scholarly journals Right temporal variant frontotemporal dementia is pathologically heterogeneous: a case-series and a systematic review

2021 ◽  
Vol 9 (1) ◽  
Author(s):  
Hulya Ulugut ◽  
Anke A. Dijkstra ◽  
Marta Scarioni ◽  
Frederik Barkhof ◽  
Philip Scheltens ◽  
...  
Keyword(s):  
Systematic Review ◽  
Frontotemporal Dementia ◽  
Case Series ◽  
Primary Progressive Aphasia ◽  
General Assumption ◽  
Large Sample Size ◽  
Pathological Evaluation ◽  
Type C ◽  
The Right ◽  
Underlying Pathology

AbstractAlthough the right temporal variant frontotemporal dementia (rtvFTD) is characterised by distinct clinical and radiological features, its underlying histopathology remains elusive. Being considered a right-sided variant of semantic variant primary progressive aphasia (svPPA), TDP-43 type C pathology has been linked to the syndrome, but this has not been studied in detail in large cohorts. In this case report and systematic review, we report the autopsy results of five subjects diagnosed with rtvFTD from our cohort and 44 single rtvFTD subjects from the literature. Macroscopic pathological evaluation of the combined results revealed that rtvFTD demonstrated either a frontotemporal or temporal evolution, even if the degeneration started in the right temporal lobe initially. FTLD-TDP type C was the most common underlying pathology in rtvFTD, however, in 64% of rtvFTD, other underlying pathologies than FTLD-TDP type C were present, such as Tau-MAPT and FTLD-TDP type A and B. Additionally, accompanying motor neuron or corticospinal tract degeneration was observed in 28% of rtvFTD patients. Our results show that in contrast to the general assumption, rtvFTD might not be a pure FTLD-TDP type C disorder, unlike its left temporal counterpart svPPA. Large sample size pathological studies are warranted to understand the diverse pathologies of the right and left temporal variants of frontotemporal dementia.

scholarly journals The Right Temporal Variant of Frontotemporal Dementia is Not Genetically Sporadic: A Case Series

2021 ◽  
pp. 1-7
Author(s):  
Hulya Ulugut Erkoyun ◽  
Sven J. van der Lee ◽  
Bas Nijmeijer ◽  
Rosalina van Spaendonk ◽  
Anne Nelissen ◽  
...  
Keyword(s):  
Frontotemporal Dementia ◽  
Genetic Variant ◽  
Autosomal Dominant ◽  
Case Series ◽  
Primary Progressive Aphasia ◽  
Clinical Syndrome ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Memory Clinics ◽  
The Right

Background: Right temporal variant frontotemporal dementia (rtvFTD) has been generally considered as a right sided variant of semantic variant primary progressive aphasia (svPPA), which is a genetically sporadic disorder. Recently, we have shown that rtvFTD has a unique clinical syndrome compared to svPPA and behavioral variant frontotemporal dementia. Objective: We challenge the assumption that rtvFTD is a sporadic, non-familial variant of FTD by identifying potential autosomal dominant inheritance and related genes in rtvFTD. Methods: We collected all subjects with a diagnosis of FTD or primary progressive aphasia who had undergone genetic screening (n = 284) and subsequently who had a genetic variant (n = 48) with a diagnosis of rtvFTD (n = 6) in 2 specialized memory clinics. Results: Genetic variants in FTD related genes were found in 33% of genetically screened rtvFTD cases; including MAPT (n = 4), GRN (n = 1), and TARDBP (n = 1) genes, whereas only one svPPA case had a genetic variant in our combined cohorts. Additionally, 4 out of 6 rtvFTD subjects had a strong family history for dementia. Conclusion: Our results demonstrate that rtvFTD, unlike svPPA, is not a pure sporadic, but a heterogeneous potential genetic variant of FTD, and screening for genetic causes for FTD should be performed in patients with rtvFTD.

scholarly journals RIGHT VS. LEFT TEMPORAL LOBE SEMIOLOGY IN DEMENTIA: LESSONS FROM TWO CASES WITH FOCAL FRONTOTEMPORAL DEMENTIA SYNDROMES

2021 ◽  
Author(s):  
Vitor Arca ◽  
Pedro Albuquerque ◽  
Victor Correia ◽  
Amanda Pires ◽  
Hugo Araújo ◽  
...  
Keyword(s):  
Frontotemporal Dementia ◽  
Temporal Lobe ◽  
Word Processing ◽  
Brain Mri ◽  
Cerebral Atrophy ◽  
Primary Progressive Aphasia ◽  
Left Temporal Lobe ◽  
Her Family ◽  
Mini Mental Status Examination ◽  
The Right

