scholarly journals Neuropathological validation of the MDS-PSP criteria with PSP and other frontotemporal lobar degeneration

2019 ◽  
Author(s):  
Stefano Gazzina ◽  
Gesine Respondek ◽  
Yaroslau Compta ◽  
Kieren S.J. Allinson ◽  
Maria G. Spillantini ◽  
...  
Keyword(s):  
Frontotemporal Dementia ◽  
Diagnostic Criteria ◽  
Sensitivity And Specificity ◽  
Frontotemporal Lobar Degeneration ◽  
Corticobasal Degeneration ◽  
Primary Progressive Aphasia ◽  
Clinical Diagnostic ◽  
Novel Therapeutic Strategies ◽  
Richardson’S Syndrome ◽  
Clinical Diagnostic Criteria

ABSTRACTBackgroundProgressive supranuclear palsy (PSP) is clinically heterogeneous. Clinical diagnostic criteria were revised in 2017, to increase sensitivity and operationalize the diagnosis of PSP Richardson’s syndrome (PSP-RS) and “variant” syndromes (vPSP).ObjectivesTo determine the (1) sensitivity and specificity of the 1996 NINDS-SPSP and 2017 MDS-PSP criteria; (2) false positive rates in frontotemporal dementia with frontotemporal lobar degeneration (FTLD); and (3) clinical evolution of variant PSP syndromes (vPSP).MethodsRetrospective multicenter review of 108 neuropathologically-confirmed PSP patients and 81 patients with other forms of FTLD: 38 behavioral variant frontotemporal dementia (bvFTD), 14 non-fluent/agrammatic variant primary progressive aphasia (nfvPPA), and 29 corticobasal degeneration (CBD), Sensitivity and specificity of the MDS-PSP criteria were compared to the NINDS-SPSP criteria at baseline. In a subset of cases, the timing and frequency of clinical features were compared across groups over six years.ResultsSensitivity for recognition of probable and possible PSP pathology was higher by MDS-PSP criteria (72.2-100%) than NINDS-SPSP criteria (48.1-61.1%). Specificity was higher by NINDS-SPSP criteria (97.5-100%) than MDS-PSP criteria (53.1-95.1%). False positives by MDS-PSP criteria were few for bvFTD (10.5-18.4%) but common for CBD and nfvPPA (fulfilling “suggestive of’ PSP). Most vPSP cases developed PSP-RS-like features within six years, including falls and supranuclear gaze palsy, distinguishing frontal presentations of PSP from bvFTD, and speech/language presentations of PSP from nfvPPA.ConclusionsThe 2017 MDS-PSP criteria successfully identify PSP, including variant phenotypes. This independent validation of the revised clinical diagnostic criteria strengthens the case for novel therapeutic strategies against PSP to include variant presentations.

O1-02-04: Behavioural variant frontotemporal dementia: Limited sensitivity of current clinical diagnostic criteria

2009 ◽  
Vol 5 (4S_Part_3) ◽  
pp. P80-P80
Author(s):  
Olivier Piguet ◽  
Michael Hornberger ◽  
Bhaskara P. Shelley ◽  
Christopher M. Kipps ◽  
John R. Hodges
Keyword(s):  
Frontotemporal Dementia ◽  
Diagnostic Criteria ◽  
Clinical Diagnostic ◽  
Clinical Diagnostic Criteria ◽  
Limited Sensitivity

Frontotemporal lobar degeneration: A consensus on clinical diagnostic criteria

Neurology ◽  
1998 ◽  
Vol 51 (6) ◽  
pp. 1546-1554 ◽  
Author(s):  
D. Neary ◽  
J. S. Snowden ◽  
L. Gustafson ◽  
U. Passant ◽  
D. Stuss ◽  
...  
Keyword(s):  
Diagnostic Criteria ◽  
Frontotemporal Lobar Degeneration ◽  
Clinical Diagnostic ◽  
Clinical Diagnostic Criteria

scholarly journals High sensitivity and specificity in proposed clinical diagnostic criteria for anti-N -methyl-D -aspartate receptor encephalitis

2017 ◽  
Vol 59 (12) ◽  
pp. 1256-1260 ◽  
Author(s):  
Alvin C C Ho ◽  
Shekeeb S Mohammad ◽  
Sekhar C Pillai ◽  
Esther Tantsis ◽  
Hannah Jones ◽  
...  
Keyword(s):  
Diagnostic Criteria ◽  
Sensitivity And Specificity ◽  
High Sensitivity ◽  
Aspartate Receptor ◽  
Clinical Diagnostic ◽  
Clinical Diagnostic Criteria

scholarly journals New Diagnostic Criteria for the Behavioural Variant of Frontotemporal Dementia

