Prevalence of Parkinson's disease and other types of Parkinsonism. A door-to-door survey in Bidasoa, Spain.

OBJECTIVES: To assess the prevalence of Parkinson's disease and parkinsonism in two Spanish populations (Irun and Hondarribia, Bidasoa Region) and to compare the results with those of similar surveys. METHODS: The survey included 2000 participants aged 65 years or older in a door-to-door, three-phase design. In the screening phase we used the SNES (Sicilian Neuro-Epidemiologic Study) screening questionnaire, which has 100% sensitivity. In phases 2 and 3 we carried out a 3-year follow-up of all cases diagnosed with parkinsonism in phase 2. Progressively stricter diagnostic criteria were chosen in order to minimize the impact of false positives on the final results. RESULTS: The prevalence of Parkinson's disease (PD) was 1.5 % (95% confidence interval, 0.9 to 2.3) and the prevalence of other types of parkinsonism (OP) was 1.1 % (95% confidence interval 0.6 to 1.9). The overall prevalence by age group was 0.4 % (65-74 years), 4.7% (75-84 years), and 2.9% (> or =85 years) for Parkinson's disease and 0.7%, 2%, and 3.9 % for parkinsonism, respectively. The other parkinsonism prevalence was 1.3 % in men and 1.6 % in women. CONCLUSIONS: These prevalence rates are similar than those found in studies made in other European countries. The prevalence of both Parkinson's disease and other types of parkinsonism increased with age, with no significant differences between men and women.

The validity of Hodkinson's Abbreviated Mental Test for dementia screening in Guipuzcoa, Spain.

The objective of this research was to evaluate the validity of Hodkinson's Abbreviated Mental Test (AMT) in screening for dementia and to identify the optimum cut-off point to use in a prevalence survey. The study included two groups of persons: (i) a random sample of 183 individuals selected from census data, 96 of whom completed the study and (ii) another 36 persons with dementia were selected from a hospital outpatients department by sampling consecutive cases. The DSM-IV criteria were used as the "gold standard" to establish a diagnosis of dementia. The AMT was administered to the 132 participants who subsequently underwent independent clinical evaluation. In the community sample, 11 persons were diagnosed with dementia and 85 without. In the total sample, a score of 7 maximizes the efficacy of the test. The sensitivity for this cut-off point is 91.5% (78.7-97.2%) and the specificity is 82.4% (72.2-89.5%). A score of 9 gives 100% sensitivity, but the proportion of false positives rises to 66%. Our results are consistent with other studies and suggest that the AMT is a valid instrument for use in screening for dementia in populations similar to the one in this study.

[The CADASIL syndrome: a model of subcortical-cortical disconnection]. / El síndrome de CADASIL: un modelo de desconexión córtico-subcortical.

INTRODUCTION: CADASIL syndrome (Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarts and Leukoencephalopathy) includes some neurological signs and symptoms (gait disturbances, epileptic seizures, pseudobulbar palsy, migraines, etc.), as well as neuropsychological dysfunctions (cognitive and executive impairment, emotional disorders and, frequently, dementia). This syndrome is a good model of white matter damage and potential disconnection syndromes. CLINICAL CASE: In this article, the neuropsychological profile of a 47 year-old woman diagnosed of CADASIL is investigated and thoroughly discussed. Results show the presence of a moderate cognitive and executive impairment, specially of memory, psychomotor abilities, executive functions and verbal fluency, but not dementia, overall suggesting the presence of a temporal-frontal-subcortical disfunction. CONCLUSION: This clinical pattern is an illuminating example of the neuropsychological consequences of the partial disconnection of prefrontal cortex from the thalamus and basal ganglia.

Prevalence of essential tremor: a door-to-door survey in bidasoa, spain.

OBJECTIVES: To assess the prevalence of essential tremor (ET) in two Spanish populations (Irun and Hondarribia, Bidasoa region) and to compare the results with those of similar surveys. METHODS: The survey included 2,000 participants aged 65 years or older in a door-to-door, two-phase design. ET was defined as postural or kinetic tremor of the head or limbs. RESULTS: ET prevalence after age adjustment was 4.8% (95% confidence interval, 3.6-6.4). Prevalence increased significantly with age and there were no sex differences. CONCLUSIONS: The age-adjusted prevalence rate of ET in people 65 years old and older in Bidasoa, Spain, is close to those described in other studies using a similar design and suggest no geographical variation. ET prevalence increases with age and has a similar distribution in males and females.

