Annals of Neurology
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Frequency and distribution of Alzheimer's disease in Europe: A collaborative study of 1980–1990 prevalence findings Abstract We reanalyzed and compared current prevalence estimates of Alzheimer's disease in Europe. Studies characterized as follows qualified for comparison: dementia defined by the Diagnostic and Statistical Manual for Mental Disorders , 3rd edition, or equivalent criteria; Alzheimer's disease diagnosed by the National Institute of Neurological and Communicative Disorders and Stroke‐Alzheimer's Disease and Related Disorders Association or equivalent criteria; case‐finding through direct individual examination; appropriate sample size; and inclusion of institutionalized persons. Of the 23 European surveys of dementia considered, six fulfilled the inclusion criteria. When age and sex were considered, there were no major geographic differences in the prevalence of Alzheimer's disease across Europe. Overall European prevalence (per 100 population) for the age groups 30 to 59, 60 to 69, 70 to 79, and 80 to 89 years was, respectively, 0.02, 0.3, 3.2, and 10.8. Prevalence increased exponentially with advancing age and, in some populations, was consistently higher in women. Prevalence remained stable over 15 years in one study.
Annals of Neurology - Tập 30 Số 3 - Trang 381-390 - 1991
<i>PLA2G6</i> mutations and Parkinson's disease
Annals of Neurology - Tập 67 Số 1 - Trang 147-148 - 2010
Immunological and pathological study of anti‐Ri–associated encephalopathy Abstract A patient with high titers of the anti‐Ri antibody died 3 years after a progressive course with ataxia, opsoclonus, dementia, and peripheral neuropathy. At autopsy, no tumor was found. The nervous system exhibited severe Purkinje cell loss and contained perivascular and interstitial inflammatory infiltrates, particularly involving the brainstem. B and CD4 cells predominated in the perivascular spaces and CD8 cells in the interstitial infiltrates. Complement reactivity and natural killer cells were present and predominated in areas with more intense inflammatory infiltrates. Deposits of IgG were detected in the cytoplasm and nuclei of some neurons, particularly those in the brainstem tegmentum. The proportion of anti‐Ri IgG in the total IgG extracted from various areas of the brain, serum, and cerebrospinal fluid was determined by quantitative western blot analysis. Anti‐Ri reactivity was identified in immunoblots of all regions of the brain, but it predominated in basis pontis and dorsal mesencephalon. Our findings support the hypothesis of an autoimmune basis for the disorder and suggest that an antibody‐mediated mechanism may play a role in its pathogenesis.
Annals of Neurology - Tập 36 Số 6 - Trang 896-902 - 1994
An attention modulated response to disgust in human ventral anterior insula Abstract The human brain is expert in analyzing rapidly and precisely facial features, especially emotional expressions representing a powerful communication vector. The involvement of insula in disgust recognition has been reported in behavioral and functional imaging studies. However, we do not know whether specific insular fields are involved in disgust processing nor what the processing time course is. Using depth electrodes implanted during presurgical evaluation of patients with drug‐refractory temporal lobe epilepsy, we recorded intracerebral event‐related potentials to human facial emotional expressions, that is, fear, disgust, happiness, surprise, and neutral expression. We studied evoked responses in 13 patients with insular contacts to specify the insular fields involved in disgust processing and assess the timing of their activation. We showed that specific potentials to disgust beginning 300 milliseconds after stimulus onset and lasting 200 milliseconds were evoked in the ventral anterior insula in four patients. The occurrence and latency of event‐related potentials to disgust in the ventral anterior insula were affected by selective attention. The analysis of spatial and temporal characteristics of insular responses to disgust facial expression lead us to underline the crucial role of ventral anterior insula in the categorization of facial emotional expressions, particularly the disgust. Ann Neurol 2003
Annals of Neurology - Tập 53 Số 4 - Trang 446-453 - 2003
Cognition and anatomy in three variants of primary progressive aphasia Abstract We performed a comprehensive cognitive, neuroimaging, and genetic study of 31 patients with primary progressive aphasia (PPA), a decline in language functions that remains isolated for at least 2 years. Detailed speech and language evaluation was used to identify three different clinical variants: nonfluent progressive aphasia (NFPA; n = 11), semantic dementia (SD; n = 10), and a third variant termed logopenic progressive aphasia (LPA; n = 10). Voxel‐based morphometry (VBM) on MRIs showed that, when all 31 PPA patients were analyzed together, the left perisylvian region and the anterior temporal lobes were atrophied. However, when each clinical variant was considered separately, distinctive patterns emerged: (1) NFPA, characterized by apraxia of speech and deficits in processing complex syntax, was associated with left inferior frontal and insular atrophy; (2) SD, characterized by fluent speech and semantic memory deficits, was associated with anterior temporal damage; and (3) LPA, characterized by slow speech and impaired syntactic comprehension and naming, showed atrophy in the left posterior temporal cortex and inferior parietal lobule. Apolipoprotein E ε4 haplotype frequency was 20% in NFPA, 0% in SD, and 67% in LPA. Cognitive, genetic, and anatomical features indicate that different PPA clinical variants may correspond to different underlying pathological processes.
