Probable Frontotemporal Dementia Research Articles

Mortality in FTD: An Australian perspective

AbstractTHEME: Clinical manifestationsTOPIC: Neuropsychiatry & beh neurologySUBTOPIC: NeuropsychiatryBackgroundSurvival duration in frontotemporal dementia (FTD) remains inconsistent, depending on the clinical phenotype with different risk factors identified. Risk factors to mortality include the FTD‐MND presentation, a genetic predisposition (Agarwal S et al. 2019), deficits in neuropsychological tests, imaging changes (Agarwal et al. 2019; Hodges et al. 2003) and non‐tau pathology (Roberson et al. 2005; Hodges et al. 2003). We aimed to identify survival duration and cause of death in this group.MethodAll inpatients admitted to Neuropsychiatry, based at the Royal Melbourne Hospital, in metropolitan Australia from 1992 – 2014, who were diagnosed with probably FTD of any subtype were included. Linkage data was obtained by the Australian Institute of Health and Welfare (AIHW). We reviewed their clinical information including demographics, age of symptom onset, type of presenting symptom, neuroimaging and diagnosis.ResultOf the 528 individual inpatients identified with a dementia, there were n=100 who had a diagnosis of probable FTD. There were n=76 who had a behavioural‐variant FTD (bv‐FTD), n=14 who had a language‐variant FTD (lang‐FTD) and n=10 who had FTD‐MND. 67% of the group had died. Overall median duration of survival was 10.5 years (95% CI 7.9, 12.2). There were no differences in age of death nor age onset between the three FTD‐subtypes (p=0.479, p=0.289, respectively). As expected, there were significant differences in the median survival duration of the three FTD subtypes, with FTD‐MND having the shortest duration (p=0.004). Causes of death based on ICD‐10 were predominantly dementia‐related (26.3% Other degenerative diseases of nervous system; 17.5% unspecified dementia) but also included acute myocardial infarction (7%) and alcohol‐related (7%). Standardised mortality ratios (SMRs) revealed that compared to population norms, people with FTD had 8x mortality rate.ConclusionNot unsurprisingly, FTD‐MND had the shortest duration compared to the other subtypes of FTD. The duration of survival was comparable to other studies. Causes of death revealed that a dementia was identified in almost half of cases. High SMRs suggest that FTD is a particularly “fatal” type of dementia in younger people. Investigation into risk factors to death is required.

Effects of Palmitoylethanolamide Combined with Luteoline on Frontal Lobe Functions, High Frequency Oscillations, and GABAergic Transmission in Patients with Frontotemporal Dementia.

Frontotemporal dementia (FTD) is a presenile neurodegenerative disease for which there is no effective pharmacological treatment. Recently, a link has been proposed between neuroinflammation and FTD. Here, we aim to investigate the effects of palmitoylethanolamide (PEA) combined with luteoline (PEA-LUT), an endocannabinoid with anti-inflammatory and neuroprotective effects, on behavior, cognition, and cortical activity in a sample of FTD patients. Seventeen patients with a diagnosis of probable FTD were enrolled. Cognitive and neurophysiological evaluations were performed at baseline and after 4 weeks of PEA-LUT 700 mg×2/day. Cognitive effects were assessed by Neuropsychiatric Inventory (NPI), Mini-Mental State Examination, Frontal Assessment Battery (FAB), Screening for Aphasia in Neurodegeneration, Activities of Daily Living-Instrumental Activities of Daily Living, and Frontotemporal Lobar Degeneration-modified Clinical Dementia Rating scale. To investigate in vivo neurophysiological effects of PEA-LUT, we used repetitive and paired-pulse transcranial magnetic stimulation (TMS) protocols assessing LTP-like cortical plasticity, short-interval intracortical inhibition, long-interval intracortical inhibition (LICI), and short-latency afferent inhibition. Moreover, we used TMS combined with EEG to evaluate the effects on frontal lobe cortical oscillatory activity. Treatment with PEA-LUT was associated with an improvement in NPI and FAB scores. Neurophysiological evaluation showed a restoration of LICI, in particular at ISI 100 ms, suggesting a modulation of GABA(B) activity. TMS-EEG showed a remarkable increase of TMS-evoked frontal lobe activity and of high-frequency oscillations in the beta/gamma range. PEA-LUT could reduce behavioral disturbances and improve frontal lobe functions in FTD patients through the modulation of cortical oscillatory activity and GABA(B)ergic transmission.

