The Diagnostic Challenge of the Late-Onset Frontal Lobe Syndrome

2017 ◽  
Vol 78 (9) ◽  
pp. e1197-e1203 ◽  
Author(s):  
Everard G. B. Vijverberg ◽  
Flora Gossink ◽  
Welmoed Krudop ◽  
Sietske Sikkes ◽  
Cora Kerssens ◽  
...  
2018 ◽  
Vol 31 (5) ◽  
pp. 743-748 ◽  
Author(s):  
Flora T. Gossink ◽  
Everard Vijverberg ◽  
Welmoed Krudop ◽  
Philip Scheltens ◽  
Max L. Stek ◽  
...  

ABSTRACTA late onset frontal lobe syndrome (LOF) refers to a clinical syndrome with apathy, disinhibition, or stereotypical behavior arising in middle or late adulthood. Diagnostics are challenging, and both clinicians and patients need reliable predictors of progression to improve clinical guidance. In this longitudinal multicenter and genetically screened prospective study, 137 LOF patients with frontal behavior (FBI score≥11) and/or stereotypical behavior (SRI≥10) were included. Progression was defined as institutionalization, death, or progression of frontal or temporal atrophy at magnetic resonance imaging (MRI) after two years of follow up. Absence of progression at MRI in addition to stable or improved Mini Mental State Examination and Frontal Assessment Battery scores after two years was indicative for non-progression. The presence of stereotypy and a neuropsychological profile with executive deficits at baseline were found to be predictive for progression, while a history and family history with psychiatric disorders were predictors for non-progression. The combination of these clinical markers had a predictive value of 80.4% (p < 0.05). In patients presenting with late onset behavioral symptoms, an appraisal of the rate of deterioration can be made by detailed mapping of clinical symptoms. Distinction of progressive discourses from non-progressive or treatable conditions is to be gained.


2015 ◽  
Vol 41 (1-2) ◽  
pp. 16-26 ◽  
Author(s):  
Welmoed A. Krudop ◽  
Annemiek Dols ◽  
Cora J. Kerssens ◽  
Niels D. Prins ◽  
Christiane Möller ◽  
...  

Background: The criteria for behavioral variant frontotemporal dementia (bvFTD) incorporate MRI and [18F]-FDG-PET. Cerebrospinal fluid (CSF) analysis is merely advised for excluding Alzheimer's disease. Aims: We aimed to assess the impact of biomarkers on diagnostic certainty and contingent changes of bvFTD diagnosis within the clinically relevant neuropsychiatric differential diagnosis of subjects with a late-onset frontal lobe syndrome (LOF). Methods: We included 137 patients with LOF, aged 45-75 years, 72% males. Biomarker disclosure was considered contributing after any substantial difference in diagnostic certainty or a diagnostic change. Percentages of contributing biomarkers were compared between three major diagnostic groups (bvFTD, psychiatry, other neurological disorders). Certainty levels in stable diagnostic groups were compared to those with a diagnostic change. Results: Biomarkers contributed in 53, 60 and 41% of the LOF patients for MRI, [18F]-FDG-PET and CSF, respectively. Biomarkers changed the diagnosis in 14% of cases towards bvFTD and in 13% from bvFTD into an alternative. Those that changed had a lower level of a priori diagnostic certainty compared to stable diagnoses. Conclusion: Our study not only supports the widely accepted use of MRI and [18F]-FDG-PET in diagnosing or excluding bvFTD, but also shows that CSF biomarkers aid clinicians in the diagnostic process.


2016 ◽  
Vol 41 (3-4) ◽  
pp. 210-219 ◽  
Author(s):  
Everard G.B. Vijverberg ◽  
Annemiek Dols ◽  
Welmoed A. Krudop ◽  
Anne Peters ◽  
Cora J. Kerssens ◽  
...  

