Bradicardia en la demencia frontotemporal

Introduction

Numerous regions of the brain, such as the medial frontal cortex, orbitofrontal cortex, insula, and amygdala, participate in the autonomic control of cardiovascular functions such as heart rate. The degenerative process in frontotemporal dementia (FTD) involves the listed anatomical structures and may therefore produce dysautonomic cardiovascular symptoms.

Aim

To observe whether or not non-cardiogenic bradycardia was more frequent in a group of patients with FTD than in subjects with mild cognitive impairment or dementia of a different aetiology.

Patients and method

Once patients with primary cardiac arrhythmia were excluded, we registered the heart rates of 258 patients with cognitive symptoms (36 with FTD, 22 with Alzheimer disease, 23 with vascular dementia, 10 with other dementias, and 167 with non-dementia cognitive impairment).

Results

Bradycardia (< 60 beats/minute) was significantly more frequent in patients with FTD. This difference remained significant after excluding subjects undergoing treatment with a potentially bradycardic effect. Bradycardia was more prevalent in behavioural FTD cases than in cases of the aphasic variant, and we detected a trend toward higher frequency among patients with more pronounced right hemisphere atrophy. Moreover, mean systolic blood pressure in FTD patients was lower than in other participants, and systolic hypotension (< 120 and < 100 mm Hg) was more prevalent.

Conclusion

Bradycardia was more frequent in the FTD sample than in other patients with cognitive symptoms. Further investigations will be necessary before we may consider bradycardia to be a sign supporting diagnosis of FTD or its behavioural variant.     

Alzheimer's disease and frontal variant of frontotemporal dementia

The aim of this study was to investigate whether a brief neuropsychological battery consisting of a limited number of cognitive tests and an evaluation of the behavioural domains intended to discriminate between frontotemporal dementia (fv–FTD) and Alzheimer's disease (AD), constitutes a useful instrument for making a differential clinical diagnosis between these two pathologies. Nineteen fv–FTD and 39 AD patients were compared on cognitive tasks (assessing memory, executive functions, language and constructional praxis) and on the NPI behavioural assessment. A stepwise discriminant analysis was performed to identify the linear combination of cognitive and behavioural measures able to best discriminate between the two groups. One test for each of the investigated cognitive domains (Delayed Prose Recall, FAS verbal fluency, Boston naming test, Rey's Figure A Copy) and the four subscales of the Neuropsychiatry Inventory (NPI) which best differentiated between fv–FTD and AD patients (apathy, disinhibition, euphoria, aberrant motor behaviour) were used. The analysis selected Rey's Figure A Copy, FAS verbal fluency and NPI apathy subscale as the best discriminants between fv–FTD and AD patients. The final equation assigned 73.7% of the fv–FTD patients and 94.7% of the AD patients to the correct diagnostic group. A validation study conducted on a new independent sample of 11 fv–FTD and 22 AD patients confirmed the high sensitivity (82.6 %) and specificity (81.8%) of the diagnostic equation in assigning fv–FTD and AD patients to the correct dementia group. Although both cognitive and behavioural differences exist between FTD and AD, previous studies have aimed at differentiating the two pathologies by considering the two aspects separately and discriminant analyses were focused only on neuropsychological or neuropsychiatric evaluations. The present results emphasise the importance of rating both cognitive and behavioural clinical features of the two syndromes as objectively as possible to improve differential diagnostic accuracy.     

Cognitive endophenotypes in OCD: A study of unaffected siblings of probands with familial OCD

Background

Impairments in executive functions and non-verbal memory are considered potential endophenotype markers of obsessive–compulsive disorder (OCD). For the neuropsychological deficits to be considered endophenotypes, they should be demonstrable in unaffected family members.

Aim

To compare the neuropsychological performance in unaffected siblings of probands with familial OCD with that of individually matched healthy controls.

Methods

Twenty-five unaffected siblings of OCD probands with familial OCD, and 25 individually matched healthy controls were assessed with tests of attention, executive function, memory and intelligence.

Results

Unaffected siblings showed significant deficits in tests of decision making and behavioural reversal i.e., the Iowa Gambling Task (IGT) and the Delayed Alternation Test (DAT) respectively, but performed adequately in other tests.

