Communication-related experiences of individuals in the early phase of semantic dementia and their families: an interview study.

BACKGROUND: Semantic dementia (SD), a subtype of frontotemporal dementia, manifests as verbal symptoms, including social and behavioural deficits, associated with focal atrophy of the frontotemporal lobes. This study aimed to clarify the experiences of individuals with early-onset SD receiving speech and language rehabilitation (hereafter referred to as 'rehabilitation'), with the intent of making it routine, as well as the experiences of their families. METHODS: Individual interviews were conducted with nine families with members who had adopted rehabilitation. Verbatim transcripts were used as data, and analyzed inductively according to the content analysis process. RESULTS: The family members realised the changes in the personality and behaviour of the individual with SD early, to the extent that they thought the individual with SD was different from before and were distressed by the loss of verbal communication. Nevertheless, the family members found a way to communicate by maintaining residual functions through rehabilitation and utilising their unique relationship with the individual with SD. CONCLUSIONS: It is important to carefully explain the characteristics of the disease and the long-term significance of rehabilitation to individuals with SD and their families in the early stages of the disease.

Analysis of factors related to cognitive impairment in a community-based, complete enumeration survey in Japan: the Nakayama study.

BACKGROUND: The number of patients with cognitive disorders is rapidly increasing in the world, becoming not only a medical problem, but also a social problem. There have been many reports that various factors are associated with cognitive dysfunction, but the factors have not yet been fully identified. This was a community-based complete enumeration study which aimed to identify risk and protective factors for dementia. METHODS: The first phase included all residents aged 65 years or older in a town in Japan. They completed many examinations, such as living conditions questionnaires, physical examination, Mini-Mental State Examination, and brain magnetic resonance imaging. The participants with suspected cognitive impairment underwent additional examinations for detailed evaluation in the second phase. Statistical analysis was performed to identify risk and protective factors for dementia after all participants were diagnosed. RESULTS: There were 927 participants in the baseline evaluation; 611 (65.9%) were healthy, 165 (17.8%) had mild cognitive impairment (MCI), and 151 (16.3%) had dementia. The age-standardised prevalence of dementia was 9.5%. Statistical analyses for amnestic MCI and Alzheimer's disease showed that risk factors for cognitive decline were diabetes mellitus, low activities of daily living, and living alone, and that protective factors were history of exercise and drinking habit. CONCLUSION: The present findings suggest that several lifestyle-related diseases and factors are associated with cognitive decline. These results support similar findings from previous studies and will be helpful for preventing dementia in the future.

[Retrograde S(9+)10 Segmentectomy without Interlobar Fissurelectomy].

BACKGROUNDS: With increase of patients with a small-sized lung cancer, there is an increasing need for minimally invasive lung segmentectomy that can preserve respiratory function. We perform S(9+)10 segmentectomy with retrograde dissection of the pulmonary vein, bronchus, pulmonary artery, in order, without interlober fissurelectomy and staple dissection of the peripheral lung parenchyma. METHODS: Seven patients who underwent retrograde S(9+)10 segmentectomy between June, 2021 and May, 2022 in our hospital were retrospectively reviewed. RESULTS: No patient was converted to the open thoracotomy, without any complications including prolonged air leakage. The average operation time was 171 minutes( range 125 to 221), amount of bleeding was 25 ml( range 0 to 75). Median duration of chest tube insertion was 4 days( range 3 to 6), length of stay after surgery was 6 days (range 5 to 9). Pathologic stage showed pT1mi in 3 patients, pT1a in 3 patients, pT2a in 1 patient. No local recurrence was seen at this time. CONCLUSIONS: Retrograde S(9+)10 segmentectomy is feasible and facilitates interlobar procedure at the time of repeated segmentectomy or completion lobectomy.

Secular trends in the prevalence of dementia based on a community-based complete enumeration in Japan: the Nakayama Study.

