Biomedical subjects

R Perneczky

Publications and source records attributed to R Perneczky.

10 recordsLinked to original sources

PubMed2006

[Initial symptoms, survival and causes of death in 115 patients with frontotemporal lobar degeneration].

The early and differential diagnosis of the clinical phenotypes of frontotemporal lobar degeneration (FTLD), including frontotemporal dementia (FTD), semantic dementia (SD) and non-fluent progressive aphasia (NFPA), can be challenging. It may be difficult not only to differentiate these conditions from normal aging, psychiatric disorders, and other dementias, but also to distinguish between them. For early diagnosis, information on the initial and presenting symptoms of the FTLD phenotypes is essential. In the present study caregivers of 78 patients with FTD, 20 patients with SD and 17 patients with PA were interviewed about initial symptoms. In patients with FTD, the most frequent initial symptoms were alterations of personality, followed by forgetfulness and word finding difficulty. Patients with SD presented with word finding difficulty and behavioral disturbances. Almost all patients with PA developed word finding difficulty as the first manifestation of their disorder. Diagnostic latency - the time from disease onset to diagnosis was 4.1 years in FTD, 4.2 years in SD and 3.1 years in PA. Caregivers, and in some cases also patients, should be educated about the likely course and mortality of FTLD. To obtain information about survival time and cause of death associated with FTLD we analyzed follow-up data on 106 patients of whom 25 had died. The median survival time from the occurrence of first symptoms was 14 years. Mortality risk was significantly higher in patients with an early disease onset. Causes of death were varied, but pneumonia and sudden unexplained deaths were particularly frequent.

Age of Onset↗

PubMed2006

Longitudinal changes of cerebral glucose metabolism in semantic dementia.

BACKGROUND: Semantic dementia (SD). OBJECTIVE: To identify the pattern of decline of cerebral glucose metabolism in SD using cerebral (18)F-fluoro-2-desoxy-D-glucose positron emission tomography scanning ((18)F-FDG-PET). METHODS: Eight patients with SD underwent (18)F-FDG-PET at baseline and at re-examination in average 15 months later. RESULTS: Compared with healthy control subjects, patients with SD showed a significant asymmetrical (left > right) hypometabolism of the temporal lobes, particularly of the anterior poles, at baseline. At follow-up, we observed a deterioration of cognitive abilities. However, in addition to the temporal lobes no other cortical or subcortical region showed a significant reduction of glucose metabolism except the anterior cingulate cortex (pcorr < 0.05). CONCLUSION: Subtle functional changes suffice to produce significant neuropsycho- logical deterioration.

Age of Onset↗

PubMed2006

Behavioral disturbances in the course of frontotemporal dementia.

BACKGROUND: Behavioral disturbances are prominent in frontotemporal dementia (FTD), and their occurrence has been the topic of several investigations. Nonetheless, the prevalence and severity of behavioral disturbances of patients with FTD in different degrees of dementia severity have rarely been studied. OBJECTIVE: The aim of this study was to assess and compare the prevalence and severity of behavioral disturbances in patients with mild FTD and in patients with moderate/severe dementia. METHODS: We included 21 outpatients with mild FTD [Clinical Dementia Rating (CDR) = 1] and 19 patients with moderate or severe dementia (CDR = 2 or 3) in this study. Behavioral disturbances were assessed using the Neuropsychiatric Inventory (NPI). RESULTS: We found a statistically significant difference in the total NPI scores between patients with mild FTD and patients with moderate or severe FTD, the latter scoring higher. Apathy was the most prevalent symptom in both patient groups (90.5 and 100%). Except appetite and eating disturbance, which appeared in 77.8% of the patients with moderate/severe dementia, all other symptoms were clearly less common (<50%). CONCLUSION: The results highlight the variability of behavioral disturbances in mild and moderate/severe stages of FTD.

Age of Onset↗

PubMed2006

Schooling mediates brain reserve in Alzheimer's disease: findings of fluoro-deoxy-glucose-positron emission tomography.

BACKGROUND: Functional imaging studies report that higher education is associated with more severe pathology in patients with Alzheimer's disease, controlling for disease severity. Therefore, schooling seems to provide brain reserve against neurodegeneration. OBJECTIVE: To provide further evidence for brain reserve in a large sample, using a sensitive technique for the indirect assessment of brain abnormality (18F-fluoro-deoxy-glucose-positron emission tomography (FDG-PET)), a comprehensive measure of global cognitive impairment to control for disease severity (total score of the Consortium to Establish a Registry for Alzheimer's Disease Neuropsychological Battery) and an approach unbiased by predefined regions of interest for the statistical analysis (statistical parametric mapping (SPM)). METHODS: 93 patients with mild Alzheimer's disease and 16 healthy controls underwent 18F-FDG-PET imaging of the brain. A linear regression analysis with education as independent and glucose utilisation as dependent variables, adjusted for global cognitive status and demographic variables, was conducted in SPM2. RESULTS: The regression analysis showed a marked inverse association between years of schooling and glucose metabolism in the posterior temporo-occipital association cortex and the precuneus in the left hemisphere. CONCLUSIONS: In line with previous reports, the findings suggest that education is associated with brain reserve and that people with higher education can cope with brain damage for a longer time.

Aged↗

PubMed2006

Progression to dementia in clinical subtypes of mild cognitive impairment.