Conclusions

Our study suggests that the deficits in decision making and behavioural reversal could be potential endophenotypes in OCD. These deficits are consistent with the proposed neurobiological model of OCD involving the orbitofrontal cortex. Future studies could couple cognitive and imaging strategies to identify neurocognitive endophenotypes in homogenous samples of OCD.     

Accuracy of single-photon emission computed tomography in differentiating frontotemporal dementia from Alzheimer's disease

Background

Alzheimer''s disease (AD) and frontotemporal dementia (FTD) are the commonest causes of presenile dementia. In the absence of a biological marker, diagnosis is reliant on clinical evaluation. Confirmation is often sought from neuroimaging, including single‐photon emission computed tomography (SPECT). Most previous SPECT studies lack pathological validation.

Aim

To examine the accuracy of SPECT in differentiating FTD from AD in patients with subsequent pathological confirmation.

Methods

Technetium‐99‐labelled hexamethyl propylene amine oxime SPECT images obtained at initial evaluation in 25 pathologically confirmed cases of FTD were examined. These images were visually rated by an experienced blinded nuclear medicine consultant and compared with those of 31 patients with AD, also with pathological validation.

Results

A reduction in frontal cerebral blood flow (CBF) was more common in FTD and was of diagnostic value (sensitivity 0.8, specificity 0.65 and likelihood ratio (LR) 2.25; 95% CI 1.35 to 3.77). A pattern of bilateral frontal CBF reduction without the presence of associated bilateral parietal CBF change is diagnostically more accurate (sensitivity 0.80, specificity 0.81 and +LR 4.13, 95% CI 1.96 to 8.71). Diagnostic categorisation (FTD or AD) on the basis of SPECT alone was less accurate than clinical diagnosis (based on neurology and detailed neuropsychological evaluation). One patient with FTD was initially clinically misdiagnosed as AD, owing to the lack of availability of full neuropsychological assessment. However, SPECT correctly diagnosed this patient, providing a diagnostic gain of 4%.

Conclusion

Technetium‐99‐labelled hexamethyl propylene amine oxime SPECT CBF patterns provide valuable information in the diagnosis of FTD and AD. These data can be better used as an adjunct to clinical diagnosis if pathology is to be correctly predicted in life.Frontotemporal dementia (FTD) is a cortical dementia distinct from other dementing illnesses. It typically presents with personality/behavioural change and decline in social conduct with early loss of insight.^1,2 In the absence of biological markers, the pathological detection of characteristic histological changes remains the gold standard of diagnosis. In life, diagnosis is primarily based on patterns of neurological and neuropsychological findings. However, differentiation from other dementias can be difficult and demands an astute qualitative analysis of various behaviours and neuropsychological test performances.³ With a paucity of experienced neuropsychologists, additional and independent diagnostic information is often sought through imaging, be it structural (CT and MRI) and/or functional (single‐photon emission computed tomography (SPECT) and positron emission tomography).SPECT is used to evaluate patients with dementia and can show purported characteristic changes in FTD and in Alzheimer''s disease (AD).^4,5,6,7,8,9 The technique provides a method of evaluating blood flow in various regions of the brain, which reflects areas of poor function by showing reductions in regional cerebral blood flow (CBF). It has been shown that posterior changes in regional CBF are common in AD,^4,5,6,7 whereas in FTD anterior changes are prevalent^7,8,9 and posterior changes rare.⁷However, CBF changes are neither wholly specific nor invariable in various dementing illnesses. Masterman et al¹⁰ looked at the value of bitemporal hypoperfusion in diagnosing AD, and found that, although a sensitive measure for detecting dementia (0.75), it was poorly specific for AD (0.55). Consequently, bitemporal hypoperfusion on SPECT can be a non‐specific finding in various forms of dementia and is not exclusive to AD. Starkstein et al¹¹ reported deficits in CBF in the frontal (especially orbitofrontal) and anterior temporal cortices in FTD. However, they provided neither the measure of the diagnostic accuracy of SPECT in FTD nor of the diagnostic gain it may provide. Most of these studies are also limited by the fact that the dementia groups are defined clinically. The clinical diagnostic accuracy of FTD in life varies hugely between 14–85%.^12,13,14A few studies have looked at the accuracy of clinical and SPECT findings in relation to the final pathological diagnoses.^{15,16,17,18,19} Although these studies found that SPECT findings do correlate with dementia type, they failed to enquire whether SPECT provides any additional diagnostic gain over clinical judgement. These studies are also severely limited by the small numbers of patients in the FTD groups.The aims of this study include evaluation of the diagnostic accuracy of SPECT in differentiating FTD from AD at initial assessment in a group of patients with final pathological confirmation of diagnosis. We also examined the diagnostic gain SPECT may provide over clinical diagnosis of FTD from among this group of patients with FTD and AD.     