BACKGROUND: The number of dementia patients is increasing worldwide, especially in Japan, which has the world's highest ageing population. The increase in the number of older people with dementia is a medical and socioeconomic problem that needs to be prevented, but the actual situation is still not fully understood. METHODS: Four cross-sectional studies on dementia were conducted in 1997, 2004, 2012, and 2016 for complete enumeration of all residents aged 65 years and older. We examined the secular trends in the prevalence of all-cause dementia, Alzheimer's disease (AD), vascular dementia (VaD), and other/unclassified dementia. RESULTS: The age-standardised prevalence of all-cause dementia significantly increased (4.5% in 1997, 5.7% in 2004, 5.3% in 2012, 9.5% in 2016; P for trend <0.05). Similar trends were observed for AD (1.7%, 3.0%, 2.5% and 4.9%, respectively; P for trend <0.05) and other/unclassified dementia (0.8%, 1.0%, 1.0% and 2.2%, respectively; P for trend <0.05), whereas no significant change in VaD was seen (2.1%, 1.8%, 1.8%, 2.4%, respectively; P for trend = 0.77). The crude prevalence of all-cause dementia and AD increased from 1997 to 2016 among participants aged 75-79 years and ≥85 years (all P for trend <0.05). Similar trends were observed for other/unclassified dementia among participants aged ≥80 years (all P for trend <0.05), but not in VaD. CONCLUSIONS: The prevalence of dementia has increased beyond the ageing of the population, suggesting that factors in addition to ageing are involved in the increase in the number of older people with dementia. To control the increase in the number of older people with dementia, elucidation of secular trends in the incidence, mortality, and prognosis of dementia as well as the factors that promote and protect against dementia, and development of preventive strategies are necessary.

[Metaplastic Thymoma Underwent Surgery:Report of a Case].

Metaplastic thymoma is a rare histologic variant of thymic epithelial tumors and is characterized by a biphasic growth pattern. We herein report the case of 44-year-old woman who underwent surgery for metaplastic thymoma. Computed tomography scan revealed a well-circumscribed mediastinal tumor: 56 mm in diameter with homogenous enhancement. The tumor was suspected to be a non-invasive thymoma, and thymomectomy with resection of the surrounding thymus was performed using thoracoscopy. The resected tumor measured 60 mm and was grossly well-encapsulated. The cut surface was gray to white and homogenous. Microscopically, the epithelial components took the form of an anastomosing nest to broad trabeculae intertwining with the bundle of spindle cells. Mitosis was not found and the Ki-67 index was < 1%. Cytokeratin 5/6 was strongly positive in the epithelial components composed of polygonal cells. Terminal deoxynucleotidyl transferase positive immature T cells were not observed. Based on these pathologic findings, the tumor was identified as metaplastic thymoma.

Sleep disturbances are key symptoms of very early stage Alzheimer disease with behavioral and psychological symptoms: a Japan multi-center cross-sectional study (J-BIRD).

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 (ß = 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.

Comparison of the utility of everyday memory test and the Alzheimer's Disease Assessment Scale-Cognitive part for evaluation of mild cognitive impairment and very mild Alzheimer's disease.

AIM: The purpose of this study was to compare the utility of the Rivermead Behavioural Memory Test (RBMT) and the Alzheimer's Disease Assessment Scale-Cognitive part (ADAS-Cog) for the evaluation of mild cognitive impairment (MCI) or very mild Alzheimer's disease (AD). METHODS: The discriminative abilities of RBMT and ADAS-Cog were compared in the very early stage of AD or MCI patients. Furthermore, we evaluated the difference in both RBMT score and ADAS-Cog score between different severities. RESULTS: Evident superiority in the false negative rate was observed in RBMT over ADAS-Cog in MCI or very mild AD. In addition, 86.7% of the subjects overlooked by ADAS-Cog were correctly detected by RBMT profile score. However, the RBMT score falls in the very early stages and the range of the RBMT score is rather narrow. As a result, it is difficult to evaluate status and follow the progression in severer cases. In contrast to RBMT, the ADAS-Cog score has a wide range and can evaluate and follow the severity in more severe cases. CONCLUSION: RBMT is more useful than ADAS-Cog in evaluating patients with MCI or very mild AD.