CASES OF FRONTO TEMPORAL DEMENTIA PRESENTING WITH PSYCHOSIS

Introduction Fronto Temporal Dementia presents in varied clinical situations, here we are presenting 2 cases that presented to Neurologist with Behavioural symptoms with No cognitive decline and symptoms suggestive of Psychosis. Both the cases were treated with neuroleptics and were considered to be Psychosis. MRI of the brain and Labs were normal. Only PET CT Scan of the brain showed probable Fronto Temporal Dementia. Methods Report of case series Results CASE 1 • 62 Year old female from middle socio economic status, Homemaker with Family history of psychiatric illness in brother was referred by the neurology team with behavioural manifestations, Initially presented with irrelevant talk, talking to self since last 2 years. She gradually started becoming withdrawn, but managed to be independent in her Activities of Daily Living till few months back.Since the last couple of months she was not interested to her house hold chores, confining herself to indoors, and reduced self care. Since couple of months predominantly silent, occasionally responds to what family says and at times does the opposite. No hallucinations and delusions. No history of clear cognitive decline except for occasionally misplacing things which was felt normal. On examination: she was able to walk, mute, active and passive negativism. Labs and MRI brain done were within normal limits. No other significant medical history was present. A Diagnosis of “Catatonia” was made, Trial of lorazepam was given with mild improvement in speech and communication, food intake was better, obeying simple commands better. In discussion with neurologist and a trial of ECT was considered. AS the improvement was marginal a PET CT Scan was done which was Suggestive of Fronto Temporal Dementia. CASE 2 • A 60 year old lady, divorced in a couple of years after marriage, coping well worked as a teacher, Pre-morbidly stubborn and demanding, Family history of depressive disorder in first degree relative Behavioural changes noted at the age of 50 years manifested by being withdrawn, reduced involvement in regular activities, increased food consumption reduced self care, would pass urine stools at inappropriate places and be unmindful. Admitted at general hospital-diagnosed to have Hypertension, Diabeted Mellitus, Hypothyroidism and Irritable Bowel Syndrome. She occasionally mentioned people were talking about her but difficult to elicit other psychotic Symptoms. She was shifted to old age home at Hyderabad where she had leg pains and diagnosed as RLS and responded to treatment. MRI of the brain was normal. During subsequent follow up with the Neurologist she was noted to be “depressed” and started on Escitalopram-unclear response. In the next few months the episodes of passing stools inappropriately came down, hyperphagia persisted and would occasionally report of apprehension that someone may harm her and could see some vague shadows. This prompted her to be started on Amsulpride but stopped in few days as developed EPS at about 200mg/day. Despite stopping medications she started having significant increase in appetite, would start intruding into other peoples rooms and take whatever food items were there. She was subsequently told not to come into the common mess as she would consume large proportions of food from others plate as well. She was threatened to be thrown out of the old age home and they only agreed if she will be restricted to room. Being off all medications, noted to have occasional forgetfulness. Her “hyperphagia” continued and started developing involuntary Right upper limb choreo-athetotic movements. She also was noted to have repeated touching and rubbing of the face. A PET CT scan of the Brian was suggestive of Fronto Temporal Dementia. Conclusions A diagnosis of Fronto temporal Dementia should be suspected in all cases presenting with late onset psychosis and mild cognitive disturbances. Implication of the the diagnosis and treatment will be discussed. This research was funded by NIL

Heart and brain: A new association – Report of a family with “probable frontotemporal dementia and heart block”

Heart and brain: A new association – Report of a family with “probable frontotemporal dementia and heart block”

A Pilot Study Investigating a Novel Non-Linear Measure of Eyes Open versus Eyes Closed EEG Synchronization in People with Alzheimer’s Disease and Healthy Controls