The Frontal Assessment Battery (FAB): normative data from an Italian sample and performances of patients with Alzheimer's disease and frontotemporal dementia

The Frontal Assessment Battery (FAB) is a short neuropsychological tool aiming to assess executive functions at the bedside. Two-hundred and thirty-six normal controls were administered the FAB and three other tasks assessing attentional and executive functions. The FAB was also administered to 28 patients suffering from mild Alzheimer's disease (AD, n. 15 subjects) or frontotemporal dementia (FTD, n. 13 subjects). The FAB showed good concurrent and discriminant validity and high internal consistency. Test-retest and inter-rater reliability were fairly good. A multiple regression analysis showed a significant positive effect of education and a negative effect of age. Cut-off values of non-parametric distribution were computed. A difference on FAB scores within dementia patients was observed, with subjects with FTD performing worse than patients suffering from AD.     

Traducción al español y validación de una batería ejecutiva (BE25) y su versión abreviada (ABE12) para la detección de disfunción ejecutiva en demencias

Introduction

There is a need for clinically administered instruments capable of detecting executive dysfunction in dementia.

Objective

The translation and validation of Executive Battery 25 (EB25) and a short version for screening of executive dysfunction in dementia. Methods: The original battery was translated and validated using convergent and divergent correlation in 66 mild dementia patients (CDR 1) matched with 66 controls. EB25 consists of 25 items which detect executive dysfunction. Convergent correlation was made with 7 tests assessing executive dysfunction, the Frontal Systems Behaviour Scale (FrSBe) and Disability Fast Assessment Scale.

Results

Patients had higher scores than controls and correlated with the Stroop Test, verbal fluency test and Frontal Behaviour Inventory. Only 12 out of 25 items were needed to separate both groups, which were used to build an abbreviated Executive Battery with equal psychometric properties and discriminative power. The cut-off point for EB25 was 12, and 7 for the abbreviated version. A cut-off point of 12 was able to discriminate between ¿Alzheimer's disease? (AD) and frontotemporal lobe dementia (FTLD).

Conclusions

EB25 and AEB12 enable executive dysfunction to be detected in mild dementia. On the other hand, AEB12 exhibits better psychometric properties than the original battery, allowing discrimination between AD and FTLD and is completed in less time.     

Estadio evolutivo de los pacientes con enfermedad de Alzheimer que acuden a la consulta especializada en España. Estudio EACE

Introduction

The diagnostic paradigm of Alzheimer disease (AD) is changing; there is a trend toward diagnosing the disease in its early stages, even before the complete syndrome of dementia is apparent. The clinical stage at which AD is usually diagnosed in our area is unknown. Therefore, the purpose of this study is to describe the clinical stages of AD patients at time of diagnosis.

Methods

Multicentre, observational and cross-sectional study. Patients with probable AD according to NINCDS-ARDRA criteria, attended in specialist clinics in Spain, were included in the study. We recorded the symptom onset to evaluation and symptom onset to diagnosis intervals and clinical status of AD (based on MMSE, NPI questionnaire, and CDR scale).

Results

Participants in this study included 437 specialists representing all of Spain's autonomous communities and a total of 1,707 patients, of whom 1,694 were included in the analysis. Mean MMSE score was 17.6±4.8 (95% CI: 17.4-17.9). Moderate cognitive impairment (MMSE between 10 and 20) was detected in 64% of the patients, and severe cognitive impairment (MMSE<10) in 6%. The mean interval between symptom onset and the initial primary care visit was 10.9±17.2 months (95% CI: 9.9-11.8), and the interval between symptom onset and diagnosis with AD was 28.4±21.3 months.

Conclusions

Results from the EACE show that most AD patients in our area have reached a moderate clinical stage by the time they are evaluated in a specialist clinic.     

