OBJECTIVE: To measure the frequency dynamics of cervical and ocular vestibular evoked myogenic potentials in healthy subjects and patients with Ménière's disease. STUDY DESIGN: A prospective cohort study. SETTING: A university teaching hospital. SUBJECTS: Eight healthy volunteers (16 ears) and 12 adult patients with unilateral Ménière's disease (8 with definite ...

Continuous intraduodenal infusion of levodopa/carbidopa (Duodopa®) via PEJ tubes is increasingly used in patients with advanced stages of Parkinson's disease. Tube-related complications such as kinking or coiling have been frequently reported. We herein describe two cases of tube dysfunction in patients with Parkinson's disease and continuous Duodopa® treatment due to ...

Background: The histopathologic pattern of clonal seborrheic keratosis (SK) is quite similar to the nested pattern of pagetoid Bowen's disease [squamous cell carcinoma in situ (SCCIS)], and differentiation between the two can be challenging, especially when only small pieces are available for interpretation. Methods: Eleven examples of clonal SK and ...

MicroRNAs play a crucial role in Chronic Lymphocytic Leukemia (CLL). We investigated whether microRNAs can discriminate patients with a progressive disease from those with a stable disease. We analyzed microRNA expression on leukemic cells isolated from 358 sequential samples of 114 patients with either stable or progressive disease. We find ...

Stroke and dementia are closely associated, whether in the form of vascular cognitive impairment or Alzheimer's disease. Alzheimer's disease and stroke share very similar risk factor profiles and may be prevented with similar modification programs. We are dealing with a present and future epidemic that will fundamentally affect health care ...

Bilateral striopallidodentate calcification, usually termed Fahr's disease, can give rise to various clinical manifestations including hyperkinetic movement disorders or a hypokinetic Parkinsonian syndrome, behavioural and mood changes, cognitive deficits and even frank dementia. We describe four patients all of whom underwent a detailed scintigraphic, neuroradiological and clinical work-up: two had ...

The boundary between vascular dementia and Alzheimer disease (AD) continues to be unclear. Some posit that gradually progressive vascular dementia, as with small vessel disease, is simply vascular disease plus AD. Because AD presents a characteristic pattern on fluorodeoxyglucose positron emission tomography, we sought to determine whether the fluorodeoxyglucose pattern ...

To investigate the relationship between bullous pemphigoid (BP) and neurologic disease. Case-control study. Tertiary care center for immunobullous diseases and skin tumor clinics at a university hospital in Oxford, England. Ninety consecutive patients with BP and 141 controls. Age-adjusted prevalence of neurologic disease in patients and controls. Time interval between ...

Cholinesterase inhibitors (ChEIs) are widely licensed for the symptomatic treatment of Alzheimer’s disease, but their use has also been examined in a wide variety of neurological disorders besides Alzheimer’s disease, and this article reviews these uses. The evidence currently available suggests that ChEIs may possibly have a role in the ...

The prevention of dementia, and particularly of Alzheimer's disease, is a major challenge for researchers and clinicians. In this article, the mixture of evidence, observations and hypotheses in the current literature is categorized into four avenues for possible preventive interventions, as suggested by the NIH State-of-the-Science Conference. The main categories ...

Thirty same-sex twin pairs were identified in which both members were assessed at baseline and one twin subsequently developed dementia, at least 3 years subsequent to the baseline measurement, while the partner remained cognitively intact for at least three additional years. Eighteen of the 30 cases were diagnosed with Alzheimer's ...

INTRODUCTION: Dementia is a growing health and social concern for all Australians. Whilst the prevalence of dementia amongst Australia's indigenous people is unclear, there is some evidence that dementia rates are five times that of the general Australian population. To date no studies have examined dementia knowledge levels in indigenous ...

Extracellular fibrous amyloid deposits or intracellular inclusion bodies containing abnormal protein aggregates are pathological hallmarks of several neurodegenerative disorders and it has been hotly debated whether these aberrant protein structures merely occur as a consequence of disease or actually participate in a pathogenic cascade which culminates in neural dysfunction and ...

Dementia with Lewy bodies (DLB) and Parkinson's disease dementia (PDD) are the two most common types of dementing neurodegenerative disease after Alzheimer's disease (AD). Both of these conditions are often diagnosed late or not at all. Selective literature review. The severe cholinergic and dopaminergic deficits that are present in both ...

Meyer's dysplasia strongly mimics Legg-Calve-Perthes' disease despite differing markedly in eventual outcome. This study presents the clinicoradiologi-cal features which differentiate it from Perthes' disease in a group of 178 children with a preliminary diagnosis of Perthes' disease, of whom nine were subsequently diagnosed with Meyer's dysplasia. All had a near ...

The terms "dementia" and "Alzheimer's disease" are often wrongly used as if they were synonyms. Dementia is a clinical syndrome whose main element is memory impairment; it is due to Alzheimer's disease in more than 75% of cases. Alzheimer's disease, on the other hand, is a neuropathological entity that is ...