Background: The incidence of Alzheimer disease (AD) is increasing with the ageing population. The development of low cost non-invasive diagnostic aids for AD is a research priority. This pilot study investigated whether an approach based on a novel dynamic quantitative parametric EEG method could detect abnormalities in people with AD. Methods: 20 patients with probable AD, 20 matched healthy controls (HC) and 4 patients with probable fronto temporal dementia (FTD) were included. All had detailed neuropsychology along with structural, resting state fMRI and EEG. EEG data were analyzed using the Error Reduction Ratio-causality (ERR-causality) test that can capture both linear and nonlinear interactions between different EEG recording areas. The 95% confidence intervals of EEG levels of bi-centroparietal synchronization were estimated for eyes open (EO) and eyes closed (EC) states. Results: In the EC state, AD patients and HC had very similar levels of bi-centro parietal synchronization; but in the EO resting state, patients with AD had significantly higher levels of synchronization (AD = 0.44; interquartile range (IQR) 0.41 vs. HC = 0.15; IQR 0.17, p < 0.0001). The EO/EC synchronization ratio, a measure of the dynamic changes between the two states, also showed significant differences between these two groups (AD ratio 0.78 versus HC ratio 0.37 p < 0.0001). EO synchronization was also significantly different between AD and FTD (FTD = 0.075; IQR 0.03, p < 0.0001). However, the EO/EC ratio was not informative in the FTD group due to very low levels of synchronization in both states (EO and EC). Conclusion: In this pilot work, resting state quantitative EEG shows significant differences between healthy controls and patients with AD. This approach has the potential to develop into a useful non-invasive and economical diagnostic aid in AD.

Variante conductual de la demencia frontotemporal como forma de presentación de la degeneración corticobasal

The behavioural variant of frontotemporal dementia is characterised by progressive social, cognitive and personality deterioration associated with several molecular pathologies of frontotemporal lobar dementia (FTLD): FTLD-tau, FTLD-TDP and FTLD-FUS. Its diagnosis requires pathological studies. A 61-year-old male, with a three-year progressive history of behavioural disorder, apathy, poor language skills, perseveration, lack of empathy, bulimia and executive dysfunction. Neuroimaging revealed right-dominant frontal cortical atrophy, and a single-photon emission tomography brain scan showed bilateral frontal hypoperfusion with thalamic and caudate involvement. Clinically, he was diagnosed with probable frontotemporal dementia, behavioural variant. On his death, his brain was donated to the Neurological Tissue Bank and the neuropathological diagnosis was corticobasal degeneration. Corticobasal degeneration is one of the FTLD-tau tauopathies. The 2013 diagnostic criteria for corticobasal degeneration include executive dysfunction and behavioural and personality disorders similar to those of this patient as a clinical phenotype. The anatomoclinical case presented illustrates the absence of any correlation between the clinical phenotype and the underlying neuropathological diagnosis in frontotemporal dementia, and the need to conduct a histopathological study in order to reach a definitive diagnosis.

Clinical overlap between behavioral variant of frontotemporal dementia and bipolar disorder: A case report

The behavioral variant of frontotemporal dementia (FTD) often begins with psychiatric symptoms, including changes in personal conduct and/or interpersonal behavior. Prior to developing cognitive impairment, differentiating FTD from primary psychiatric disorders might be challenging.This work presents a case of a manic episode with psychotic features in a 61-year-old man, whom personality changes and daily life difficulties arouse and persist after optimal management of the active manic and psychotic symptoms. Neuropsychological assessment detailed severe deficits among visuospatial and planning performances. Structural neuroimaging (CT-scan) primary revealed a global pattern of brain volume reduction. Severe perfusion deficits on frontal and both parietal lobes were shown on 99mTc-HMPAO single-photon emission computed tomography (SPECT). The hypothesis of probable FTD (behavioral variant) was established.The present case highlights how putative atypical and late-onset forms of bipolar disorder (BD) might instead progress to FTD. Several links are being advanced between the BD and FTD, for instance the close involvement of the C9ORF72 gene in a group of BD patients which progresses to dementia. These relations have actually been on focus recently. The field is however still relatively unexplored.Disclosure of interestThe authors have not supplied their declaration of competing interest.

