Creutzfeldt-Jakob disease.

This label is applied to a group of patients whose neurological and psychiatric symptoms are somewhat varied and whose brains show pathological changes which are basically similar, although their distribution and extent in the nervous system may vary (Siedler and Malamud, 1963). In the early 19203 A. Jakob (1921, 1923) described five patients aged between 30 and 50 who had presented with somewhat comparable symptoms and in whom the brains showed reasonably similar pathological changes. The patients complained ofabnormal sensations in the limbs and tended to become spastic. Jerky movements occurred and mental changes developed which usually led to profound dementia. There were often bulbar signs such as aphonia and dysphagia. In necropsied cases there was degeneration in the brain with loss of numbers of nerve cells, degenerative changes in many of the Fig. 1 Coronal slice through the cerebra, surviving cells, and an increase in glial cells. These of a case of Creutzfeldt-Jakob disease in + changes were found in the cortex, basal ganglia, and cortical ribbon was not narrowed and the motor nuclei of the brain stem and spinal cord. In not appreciably enlarged. the brain stem and spinal cord the changes were comparable with those seen in amyotrophic lateral sclerosis (motor neurone disease). The similarities, both clinical and pathological, between Jakob's cases are great enough to enable one to feel that they are representative of a fairly specific disease process. Speilmeyer suggested that a patient described by Creutzfeldt in 1920 was also suffering from a similar disease. The brain of this girl, aged 20, showed somewhat similar pathological changes and Spielmeyer therefore suggested the name CreutzfeldtJakob disease. It is, I think, a pity that this case has been included as an example of the syndrome. Since tw that time there have been numerous reports; Kirschbaum (1968) collected 150 cases from the literature and there have been several later reports. We have examined 12 cases of the syndrome. Macroscopically the brain may appear normal (Fig. 1), apart from slight generalized ventricular dilatation, though some cases show a much thinned cortical ribbon with marked loss of white matter, Fig. 2 Similar slice from a case of Creu, causing considerable ventricular dilatation (Fig. 2). disease in which there was great narrowin, Microscopically all of our cases showed status ribbon, marked widening of the sulci, and spongiosus in the grey matter of the cortex (Figs. 3, ventricular dilatation. 97 hemispheres which the ventricles were