Abstract: Dementia, as a clinical syndrome, has many potential causes. The majority of cases are due to neurodegenerative diseases and/or vascular disease with relatively gradual progressive clinical courses. However, dementia can be rapidly progressive with time courses measured in only weeks or a few months. These more rapidly progressive dementias ...

Leukoaraiosis is a common finding in stroke patients and has been strongly associated with risk of incident stroke and dementia. Leukoaraiosis may also be an independent predictor of stroke outcomes. There is increasing evidence from neuroimaging to support the concept that some leukoaraiosis is caused by white matter infarcts, which ...

Vascular and neurodegenerative disease commonly cooccur in older persons. We review findings from the Rush Religious Orders Study and Memory and Aging Project. Both studies enroll subjects without diagnosed dementia, perform annual evaluations, and obtain autopsies proximate to death. We found that macroscopic infarcts are common, lower the threshold for ...

Binswanger disease is a common cause of vascular dementia in the elderly. This report up-dates the pathological and clinical findings, imaging identification emphasizing recent advances, and diagnosis of this condition.

In recent years, a considerable number of new sporadic or hereditary small artery diseases of the brain have been detected which preferably occur in younger age, below 45 years. Cerebral microangiopathies constitute an appreciable portion of all strokes. In middle aged patients, hereditary cerebral small vessel diseases have to be ...

Early Alzheimer's Disease Clinical Practice, N Engl J Med 2010:362;2194-2201. In the second paragraph of the Treatment Options section (page 2197), the parenthetical information given in the second sentence, regarding the Alzheimer's Disease Assessment Scale for cognition, should have read, "a scale ranging from 0 to 70, with higher scores ...

A substantial number of individuals with Parkinson's disease who display impaired postural stability experience accelerated cognitive decline and an increased prevalence of dementia. To date, studies suggest that this relationship, believed to be due to involvement of nondopaminergic circuitry, occurs later in the disease process. Research has yet to adequately ...

Hippocampal changes may be a useful biomarker for Alzheimer's disease if they are specific to dementia sub-type. We compare hippocampal volume and shape in population-based incident cases of Alzheimer's disease and vascular dementia (VaD). Participants are Japanese-American men from the Honolulu Asia Aging Study. The following analysis is based on ...

In Alzheimer disease, CSF biomarkers and nuclear imaging are of particular interest. Many studies investigated only one technique, limiting comparison. Here, in 76 patients blinded 99mTc-SPECT was compared to CSF. Sensitivity of CSF was 92%; and 51% for SPECT. Specificity favored SPECT (90 vs. 80%). Both techniques showed no coherence ...

A diagnosis of dementia is devastating at any age but diagnosis in younger patients presents a particular challenge. The differential diagnosis is broad as late presentation of metabolic disease is common and the burden of inherited dementia is higher in these patients than in patients with late-onset dementia. The presentation ...

BACKGROUND: Since normal pressure hydrocephalus (NPH) was first described in 1965, neurologists have been involved in the initial diagnostic evaluation for it but have rarely followed patients specifically to monitor response to therapy after shunt surgery. REVIEW SUMMARY: The potential role for the neurologist in the longitudinal management of NPH ...

Dementia associated with Parkinson's disease (PDD) is a common problem and one that is associated with significant morbidity and mortality. Over the past decade, increasing research efforts and funding have been directed toward an improved understanding of PDD. Despite these efforts, fundamental gaps remain in our knowledge. Consequently, therapeutic progress ...

Alzheimer's disease (AD), frontotemporal dementia (FTD) and semantic dementia (SD) are neurodegenerative diseases that differ in their socioemotional presentations. Mutual gaze (i.e. when two individuals make eye contact) is a building block of social behavior that may be differentially affected by these diseases. We studied 13 AD patients, 11 FTD ...

Small vessel disease (SVD) is responsible for brain chronic circular disorder, and accounts for 20%-30% cases of ischemic stroke as well as cerebral hemorrhage, and to a great extent, encephalopathy. Binswanger's disease and multiple small strokes, which are common in older people, are also closely associated with SVD. These disorders ...

The purpose of this study was to explore the sensitivity and specificity of the Clock Drawing Test by using a widely employed four-point scoring system to discriminate between patients with Alzheimer's disease or vascular dementia. Receiver operating characteristic analysis indicated that the Clock Drawing Test was able to distinguish between ...

Cognitive impairment and dementia are more common in patients with Parkinson disease (PD) than age-matched controls and appear to become more frequent as PD progresses. However, estimates of dementia in patients with PD have varied widely, likely due in part to differences in case definition, case ascertainment and methodology. First, ...

The prevalence of cognitive impairment and dementia in Parkinson's disease (PD) is high and can potentially occur as the result of multiple differing pathologies. Neuroimaging has provided evidence of decreased cortical volume, increased white matter diffusion changes, and decreased resting metabolic activity that appears to begin prior to the onset ...