The effects of behavioral and psychological symptoms on caregiver burden in frontotemporal dementia, Lewy body dementia, and Alzheimer's disease: clinical experience in China

ABSTRACTBackground and aims: Caregivers of individuals with neurodegenerative diseases, including frontotemporal dementia (FTD), Lewy body dementia (DLB), and Alzheimer's disease (AD), experience high levels of psychological and physical stress, likely due to behavioral and psychological symptoms of dementia (BPSD). This study is the first to simultaneously evaluate the effects of BPSD on caregiver burden in these three types of dementia.Method: A total of 214 dementia patients, including probable FTD (n = 82), DLB (n = 22), and AD (n = 110), as well as their primary caregivers, were assessed using psychological inventories and cognitive evaluation. The FTD group was further divided into the three established clinical variants: behavioral variant frontotemporal dementia (bvFTD, n = 51), non-fluent variant primary progressive aphasia (nfvPPA, n = 15), and semantic variant primary progressive aphasia (svPPA, n = 16). Cognitive impairment and neuropsychiatric symptoms were assessed using the Mini Mental State Examination, Montreal Cognitive Assessment, Clock Drawing Test, and Neuropsychiatric Inventory (NPI), respectively. Caregiver burden was assessed using the Zarit Burden Inventory (ZBI).Results: FTD patients had higher NPI and ZBI scores than DLB and AD patients, whose scores were similar. Logistic regression analysis revealed that the factors influencing caregiver burden for each group were: FTD: total NPI scores, agitation, and aberrant motor behavior; bvFTD: total NPI scores; DLB: total NPI scores; and AD: total NPI scores, onset age, apathy, and ADL. Caregivers of bvFTD patients had the highest levels of burden, which were significantly greater than for caregivers of nfvPPA, svPPA, DLB, and AD patients.Conclusion: BPSD was highly correlated with emotional burden in caregivers of FTD, DLB, and AD patients. The highest burden was observed in bvFTD caregivers.

The value of FDG-PET/CT by using 3-dimensional stereotactic surface projection software analysis in the differential diagnosis of dementia

To retrospectively reevaluate brain fluor-18-fluorodeoxyglucose positron emission tomography (FDG-PET) imaging studies with 3-dimensional stereotactic surface projection (NEUROSTAT) software in order to detect changes in regional brain metabolism and to find out its contribution to the final diagnosis. A total of 48 cases were included in this study. According to clinical evaluation and neuropsychometric test results, there were 17 (35%) patients with probable Alzheimer disease (AD), 17 (35%) patients with probable frontotemporal dementia (FTD), and 14 (30%) patients with undefined advanced dementia. Brain FDG-PET imaging studies were interpreted visually and also using 3-dimensional stereotactic surface projection. Clinic and PET findings were consistent in 20 patients and inconsistent in 14 patients. When consensus diagnosis was taken as the reference, the sensitivity, specificity, accuracy, and positive and negative predictive values of FDG-PET imaging were 93%, 85%, 90%, 90%, and 89% respectively, for AD diagnosis. The same values were 85%, 93%, 90%, 89%, and 90%, respectively, for FTD definition. Using automatized programs that enable quantitative evaluation of regional brain glucose metabolism, in addition to visual evaluation, may increase diagnostic efficiency, as well as minimize interobserver and/or intercenter variability.

Head-to-Head Comparison of 11C-PiB and 18F-AZD4694 (NAV4694) for β-Amyloid Imaging in Aging and Dementia