Background: Case 1: a 59-year old man presented to our service with 4 years of progressive cognitive and behavioral symptoms. He became forgetful and experienced difficulties managing his payments. After 4 years he could no longer recognise his relatives. Cognitive assessment showed a mini-mental status examination of 17/30. MRI and SPECT revealed respectively focal atrophy and hipoperfusion of the frontal regions and anterior right temporal lobe. Case 2: a 72-year-old woman was brought to evaluation with a 5-years history of progressive language and behavioral deterioration. Her family reported early speech errors and behavioral changes, with a marked aggressiveness, ritualistic behaviors and hyperorality. Cognitive evaluation revealed a MMSE of 6/30 mainly due to a relatively fluent afasia. Brain MRI showed asymmetric cerebral atrophy, more prominent in the anterior left temporal lobe. Objective: N/H Methods: N/H Results: N/H Conclusion: We describe two cases of suspected frontotemporal dementia (FTD) syndromes. The left ATL may receive proportionately more input from the lexical and phonological centers subserving word processing. The right ATL may receive more input from right-lateralized emotion processing hubs. Focal atrophy of the left anterior temporal lobe has been associated with the semantic type of primary progressive aphasia evolving to semantic dementia. In contrast, focal atrophy of the right temporal lobe has recently been described as a controversial entity reported as the right temporal variant of FTD.

scholarly journals A systematic review of the prevalence of depression, anxiety, and apathy in frontotemporal dementia, atypical and young-onset Alzheimer’s disease, and inherited dementia

2020 ◽  
pp. 1-20
Author(s):  
Jessica D. Collins ◽  
Susie M. D. Henley ◽  
Aida Suárez-González
Keyword(s):  
Systematic Review ◽  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Frontotemporal Dementia ◽  
Neuropsychiatric Symptoms ◽  
Primary Progressive Aphasia ◽  
Small Sample ◽  
Cortical Atrophy ◽  
Sample Sizes ◽  
Young Onset

ABSTRACT Objectives: Depression, anxiety, and apathy are the most commonly reported neuropsychiatric symptoms (NPS) in Alzheimer’s disease (AD). Understanding their prevalence in rarer dementias such as frontotemporal dementia (FTD), primary progressive aphasia (PPA), posterior cortical atrophy (PCA), young-onset AD (YOAD), and inherited dementias has implications for both clinical practice and research. In this study, we aimed to examine the current state of knowledge of the prevalence of these three NPS in less prevalent dementias. Design: We conducted a systematic review based on searches of EMBASE, PsycINFO, and PubMed up to September 2019. Results: 47 articles meeting inclusion criteria were identified. Depression, anxiety, and apathy were commonly reported across the phenotypes studied but their prevalence showed large variation between studies. Apathy showed the highest reported frequency in FTD (50–100% across studies), behavioral variant frontotemporal dementia (bvFTD) (73–100%), and YOAD (44–100%). Anxiety was frequently reported in FTD (0–100%) and bvFTD (19–63%). Depression showed the highest prevalence in FTD (7–69%) and YOAD (11–55%). Among the three variants of PPA, sv-PPA is the one most investigated (seven articles). Three or fewer articles were identified examining NPS in the remaining PPA variants, PCA, familial AD, and familial FTD. Inconsistency in the tools used to measure symptoms and small sample sizes were common methodological limitations. Conclusions: Future studies should consider the inclusion of larger sample sizes (e.g. through multicenter collaborations) and the use of harmonized protocols that include the combination of caregiver and patient-derived measures and symptom-specific questionnaires. More research is needed on the phenotype-specific barriers and facilitators for people living with dementia to successfully engage in self-reports of NPS.

scholarly journals Anatomical Evaluation of Root and Root Canal Configuration of Permanent Maxillary Dentition in the Population of the Kingdom of Saudi Arabia

2022 ◽  
Vol 2022 ◽  
pp. 1-12
Author(s):  
Mohammed Mashyakhy ◽  
Mohammed Awawdeh ◽  
Abdulaziz Abu-Melha ◽  
Bushra Alotaibi ◽  
Nada AlTuwaijri ◽  
...  
Keyword(s):  
Systematic Review ◽  
Saudi Arabia ◽  
Sample Size ◽  
Root Canal ◽  
Case Series ◽  
Deciduous Teeth ◽  
Large Sample Size ◽  
International Studies ◽  
Type I ◽  
Kingdom Of Saudi Arabia