Risk of progression from mild memory impairment to clinically diagnosable Alzheimer's disease in a Japanese community (from the Nakayama Study).

BACKGROUND: Memory impairment has been proposed as the most common early sign of Alzheimer's disease (AD). The aims of this work were to evaluate the risk of progression from mild memory impairment/no dementia (MMI/ND) to clinically diagnosable AD in a community-based prospective cohort and to establish the risk factors for progression from MMI/ND to AD in the elderly. METHODS: Elderly subjects aged over 65 years were selected from the participants in the first Nakayama study. MMI/ND was defined as memory deficit on objective memory assessment, without dementia, impairment of general cognitive function, or disability in activities of daily living. A total of 104 MMI/ND subjects selected from 1242 community-dwellers were followed longitudinally for five years. RESULTS: During the five-year follow-up, 11 (10.6%) subjects were diagnosed with AD, five (4.8%) with vascular dementia (VaD), and six (5.8%) with dementia of other etiology. Logistic regression analysis revealed that diabetes mellitus (DM) and a family history of dementia (within third-degree relatives) were positively associated with progression to AD, while no factor was significantly associated with progression to VaD or all types of dementia. CONCLUSIONS: DM and a family history of dementia were significant risk factors for progression from MMI/ND to clinically diagnosable AD in the elderly in a Japanese community.

Clinical profiles of late-onset semantic dementia, compared with early-onset semantic dementia and late-onset Alzheimer's disease.

BACKGROUND: Semantic dementia (SD) has been recognized as a representative of dementia with presenile onset; however, recent epidemiological studies have shown that SD also occurs in the elderly. There have been few studies about the differences of clinical profiles between early-onset SD (EO-SD) and late-onset SD (LO-SD). Age-associated changes in the brain might cause some additional cognitive and behavioural profiles of LO-SD in contrast to the typical EO-SD cases. The aim of the present study was to clarify the characteristics of neuropsychological, and behavioural and psychological symptoms of dementia (BPSD) profiles of LO-SD patients observed in screening tests in comparison with EO-SD patients and late-onset Alzheimer's disease (LO-AD) patients as controls. METHODS: Study participants were LO-SD (n = 10), EO-SD (n = 15) and LO-AD (n = 47). We examined the Mini-Mental State Examination (MMSE), the Raven's Coloured Progressive Matrices (RCPM), the Short-Memory Questionnaire (SMQ), the Neuropsychiatric Inventory (NPI) and the Stereotypy Rating Inventory (SRI). RESULTS: Both SD groups scored significantly lower than the LO-AD patients in 'naming' of the MMSE. In the 'construction' score of the MMSE and the RCPM score, however, the LO-SD patients as well as the LO-AD patients were significantly lower than the EO-SD patients. In the SMQ score, 'euphoria' and 'disinhibition' scores of the NPI, the SRI total and subscale scores, both SD groups were significantly higher, whereas in the 'delusion' score of the NPI, both SD groups were significantly lower than the LO-AD patients. CONCLUSIONS: Visuospatial and constructive skills of LO-SD patients might be mildly deteriorated compared with EO-SD patients, whereas other cognitive and behavioural profiles of LO-SD are similar to EO-SD. Age-associated changes in the brain should be considered when we diagnose SD in elderly patients.

Diaphragmatic paraganglioma protruding into the right thoracic cavity.

Paragangliomas in the diaphragm are extremely rare. We report the case of a 27-year-old woman with a nonfunctioning paraganglioma protruding superiorly from the right diaphragm. The patient underwent an anterior thoracotomy, and a supradiaphragmatic tumor (70 mm in diameter), which compressed the inferior vena cava and the right hepatic vein, was completely resected by combined partial resection of the right diaphragm and pericardium. To our knowledge, this is the first report of a paraganglioma situated both on the diaphragm and close to the inferior vena cava and hepatic vein. KEY POINTS.

