Cognitive Reserve Hypothesis in Frontotemporal Dementia: Evidence from a Brain SPECT Study in a Series of Greek Frontotemporal Dementia Patients.

BACKGROUND AND OBJECTIVE: Cognitive reserve (CR) mediates the clinical expression of brain pathology in Alzheimer's disease, while there are much less relevant data in frontotemporal dementia (FTD). In the present study we examined whether CR, measured using the Cognitive Reserve Index (CRI), correlated with regional cerebral blood flow (rCBF) in Greek FTD patients. METHODS: Eighty FTD patients, i.e., 47 with behavioral variant FTD (bvFTD) and 33 with primary progressive aphasia (PPA), were enrolled into this study. CR was assessed using the CRI questionnaire, which provides a total score (CRI) and 3 subscores, i.e., CRI-education, CRI-working activity, and CRI-leisure time. The FTD-Clinical Dementia Rating Scale was used to assess the severity of dementia and a brain SPECT study was performed to measure rCBF. Finally, multiple regression analyses were conducted to explore correlations between CR indices and frontotemporal rCBF. RESULTS: In both the bvFTD and the PPA groups, higher scores in the CRI, CRI-education, and CRI-leisure time correlated with lower rCBF in the bilateral frontal and left temporal cortex, respectively, controlling for age, sex, time since symptom onset, and disease severity. CONCLUSION: In the present study, lifetime participation in leisure time activities was found to mitigate the burden of disease in bvFTD and PPA patients. Moreover, FTD patients with a higher educational attainment were able to cope better with greater brain damage. Determination of the most suitable activities to build an adequate level of CR is crucial for dementia prevention.

Frontotemporal Lobar Degeneration-Modified Clinical Dementia Rating (FTLD-CDR) Scale and Frontotemporal Dementia Rating Scale (FRS) Correlation With Regional Brain Perfusion in a Series of FTLD Patients.

Severity assessment scales for frontotemporal lobar degeneration (FTLD) have been recently introduced. In the present study, the authors examined whether the FTLD-modified Clinical Dementia Rating (FTLD-CDR) scale and the Frontotemporal Dementia Rating Scale (FRS) correlated with regional brain perfusion in Greek FTLD patients. A total of 47 behavioral variant frontotemporal dementia (bvFTD) patients and 33 primary progressive aphasia (PPA) patients were assessed for demographic data, cognitive reserve (CR), and severity of dementia and underwent brain single-photon emission computed tomography. Both scales were valid in the bvFTD group, predicting frontal lobe perfusion. In the PPA group, both scales were found to predict temporal hypoperfusion only after accounting for CR, which could imply a potential role for reserve in PPA patients.

Adaptation of the Cognitive Reserve Index Questionnaire (CRIq) for the Greek population.

Cognitive reserve (CR) is thought to reflect the cumulative brain potential derived from various cognitively demanding activities throughout the entire life. It seems to mediate both one's cognitive performance and clinical expression of different brain pathologies, such as Alzheimer's disease. Many researchers have tried to assess CR by using proxies, such as educational and occupational level, participation in leisure time activities and intelligence, alone or in various combinations. Recently, a new tool for measuring CR status was constructed, the Cognitive Reserve Index questionnaire (CRIq), comprising of all known CR proxies. CRIq also takes into account the amount of time spent during each of these activities, thus capturing the core idea behind CR theory: its active day to day formulation during all age stages. Aim of the present study was to adapt CRIq for the Greek population. The questionnaire was administered to 591 participants (age range 18-89) stratified in three age groups (young adults, middle-aged, elderly). The middle-aged group showed higher total CRI as well as CRI-Education, CRI-WorkingActivity and CRI-LeisureTime scores compared to both other groups, reflecting more years of engagement in all activities. Gender also influenced CRI scores, with men scoring higher than women, again resulting from historical and social perspectives. Overall, the CRIq showed satisfactory internal consistency, was easy to administer and its adaptation process provided solid and interpretable results. The Greek version of CRIq enriches existing dementia research methodology and allows for valid results in an ever growing field.

Early onset degenerative dementias: demographic characteristics and etiologic classification in a tertiary referral center.