(11)C-Pittsburgh compound-B ((11)C-PiB) is the benchmark radiotracer for imaging of β-amyloid (Aβ) plaque in Alzheimer disease (AD). (18)F-labeled Aβ tracers subsequently developed for clinical use show higher nonspecific white matter binding and, in some cases, lower cortical binding in AD that could lead to less accurate interpretation of scans. We compared the cortical and white matter binding of a new (18)F-labeled Aβ tracer, (18)F-AZD4694 (recently renamed NAV4694), with (11)C-PiB in the same subjects. Forty-five participants underwent PET imaging with (11)C-PiB and (18)F-AZD4694 (25 healthy elderly controls [HCs], 10 subjects with mild cognitive impairment, 7 subjects with probable AD, and 3 subjects with probable frontotemporal dementia). Images were coregistered so that region-of-interest placement was identical on both scans, and standardized uptake value ratios (SUVRs) using the cerebellar cortex as a reference region were calculated between 40 and 70 min after injection for both tracers. (18)F-AZD4694 showed reversible binding kinetics similar to (11)C-PiB, reaching an apparent steady state at 50 min after injection. Both radiotracers showed a similar dynamic range of neocortical SUVR (1.1-3.3 and 1.0-3.2 SUVR for (11)C-PiB and (18)F-AZD4694, respectively) and identical low nonspecific white matter binding, with frontal cortex-to-white matter ratios of 0.7 ± 0.2 and 1.3 ± 0.2 for both radiotracers in HCs and AD subjects, respectively. There was an excellent linear correlation between (11)C-PiB and (18)F-AZD4694 neocortical SUVR (slope of 0.95, r = 0.99, P < 0.0001). (18)F-AZD4694 displays imaging characteristics nearly identical to those of (11)C-PiB. The low white matter and high cortical binding in AD indicate that this tracer is well suited to both clinical and research use.

Variante frontal de la enfermedad de Alzheimer. Dos casos confirmados anatomopatológicamente y revisión de la bibliografía

Alzheimer's disease (AD) is the most frequent degenerative dementia in our setting. In most patients the initial manifestations of the disease consist in a selective and progressive compromise of memory. Yet, it is not a homogeneous process and in some cases the mode of presentation can be atypical. The presentation of AD in the form of an early disorder affecting personality, behaviour and the executive functions has been called the frontal variant of AD. In our case, its definitive diagnosis was only possible by means of a histological analysis, given the fact that the applicable clinical criteria were then insufficient to reach a diagnosis of this atypical form of AD. We report the cases of two patients, one female and one male aged 60 and 52 respectively, who presented a progressive picture of cognitive impairment with initial involvement of the executive functions and personality changes, together with mood disorders. As a result, the initial diagnosis was one of probable frontotemporal dementia. However, in both cases, the autopsy revealed data consistent with a diagnosis of AD, with a distribution of the pathology that essentially affected the frontal lobes. AD has a heterogeneous form of presentation, which can give rise to errors in its initial diagnosis, since current clinical criteria do not take this clinical variability sufficiently into account. We therefore consider it important to pay attention to the atypical forms of AD with the aim of developing new diagnostic methods that allow AD to be distinguished from other degenerative processes.

Behavioral correlates of cerebrospinal fluid amino acid and biogenic amine neurotransmitter alterations in dementia

BackgroundBehavioral and psychological signs and symptoms of dementia (BPSD) are a heterogeneous group of behavioral and psychiatric disturbances occurring in dementia patients of any etiology. Research suggests that altered activities of dopaminergic, serotonergic, (nor)adrenergic, as well as amino acid neurotransmitter systems play a role in the etiopathogenesis of BPSD. In this study we attempted to identify cerebrospinal fluid (CSF) neurochemical correlates of BPSD to provide further insight into its underlying neurochemical pathophysiological mechanisms. MethodsPatients with probable Alzheimer’s disease (AD; n = 202), probable AD with cerebrovascular disease (n = 37), probable frontotemporal dementia (FTD; n = 32), and probable dementia with Lewy bodies (DLB; n = 26) underwent behavioral assessment and lumbar puncture. CSF levels of six amino acids and several biogenic amines and metabolites were analyzed using ultraperformance liquid chromatography with fluorescence detection and reversed-phase high-performance liquid chromatography with fluorescence detection. ResultsIn the AD patients, CSF homovanillic acid/5-hydroxyindoleacetic acid (HVA/5HIAA) ratios correlated positively with anxieties/phobias, whereas CSF levels of taurine correlated negatively with depression and behavioral disturbances in general. In FTD patients, CSF levels of glutamate correlated negatively with verbally agitated behavior. In DLB patients, CSF levels of HVA correlated negatively with hallucinations. ConclusionsSeveral neurotransmitter systems can be linked to one specific behavioral syndrome depending on the dementia subtype. In addition to biogenic amines and metabolites, amino acids seem to play a major role in the neurochemical etiology of BPSD as well.

