The dementia with Lewy bodies (DLB) Consortium has revised criteria for the clinical and pathologic diagnosis of DLB incorporating new information about the core clinical features and suggesting improved methods to assess them. REM sleep behavior disorder, severe neuroleptic sensitivity, and reduced striatal dopamine transporter activity on functional neuroimaging are given greater diagnostic weighting as features suggestive of a DLB diagnosis. The 1-year rule distinguishing between DLB and Parkinson disease with dementia may be difficult to apply in clinical settings and in such cases the term most appropriate to each individual patient should be used. Generic terms such as Lewy body (LB) disease are often helpful. The authors propose a new scheme for the pathologic assessment of LBs and Lewy neurites (LN) using alpha-synuclein immunohistochemistry and semiquantitative grading of lesion density, with the pattern of regional involvement being more important than total LB count. The new criteria take into account both Lewy-related and Alzheimer disease (AD)-type pathology to allocate a probability that these are associated with the clinical DLB syndrome. Finally, the authors suggest patient management guidelines including the need for accurate diagnosis, a target symptom approach, and use of appropriate outcome measures. There is limited evidence about specific interventions but available data suggest only a partial response of motor symptoms to levodopa: severe sensitivity to typical and atypical antipsychotics in approximately 50%, and improvements in attention, visual hallucinations, and sleep disorders with cholinesterase inhibitors.
Although essential tremor (ET) is considered the most prevalent adult movement disorder, the available information on its prevalence and distribution worldwide is not completely understood. We investigated the prevalence and distribution of ET in three elderly Spanish populations using a door-to-door, two-phase approach. A brief screening instrument was administered on May 1, 1994 to subjects over 64 years old taken from the census of one urban municipality of Greater Madrid (quarter of Margaritas, Getafe), one urban district of Madrid (Lista), and one rural site (Arévalo county, Avila) (N = 5278). Study subjects were limited to those who screened positively (N = 472). To increase reliability, each patient was examined by 3 experienced neurologists, and was classified as having ET only when all 3 neurologists agreed (183 of 472). The present study was part of a large-scale epidemiological survey of neurological diseases, and served as a baseline investigation in a 3-year incidence study. Accordingly, 41 ET patients were identified when evaluating subjects who had screened positively for dementia, stroke, or parkinsonism, despite the fact that they had screened negatively for tremor; furthermore, 32 additional ET prevalent cases were detected when evaluating subjects who had screened positively for tremor in the second cross-sectional study (May 1, 1997), although they had screened negatively for tremor in the first cross-sectional study. We identified 256 persons (152 women, 104 men) with ET; of these, 87 patients (34.0%) reported having an affected relative. Two hundred and four (79.7%) of the subjects with ET were detected through this screening and had not been diagnosed previously. The prevalence of ET was 4.8% (95% CI = 4.2-5.4) for the total population; 4.6% (95% CI = 3.7-5.4) in men and 5.0% (95% CI = 4.2-5.8) in women. Age-specific prevalence increased with advancing age for both men and women. Despite the variability in worldwide data, ET is a frequently encountered disorder in elderly people. Furthermore, as ET may be seen as a relatively benign condition, a large proportion of patients may never seek neurological attention.
The prevalence of Parkinson's disease (PD) and other types of parkinsonism in three elderly populations of central Spain was investigated using a door-to-door, two-phase approach. This design called for the administration of a brief questionnaire to subjects 65 years of age or older taken from the census of one urban municipality of Greater Madrid (Margaritas, Getafe), one rural site (Arévalo County, Avila), and one urban district of Madrid (Lista) in Spain (N = 5,278). Study neurologists extensively investigated those subjects who screened positively. The diagnoses, based on specified criteria, were reviewed to increase reliability across neurologists. We found 118 subjects with parkinsonism: 81 affected by PD (68.6%), 26 drug-induced parkinsonism (22.0%), 6 parkinsonism in dementia (5.1%), 3 vascular parkinsonism (2.5%), and 2 unspecified parkinsonism (1.7%). The prevalence was 2.2% (95% confidence interval [CI], 1.8-2.6) for all types of parkinsonism and 1.5% (95% CI, 1.2-1.8) for PD. The prevalence estimates of parkinsonism and PD increased with age, declining at 85 years and over. Age prevalence ratios were higher for men. Twenty-three subjects (28.4%) of the subjects with PD were detected through the screening and had not been diagnosed previously. Overall prevalence estimates of PD and other types of parkinsonism in central Spain rank at levels similar to those recently reported for other European and non-European elderly populations. Despite improvement in access to health services, an important proportion of PD patients may never seek neurological attention.
We compared CSF and serum levels of iron, copper, manganese, and zinc, measured by atomic absorption spectrophotometry, in 26 patients patients with Alzheimer's disease (AD) without major clinical signs of undernutrition, and 28 matched controls. CSF zinc levels were significantly decreased in AD patients as compared with controls (p < 0.05). The serum levels of zinc, and the CSF and serum levels of iron, copper, and manganese, did not differ significantly between AD-patient and control groups. These values were not correlated with age, age at onset, duration of the disease, and scores of the MiniMental State Examination in the AD group. Weight and body mass index were significantly lower in AD patients than in controls. Because serum zinc levels were normal, the possibility that low CSF zinc levels were due to a deficiency of dietary intake seems unlikely. However, it is possible that they might be related to the interaction of beta-amyloid and/or amyloid precursor protein with zinc, that could result in a depletion of zinc levels.
We compared CSF and serum levels of iron, copper, manganese, and zinc, measured by atomic absorption spectrophotometry, in 37 patients with Parkinson's disease (PD) and 37 matched controls. The CSF levels of zinc were significantly decreased in PD patients as compared with controls (p < 0.05). The serum levels of zinc, and the CSF and serum levels of iron, copper, and manganese, did not differ significantly between PD-patient and control groups. There was no influence of antiparkinsonian therapy on CSF levels of none of these transition metals. These values were not correlated with age, age at onset, duration of the disease, scores of the Unified Parkinson Disease Rating Scale of the Hoehn and Yahr staging in the PD group, with the exception of CSF copper levels with the duration of the disease (r = 0.38, p < 0.05). These results suggest that low CSF zinc concentrations might be related with the risk for PD, although they could be related with oxidative stress processes.
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