3-12-10 Familial progressive supranuclear palsy. Clinical polymorphism

September 8, 2017 | Autor: José Ramón Ara | Categoria: Polymorphism, Progressive Supranuclear Palsy, Clinical Sciences, Neurosciences
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Dementia, Cognitive Decline, Aging free radicals and excess glutamate. Numerous epidemiological studies indicate that populations taking antiinflammatory drugs have a sharply reduced prevalence of AD. One small clinical trial with indomethacin showed arrest of the disease over a 6 month period. Therapeutic intervention in key inflammatory processes holds great promise for the amelioration of AD and possibly other neurodegenerative disorders.

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Prevalence of dementia1 syndromes in a population of Uruguay. Study of “Villa del Cerro”

C. Ketzoian, S. Romero, E. Dieguez, G. Coirolo, I. Rega, R. Caseres, C. Chouza. Neuroepidemio/ogy Section, Laboraroty of Neuropsychology. lnsfitute of Neurobg~ School of Medicine, University of the Repub/ic, Montevideo, Uruguay This investigation determined the point-prevalence of dementia1 syndromes in a population of Uruguay. The findings were derives from a large-scale morbidity survey involving more than 21.000 inhabitants conducted in ‘Villa del Cerro”, a neighbourhood of Montevideo, capital city of Uruguay. Both the house-hold and the institutionalized populations of the “Villa del Cerro” were included. It is a descriptive, transversal-type, two-phase, epidemiological study. The reference date for the transversal cut was fixed for September lst, 1993. In phase I, a screening questionnaire was applied in order to select possible cases; in phase II we examined the selected cases (306) using a standardized protocol, in order to study intelligence, memories, language, praxias and gnosias, and the Folstein minimental test. Results were limited to persons 40 years old or older. Prevalence rates will be presented, total and specific by age, sex and socio-economic level. The findings concerning the etiology of dementia1 syndromes in this community-based survey will be discussed.

3-12-08 T.A. Walzer, Neurological

Phenomenology of depressive Alrheimer’s disease (AD)


E. Chemerinski, L. Sabe, S.E. Starkstein. Research, Buenos Aires, Argentina


Raul Carrea



Objective: To examine the specificity of depressive symptoms among patients with AD. Methods: We assessed 31 patients with AD and a depressed mood, 31 AD patients without depression (matched for age, gender, and Mini-Mental scores), 31 depressed patients without dementia (matched for age and gender), and 8 normal controls. Patients were examined with a comprehensive psychiatric scale. Results: AD-depressed patients showed significantly less worrying and loss of appetite than depressed patients without AD. ADdepressed patients showed significantly more worrying, anxiety, ideas of reference, social withdrawal, loss of appetite, and psychotic symptoms than AD patients without a depressive mood. Finally non-depressed AD patients reported significantly more thinking problems and difficulties of concentration than age-comparable controls. Conclusions: There were 3 important findings: 1) the phenomenology of depression in AD is similar to primary (i.e., no known brain injury) depression; 2) autonomic and affective symptoms of depression are significantly more prevalent among AD patients with a depressive mood; and 3) thinking problems should be excluded from the diagnostic criteria of depression in AD.


Neurocognitive scales standardisation for dementia study: Adas Cog. and Mattis Dementia Scale (MDS)

E. Labos, C. Atlasovich, S. Vanotti, A. Renato. Language of Medicine, Universiry of Buenos Aires, Argentina



This study reports the results obtained by the assesment of control healthy subjects with two scales: Adas Cog. and MDS. The specific objectives are: (1) To introduce the Rioplatense Spanish version from Adas Cog. based on the Greco Group’s French version. (2) To determine the major variability subtest in both scales considering the following variables: sex, age, level of instruction and dominance. Method and Material: 188 subjects, 57 male and 131 female, age: M = 69.46 f 7.66 studied by MDS and 147 subjects, 53 male and 94 female, age: M = 66.01 f 10.76 studied by Adas Cog. Results: The mean score for MDS was 133.6 f 5.65 and for Adas Cog., 5.39 f 3.09. Age and level of instruction were the most significative variables to determine the total score’s means. Regarding age, the most sensible items showing a progressive decrease were: Word Recall and Word Recognition in Adas cog. All the subtests under MDS show a steady decrease curve related to age and level of instruction variables. The data analysis allows us to cathegorize the results from items and subtests showing the most sensible results in the control group. The study permits us to validate and standardize both Scales in Dementia in our population.