[Neuropsychological implications of Crouzon syndrome: a case report]. / Implicaciones neuropsicológicas del síndrome de Crouzon: estudio de un caso.

INTRODUCTION: Crouzon syndrome (CS) has traditionally been associated with moderated mental retardation. However, very few studies have quantified in these patients the degree of this mental retardation, and even less research has been carried out to evidenciate the typical neuropsychological profile, including affective, cognitive and executive functions. CLINICAL CASE: We present a single case-study of a 16 year-old, right-handed female affected of CS. Neuropsychological functions assessed included attention, concentration, memory, visual perception, language, arithmetic processes, praxis, executive functions, reasoning and general intelligence. Deficient results have been obtained in learning and memory, attention, language, graphic praxis, comprehension of social situations and executive functions (frontal lobes). Nevertheless, the patient kept within normal limits her spatial and temporal orientation, visual perception, visual-spatial and manipulative abilities and arithmetic processes. CONCLUSIONS: Results suggest that the intellectual defect in CS is not general and uniform and that some intellectual abilities are preserved, while others are affected in different degrees. The importance of carrying out a thorough neuropsychological assessment in these cases is emphasized.

[Causes of blindness and disability pensions in the eastern metropolitan area of Santiago]. / Causas de ceguera y pensión de invalidez en el Area Metropolitana Oriente de Santiago.

Between June 1983 and September 1987, 2799 disability pensions were given in the East Santiago Area, population 676,269. Of them, 222 (7.9%) were due to ophthalmological causes. Blindness as defined by the WHO was present in 57 cases. It was caused by myopia (33%), glaucoma (21%), diabetic retinopathy (9.8%), retinal detachment, cataract and optic atrophy (5.2%) and ocular trauma (3.5%).

Apraxia in a patient with lesion located in right sub-cortical area. Analysis of errors.

This report describes a case of apraxia caused by a right sided sub-cortical lesion. Performance on test for apraxia was impaired. A new type of error which involved demonstrating the use of objects on one's own body is reported. Some theoretical interpretations of the findings are discussed.

[Alexia without agraphia, right homonymous hemianopsia, and color anomia. Report of four cases with location of the lesion by computerized axial tomography of the brain (author's transl)]. / Alexia sin agrafia, hemianopsia homónima derecha y anomia de colores. A propósito de cuatro observaciones con topografía conocida mediante TAC craneal.

Two patients with left occipitotemporal hematomas and another two with ischemic lesions in the territory of the left posterior cerebral artery presented alexia without agraphia, right homonymous hemianopsia, and a disturbance in the naming of colors. The etiology and the location of the lesion was demonstrated by computerized axial tomography in all four cases. After a period of follow-up of more than one year a good evolution was substantiated in the patients with hematoma. The mechanisms underlying the appearance of symptoms are discussed, and a comment is made on the importance of the splenium of the corpus callosum in the transfer of visual impulses, with the dissociations appearing in the disconnection syndromes due to lesions at this level.

[Alcoholism, polyneuropathy, and liver disease. A prospective electrophysiologic study]. / Alcoholismo, polineuropatía y enfermedad hepática. Estudio prospectivo electrofisiológico.

A prospective clinical and electrophysiologic study was performed in 50 alcoholic patients in order to assess the incidence and clinical manifestations of alcoholic polyneuropathy, the value of the electrophysiologic parameters studied, and the influence of liver disease on the appearance of neuropathy. The proposed criteria for polyneuropathy were fulfilled by 24% of the patients. Abolition of the Achillean reflexes was the most frequent clinical finding, while reduced amplitude of muscular potential was the most sensitive electrophysiologic parameter. There were no differences in alcohol consumption, frequency of liver disease, and degree of alteration of liver function in cirrhotics between patients with and without polyneuropathy.