Olfactory dysfunction is common in subjects with Parkinson's disease. The pathophysiology of such dysfunction, however, remains poorly understood. Neurodegeneration within central regions involved in odour perception may contribute to olfactory dysfunction in Parkinson's disease. Central cholinergic deficits occur in Parkinson's disease and cholinergic neurons innervate regions, such as the limbic ...

Resting-state or intrinsic connectivity network functional magnetic resonance imaging provides a new tool for mapping large-scale neural network function and dysfunction. Recently, we showed that behavioural variant frontotemporal dementia and Alzheimer's disease cause atrophy within two major networks, an anterior 'Salience Network' (atrophied in behavioural variant frontotemporal dementia) and a ...

To identify predictors of the progression from pre-dementia stages of cognitive impairment in Alzheimer's disease is relevant to clinical management and to substantiate the decision of prescribing antidementia drugs. Longitudinal study of a cohort of elderly adults with amnestic mild cognitive impairment and healthy controls, carried out to estimate the ...

Alzheimer's disease imposes a significant public health burden that will only worsen as the population ages. Thus, there is considerable motivation to develop effective strategies to treat, or more ideally, prevent the disease. Epidemiologic evidence has suggested that non-steroidal anti-inflammatory drugs (or NSAIDs) may be neuro-protective. However, this evidence is ...

A series of negative clinical trials of disease-modifying agents for Alzheimer's disease has increased pessimism regarding the prospects for important therapeutic advances. But limited efficacy may be attributed in part to the advanced degree of neurodegeneration present at the onset of dementia. To optimize the likelihood of success, it is ...

A goal of many clinical trials for Alzheimer's disease is to develop disease-modifying therapies for the pre-dementia period of the Alzheimer's disease process. However, there are no clinical criteria for characterizing subjects in this range. The construct of amnestic mild cognitive impairment has been extensively investigated and may provide useful ...

Frontotemporal dementia is the second most common early onset dementia after Alzheimer disease. Frontotemporal dementias are a complex group of dementias. The clinical diagnosis can be perplexing because of concurring psychiatric and neurologic syndromes. Frontotemporal lobar degeneration, the underlying pathology, represents an emerging group of proteinopathies. Genetic factors play an ...

Dementia is a common feature in Parkinson disease (PD), the time of onset determining how patients are classified. Those patients where dementia develops prior to parkinsonism or during the first year of disease are designated as having dementia with Lewy bodies (DLB). In those where dementia develops over a year ...

Dementia is a terminal disease, associated with great suffering and difficult decisions in the severe stage. The decision-making process is characterized by uncertainty because of lack of scientific evidence in treatments and by the need to reconcile conflicting points of view. In intercurrent diseases, aggressive interventions are used without consideration ...

The increase in life expectancy seen in many countries has been accompanied by an increase in the number of people living with dementia and a growing need for health care. The large number of affected individuals emphasizes the need to identify causes for the phenotypes associated with diseases such as ...

Alzheimer's disease is a neurodegenerative illness. The Mini Mental State Examination (MMSE), Cognitive Abilities Screening Instrument (CASI) and Clinical Dementia Rating (CDR) are applied to assess whether a person suffers Alzheimer's disease and determine its severity. There are many confounding factors within those assessments, medical professionals need to bear in ...

BACKGROUND: The aim of this short communication is to share my personal experience of caring for a terminally ill family member who was afflicted with Pick's disease. I tell my story in the hope of increasing society's awareness on this little known but devastating disease and assisting those who might ...

Behavioral changes and cognitive decline are the core clinical manifestations in the behavioral variant of frontotemporal dementia (bv-FTD). The behavioral changes may include characteristic stereotypic movements. These movements, although without clear purpose, are not involuntary. Involuntary movements are usually not seen in FTD.Two patients with involuntary choreoathetoid movements but otherwise ...

Serum or plasma progranulin (GRN) is a highly accurate of GRN-related frontotemporal lobar degeneration, which is caused by loss-of-function mutations in the GRN gene. Both null mutations and missense mutations in GRN have also been observed in patients with Alzheimer's disease. Here, the evidence for a role of circulating GRN ...

Primary progressive aphasia (PPA) is a disorder of declining language that is a frequent presentation of neurodegenerative diseases such as frontotemporal lobar degeneration. Three variants of PPA are recognized: progressive nonfluent aphasia, semantic dementia, and logopenic progressive aphasia. In an era of etiology-specific treatments for neurodegenerative conditions, determining the histopathological ...

A significant expansion of knowledge in the last few years, especially in the molecular biology of frontotemporal dementia (FTD) is summarized. This condition, formerly known as Pick's disease and considered rare, is estimated to be 12-15% of all dementias and 30-50% early onset ones. The clinical picture is protean, mainly ...

The development of acetyl-cholinesterase inhibitors, and the prospect of future therapies to prevent, or modify, the course of Alzheimer's disease necessitates greater accuracy in diagnosis of this heterogeneous disease. Current diagnosis is based on clinical criteria and neuropathology. This is not always sufficient, and the development of sensitive and specific ...