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Familial progressive polymorphism


palsy. Clinical

J.L. Capablo, E. Marta, E. Mostacero I, J.A. Gimenez-Mas 2, I. Ferrer 3, J.R. Ara. Department of Neurology: 2 Department of Neuropathology, Miguel Servet Hospital, ’ Clinic0 Hospital, Zaragoza, 3 Department of Neuropafho/ogy frincipes de Espaia Hospital, Barcelona, Spain The original clinical picture of progressive supranuclear palsy (PSP) is characterized by supranuclear ophthalmoplegia, axial dystonia in extension, pseudobulbar palsy and mental changes. The familial cases of PSP up until published are very limited. We described a family with PSP involving three members with confirmation histopathological in one patient and differential clinical spectrum between individuals. Patients: Case 1. A-year-old 80 woman, presented a moderate global deterioration affecting performance intelligence quontient (IQ) to a greater extent than verbal IQ, compulsive behaviors disturbances and marked motor perseveration, impaired attention, poor abstract thinking, mild memory loss with preservation of short-term memory. Later, it was followed by dysarthria and progressive impairment cognitive until a profound dementia. She died at age 66 years. The neuropathological examination confirmed accumulation of neurofibrillary tangles (NFT), nerve cell loss and gliosis in pallido-subthalamic area; and zona compacta substantia nigra; and peri-aqueductal grey matter; and reticular subtantia of the brainstem. In immunohistochemical studies of NFT were positive for TAU and weakly positive for ubiquitin. In the frontal cortex were detected small number of NFT positive for TAU and moderate neuronal loss. Case 2. Sister, a 61 years-old man, the first manifestations was a dementia with impaired for frontal lobe function, followed by dysarthria and dysphagia. He died at age 67 years. Case 3. Sister, a 56 years-old-woman presented with pseudobulbar palsy. Dysarthria, dysphagia and emotional incontinence. The tongue is contracted and movements slow. Also hearing loss, gait disturbances and pyramidal signs developed. The patient died at age 58 years. We reported a family with PSP. The clinical symtoms varied between yours members with atypical clinical findings beyond those noted in the original descriptions of this disorders. It was not found supranuclear opthalmoplegia up until now considered the most important distinguishing feature of PSP. Two patients developed a dementia with greatest impainent of frontal lobe function as clinical picture. We conclude that in PSP: l.- Genetic factors play an important role; 2.- The clinical features show wide variations. This support that the frequency of individual and familial PSP may be much greater and misdiagnosis are common.


Tropicamide test in the clinical evaluation Alzheimer dementia (preliminary data)


L. Pasqui, G. D’Andrea, A. Monaco, C. Lanzieri, I. Ponza’. Department of Neurology and Oftalmolog~ Esfe Hospital, /fa/y, ’ Depament of Neurolog)! Padua, /ta/y The diagnosis of Alzheimer dementia is still a difficult task. However recently it has been reported that, as in individual affected by Down syndrome, patients with probable Alzheimer’s disease exhibit an hypersensitivity in pupil response dilatation to a cholinergic antagonist tropicamide. In order to confirm these results and evaluate the possible usefulness of tropicamide test in Alzheimer’s disease,. we studied six patients affected by probable Alzheimer dementia aged between 56-75, one affected by multiinfarct dementia aged 55 and three normal control matched for age with patients. Before been evaluated, all subjects were seated in semidarkened room and, after 5 minutes, the pupil diameter was measured with a computerized pupillometer. In same subjects, free of any ocular illness, two drops of low tropicamide solution (0.05%) were placed in both eyes The pupil diameter was measured after 30 and 60 min. in same environmental conditions. Demented Alzheimer-Type showed a mean of pupil diameters bigger both at 30 (30%) and 60 (36%) minutes in comparison to the basal condition and control subjects (mean 18% and 19.5% respectively). The patient with multi-infarct dementia exhibit an increase similar to control (19 and 19%). These results further suggest that Alzheimer-Type patients are characterized by a hypersensitivity in the pupil response to cholinergic antagonist and perhaps tropicamide can be helpful test in the precoce diagnosis of Alzeimer’s disease.

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Population epidemiologic survey of dementia Catanduva, Brazil: Preliminary results

E. Herrera, P. Caramelli, A.S.B. of Internal Medicine, University and Departments of Neurology Paul0 Faculty of Medicine, S8o


Silveira, S.C. Mathias, R. Nitrini. Department of Catanduva Faculty of Medicine, Catanduva and Medical Informatics, University of Sk50 Paula, Brazil

Objective: To report the preliminary findings on the prevalence a population-based study in the city of Catanduva, Brazil.

of dementia


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