Annals of Neurology - Tập 55 Số 3 - Trang 335-346 - 2004
Glucose metabolism and serotonin receptors in the frontotemporal lobe degeneration Abstract In patients with the frontal variant of frontotemporal lobar degeneration (fv‐FTLD), behavioral abnormalities may vary from apathy with motor slowness (apathetic form) to disinhibition with agitation (disinhibited form). These clinical presentations may be related to specific regional cerebral dysfunction and to deficit in the serotoninergic system. We studied cerebral glucose uptake using 18 F‐fluorodeoxyglucose and positron emission tomography in 18 patients fulfilling clinical criteria for fv‐FTLD and showing, respectively, an apathetic or disinhibited behavioral syndrome. In eight of these patients, we also evaluated the 5‐hydroxytryptamine‐2A receptor cerebral receptor distribution with [11 C]MDL and positron emission tomography. We found a reduction of frontal glucose metabolism in the whole group of fv‐FTLD patients. Apathetic syndrome was associated with a prevalent dorsolateral and frontal medial hypometabolism, whereas the disinhibited syndrome demonstrated a selective hypometabolism in interconnected limbic structures (the cingulate cortex, hippocampus/amygdala, and accumbens nucleus). The in vivo measurements of [11 C]MDL indicated a significant reduction of 5‐hydroxytryptamine‐2A receptors in orbitofrontal, frontal medial, and cingulate cortices. These 18 F‐fluorodeoxyglucose positron emission tomography changes can be considered as specific functional markers of the different behavioral presentations in fv‐FTLD. The serotoninergic system dysfunction provides a rationale for therapeutic trials with selective serotonin reuptake inhibitors. Ann Neurol 2005;57:216–225
Annals of Neurology - Tập 57 Số 2 - Trang 216-225 - 2005
Primary progressive aphasia Abstract Primary progressive aphasia (PPA) is a focal dementia characterized by an isolated and gradual dissolution of language function. The disease starts with word‐finding disturbances (anomia) and frequently proceeds to impair the grammatical structure (syntax) and comprehension (semantics) of language. The speech output in PPA can be fluent or nonfluent. Memory, visual processing, and personality remain relatively well‐preserved until the advanced stages and help to distiguish PPA from frontal lobe dementia and the typical forms of Alzheimer's disease. The term “semantic dementia” was originally introduced to designate a different group of patients with a combination of verbal and visual processing deficits. In practice, however, this diagnosis is also being used in a variant sense to denote a subtype of PPA with fluent speech and impaired comprehension, even in the absence of visual processing deficits. Insofar as the diagnosis of semantic dementia can have these two different meanings, it is important to specify whether it is being used in the original sense or to denote a subtype of PPA. Structural and physiological neuroimaging confirms the selective predilection of PPA for the left hemisphere, especially for its language‐related cortices. A few patients with PPA display the neuropathological markers of Alzheimer's disease, but in an unusual distribution. The majority of the autopsies in PPA have shown either Pick's disease or lobar atrophy without distinctive histopathology. The suggestion has been made that PPA and frontal lobe dementia constitute phenotypical variations of a unitary disease process within the “Pick‐lobar atrophy” spectrum. Recent advances in chromosome 17‐linked dementias justify a rigorous search for tau polymorphisms and tauopathy in sporadic PPA. An informed approach to this syndrome will increase the effectiveness with which clinicians can address the unique challenges associated with the diagnosis and care of PPA. Ann Neurol 2001;49:425–432
Annals of Neurology - Tập 49 Số 4 - Trang 425-432 - 2001
Slowly progressive aphasia without generalized dementia Abstract Six right‐handed patients experienced a slowly progressing aphasic disorder without the additional intellectual and behavioral disturbances of dementia. The symptoms almost universally started in the presenium. The initial difficulty was an anomic aphasia in five of the patients and pure word deafness in the sixth. Continuous and gradual deterioration occurred in the five patients who presented with an anomic aphasia. They eventually experienced additional impairment of reading, writing, and comprehension. In four patients, other areas of comportment were not involved within the 5 to 11 years of follow‐up. A more generalized state of dementia may have emerged in the other two patients, but only after 7 years of progressive and debilitating aphasia. Neurodiagnostic procedures were consistent with preferential involvement of the left perisylvian region. In one patient, cortical biopsy did not show any pathognomonic change; specifically, no neurofibrillary tangles, amyloid plaques, neuronal inclusions, or gliosis were seen. This condition may constitute a syndrome of relatively focal cerebral degeneration with a predilection for the left perisylvian region.