Aim. This study is aimed at combining the sample sizes of all studies on permanent maxillary teeth conducted in different regions of the Kingdom of Saudi Arabia (KSA) to obtain a large sample size that represents the population of the KSA. The outcome of these combined studies is compared with international studies in terms of the number of roots, number of canals, and canal configurations on the basis of Vertucci’s classification. Methodology. The studies were systematically reviewed using the Preferred Reporting Items for Systematic Review and Meta-analysis chart. Studies were included in the analysis if they were conducted in the KSA, involved permanent human maxillary teeth, and had a sample of more than 10 teeth (power). By contrast, studies were excluded if they involved deciduous teeth in the sample size, investigated nonhuman teeth, were not conducted in the KSA, and were case reports, case series, review studies, and anomalies. Relevant literature was searched from PubMed, Scopus, Web of Science, Embase, Cochrane, and Direct Science by two calibrated teams, starting in August 2020, without time limits or language restrictions. Results. The database searches and cross-referencing identified a total of 19 relevant studies. All maxillary canines ( N = 1,018 ) had one root, whereas 98.4% had one canal and 98.3% had Vertucci type I. Moreover, 63.2% of the maxillary first premolars had two roots, and 91.4% had two canals. The most common Vertucci root canal configuration was type IV (64.6%). The maxillary second premolars mostly had one root (84.4%) and one canal (50.4%). The most common canal configuration was Vertucci type I (47.1%). The majority of maxillary first molars had three roots (98.9%), 48.7% of which had three canals, and 46.4% had four canals. The most prevalent feature of the canal morphology of mesiobuccal roots was Vertucci type II (35.3%). The investigated maxillary second molars had three roots, 88.0% of which had three canals. Conclusion. This systematic review represents the Saudi population since samples were combined from different studies from different regions of the country. Variations in findings were observed in the same group of teeth from different regions and the same region, while the overall combined samples results fell within the range of other international studies.

scholarly journals 593 Colonic Perforation Following Major Burns: A Systematic Review

2021 ◽  
Vol 108 (Supplement_6) ◽  
Author(s):  
M Fadel ◽  
M Iskandarani ◽  
J Cuddihy ◽  
I Jones ◽  
D Collins ◽  
...  
Keyword(s):  
Systematic Review ◽  
Case Reports ◽  
Total Body Surface Area ◽  
Clinical Signs ◽  
Case Series ◽  
Systemic Effect ◽  
Colonic Perforation ◽  
Severe Burns ◽  
Gastrointestinal Stasis ◽  
The Right

Abstract Introduction Major burns complicated by stress ulceration and perforation of the stomach or duodenum is a recognised clinical phenomenon. Colonic perforation in burns patients is seemingly uncommon, and the overall incidence, clinical signs/diagnosis of perforation, intervention required, and mortality is incompletely described in the literature. Method We performed a systematic review of the literature on severe burns resulting in colonic perforation during admission. Relevant studies from January 1975 to June 2020 were retrieved from MEDLINE and EMBASE databases. Patient demographics, total body surface area (TBSA), site of colonic perforation, management and overall outcome were extracted. We present a case series of five major burns patients who had colonic perforations in our Specialist Burns Centre. Results We identified 54 studies, of which nine (two case series and seven case reports) met the inclusion criteria. In most cases, the TBSA associated with a colonic perforation was ≥ 30% (10/16 patients, 63%) and the abdomen was involved in 9/16 patients (56%). Perforations mainly affected the right colon (12/16 patients, 75%), usually occurring after the second week of admission (13/16 patients, 81%). Right-sided colonic perforations were associated with an increased mortality rate compared to left-sided perforations (42% vs 25%). Conclusions The current literature, mainly limited to case series and case reports, confirms that colonic perforations in burns patients are rare. The resulting perforation is related to the systemic effect of burn injuries including sepsis and gastrointestinal stasis. We have identified patients who are at higher risk of developing colonic perforations in order to prompt early diagnosis and intervention.

Amyloid pet in primary progressive aphasia: case series and systematic review of the literature

2016 ◽  
Vol 264 (1) ◽  
pp. 121-130 ◽  
Author(s):  
Alberto Villarejo-Galende ◽  
Sara Llamas-Velasco ◽  
Adolfo Gómez-Grande ◽  
Verónica Puertas-Martín ◽  
Israel Contador ◽  
...  
Keyword(s):  
Systematic Review ◽  
Case Series ◽  
Primary Progressive Aphasia ◽  
Amyloid Pet ◽  
Review Of The Literature ◽  
Progressive Aphasia ◽  
Primary Progressive

Frontotemporal Dementia

2008 ◽  
Author(s):  
David M. Blass
Keyword(s):  
Frontotemporal Dementia ◽  
Cognitive Abilities ◽  
Psychiatric Symptoms ◽  
Age Of Onset ◽  
Hippocampal Sclerosis ◽  
Case Series ◽  
Primary Progressive Aphasia ◽  
Temporal Lobes ◽  
People With Dementia ◽  
Behavioral Abnormalities

