Clinical and Neuropsychological Comparisons of Early-Onset Versus Late-Onset Frontotemporal Dementia: A CREDOS-FTD Study

2015 ◽  
Vol 45 (2) ◽  
pp. 599-608 ◽  
Author(s):  
Byoung Seok Ye ◽  
Seong Hye Choi ◽  
Seol-Heui Han ◽  
SangYun Kim ◽  
Dong-Won Yang ◽  
...  
2019 ◽  
Vol 20 (16) ◽  
pp. 3903 ◽  
Author(s):  
Miriam Ciani ◽  
Cristian Bonvicini ◽  
Catia Scassellati ◽  
Matteo Carrara ◽  
Carlo Maj ◽  
...  

Frontotemporal dementia (FTD) is a common form of dementia among early-onset cases. Several genetic factors for FTD have been revealed, but a large proportion of FTD cases still have an unidentified genetic origin. Recent studies highlighted common pathobiological mechanisms among neurodegenerative diseases. In the present study, we investigated a panel of candidate genes, previously described to be associated with FTD and/or other neurodegenerative diseases by targeted next generation sequencing (NGS). We focused our study on sporadic FTD (sFTD), devoid of disease-causing mutations in GRN, MAPT and C9orf72. Since genetic factors have a substantially higher pathogenetic contribution in early onset patients than in late onset dementia, we selected patients with early onset (<65 years). Our study revealed that, in 50% of patients, rare missense potentially pathogenetic variants in genes previously associated with Alzheimer’s disease, Parkinson disease, amyotrophic lateral sclerosis and Lewy body dementia (GBA, ABCA7, PARK7, FUS, SORL1, LRRK2, ALS2), confirming genetic pleiotropy in neurodegeneration. In parallel, a synergic genetic effect on FTD is suggested by the presence of variants in five different genes in one single patient. Further studies employing genome-wide approaches might highlight pathogenic variants in novel genes that explain the still missing heritability of FTD.


2008 ◽  
Vol 15 (12) ◽  
pp. 1412-1415 ◽  
Author(s):  
B. Borroni ◽  
C. Agosti ◽  
G. Bellelli ◽  
A. Padovani

2021 ◽  
Author(s):  
Nan Lin ◽  
Jing Gao ◽  
Chenhui Mao ◽  
Heyang Sun ◽  
Qiang Lu ◽  
...  

Abstract Background. Alzheimer’s disease (AD) and frontotemporal dementia (FTD) are the two main types of dementia. We aim to investigate the difference between AD and FTD by use of multimodal EEG analyses. Additionally, the difference in correlations between EEG and clinical data was also investigated.Methods. Thirty-one patients diagnosed with AD and 15 patients with FTD were recruited (2008.1-2020.2), along with 24 healthy controls. Clinical data were reviewed. EEG microstate analysis, spectral analyses, and connectivity analysis were performed. Results. Microstate duration was increased in AD for microstate B and increased in FTD for microstate A compared to controls. Correspondingly, microstate C occurrence was decreased in both dementia groups, compared to control group. After divided into early onset and late onset AD, increased mean duration and reduced mean occurrence were observed in early onset AD, compared to late onset AD, with no significant difference in visual EEG score. CSF Aβ42 was correlated to microstate B coverage in AD (r = -0.833, P = 0.010), and microstate D occurrence in FTD (r = 0.786, P = 0.021). ADL and MMSE were also related to visual EEG score and microstate, but for different variables in the two dementia groups. Spectral analysis revealed decreased power in 8-30 Hz and increased power in delta band in both dementia. AD had higher spectral power in the temporal region, compared to FTD. Reduced alpha and beta coherences were demonstrated in AD in bilateral frontal, fronto-temporal, and fronto-occipital connections, and in FTD in the right frontal and fronto-temporal connections. Conclusions. Multimodal EEG analyses show different results between AD and FTD. Reduced coherence is across more brain areas in AD, including intra-anterior and anterior-posterior regions, compared to FTD, which only had frontal-temporal connectivity involved. Spectral analysis revealed a general EEG slowing. Increased microstate duration and decreased occurrence may be attributed to EEG slowing, for different classes in different types of dementia. Microstate may be more sensitive than visual EEG inspection. The correlations with clinical severity and biomarkers indicate that EEG is a potential biomarker for diagnosis and disease assessment.


2021 ◽  
Vol 15 ◽  
Author(s):  
Nan Lin ◽  
Jing Gao ◽  
Chenhui Mao ◽  
Heyang Sun ◽  
Qiang Lu ◽  
...  