Salvage Surgery Post Definitive Chemoradiotherapy and Durvalumab for Lung Cancer.

Although concurrent chemoradiotherapy (CRT) followed by consolidation immunotherapy considerably improves the duration of survival in patients with unresectable stage III non-small cell lung cancer (NSCLC), few data are available on the management of local relapse after therapy. We present a patient with initially unresectable NSCLC who underwent a right upper lobectomy with reconstruction of the bronchus and pulmonary artery after definitive CRT, followed by consolidation durvalumab. No postoperative complications occurred, and he was recurrence-free at the 10-month follow-up. Salvage surgery might be a viable option for local relapse of NSCLC treated with definitive CRT and durvalumab.

Transition of distinctive symptoms of semantic dementia during longitudinal clinical observation.

BACKGROUND/AIMS: The aim of this study is to examine the clinical symptoms in a number of semantic dementia (SD) patients and to reveal the longitudinal progression and clinical course of these distinctive symptoms of SD. METHODS: 19 consecutive SD patients were examined. Symptoms were classified into 23 distinct categories: behavioral symptoms, language and cognitive symptoms and symptoms concerning the impairment of activities of daily living (ADL). We divided patients into two subgroups, left- and right-dominant SD, and compared the onset of each symptom. RESULTS: Language impairments occurred as the initial symptom in 16 cases. At the first examination, all cases showed both anomia and impairment of word comprehension. By around 3 years after onset, almost all language impairments were observed. Approximately 3-5 years after onset, prosopagnosia and behavioral symptoms appeared. Around the period when the loss of the language faculty and apathy became remarkable, impairment of ADL appeared. Patients spent all day in bed at this stage. Moreover, prosopagnosia appeared significantly earlier in right-dominant SD. CONCLUSION: Our findings clarify the progression of distinctive symptoms of SD patients. It is necessary to create a treatment strategy for SD patients with such a disease-specific course of SD.

[Evaluation of Semantic Dementia Patient].

Semantic dementia (SD) is a clinical syndrome characterized by selective and progressive semantic memory impairment due to frontotemporal lobar degeneration (FTLD). Semantic memory disorders appear in every cognitive fields, be it language or recognition of familiar people and objects. Left-right asymmetry produces a distinctive clinical symptom of anterior temporal lobe atrophy. Progressive gogi (word-meaning) aphasia, i.e., word comprehension deficits with severe anomia, is prominent in left-dominant patients, whereas progressive prosopagnosia, i.e., difficulty recognizing familiar faces, is prominent in right-dominant patients. Loss of semantic memory of objects is observed in advanced stages of the disease. Behavioral symptoms are less prominent in the early stages; however, behavioral changes in patients with gogi aphasia are observed even in their initial visits. In addition to neuroimaging and neuropsychological evaluation of semantic memory, it will be useful if clinicians pay attention to behavioral changes of patients with gogi aphasia.

The association between semantic dementia and surface dyslexia in Japanese.

One theory about reading suggests that producing the correct pronunciations of written words, particularly less familiar words with an atypical spelling-sound relationship, relies in part on knowledge of the word's meaning. This hypothesis has been supported by reports of surface dyslexia in large case-series studies of English-speaking/reading patients with semantic dementia (SD), but would have increased credibility if it applied to other languages and writing systems as well. The hypothesis predicts that, of the two systems used to write Japanese, SD patients should be unimpaired at oral reading of kana because of its invariant relationship between orthography and phonology. By contrast, oral reading of kanji should be impaired in a graded fashion depending on the consistency characteristics of the kanji target words, with worst performance on words whose component characters take 'minority' (atypical) pronunciations, especially if the words are of lower frequency. Errors in kanji reading should primarily reflect assignment of more typical readings to the component characters in these atypical words. In the largest-ever-reported case series of Japanese patients with semantic dementia, we tested and confirmed this hypothesis.

Two cases of frontotemporal dementia with predominant temporal lobe atrophy.