Early onset dementia (EOD) is a major diagnostic challenge as it often presents with atypical features and may be attributed to treatable diseases. Primary degenerative dementias (Alzheimer's disease-AD, frontotemporal lobar degeneration-FTLD, Lewy body dementia-LBD), although traditionally considered to affect older people, are still a main cause of EOD. 491 demented patients were assessed from January 1, 2003 to December 31, 2010 in the Neurology Department of a tertiary referral center. Patients were classified as AD, behavioral variant frontotemporal dementia (bvFTD), non-fluent agrammatic variant primary progressive aphasia (naPPA), semantic variant PPA (svPPA), corticobasal degeneration (CBD), or progressive supranuclear palsy (PSP) who also met criteria for naPPA and LBD. Finally, their demographic characteristics were analysed, according to age at onset (EOD <65 years, late onset dementia-LOD ≥65 years). From the 491 patients, 137 (27.9 %) were EOD. In the EOD group, 52 (38 %) were diagnosed with bvFTD, 34 (24.8 %) with AD, 27 (19.7 %) with naPPA, 10 (7.2 %) with svPPA, 12 (8.8 %) with CBD or PSP, and 2 (1.5 %) with LBD. Demographic characteristics did not differ significantly among diagnostic categories in the EOD group, while in the LOD group FTLD patients were younger and more frequently men compared to both AD and LBD patients. EOD patients had more years of education than LOD patients. Degenerative disorders as causes of EOD are not rare. High clinical alertness is warranted to achieve correct and timely diagnosis.

Spinal cord involvement in chronic inflammatory demyelinating polyradiculoneuropathy: a clinical and MRI study.

Concomitant central nervous system (CNS) involvement in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is rare. Although the spinal nerve roots may present MRI abnormalities in CIDP, hitherto, the spinal cord has been investigated in a single study. We retrospectively investigated clinically and with MRI a cohort of patients with definite CIDP diagnosis (EFNS/PNS criteria) for evidence of brain and spinal cord involvement, who were initially admitted in our department during the last 4 years. Among 12 patients with CIDP (men: 8, mean age: 59.3 years, mean disease duration: 3.8 years), nine patients had their MRI scan during a clinical relapse and three during remission. Brain MRI did not document typical multiple sclerosis lesions in any patient. We did not identify any MRI abnormalities in ten patients without clinical evidence of spinal cord involvement. Conversely, MRI disclosed extensive lesions of the thoracic cord in two patients with an overt spinal cord syndrome, whom we describe. This represents the biggest MRI study of CIDP patients who have been investigated for spinal cord involvement. Our data support earlier observations that a minority of CIDP patients may additionally develop CNS involvement of variable degree.

The frontotemporal dementias in a tertiary referral center: classification and demographic characteristics in a series of 232 cases.

BACKGROUND: Frontotemporal lobar degeneration (FTLD) comprises of behavioral variant frontotemporal dementia (bvFTD) and primary progressive aphasia (PPA) with its 3 main variants, namely nonfluent/agrammatic (naPPA), semantic (svPPA) and logopenic (lvPPA). Recently a clinical syndrome with predominant right temporal atrophy was recognized (rvFTD). FTLD often overlaps with parkinsonism plus syndromes such as corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP), as well as with motor neuron disease (FTD-MND). While FTLD syndromes were thought to be rare and difficult to diagnose ante mortem, revised diagnostic criteria as well as recent studies highlighted the plausibility of accurate clinical diagnosis. METHODS: 232 FTLD patients were assessed from January 1, 2003 to December 31, 2010 in the Neurology Department of a tertiary referral center. Patients were classified as bvFTD, naPPA, svPPA, lvPPA, CBD/PSP and rvFTD and their demographic characteristics were analyzed. RESULTS: From the 232 patients, 111 (47.8%) were diagnosed with bvFTD, 56 (24.1%) with naPPA, 21 (9.1%) with svPPA, 6 (2.6%) with lvPPA, 20 (8.6%) with CBD or PSP and 18 (7.8%) with rvFTD. 44% of the patients were under 65 years old at onset of symptoms, while only 4.3% reported family history of dementia. FTLD subgroups did not differ with respect to demographic characteristics, but early onset cases had higher educational level. DISCUSSION: FTLD represents a syndrome with different but clinically distinguishable phenotypes. Cultural, educational and socioeconomic status differences might regulate patients' access to medical care and therefore influence age of reported onset and prevalence of FTLD in clinical studies. High clinical alertness and sensitive neuropsychological tests could lead to timely clinical diagnosis in a common presenile type of dementia.

Autoimmune autonomic ganglionopathy after a CNS infection.

We report the first Greek case of autoimmune autonomic ganglionopathy seropositive for antibodies against ganglionic acetylcholine receptors, unique for an antecedent viral cerebellitis and long, slowly progressive course, with improvement after treatment with pyridostigmine.

Diagnostic uncertainty in a severely demented male patient: a case report.

INTRODUCTION: Current trends in dementia research focus on early and accurate diagnosis. In clinical practice however, this is not always possible, as multiple underlying pathologies produce mixed dementia syndromes. Furthermore, patients with severe dementia are often underestimated. CASE PRESENTATION: We present a case of a 71 year old Caucasian male with severe Alzheimer's Disease, bedridden and fully dependent in activities of everyday living, whose general cognitive function is almost intact. We emphasize on the diverse underlying pathologies contributing to this intriguing clinical presentation and to diagnostic uncertainty. CONCLUSION: Understanding the complexity of the dementia process in every patient using a multidimensional approach, contributes to more rational management strategies and finally to high quality care for patients and caregivers.