Pyramidal and extrapyramidal scale (PEPS): a new scale for the assessment of motor impairment in vascular cognitive impairment associated with small vessel disease

Objective

Vascular cognitive impairment associated with small vessel disease (sVCI) may manifest as both cognitive and motor dysfunctions. However, few instruments exist for systematically assessing motor symptoms in sVCI, even though many neuropsychological tests exist to evaluate cognitive function. We developed a new scale for assessing motor impairments and evaluated the reliability and validity of the scale in patients with sVCI.

Methods

A new motor scale, called the PEPS (Pyramidal and Extra Pyramidal Scale for sVCI), consisted of 34 items (for 60 total points) with 5 subdomains: corticospinal, corticobulbar, extrapyramidal signs, gait abnormalities, and gait severity. The PEPS was compared between 75 patients with sVCI and 73 control patients who had dementia or mild cognitive impairment (MCI) without ischemia.

Results

The PEPS had good interrater and test-retest reliability, and it was moderately to highly correlated with the UPDRS, NIHSS, MMSE, CDR, and ADL scales. An optimal cut-off score of PEPS to discriminate dementia or MCI patients with ischemia from those without ischemia was 6.5 with a sensitivity of 88% and a specificity of 100%.

Conclusion

The PEPS is a reliable and valid scale that can be used to assess and monitor motor impairment in patients with vascular cognitive impairment due to small vessel disease.     

Comparison of quantitative EEG between patients with Alzheimer’s disease and those with Parkinson’s disease dementia

Objective

Dementia frequently occurs in Parkinson’s disease (PD) but its pathophysiological basis is little known. Comparative EEG studies of Alzheimer’s disease (AD) and Parkinson’s disease dementia (PDD) are still rare, but could provide knowledge on the different pathophysiological mechanisms involved. The objective of the present study was to comparatively evaluate the absolute power and coherence on the EEG for patients with AD and PDD.

Methods

This study assessed 38 adults with AD, 12 with PDD, 31 with Parkinson’s disease without dementia, and 37 controls (CG) by a neurological evaluation, CERAD neuropsychological battery, executive functions tests and qEEG, calculating global absolute powers for the delta, theta, alpha and beta bands and inter- and intra-hemispheric coherences.

Results

The delta and theta powers were highest in PDD and lowest in CG (p < 0.05). The beta frontal-occipital inter-hemispheric coherence was highest in PDD (p < 0.05). Whereas, alpha and beta frontal inter-hemispheric coherence was highest in PDD and lowest in AD (p < 0.05).

Conclusion

These results suggest that qEEG power and coherence measures are different in AD and PDD.

Significance

These qEEG differences must be related to the distinct mechanisms of cortical neural connections in AD and PDD.     

Qualitative aspects of learning, recall, and recognition in dementia

Objective:

To determine whether learning and serial position effect (SPE) differs qualitatively and quantitatively among different types of dementia and between dementia patients and controls; we also wished to find out whether interference affects it.

Materials and Methods:

We administered the Malayalam version of the Rey Auditory Verbal Learning Test (RAVLT) to 30 cognitively unimpaired controls and 80 dementia patients [30 with Alzheimer''s disease (AD), 30 with vascular dementia (VaD), and 20 with frontotemporal dementia (FTD)] with mild severity on the Clinical Dementia Rating Scale.

Results:

All groups were comparable on education and age, except the FTD group, who were younger. Qualitatively, the learning pattern and SPE (with primacy and recency being superior to intermediate) was retained in the AD, VaD, and control groups. On SPE in free recall, recency was superior to intermediate in the FTD group (P < 0.01 using Bonferroni correction). On recognition, the AD and VaD groups had more misses (P < 0.01), while the FTD group had more false positives (P < 0.01).

Conclusion:

Quantitative learning is affected by dementia. The pattern of qualitative learning remains unaltered in dementia in the early stages.     

Demencia frontotemporal variante conductual: aproximación clínica y terapéutica

Introduction

Behavioural variant frontotemporal dementia (bvFTD) is the most frequent presentation in the clinical spectrum of frontotemporal dementia (FTD) and it is characterised by progressive changes in personality and conduct. Major breakthroughs in molecular biology and genetics made during the last two decades have lent us a better understanding of this syndrome, which may be the first manifestation in many different neurodegenerative diseases.