Frontotemporal dementia (FTD) is a subtype of frontotemporal lobar degeneration, which also includes semantic dementia (SD) and progressive non-fluent aphasia. Frontotemporal dementia is characterized by changes in personality and behavioral abnormalities, generally associated with predominant frontal lobe atrophy. Conversely, SD is typically characterized by Gogi (word meaning) aphasia based on semantic memory impairment and is associated with predominant temporal lobe atrophy. However, in the present cases, we diagnosed FTD on the basis of clinical symptoms, such as disinhibition, indifference, and stereotypy, without semantic memory impairment, even though neuroimaging showed predominant temporal lobe atrophy. We suggest that clinical symptoms are the most important cues for an accurate clinical diagnosis and there is no exclusive relationship between the syndrome and atrophy of the temporal lobes.

Cognitive function and psychiatric symptoms in early- and late-onset frontotemporal dementia.

BACKGROUND/AIM: Some recent studies mentioned that late-onset frontotemporal dementia (FTD) is more common than previously assumed. Although much research has been done in the field, there are no systematic studies which have compared clinical characteristics of early- and late-onset FTD. The aim of this study was to compare cognitive function and psychiatric symptoms in patients with early- and late-onset FTD. METHODS: Study participants were consecutive outpatients. There were 35 FTD patients; their mean age at onset was 63.0 years. We studied sex, education, duration from onset to consultation, Clinical Dementia Rating (CDR) scores, Mini-Mental State Examination (MMSE) scores, Raven's Coloured Progressive Matrices (RCPM) scores, and Neuropsychiatric Inventory (NPI) scores at first consultation of early- and late-onset FTD patients. RESULTS: There were no significant differences in sex ratio, education, CDR scores, and duration from onset to consultation. There were significant differences in the total MMSE scores, 'three-word recall task', 'construction task', and RCPM scores; late-onset groups scored significantly lower than early-onset groups. There were significant differences in the apathy domain of NPI and total NPI scores; late-onset groups scored significantly higher than early-onset groups. CONCLUSION: Late-onset FTD patients may have memory and visuospatial deficits in addition to their behavioural changes, even if they are clinically diagnosed according to consensus diagnostic criteria. They also present more apathy, and they may have a different histolopathological background.

Gustatory Dysfunction as an Early Symptom of Semantic Dementia.

OBJECTIVE: To investigate the gustatory function in patients with semantic dementia (SD). METHODS: Detection and recognition thresholds of the 4 basic tastes (sweet, salty, sour, and bitter), taste discrimination, and taste identification were evaluated in 18 patients with SD, 18 patients with Alzheimer disease (AD), and 22 healthy controls. RESULTS: Total detection and recognition threshold values were significantly higher in the SD and AD groups than in the control group. Patients with early-stage SD (Clinical Dementia Rating Scale score 0.5) exhibited significantly higher detection and recognition thresholds relative to controls, while increases in recognition threshold were only noted in patients with AD. Patients with SD exhibited significantly higher thresholds for the detection of sweet and salty tastes and the recognition of salty, sour, and bitter tastes, while patients with AD exhibited significantly higher thresholds only for the recognition of salty and sour tastes. Taste discrimination was preserved, whereas taste identification was disturbed, in both the SD and AD groups. CONCLUSIONS: Gustatory dysfunction at both the sensory and semantic levels may be among the early symptoms of SD. Although patients with SD had difficulty detecting sweet tastes, they more easily recognized these tastes than others, which may explain their strong preference for sweets.

Differences of Behavioral and Psychological Symptoms of Dementia in Disease Severity in Four Major Dementias.

