-way repeated-measures analysis of variance), with increased rCBF in iNPH+ImpAPA but not in iNPH-ImpAPA. The significant group-by-shunt interaction persisted if change in NPI dysphoria scores was used as a covariate (F1, 27  = 8.33, P = 0.008).Conclusions: Our findings suggest that right caudate dysfunction might cause apathy in iNPH patients. [ABSTRACT FROM AUTHOR]

Background Cerebral small vessel disease (SVD) is frequently observed in patients with Alzheimer's disease (AD). However, the association between SVD and clinical symptoms exhibited by patients with AD remains unclear. This study examined the association of SVD as observed on magnetic resonance imaging (MRI) with behavioural and psychological symptoms of dementia and cognitive function of patients with probable AD. Methods A total of 163 consecutive patients (55 men, 108 women) with probable AD were included in this cross-sectional study of a prospective cohort. Patients were divided into two groups based on the presence or absence of cerebral SVD [white matter hyperintensities (WMH) grade 0/1 (Fazekas scale) and no lacunes: SVD absent, WMH grade 2/3 (Fazekas scale) or the number of lacunes ≥1: SVD present]. Cognitive functions were assessed using the Mini mental state examination, word recall and recognition subtests in the Alzheimer's Disease Assessment Scale-Cognitive Subscale, as well as the letter fluency task and the category fluency task. Psychiatric symptoms were rated according to Neuropsychiatric Inventory. Results Patients with probable AD with cerebral SVD had significantly more delusions and depression than those without SVD. No significant differences were observed in other neuropsychiatric symptoms, MMSE or word recall and recognition tests between both groups. Conclusions Our results suggest that cerebral SVD observed on MRI of patients with AD is associated with delusions and depression. Copyright © 2012 John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]

Background: Sleep disturbances in Alzheimer disease (AD) may affect behavioral and psychological symptoms of dementia (BPSD). Our aim was to elucidate the associations between sleep disturbances and other BPSD at different stages of AD., Methods: This investigation was part of a multicenter-retrospective study in Japan (J-BIRD). Eligible for final analyses were 684 AD patients. Global severity of dementia was estimated using the Clinical Dementia Rating (CDR) scale. BPSD were assessed using the Neuropsychiatric Inventory (NPI). We analyzed the relationships between sleep disturbances and BPSD at different stages of AD according to the CDR score., Results: Among the 684 AD patients, 146 (21.3%) had sleep disturbances. Patients with very early AD (CDR 0.5) and sleep disturbances had significantly more BPSD than those without sleep disturbances, as indicated by the higher prevalence of the following four NPI items: anxiety, euphoria, disinhibition, and aberrant motor behavior. In AD at CDR 2, (moderate AD) only one NPI item (irritability) was affected, while none was affected at CDR 1 (mild AD) and 3 (severe AD). Multiple regression analyses were performed in those with AD having various CDR scores. At CDR 0.5, the presence of sleep disturbances was associated with a high total NPI score (β = 0.32, p < 0.001). However, other factors, including cognitive decline, age, gender, and years of education, were not significantly associated with the NPI score. At CDR 1 and 2, no factor was significantly related to BPSD., Conclusion: Sleep disturbances were strongly associated with other BPSD in the very early stage of AD. Copyright © 2016 John Wiley & Sons, Ltd., (Copyright © 2016 John Wiley & Sons, Ltd.)