Development

We reviewed the main epidemiological, clinical, diagnostic and therapeutic aspects of bvFTD. Most cases manifest sporadically and the average age of onset is 58 years. Current criteria for bvFTD propose three levels of diagnostic certainty: possible, probable, and definite. Clinical diagnosis is based on a detailed medical history provided by family members and caregivers, in conjunction with neuropsychological testing. Treatments which have been used in bvFDT to date are all symptomatic and their effectiveness is debatable. New drugs designed for specific molecular targets that are implicated in frontotemporal lobar degeneration are being developed.

Conclusions

BvFDT is a frequent cause of dementia. It is a non-specific syndrome associated with heterogeneous histopathological and biomolecular findings. The definition of clinical subtypes complemented by biomarker identification may help predict the underlying pathology. This knowledge, along with the development of drugs designed for molecular targets, will offer new treatment possibilities.     

Longitudinal cognitive biomarkers predicting symptom onset in presymptomatic frontotemporal dementia

Introduction

We performed 4-year follow-up neuropsychological assessment to investigate cognitive decline and the prognostic abilities from presymptomatic to symptomatic familial frontotemporal dementia (FTD).

Methods

Presymptomatic MAPT (n?=?15) and GRN mutation carriers (n?=?31), and healthy controls (n?=?39) underwent neuropsychological assessment every 2 years. Eight mutation carriers (5 MAPT, 3 GRN) became symptomatic. We investigated cognitive decline with multilevel regression modeling; the prognostic performance was assessed with ROC analyses and stepwise logistic regression.

Results

MAPT converters declined on language, attention, executive function, social cognition, and memory, and GRN converters declined on attention and executive function (p?<?0.05). Cognitive decline in ScreeLing phonology (p?=?0.046) and letter fluency (p?=?0.046) were predictive for conversion to non-fluent variant PPA, and decline on categorical fluency (p?=?0.025) for an underlying MAPT mutation.

Discussion

Using longitudinal neuropsychological assessment, we detected a mutation-specific pattern of cognitive decline, potentially suggesting prognostic value of neuropsychological trajectories in conversion to symptomatic FTD.

     

Health related quality of life changes in somatising patients after individual versus group cognitive behavioural therapy: A randomized clinical trial

Objective

To assess changes in health related quality of life after a cognitive behavioural program for patients diagnosed with abridged somatization disorder in primary care.

Method

A multicentre, randomized, parallel group, controlled trial was designed. 168 patients were recruited from 29 primary health care centres in Spain and were randomly assigned to one of the three study arms: treatment as usual improved with Smith's norms, individual cognitive behavioural treatment, and group cognitive behavioural treatment. Health-related quality of life was assessed using SF-36 Health Survey.

Results

Individual cognitive behavioural treatment achieves greater changes in health related quality of life than group cognitive behavioural therapy and treatment as usual. Improvement in health related quality of life was fully observed at 12 month, and partially at 6 months. The modality of intervention interacts with time in all dimensions except for Physical functioning and Vitality. Patients who received individual cognitive behavioural therapy treatment had better scores in Physical and Mental health summary measures at 12 month follow-up.

Conclusions

Individual cognitive behavioural treatment is the most effective way to improve health related quality of life in abridged somatization disorder patients, and its effects are sustained over time. Also, regardless of the type of intervention, physical functioning improves compared with treatment as usual.     

Perceptual and response interference in Alzheimer’s disease and mild cognitive impairment

Objectives

The ability to resolve conflicts is indispensable to the function of daily life and decreases with cognitive decline. We hypothesized that subjects with different levels of cognitive impairment exhibit different conflict resolution performances and may be susceptible to interference effects at different stages.

Methods

Sixteen normal controls (NC), 15 mild cognitive impairment (MCI) and seven Alzheimer’s disease (AD) patients were recruited to perform in a modified Eriksen flanker task.

Results

We observed that the AD and MCI patients exhibited smaller accuracy rate and longer response time compared to NC subjects. Longer N2 and P300 latencies were observed in the AD group. Furthermore, the MCI group showed a longer latency than the NC group in the P300 latency. The magnitude of the perceptual and response interference effects was larger in the AD group than the other groups, and the MCI group significantly differed from the NC group at the perceptual level.

Conclusion

The ability to resolve conflict decreased with impaired cognition and the perceptual and response interference effects may be useful in distinguishing MCI and AD.

