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Biomedical subjects

J Muruzabal

Publications and source records attributed to J Muruzabal.

4 recordsLinked to original sources

Prevalence of Alzheimer's disease and other dementing disorders in Pamplona, Spain.

We investigated the prevalence of dementing disorders in the city of Pamplona, Spain, using a door-to-door two-phase approach. We first administered the Cambridge Examination of Mental Disorders of the Elderly (CAMDEX) to all survivors, as of March 1, 1991, of a probability sample of the total population identified in 1989 (n = 1,127). Using specified diagnostic criteria, the study neurologists extensively investigated those subjects who screened positive on CAMDEX. We found 194 subjects affected by dementia, 119 had Alzheimer's disease; 51 vascular or mixed dementia, and 16 secondary dementia. The prevalence of both dementia and Alzheimer's disease increased steeply with advancing age and was consistently higher in women. The prevalence of combined vascular and mixed dementia increased less rapidly with age, and was generally higher in men. Alzheimer's disease was the most common type of dementia. Our prevalence figures for dementia and Alzheimer's disease are similar to those previously reported in Europe.

Age Factors↗

Cortical mechanisms mediating asterixis.

We describe a patient with chronic renal failure who suffered multifocal action-induced jerks. Electromyography (EMG) recorded the typical silence of asterixis. Back-averaging the EEG activity preceding the EMG silent periods in the forearm showed a biphasic wave antedating the asterixis by 23 ms. Somatosensory evoked potentials (SEPs) after median nerve stimulation were pathologically enlarged on both hemispheres. Brain-mapping of the biphasic wave preceding asterixis and the giant SEPs indicated a common origin in the sensorimotor cortex. This observation provides further documentation of a cortical origin for some types of asterixis in humans.

Brain Mapping↗

Abnormal muscle and skin mitochondria in family with myoclonus, ataxia, and deafness (May and White syndrome).

A mother and two of her daughters had deafness and cortical reflex myoclonus; the mother also had mild truncal ataxia. Muscle and skin biopsy specimens revealed abundant ragged-red fibres and abnormal mitochondria. The son of one of the daughters had sensorineural deafness. Three other grandchildren were asymptomatic. The two daughters also had diabetes mellitus, hypertension and cardiomyopathy. Another daughter died of renal failure. The mother lost her hearing in her 70s, one daughter in her 30s, and the other daughter and the grandson in their 20s. The mother has had transient episodes (24-48 hours) of temporal disorientation, severe action myoclonus, and ataxia for about eight years. This is the first reported family with inherited deafness, myoclonus, and ataxia with mitochondrial pathology.

Adolescent↗