Frontotemporal dementia (FTD) is a family of neurodegenerative diseases and syndromes that most commonly involve the frontal and temporal lobes, producing dramatic alterations of personality, behavior, language, and other cognitive abilities (McKhann et al., 2001). Age of onset tends to be younger than in Alzheimer’s disease (AD), with most patients becoming symptomatic in the sixth decade of life. Although population-based epidemiologic studies of FTD have found a prevalence of approximately 5–10 per 100,000 in patients 50 years of age and older, autopsy-based case series have found that approximately 5% to15% of people with dementia have FTD, a discrepancy suggesting that many cases go undiagnosed during life (Rosso et al., 2003). Recent advances in clinicopathologic correlation have revealed that a number of neurologic conditions previously conceived of as independent disease entities such as progressive supranuclear palsy (PSP), corticobasilar degeneration (CBD), and hippocampal sclerosis dementia are in many cases better classified in the FTD family (McKhann et al., 2001; Blass et al., 2004). Many patients with FTD develop other neurologic syndromes as well, including parkinsonism and amyotrophic lateral sclerosis (ALS). FTD is actually a group of clinical syndromes with overlapping neuropathologies. The clinical expression of the disease relates primarily to the anatomic location of disease involvement rather than the neuropathologic subtype; there are many such subtypes. Clinical variants are most distinct early in the disease course, when the degree of anatomic involvement may be limited to discrete regions. As the disease spreads through the brain, many patients have symptoms that become complex and take on char acteristics of other variants. The first clinical FTD variant is one in which behavioral abnormalities and personality changes dominate the clinical presentation. This syndrome is usually associated with disease involvement of the frontal and anterior temporal lobes. In addition, there are two language presentations: primary progressive aphasia and semantic dementia (McKhann et al., 2001). The neurologic syndromes of PSP, CBD, and ALS with dementia are familiar to the neurologist because of their neurologic symptoms; it is noteworthy that patients with any of the previously mentioned syndromes routinely develop the psychiatric symptoms reviewed below.

scholarly journals A clinical-radiological framework of the right temporal variant of frontotemporal dementia

Brain ◽  
2020 ◽  
Vol 143 (9) ◽  
pp. 2831-2843 ◽  
Author(s):  
Hulya Ulugut Erkoyun ◽  
Colin Groot ◽  
Ronja Heilbron ◽  
Anne Nelissen ◽  
Jonathan van Rossum ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Frontotemporal Dementia ◽  
Diagnostic Criteria ◽  
Primary Progressive Aphasia ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Behavioural Changes ◽  
The Right ◽  
Mental Slowness

Abstract The concept of the right temporal variant of frontotemporal dementia (rtvFTD) is still equivocal. The syndrome accompanying predominant right anterior temporal atrophy has previously been described as memory loss, prosopagnosia, getting lost and behavioural changes. Accurate detection is challenging, as the clinical syndrome might be confused with either behavioural variant FTD (bvFTD) or Alzheimer’s disease. Furthermore, based on neuroimaging features, the syndrome has been considered a right-sided variant of semantic variant primary progressive aphasia (svPPA). Therefore, we aimed to demarcate the clinical and neuropsychological characteristics of rtvFTD versus svPPA, bvFTD and Alzheimer’s disease. Moreover, we aimed to compare its neuroimaging profile against svPPA, which is associated with predominant left anterior temporal atrophy. Of 619 subjects with a clinical diagnosis of frontotemporal dementia or primary progressive aphasia, we included 70 subjects with a negative amyloid status in whom predominant right temporal lobar atrophy was identified based on blinded visual assessment of their initial brain MRI scans. Clinical symptoms were assessed retrospectively and compared with age- and sex-matched patients with svPPA (n = 70), bvFTD (n = 70) and Alzheimer’s disease (n = 70). Prosopagnosia, episodic memory impairment and behavioural changes such as disinhibition, apathy, compulsiveness and loss of empathy were the most common initial symptoms, whereas during the disease course, patients developed language problems such as word-finding difficulties and anomia. Distinctive symptoms of rtvFTD compared to the other groups included depression, somatic complaints, and motor/mental slowness. Aside from right temporal atrophy, the imaging pattern showed volume loss of the right ventral frontal area and the left temporal lobe, which represented a close mirror image of svPPA. Atrophy of the bilateral temporal poles and the fusiform gyrus were associated with prosopagnosia in rtvFTD. Our results highlight that rtvFTD has a unique clinical presentation. Since current diagnostic criteria do not cover specific symptoms of the rtvFTD, we propose a diagnostic tree to be used to define diagnostic criteria and call for an international validation.