Annals of Neurology - Tập 11 Số 6 - Trang 592-598 - 1982
Amyotrophic lateral sclerosis: Problems and prospects Amyotrophic lateral sclerosis (ALS) is a lethal degenerative disorder of motoneurons, which may occur concurrently with frontotemporal dementia. Genetic analyses of the ∼10% of ALS cases that are dominantly inherited provide insight into ALS pathobiology. Two broad themes are evident. One, prompted by investigations of the SOD1 gene, is that conformational instability of proteins triggers downstream neurotoxic processes. The second, from studies of the TDP43, FUS, and C9orf72 genes, is that perturbations of RNA processing can be highly adverse in motoneurons. Several investigations support the concept that non‐neuronal cells (microglia, astroglia, oligodendroglia) participate in the degenerative process in ALS. Recent data also emphasize the importance of molecular events in the axon and distal motoneuron terminals. Only 1 compound, riluzole, is approved by the US Food and Drug Administration for ALS; several therapies are in clinical trials, including 2 mesenchymal stem cell trials. The challenges and unmet needs in ALS emphasize the importance of new research directions: high‐throughput sequencing of large DNA sets of familial and sporadic ALS, which will define scores of candidate ALS genes and pathways and facilitate studies of epistasis and epigenetics; infrastructures for candidate gene validation, including in vitro and in vivo modeling; valid biomarkers that elucidate causative molecular events and accelerate clinical trials; and in the long term, methods to identify environmental toxins. The unprecedented intensity of research in ALS and the advent of extraordinary technologies (rapid, inexpensive DNA sequencing; stem cell production from skin‐derived fibroblasts; silencing of miscreant mutant genes) bode well for discovery of innovative ALS therapies. Ann Neurol 2013;74:309–316
Annals of Neurology - Tập 74 Số 3 - Trang 309-316 - 2013
Movement‐related cortical potentials in writer's cramp Abstract Movement‐related cortical potentials in response to simple, self‐paced, brisk index finger abduction movements were recorded in patients with simple and complex writer's cramp and compared with those of age‐matched control subjects. Analysis of the movement‐related cortical potential waveforms showed that the Bereitschaftspotential, the peak of the negative slope, and the frontal peak of the motor potential did not differ in the two groups, except for the average amplitude of the early part of the negative‐slope peak, which was decreased in the patient group during the interval of 300 to 200 msec prior to electromyographic onset. This finding was restricted to the electrodes overlying the contralateral and midline central electrodes. Movement‐related cortical potentials from patients and control subjects could be equally accounted for by a four‐dipole source model with sources located in the contralateral and ipsilateral sensorimotor regions and the supplementary motor area. There was a trend for a reduction in the strength of the sensorimotor sources active during the premotor period in the patient group, but the difference did not reach a significant level for any individual source. No differences were found between the movement‐related cortical potentials elicited by movements of the affected and unaffected hand, or between those of patients with simple or complex hand cramps. This result suggests a deficiency of contralateral motor cortex activation just prior to the initiation of voluntary movements in patients with focal dystonia.
Annals of Neurology - Tập 38 Số 6 - Trang 862-868 - 1995
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