Background/Aims: We aimed to prospectively assess the diagnostic accuracy of the revised criteria for behavioural variant frontotemporal dementia (bvFTD) among subjects presenting with a frontal lobe syndrome in middle-late adulthood. Methods: Patients were included based on a predominant behavioural clinical presentation, a Frontal Behavioural Inventory (FBI) score of ≥11 and/or a Stereotypy Rating Inventory (SRI) score of ≥10. At baseline, the fulfilment of the international consensus criteria for behavioural variant FTD (FTDC) was systematically recorded. The 2-year follow-up consensus diagnosis was used as the gold standard to calculate sensitivity and specificity of the FTDC criteria for possible and probable bvFTD. Results: Two-year follow-up data were available for 116 patients (85%). Two-year follow-up consensus diagnoses consisted of probable/definite bvFTD (n = 27), other dementia (n = 30), psychiatric disorders (n = 46) and other neurological disorders (n = 13). Sensitivity for possible bvFTD was 85% (95% CI 70-95%) at a specificity of 27% (95% CI 19-37%). Sensitivity for probable bvFTD was 85% (95% CI 69-95%), whereas their specificity was 82% (95% CI 73-89%). Conclusions: We found a good diagnostic accuracy for FTDC probable bvFTD. However, the specificity for FTDC possible bvFTD was low. Our results reflect the symptomatic overlap between bvFTD, other neurological conditions and psychiatric disorders, and the relevance of adding neuroimaging to the diagnostic process.


2014 ◽  
Vol 10 ◽  
pp. P363-P363
Author(s):  
Samantha Stefanie Orasji ◽  
Welmoed Krudop ◽  
Cora Kerssens ◽  
Max Stek ◽  
Frederik Barkhof ◽  
...  

2015 ◽  
Vol 23 (10) ◽  
pp. 1056-1066 ◽  
Author(s):  
Welmoed A. Krudop ◽  
Cora J. Kerssens ◽  
Annemiek Dols ◽  
Niels D. Prins ◽  
Christiane Möller ◽  
...  

2006 ◽  
Vol 14 (7S_Part_12) ◽  
pp. P657-P658
Author(s):  
Yolande A.L. Pijnenburg ◽  
Everard Vijverberg ◽  
Welmoed A. Krudop ◽  
Flora T. Gossink ◽  
Niels D. Prins ◽  
...  

Author(s):  
Abdul Jaleel ◽  
Pavithran V. M. ◽  
Shanavas Cholakkal ◽  
Vineeth Kadangot Kuthampulli

Abstract Inverted papilloma is an uncommon tumor mostly arising from the lateral wall of the nasal cavity and displays a benign but locally aggressive behavior. Intracranial extension is an extremely rare presentation of inverted papilloma. Extension occurs either as a benign lesion or due to malignant transformation. We report a case of concurrent inverted papilloma and squamous cell carcinoma presenting with epistaxis and recent-onset altered behavior and memory impairment. After literature review of similar cases having inverted papilloma with intracranial extension, we could identify a total of 12 cases, most of which were recurrences of a primary inverted papilloma that were resected before extension into the cranial cavity. Most cases were of extradural extension, and intradural spread resulted in poor prognosis on follow-up. Concurrent inverted papilloma and squamous cell carcinoma extending into the anterior cranial fossa and frontal lobe is a very rare clinical entity and can present as frontal lobe syndrome.


2015 ◽  
Vol 40 (3-4) ◽  
pp. 121-129
Author(s):  
Welmoed A. Krudop ◽  
Sjanne Bosman ◽  
Jeroen J.G. Geurts ◽  
Sietske A.M. Sikkes ◽  
Nicolaas A. Verwey ◽  
...  

Aims: A clinical frontal lobe syndrome (FLS) is generally attributed to functional or structural disturbances within frontal-subcortical circuits. We studied the distribution of pathological brain changes in FLS. Additionally, the prevalence of FLS among various disorders was studied. Methods: We systematically screened clinical files of donors to the Netherlands Brain Bank (n = 2,814) for FLS. A total of 262 FLS cases were identified, and the distribution of postmortem pathological changes within the frontal-subcortical circuits was extracted from their neuropathological reports. Results: In 244 out of 262 patients (93%), pathological changes within the frontal-subcortical circuits were found: 90 subjects (34%) with frontal cortical pathology and 18 (7%) with pathology restricted to subcortical grey matter nuclei, whereas 136 subjects (52%) showed both cortical and subcortical pathology. In 18 subjects (7%), no pathology was found in the examined areas. The prevalence of FLS was highest in frontal-temporal lobar degeneration, followed by progressive supranuclear palsy and vascular dementia [χ2(6, n = 1,561) = 222.64, p < 0.01]. Conclusion: In this large brain bank study, the distribution of pathological changes in subjects with FLS was shown to be frontal-subcortical for the first time. A minority of FLS cases had pathology in the subcortical regions only or no frontal pathology at all.


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