scholarly journals Safety, Immunogenicity, and Efficacy of COVID-19 Vaccine in Children and Adolescents: A Systematic Review

2021 ◽  
Author(s):  
Meng Lv ◽  
Xufei Luo ◽  
Quan Shen ◽  
Ruobing Lei ◽  
Xiao Liu ◽  
...  
Keyword(s):  
Systematic Review ◽  
Immune Response ◽  
Clinical Trials ◽  
Children And Adolescents ◽  
Protective Efficacy ◽  
Case Reports ◽  
Meta Analysis ◽  
Case Series ◽  
Large Sample Size ◽  
Protective Effects

AbstractAimTo identify the safety, immunogenicity, and protective efficacy of COVID-19 vaccine in children and adolescents.MethodsWe conducted a systematic review. Databases including PubMed, Web of Science, WHO COVID-19 database, and CNKI were searched on 23 July 2021. International Clinical Trials Registry Platform (ICTRP) was also searched to collect ongoing trials. We included published researches or ongoing clinical trials related to the safety, immunogenicity, and efficacy of COVID-19 vaccine in children or adolescents (aged ≤18 years). Meta-analysis was performed if the consistency of the included studies was high. If not, descriptive analyses were performed.ResultsEight published studies with 2851 children or adolescents and 28 ongoing clinical trials were included. Among eight published studies, two (25.0%) were RCTs, two (25.0%) case series, and four (50.0%) case reports. The results showed selected COVID-19 vaccines had a good safety profile in children and adolescents. Injection site pain, fatigue, headache, and chest pain were the most common adverse events. Some studies reported a few cases of myocarditis and pericarditis. Two RCTs showed that the immune response to BNT162b2 in adolescents aged 12-15 years was non-inferior to that in young people aged 16-25 years, while a stronger immune response was detected with 3μg CoronaVac injection. Only one single RCT showed the efficacy of BNT162b2 was 100% (95% CI: 75.3 to 100). Of the 28 ongoing clinical trials, twenty-three are interventional studies. Fifteen countries are conducting interventional clinical trials of COVID-19 vaccines in children and adolescents. Among them, China (10, 43.5%) and United Stated (9, 39.1%) were the top two countries with the most trials. BNT162b2 was the most common vaccine, which is under testing.ConclusionSome of the COVID-19 vaccines have potential protective effects in children and adolescents, but awareness is needed to monitor possible adverse effects after injection. Clinical trials of the COVID-19 vaccine in children and adolescents with long follow-up, large sample size, and different vaccines are still urgently needed.

scholarly journals Robotic-Assisted Simple Prostatectomy: A Systematic Review

2020 ◽  
Vol 9 (6) ◽  
pp. 1798
Author(s):  
Yakup Kordan ◽  
Abdullah Erdem Canda ◽  
Ersin Köseoğlu ◽  
Derya Balbay ◽  
M. Pilar Laguna ◽  
...  
Keyword(s):  
Systematic Review ◽  
Treatment Options ◽  
Case Series ◽  
Operation Time ◽  
Published Data ◽  
Large Sample Size ◽  
Robotic Assisted ◽  
Simple Prostatectomy ◽  
Wide Range ◽  
Laser Enucleation

Contemporary minimally invasive surgical (MIS) treatment options of patients with male Lower Urinary Tract Symptoms (LUTS) in men with prostate glands >80 mL include Holmium Laser Enucleation Prostate (HoLEP), Thulium laser VapoEnucleation Prostate (ThuVEP), and Laparoscopic (LSP) or Robotic-Assisted Simple Prostatectomy (RASP). Implementing new laser technologies is costly, and the steep learning curve of these laser techniques limit their wide range use. This promoted the use of LSP and RASP in centers with readily established laparoscopy or robotic surgery programs. The aim of this study is to review case and comparative series of RASP. We systematically reviewed published data from 2008 to 2020 on RASP and have identified 26 non-comparative and 9 comparative case series. RASP has longer operation time but less time spent in hospital and less blood loss. The outcomes of improvements in symptom score, post-voiding residual urine (PVR), postoperative PSA decline, complications, and cost are similar when compared to open and laser enucleation techniques. These outcomes position RASP as a viable MIS treatment option for patients with male LUTS needing surgical treatment for enlarged prostates. Nevertheless, prospective, randomized controlled trials (RCTs) with multicenter and large sample size are needed to confirm the findings of this systematic review.

Sign in / Sign up

Export Citation Format

Share Document