BackgroundAlzheimer’s disease (AD) and frontotemporal dementia (FTD) are the two main types of dementia. We investigated the electroencephalogram (EEG) difference and clinical correlation in early-onset Alzheimer’s disease (EOAD), and FTD using multimodal EEG analyses. EOAD had more severe EEG abnormalities than late-onset AD (LOAD). Group comparisons between EOAD and LOAD were also performed.MethodsThirty patients diagnosed with EOAD, nine patients with LOAD, and 14 patients with FTD (≤65 y) were recruited (2008.1–2020.2), along with 24 healthy controls (≤65 y, n = 18; &gt;65 y, n = 6). Clinical data were reviewed. Visual EEG, EEG microstate, and spectral analyses were performed.ResultsCompared to controls, markedly increased mean microstate duration, reduced mean occurrence, and reduced global field power (GFP) peaks per second were observed in EOAD and FTD. We found increased durations of class B in EOAD and class A in FTD. EOAD had reduced occurrences in classes A, B, and C, while only class C occurrence was reduced in FTD. The visual EEG results did not differ between AD and FTD. Microstate B showed correlations with activities of daily living score (r = 0.780, p = 0.008) and cerebrospinal fluid (CSF) Aβ42 (r = −0.833, p = 0.010) in EOAD. Microstate D occurrence was correlated with the CSF Aβ42 level in FTD (r = 0.786, p = 0.021). Spectral analysis revealed a general slowing EEG, which may contribute to microstate dynamic loss. Power in delta was significantly higher in EOAD than in FTD all over the head. In addition, EOAD had a marked increased duration and decreased occurrence than late-onset AD (LOAD), with no group differences in visual EEG results.ConclusionThe current study found that EOAD and FTD had different EEG changes, and microstate had an association with clinical severity and CSF biomarkers. EEG microstate is more sensitive than visual EEG and may be useful for the differentiation between AD and FTD. The observations support that EEG can be a potential biomarker for the diagnosis and assessment of early-onset dementias.


2014 ◽  
Author(s):  
Joseph P. Barsuglia ◽  
Michelle J. Mather ◽  
Hemali V. Panchal ◽  
Aditi Joshi ◽  
Elvira Jimenez ◽  
...  

Author(s):  
Pramod P. Singhavi

Introduction: India has the highest incidence of clinical sepsis i.e.17,000/ 1,00,000 live births. In Neonatal sepsis septicaemia, pneumonia, meningitis, osteomyelitis, arthritis and urinary tract infections can be included. Mortality in the neonatal period each year account for 41% (3.6 million) of all deaths in children under 5 years and most of these deaths occur in low income countries and about one million of these deaths are due to infectious causes including neonatal sepsis, meningitis, and pneumonia. In early onset neonatal sepsis (EOS) Clinical features are non-specific and are inefficient for identifying neonates with early-onset sepsis. Culture results take up to 48 hours and may give false-positive or low-yield results because of the antenatal antibiotic exposure. Reviews of risk factors has been used globally to guide the development of management guidelines for neonatal sepsis, and it is similarly recommended that such evidence be used to inform guideline development for management of neonatal sepsis. Material and Methods: This study was carried out using institution based cross section study . The total number neonates admitted in the hospital in given study period was 644, of which 234 were diagnosed for neonatal sepsis by the treating pediatrician based on the signs and symptoms during admission. The data was collected: Sociodemographic characteristics; maternal information; and neonatal information for neonatal sepsis like neonatal age on admission, sex, gestational age, birth weight, crying immediately at birth, and resuscitation at birth. Results: Out of 644 neonates admitted 234 (36.34%) were diagnosed for neonatal sepsis by the paediatrician based on the signs and symptoms during admission. Of the 234 neonates, 189 (80.77%) infants were in the age range of 0 to 7 days (Early onset sepsis) while 45 (19.23%) were aged between 8 and 28 days (Late onset sepsis). Male to female ratio in our study was 53.8% and 46% respectively. Out of total 126 male neonates 91(72.2%) were having early onset sepsis while 35 (27.8%) were late onset type. Out of total 108 female neonates 89(82.4%) were having early onset sepsis while 19 (17.6%) were late onset type. Maternal risk factors were identified in 103(57.2%) of early onset sepsis cases while in late onset sepsis cases were 11(20.4%). Foul smelling liquor in early onset sepsis and in late onset sepsis was 10(5.56%) and 2 (3.70%) respectively. In early onset sepsis cases maternal UTI, Meconium stained amniotic fluid, Multipara and Premature rupture of membrane was seen in 21(11.67%), 19 (10.56%), 20(11.11%) and 33 (18.33%) cases respectively. In late onset sepsis cases maternal UTI, Meconium stained amniotic fluid, Multipara and Premature rupture of membrane was seen in 2 (3.70%), 1(1.85%), 3 (5.56%) and 3 (5.56%) cases respectively. Conclusion: Maternal risk identification may help in the early identification and empirical antibiotic treatment in neonatal sepsis and thus mortality and morbidity can be reduced.


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