OBJECTIVE: To examine the outcome among patients diagnosed with different types of mild cognitive impairment (MCI). PATIENTS: A follow-up examination (average follow-up period: 3.49 +/- 2.2 years) was performed in 81 cognitively impaired, non-demented patients aged >55 years at baseline. RESULTS: 8 of 32 patients with amnestic MCI (25%), 22 of 41 patients with multiple-domain MCI (54%), and 3 of 8 patients with single non-memory MCI (37.5%) progressed to dementia. The clinical type of MCI is significantly associated with the likelihood of conversion to dementia. DISCUSSION: When the clinical syndrome of MCI evolves on a neurodegenerative basis, the multiple-domain type of MCI has a less favorable prognosis than the amnestic type and may represent a more advanced prodromal stage of dementia.

Aged↗

PubMed2006

[Validation of a Short Telephone Test (T3MS) for the Diagnosis of Cognitive Impairment].

The identification of cognitive impairment in general practice requires short but accurate tests. For epidemiologic surveys and genetic family studies cognitive tests are desirable which can be administered over the telephone. We assessed the ability of a telephone version of the Modified Mini Mental State Examination (T3MS) to identify mild cognitive impairment (MCI) and mild dementia in Alzheimer's disease (AD) and compared it with the diagnostic accuracy of the conventional Mini Mental State Examination (MMSE). The study refers to 34 patients of the outpatient clinic for cognitive disorders of the technical university of Munich of whom 18 had MCI and 16 had mild dementia in AD, respectively. The study also included 14 cognitively unimpaired age-matched probands. The T3MS and MMST were validated against an expert diagnosis base on a comprehensive diagnostic workup. Statistical analysis was performed using the receiver-operator-characteristics (ROC) method. The T3MS outperformed the MMST in the distinction between MCI patients and cognitively unimpaired individuals. In the separation between cognitively unimpaired probands and patients with mild AD the T3MS achieved a sensitivity and specificity of 100 %. The T3MS is a short and practical but accurate telephone test for the identification of mild dementia in AD for use in epidemiological surveys and genetic family studies. The interview achieves higher diagnostic precision than the MMSE and contributes to a valid assessment of cognitive performance. For the identification of mild cognitive impairment, however, the T3MS was less appropriate.

Alzheimer Disease↗

PubMed2005

The Alzheimer variant of lewy body disease: a pathologically confirmed case-control study.

The objective of the study was to identify clinical features that distinguish patients with dementia with Lewy bodies (DLB), who were classified as Alzheimer's disease (AD) patients, from patients with AD. We examined a group of 27 patients from our memory clinic, originally diagnosed with AD, of whom 6 were postmortem found to have DLB. For the present study, we compared cognitive, noncognitive and neurological symptoms between the two groups. We found that there were no differences on ratings of dementia and scales for activities of daily living. Patients with DLB performed better on the MMSE and the memory subtest of the CAMCOG, but there was no difference in any other cognitive domain. Furthermore, genetic risk factors, including family history of dementia or allele frequency of the apolipoprotein epsilon4, did not discriminate between the two groups, and there were no differences on CCT scans. Taken together, our findings suggest that Lewy body pathology may be present in patients who do not show the typical clinical features which distinguish DLB from AD.

Aged↗

PubMed2004

[Mild cognitive impairment].

From today's point of view, patients with a mild cognitive impairment (MCI) are at risk for developing a dementia. This clinical picture occurs frequently in the population and is easily recognized through simple tests and interviews conducted in the general and neurological medical practice. A goal of the diagnostics is to detect potentially redressable causes, above all depressive disorders, and to introduce a therapy. In many cases, however, MCI is an early stage of Alzheimer's disease or other neurodegenerative processes. When such a cause is suspected, the most important medical measure is to monitor the progression of the disease so that when deterioration occurs, therapy with antidementia drugs can promptly begin. In the future, patients with MCI will play an increasingly important role as the target group for the prevention of dementia.

Aged↗

PubMed2004

[Current antidementive therapy].

Current antidementive treatment can delay further progress of the symptoms of dementia and should therefore be initiated as early as possible and be of adequate duration. Wherever possible, maximum permissible doses should be given. The efficacy of current antidementive drugs in Alzheimer's disease has been sufficiently documented by relevant studies. The acetylcholinesterase inhibitors, donepezil, galantamine and rivastigmine have been approved for the treatment of mild-to-moderate Alzheimer's dementia, the NMDA inhibitor memantine is approved for moderate-to-severe Alzheimer's disease. Ginkgo biloba or piracetam are alternatives for patients with mild-to-moderate dementia, in whom acetylcholinesterase inhibitors cannot be used.

Activities of Daily Living↗

PubMed2004

[Mild cognitive disorder. Questions of definition, diagnosis, prognosis and therapy].

The syndrome of mild cognitive impairment (MCI) is heterogeneous in terms of aetiology, psychopathology, and prognosis. It is characterised by cognitive deterioration significantly exceeding the decline attributable to aging but not reaching the severity of dementia. The prevalence of MCI is estimated to be 17% in the population over 65 years old. At neuropathological examination, a large proportion of patients with mild cognitive impairment, particularly of the amnestic type, show typical features of Alzheimer's disease. The former progresses to dementia at an annual rate of 10% to 15%. In some cases, however, there is stable impairment or remission. The neurodegenerative process of Alzheimer's disease can be demonstrated in at least some patients using volumetric magnetic resonance imaging, 18-FDG positron emission tomography, or biochemical markers in the cerebrospinal fluid. It is not yet known whether patients with mild cognitive impairment or at least those with predementia Alzheimer's disease can benefit from currently available symptomatic treatments. Patients with early-stage Alzheimer's are an important target group for treatment interventions aiming at slowing the neurodegenerative process.

Adult↗