Significance

The perceptual or response interference effect may potentially be employed as a useful non-invasive probe for the clinical diagnosis of MCI and AD.     

Frontotemporal dementia: patient characteristics,cognition, and behaviour

OBJECTIVES: To describe sociodemographic data of patients with frontotemporal dementia (FTD), to compare the cognitive profile of patients with FTD with that of severity-matched patients with Alzheimer's disease using the CERAD neuropsychological battery (CERAD-NP), to investigate the frequency of behavioural disturbances, and to examine the relation between FTD-specific non-cognitive behavioural symptoms of patients with FTD with age and sex. METHODS: Fifty outpatients were diagnosed with FTD according to the Lund-Manchester consensus criteria. Cognitive impairment was assessed in 30 patients using the CERAD-NP. Severity of dementia was rated on the Clinical Dementia Rating (CDR). Eleven non-cognitive symptoms were rated by severity. To compare CERAD-NP results between patients with FTD and AD, 30 patients with AD were matched for age, sex, and global severity of cognitive performance. RESULTS: The average age at onset of first symptoms was 57.8 years. Eighteen patients (36%) had a positive family history of dementia. On the CERAD-NP patients with FTD performed significantly better than patients with AD on word list learning, delayed verbal recall and visuoconstruction (p < 0.05). There were no significant differences between FTD and AD on naming and verbal fluency tasks. The most frequent non-cognitive behavioural symptoms in FTD were loss of insight, speech abnormality, and apathy. Non-cognitive behavioural symptoms were more frequent in younger and in male than in older patients and in female patients. CONCLUSIONS: The CERAD-NP is a valuable clinical instrument for the cognitive evaluation of patients with suspected FTD. Complementary short tests of attention and executive function may be recommended. To enhance diagnostic sensitivity informant interviews should focus on non-cognitive behavioural changes, taking advantage of standardised questionnaires.     

Cognitive Impairment in Essential Tremor without Dementia

Background and purpose

Several clinical studies have demonstrated that patients with essential tremor (ET) may have cognitive deficits; however, there are no published data regarding detailed neuropsychological assessments of ET without dementia. We therefore conducted a case-control study of cognitive function in patients with ET.

Methods

The cohort for this study comprised 34 consecutive patients with ET without dementia and 33 age-matched controls, all of who completed a dementia-screening questionnaire and underwent a detailed neuropsychological investigation.

Results

Severe impairments were observed in most domains for the ET group compared to the controls, including attention, part of language function, verbal memory, and frontal executive functions.

Conclusions

Our results support the finding that the subclinical cognitive deficits characterized by attention, verbal memory impairments, and executive dysfunction are a clinical feature of ET. In addition, our results also support the finding that age at examination and educational status are the most important risk factors associated with cognitive deficits in patients with ET.     

Biomarkers study in atypical dementia: proof of a diagnostic work-up

Background

An early differentiation between Alzheimer’s Disease (AD) and other dementias is crucial for an adequate patients’ management, albeit it may result difficult for the occurrence of “atypical presentations.” Current diagnostic criteria recognize the importance of biomarkers for AD diagnosis, but still an optimal diagnostic work-up isn’t available.

Objective

Evaluate the utility and reproducibility of biomarkers and propose an “optimal” diagnostic work-up in atypical dementia.

Methods

(1) a retrospective selection of “atypical dementia cases”; (2) a repetition of diagnostic assessment by two neurologists following two different diagnostic work-ups, each consisting of multiple steps; (3) a comparison between diagnostic accuracy and confidence reached at each step by both neurologists and evaluation of the inter-rater agreement.

Results

In AD, regardless of the undertaken diagnostic work-up, a significant gain in accuracy was reached by both neurologists after the second step, whereas in frontotemporal dementia (FTD), adding subsequent steps was not always sufficient to increase significantly the baseline accuracy. A relevant increment in diagnostic confidence was detectable after studying pathophysiological markers in AD, and after assessing brain metabolism in FTD. The inter-rater agreement was higher at the second step for the AD group when the pathophysiological markers were available and for the FTD group when the results of FDG-PET were accessible.

Conclusions

In atypical cases of dementia, biomarkers significantly raise diagnostic accuracy, confidence, and agreement. This study introduces a proof of diagnostic work-up that combines imaging and CSF biomarkers and suggests distinct ways to proceed on the basis of a greater diagnostic likelihood.

