Welmoed A Krudop1, Cora J Kerssens2, Annemiek Dols2, Niels D Prins3, Christiane Möller3, Sigfried Schouws2, Wiesje M van der Flier4, Philip Scheltens3, Sietske Sikkes4, Max L Stek2, Yolande A L Pijnenburg3. 1. Alzheimer Center and Department of Neurology, VU University Medical Center, Amsterdam, the Netherlands. Electronic address: w.krudop@vumc.nl. 2. Department of Old Age Psychiatry, GGZInGeest, Amsterdam, the Netherlands. 3. Alzheimer Center and Department of Neurology, VU University Medical Center, Amsterdam, the Netherlands. 4. Alzheimer Center and Department of Neurology, VU University Medical Center, Amsterdam, the Netherlands; Department of Epidemiology and Biostatistics, VU University Medical Center, Amsterdam, the Netherlands.
Abstract
OBJECTIVE: The behavioral variant of frontotemporal dementia (bvFTD) can be difficult to diagnose because of the extensive differential diagnosis, including many other diseases presenting with a frontal lobe syndrome. We aimed to identify the diagnostic spectrum causing a late onset frontal lobe syndrome and examine the quality of commonly used instruments to distinguish between bvFTD and non-bvFTD patients, within this syndrome. METHODS: A total of 137 patients fulfilling the criteria of late onset frontal lobe syndrome, aged 45 to 75 years, were included in a prospective observational study. Diagnoses were made after clinical and neuropsychological examination, and neuroimaging and cerebral spinal fluid results were taken into account. Baseline characteristics and the scores on the Mini-Mental State Exam (MMSE), frontal assessment battery (FAB), Frontal Behavioral Inventory (FBI), and Stereotypy Rating Inventory (SRI) were compared between the bvFTD and the non-bvFTD group. RESULTS: Fifty-five (40%) of the patients received a bvFTD diagnosis (33% probable and 7% possible bvFTD). Fifty-one patients (37%) had a psychiatric disorder, including 20 with major depressive disorder. Thirty-one patients received an alternative neurological, including neurodegenerative, diagnosis. MMSE and FAB scores were unspecific for a particular diagnosis. A score above 12 on the positive FBI subscale or a score above 5 on the SRI were indicative of a bvFTD diagnosis. CONCLUSION: A broad spectrum of both neurological and psychiatric disorders underlies late onset frontal lobe syndrome, of which bvFTD was the most prevalent diagnosis in our cohort. The commonly used MMSE and the FAB could not successfully distinguish between bvFTD and non-bvFTD, but this could be achieved with the more specific FBI and SRI.
OBJECTIVE: The behavioral variant of frontotemporal dementia (bvFTD) can be difficult to diagnose because of the extensive differential diagnosis, including many other diseases presenting with a frontal lobe syndrome. We aimed to identify the diagnostic spectrum causing a late onset frontal lobe syndrome and examine the quality of commonly used instruments to distinguish between bvFTD and non-bvFTD patients, within this syndrome. METHODS: A total of 137 patients fulfilling the criteria of late onset frontal lobe syndrome, aged 45 to 75 years, were included in a prospective observational study. Diagnoses were made after clinical and neuropsychological examination, and neuroimaging and cerebral spinal fluid results were taken into account. Baseline characteristics and the scores on the Mini-Mental State Exam (MMSE), frontal assessment battery (FAB), Frontal Behavioral Inventory (FBI), and Stereotypy Rating Inventory (SRI) were compared between the bvFTD and the non-bvFTD group. RESULTS: Fifty-five (40%) of the patients received a bvFTD diagnosis (33% probable and 7% possible bvFTD). Fifty-one patients (37%) had a psychiatric disorder, including 20 with major depressive disorder. Thirty-one patients received an alternative neurological, including neurodegenerative, diagnosis. MMSE and FAB scores were unspecific for a particular diagnosis. A score above 12 on the positive FBI subscale or a score above 5 on the SRI were indicative of a bvFTD diagnosis. CONCLUSION: A broad spectrum of both neurological and psychiatric disorders underlies late onset frontal lobe syndrome, of which bvFTD was the most prevalent diagnosis in our cohort. The commonly used MMSE and the FAB could not successfully distinguish between bvFTD and non-bvFTD, but this could be achieved with the more specific FBI and SRI.
Authors: Simon Ducharme; Annemiek Dols; Robert Laforce; Emma Devenney; Fiona Kumfor; Jan van den Stock; Caroline Dallaire-Théroux; Harro Seelaar; Flora Gossink; Everard Vijverberg; Edward Huey; Mathieu Vandenbulcke; Mario Masellis; Calvin Trieu; Chiadi Onyike; Paulo Caramelli; Leonardo Cruz de Souza; Alexander Santillo; Maria Landqvist Waldö; Ramon Landin-Romero; Olivier Piguet; Wendy Kelso; Dhamidhu Eratne; Dennis Velakoulis; Manabu Ikeda; David Perry; Peter Pressman; Bradley Boeve; Rik Vandenberghe; Mario Mendez; Carole Azuar; Richard Levy; Isabelle Le Ber; Sandra Baez; Alan Lerner; Ratnavalli Ellajosyula; Florence Pasquier; Daniela Galimberti; Elio Scarpini; John van Swieten; Michael Hornberger; Howard Rosen; John Hodges; Janine Diehl-Schmid; Yolande Pijnenburg Journal: Brain Date: 2020-06-01 Impact factor: 13.501