BACKGROUND/AIMS: Behavioral and psychological symptoms of dementia (BPSDs) negatively impact the prognosis of dementia patients and increase caregiver distress. The aims of this study were to clarify the differences of trajectories of 12 kinds of BPSDs by disease severity in four major dementias and to develop charts showing the frequency, severity, and associated caregiver distress (ACD) of BPSDs using the data of a Japan multicenter study (J-BIRD). METHODS: We gathered Neuropsychiatric Inventory (NPI) data of patients with Alzheimer's disease (AD; n = 1091), dementia with Lewy bodies (DLB; n = 249), vascular dementia (VaD; n = 156), and frontotemporal lobar degeneration (FTLD; n = 102) collected during a 5-year period up to July 31, 2013 in seven centers for dementia in Japan. The NPI composite scores (frequency × severity) of 12 kinds of items were analyzed using a principal component analysis (PCA) in each dementia. The factor scores of the PCA were compared in each dementia by disease severity, which was determined with Clinical Dementia Rating (CDR). RESULTS: Significant increases with higher CDR scores were observed in 1) two of the three factor scores which were loaded for all items except euphoria in AD, 2) two of the four factor scores for apathy, aberrant motor behavior (AMB), sleep disturbances, agitation, irritability, disinhibition, and euphoria in DLB, and 3) one of the four factor scores for apathy, depression, anxiety, and sleep disturbances in VaD. However, no increases were observed in any of the five factor scores in FTLD. CONCLUSIONS: As dementia progresses, several BPSDs become more severe, including 1) apathy and sleep disturbances in AD, DLB, and VaD, 2) all of the BPSDs except euphoria in AD, 3) AMB, agitation, irritability, disinhibition, and euphoria in DLB, and 4) depression and anxiety in VaD. Trajectories of BPSDs in FTLD were unclear.

Surface dyslexia in a Japanese patient with semantic dementia: evidence for similarity-based orthography-to-phonology translation.

We studied the reading performance of a Japanese-speaking patient, TI, with bilateral but asymmetrical (left more than right) temporal-lobe atrophy, severe anomia, and poor word comprehension. Most Japanese kanji characters correspond to several different legitimate pronunciations in different contexts, with varying degrees of correspondence consistency. TI made many errors in reading aloud words that violate statistically typical character-sound correspondences, especially for less common words, but had relatively preserved ability to read aloud strings in which the assignment of the typical pronunciation for each component character yields the correct pronunciation for the whole word. The degree of consistency of character-sound correspondences affected his performance on both words and nonwords in a graded manner. One interpretation is that TI's surface dyslexic reading reflects intact direct computation of phonology from orthography, but without the additional constraint from word meaning that is, in this framework, considered critical for correct pronunciation of lower-frequency words with atypical character-sound correspondences. Another interpretation is that TI's performance reflects partially damaged lexical knowledge of whole-word orthography and phonology, coupled with spared sublexcal knowledge of character-sound correspondence rules. Whichever of these interpretations is preferred, this study offers the most detailed information yet available on the characteristics of surface dyslexia in Japanese.

The Stereotypy Rating Inventory for frontotemporal lobar degeneration.

A many behavioral disturbances, Stereotypic behaviors are among the best discriminators of Frontotemporal Lobar Degeneration (FTLD). A recent preliminary report suggests many of the behavioral symptoms, including stereotypic behaviors in FTLD patients, respond to medication with selective serotonin re-uptake inhibitors. However, there is no scale that evaluates stereotypic behaviors comprehensively. To assess the wide range of stereotypic behaviors encountered in FTLD, we developed a new instrument, the Stereotypy Rating Inventory (SRI). The SRI assesses five distinctive stereotypic behavioral disturbances often seen in patients with FTLD: eating and cooking behaviors, roaming, speaking, movements, and daily rhythm. The SRI uses the same technique as the Neuropsychiatric Inventory (NPI) in that both the frequency and the severity of each behavior are determined. The studies reported here demonstrate the content and concurrent validity, as well as inter-rater and test-retest reliability, of the instrument. Scores of FTLD patients (n=26) on the SRI were much higher than those of patients with Alzheimer's disease (n=46), patients with vascular dementia (n=26), and normal control subjects (n=40). The SRI appears to be a useful instrument for detecting stereotypic behaviors and monitoring of therapies in FTLD patients.