Tetrabenazine Treatment for Stereotypies and Tics Associated With Dementia

Stereotypies are frequently reported in frontotemporal dementia. Their pathophysiology and optimal treatment are largely unexplored. The author reports seven cases of marked stereotype in patients with probable frontotemporal dementia who were treated with tetrabenazine, a monoamine inhibitor. All patients had near-complete resolution of stereotypy. Six patients stopped tetrabenazine at some point, with return of their stereotype within 24 hours, which again improved in the four who restarted therapy. Efficacy has not waned over time. Tetrabenazine dramatically improved stereotypies in patients with dementia. This observation could increase understanding of this interesting phenotype.

Domperidone-Induced Tardive Dyskinesia and Withdrawal Psychosis in an Elderly Woman with Dementia

To report a case of probable domperidone-induced tardive dyskinesia and withdrawal psychosis in an elderly woman. A 75-year-old woman was first assessed for cognitive decline and personality changes. On clinical examination, diffuse choreoathetoid movements were noted. Chronic domperidone use seemed the most likely cause for the movement disorder and was abruptly discontinued. Within a few days, she developed a severe psychotic syndrome with Capgras delusions; the movement disorder continued unabated. Both the movement disorder and psychotic symptoms promptly remitted with risperidone treatment. Domperidone has, on rare occasions, been associated with acute extrapyramidal syndromes, especially in young children, but, to our knowledge, this is the first case of domperidone-induced tardive dyskinesia and withdrawal psychosis, adverse effects associated with chronic central nervous system dopaminergic blockade. Domperidone is a dopamine D₂ receptor antagonist that does not usually cross the blood-brain barrier. However, blood-brain barrier alterations occur with aging and dementia, which may explain central nervous system penetration of domperidone in our patient, diagnosed with probable frontotemporal dementia. The association was rated as probable on the Naranjo probability scale. Chronic use of domperidone may, on rare occasions, induce neuropsychiatric syndromes similar to those seen with the use of antipsychotics. This may be more likely in situations in which the blood-brain barrier is damaged, as in vascular and degenerative dementias.

Disruption of temporal processing in a subject with probable frontotemporal dementia

Previous research suggests that the frontal lobes are essential for temporal processing. We report a patient, MN, with probable frontotemporal dementia (FTD) who was tested on a battery of timing tasks with stimuli in the sub- and supra-second range. MN demonstrated a substantial over-estimation and under-production of target intervals on estimation and production tasks respectively but was as accurate as controls on a reproduction task. Furthermore, this deficit was markedly different for auditory and visual stimuli on production and estimation tasks; estimates of the duration of auditory stimuli were three to four times longer than for comparable visual stimuli. She performed normally on a task requiring her to judge whether a stimulus was longer or shorter than a standard duration with both sub- and supra-second stimuli. She performed well on control tasks involving estimation, production and reproduction of line lengths suggesting that her deficits were not attributable to a generalized cognitive impairment or an inability to make magnitude judgments. These data suggest that bifrontal pathology disrupts the “clock” or memory for time.

Confabulation, but not executive dysfunction discriminate AD from frontotemporal dementia

We examined confabulation and performance on frontal/executive tasks in Alzheimer's disease (AD) patients and patients with a diagnosis of probable frontotemporal dementia (FTD). Twenty-two patients with probable AD, 10 patients with probable FTD and 32 normal control subjects entered the study. Executive functions were assessed with the Modified Card Sorting test; a verbal fluency test; the Cognitive Estimation test; and the Stroop test. Confabulations were assessed with a modified version of the Confabulation Battery. The Confabulation Battery included 10 questions tapping each of the following domains: Episodic Memory (memories of personal past episodes), Semantic Memory (knowledge of famous facts and famous people), and Personal Future (personal plans). The results revealed that both AD patients and FTD patients were clearly and equally impaired on tests of executive functions. Both patients' groups confabulated across the three tasks of the Confabulation Battery, but FTD patients confabulated significantly more than AD patients on Episodic Memory and Personal Future. The results failed to provide any evidence of a correlation between the performance on frontal/executive tasks and the tendency to produce confabulatory reports. According to our results, confabulation, more than a deficit of frontal/executive functions, discriminate between AD and FTD. Therefore, screening for confabulation and, possibly, for other types of memory distortions may constitute a useful additional clinical tool in order to discriminate AD from FTD.