2011 ◽  
Vol 6 (4) ◽  
pp. 234 ◽  
Author(s):  
Yolande AL Pijnenburg ◽  
Keyword(s):  
Frontotemporal Dementia ◽  
Diagnostic Criteria ◽  
Accurate Diagnosis ◽  
Clinical Diagnostic ◽  
Starting Point ◽  
Good Starting Point ◽  
New Criteria ◽  
Clinical Diagnostic Criteria

In September 2011, the International Behavioural Variant Frontotemporal Dementia Criteria Consortium established a new set of clinical diagnostic criteria for the behavioural variant of frontotemporal dementia (bvFTD). Following the 1998 criteria by Neary et al., where five core items had to be present to make a diagnosis of bvFTD, the new criteria incorporate six clinical hallmarks and demand that at least three of these are present. Moreover, a degree of probability and a role for neuroimaging findings are introduced within the criteria. The new diagnostic criteria have a sensitivity of 76 % for probable bvFTD and a sensitivity of 86 % for possible bvFTD. They provide a good starting point for a more accurate diagnosis of bvFTD.

scholarly journals Diagnostic difficulties and possibilities of NF1-like syndromes in childhood

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Eva Pinti ◽  
Krisztina Nemeth ◽  
Krisztina Staub ◽  
Anna Lengyel ◽  
Gyorgy Fekete ◽  
...  
Keyword(s):  
Diagnostic Criteria ◽  
Neurofibromatosis Type ◽  
Early Years ◽  
Diagnostic Efficiency ◽  
Clinical Criteria ◽  
Pathogenic Variants ◽  
Clinical Diagnostic ◽  
Long Time ◽  
Diagnostic Difficulties ◽  
Clinical Diagnostic Criteria

Abstract Background Neurofibromatosis type 1 (NF1), which is caused by heterozygous inactivating pathogenic variants in the NF1, has poor phenotypic expressivity in the early years of life and there are numerous conditions, including many other tumor predisposition syndromes, that can mimic its appearance. These are collectively termed NF1-like syndromes and are also connected by their genetic background. Therefore, the NF1’s clinical diagnostic efficiency in childhood could be difficult and commonly should be completed with genetic testing. Methods To estimate the number of syndromes/conditions that could mimic NF1, we compiled them through an extensive search of the scientific literature. To test the utility of NF1’s National Institutes of Health (NIH) clinical diagnostic criteria, which have been in use for a long time, we analyzed the data of a 40-member pediatric cohort with symptoms of the NF1-like syndromes’ overlapping phenotype and performed NF1 genetic test, and established the average age when diagnostic suspicion arises. To facilitate timely identification, we compiled strongly suggestive phenotypic features and anamnestic data. Results In our cohort the utility of NF1’s clinical diagnostic criteria were very limited (sensitivity: 80%, specificity: 30%). Only 53% of children with clinically diagnosed NF1 had a detectable NF1 pathogenic variation, whereas 40% of patients without fulfilled clinical criteria tested positive. The average age at first genetic counseling was 9 years, and 40% of children were referred after at least one tumor had already been diagnosed. These results highlight the need to improve NF1-like syndromes’ diagnostic efficiency in childhood. We collected the most extensive spectrum of NF1-like syndromes to help the physicians in differential diagnosis. We recommend the detailed, non-invasive clinical evaluation of patients before referring them to a clinical geneticist. Conclusions Early diagnosis of NF1-like syndromes can help to prevent severe complications by appropriate monitoring and management. We propose a potential screening, diagnostic and management strategy based on our findings and recent scientific knowledge.

scholarly journals MRI is Unnecessary for Diagnosing Acute Achilles Tendon Ruptures: Clinical Diagnostic Criteria

2012 ◽  
Vol 470 (8) ◽  
pp. 2268-2273 ◽  
Author(s):  
David N. Garras ◽  
Steven M. Raikin ◽  
Suneel B. Bhat ◽  
Nicholas Taweel ◽  
Homyar Karanjia
Keyword(s):  
Achilles Tendon ◽  
Diagnostic Criteria ◽  
Clinical Diagnostic ◽  
Tendon Ruptures ◽  
Clinical Diagnostic Criteria
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