     

The applause sign and neuropsychological profile in progressive supranuclear palsy and Parkinson's disease

Background

The applause sign has been associated with various neurodegenerative diseases. We investigate its validity in the differential diagnosis of progressive supranuclear palsy and Parkinson's disease, and its relationship with neuropsychological tests.

Patients and methods

23 patients with progressive supranuclear palsy and 106 patients with Parkinson's disease were included and administered the following scales: progressive supranuclear palsy rating scale, unified Parkinson's disease rating scale (UPDRS), mini-mental state examination (MMSE), frontal assessment battery (FAB), neuropsychiatric inventory and three-clap test.

Results

73.9% with progressive supranuclear palsy and 21.7% with Parkinson's disease showed a positive applause sign. Only a positive applause sign, UPDRS II score and disease duration were found to be predictors of progressive supranuclear palsy. Both patient-groups showed statistically significant correlations between the applause sign and neuropsychological tests: in progressive supranuclear palsy patients MMSE correlation coefficient: 0.62 (p: 0.002) and FAB correlation coefficient: 0.48 (p: 0.02), and in Parkinson's disease patients MMSE correlation coefficient: 0.47 (p < 0.001) and FAB correlation coefficient: 0.43 (p < 0.001). Verbal fluency and inhibitory control (FAB) and writing and orientation in time (MMSE) discriminated between patients with normal and positive applause sign.

Conclusions

A positive applause sign is not specific to progressive supranuclear palsy and may also be observed in Parkinson's disease patients with altered cognition, and it's related to cortical frontal abnormalities such as language disorders and inhibitory control.     

Chronic obstructive pulmonary disease may complicate Alzheimer’s disease: a comorbidity problem

Background and aim

Chronic obstructive pulmonary disease (COPD) may be associated with worsening of cognitive performance. We studied patients with Alzheimer’s disease (AD) with and without COPD, and we analyzed, in a retrospective way, clinical and neuropsychological variables to verify if COPD plays a pejorative role on cognitive or functional autonomy in patients with dementia.

Methods

We enrolled 23 adult patients (AD-COPD) with probable AD and COPD and 23 with AD only (AD-only); they were matched for sex, age, educational level, and Mini Mental State Examination (MMSE) at the disease onset. Global cognitive status was estimated using MMSE at the first assessment and after 24 months. Memory, executive functions, praxia, and language were the other cognitive domains analyzed. The two groups were also compared for the presence of behavioral disorders (anxiety, depression).

Results

AD-COPD had worse results in executive functions screening than AD-only; no significant differences were found comparing other cognitive domains; moreover, there was no significant difference between the two groups considering the decrease in MMSE scores. AD-COPD also showed a higher presence of depression.

Discussion

COPD is known to be associated with the development of cognitive deficits, in particular, regarding for executive functions and attention, memory and logical reasoning. In this context, MMSE has a low diagnostic accuracy to underline effective cognitive impairment in AD-COPD. Our study shows a higher frequency of frontal deficits and behavioral disturbances in patients with AD and COPD than patients with AD-only. COPD could complicate the management of AD patients, thus necessitating a closer and multidisciplinary monitoring.

     

Análisis de la fluencia lectora en pacientes con la enfermedad de Alzheimer y controles asintomáticos

Introduction

Many studies highlight that an impaired ability to communicate is one of the key clinical features of Alzheimer disease (AD).

Objective

To study temporal organisation of speech in an oral reading task in patients with AD and in matched healthy controls using a semi-automatic method, and evaluate that method's ability to discriminate between the 2 groups.

Subjects and methods

A test with an oral reading task was administered to 70 subjects, comprising 35 AD patients and 35 controls. Before speech samples were recorded, participants completed a battery of neuropsychological tests. There were no differences between groups with regard to age, sex, or educational level.

Results

All of the study variables showed impairment in the AD group. According to the results, AD patients’ oral reading was marked by reduced speech and articulation rates, low effectiveness of phonation time, and increases in the number and proportion of pauses. Signal processing algorithms applied to reading fluency recordings were shown to be capable of differentiating between AD patients and controls with an accuracy of 80% (specificity 74.2%, sensitivity 77.1%) based on speech rate.

Conclusion

Analysis of oral reading fluency may be useful as a tool for the objective study and quantification of speech deficits in AD.