Rivastigmine in Frontotemporal Dementia

This preliminary open-label study aims to investigate the effects of rivastigmine, an inhibitor of acetylcholinesterase (AChE) and butyrylcholinesterase (BuChE), in 20 patients diagnosed with frontotemporal dementia (FTD). Study subjects were men and women 60-75 years of age diagnosed with probable FTD. The rivastigmine group received doses of 3-9 mg/day. The control group included matched patients receiving antipsychotics, benzodiazepines and selegiline (deprenyl). All patients completed a 12-month follow-up period. Rivastigmine treatment was well tolerated. At 12 months, there was a general amelioration of behavioural changes as demonstrated by reductions in Neuropsychiatric Inventory (p<0.001 vs baseline and control), Behavioral Pathology in Alzheimer's Disease Rating Scale (p<0.001 vs baseline and control) and Cornell Scale for Depression in Dementia scores (p<0.05 vs baseline, p<0.001 vs control) in the rivastigmine group. Caregiver burden was reduced, as shown by reduced Relative Stress Scale scores (p<0.001 vs baseline and control). Mean scores on outcome measures evaluating executive function stabilised in the rivastigmine group (p<0.05 vs controls). Rivastigmine did not prevent the disease-related deterioration of cognition as assessed using the Mini-Mental State Examination. In this open-label study, rivastigmine-treated patients were less behaviourally impaired, and caregiver burden was reduced, at 12 months, compared with baseline. The use of cholinesterase inhibitors in FTD warrants further research.

A brief neuropsychological assessment for the differential diagnosis between frontotemporal dementia and Alzheimer's disease.

The neuropsychological performance (including measures of language, semantic memory, visual and spatial perception and executive functions) of a group of 14 patients with the clinical diagnosis of probable frontotemporal dementia was compared with that of a group of 14 patients with a clinical diagnosis of probable Alzheimer's disease. The aim was to identify a specific cognitive profile of frontotemporal dementia, which could be used to select a sensitive, short evaluation for the differential diagnosis with Alzheimer's disease. Both groups were severely impaired in most tasks, including those 'frontal lobe' tests which have been suggested to play an important role in differential diagnosis. Significant differences were found only for a minority of tests (oral praxis, visual-spatial perception, and verbal fluency). A logistic regression showed that a shortened testing procedure based on four tests (Rey-Osterreith complex figure test recall, phonemic fluency, oral apraxia, and cube analysis) achieved a 70% sensitivity and 80% specificity for the correct classification of patients in the frontotemporal dementia or Alzheimer's disease group. In conclusion, a brief neuropsychological evaluation including these four tests, as well as other measures sensitive to the frontal impairment, can be useful in the differential diagnosis between the two pathologies, along with the clinical data.

Frontotemporal dementia and neuropsychology: the value of missing values

OBJECTIVETo investigate the effect of missing values due to behavioural disturbances on the neuropsychological test profile in frontotemporal dementia (FTD). The neuropsychological examination of patients with FTD poses a methodological...

Confabulation in a Patient with Fronto-Temporal Dementia and a Patient with Alzheimer's Disease

This paper describes two patients, O.I. and B.Y., with a confabulatory syndrome. O.I. was diagnosed with probable fronto-temporal dementia, whereas B.Y. met the criteria for probable Alzheimer's disease. O.I., but not B.Y., was impaired on tests of frontal/executive functions, and performed better than B.Y. on clinical tests of memory. Both patients confabulated in episodic/autobiographical memory tasks and in personal future planning tasks. B.Y. confabulated also in a semantic memory task. It is argued that the pattern of confabulation and the cognitive profile shown by the two patients is explained better by the hypothesis proposed by Dalla Barba and co-workers (Dalla Barba et al., 1997b) than by current theories of confabulation.