title: Frontotemporal dementia in Oman: cognitive behavioural profile and neuroimaging characteristics; a prospective hospital-based study.
abstract: Frontotemporal dementia is increasingly recognised as an important cause of early-onset dementia and is considered to be the second commonest neurodegenerative dementia after Alzheimer's disease. We describe the cognitive, behavioural profile and neuroimaging characteristics of 6 patients with frontal variant of Frontotemporal dementia that were evaluated at the cognitive behavioural clinic at this tertiary referral teaching hospital. All patients underwent clinical, neuropsychological, structural/functional neuroimaging, and laboratory evaluations. The male to female ratio was 1:1; mean age of onset was 54 years, and the mean duration of symptoms were 30 months. The mean scores for Addenbrooke's cognitive examination, Frontal Assessment Battery, and Mini-Mental State Examination were 70.5, 6.33 and 23.6 respectively. The mean VLOM ratio was 2.04. MRI revealed significant asymmetrical regional frontal/temporal atrophy supplemented by the evidence of circumscribed hypoperfusion in SPECT imaging. We conclude that a combination of behavioural and cognitive assessment using short bedside tests, along with structural and functional neuroimaging does facilitate early identification, and increase the diagnostic specificity of Frontotemporal dementia.
authors.affiliation: Department of Neurology, The Royal Hospital, Ministry of Health, P.O Box 1331,Seeb CPO, Muscat, PC 111, Oman. bpshelley@yahoo.com
authors.forename: Bhaskara P
authors.initials: BP
authors.lastname: Shelley
authors.forename: Jaber
authors.initials: J
authors.lastname: Al-Khabouri
authors.forename: Samir S
authors.initials: SS
authors.lastname: Hussein
authors.forename: Sameer B
authors.initials: SB
authors.lastname: Raniga
mesh_terms: D017668:Age of Onset; D000368:Aged; D001284:Atrophy / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology; D001921:Brain / Q000000981:diagnostic imaging* / Q000473:pathology* / Q000503:physiopathology; D002561:Cerebrovascular Disorders / Q000000981:diagnostic imaging / Q000209:etiology / Q000503:physiopathology; D003072:Cognition Disorders / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D003704:Dementia / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D003937:Diagnosis, Differential; D018450:Disease Progression; D042241:Early Diagnosis; D005260:Female; D006760:Hospitalization / Q000706:statistics & numerical data; D006801:Humans; D040582:Inheritance Patterns; D008279:Magnetic Resonance Imaging; D008297:Male; D001523:Mental Disorders / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D008875:Middle Aged; D009483:Neuropsychological Tests; D009850:Oman; D010375:Pedigree; D011237:Predictive Value of Tests; D011446:Prospective Studies; D015899:Tomography, Emission-Computed, Single-Photon