Alzheimer A. Über eine eigenartige Erkrankung der Hirnrinde Allgemeine Zeitschrift fur Psychiatrie und Psychisch-gerichtliche Medizin. 1907 Jan ; 64():146-8.
The English translation of Dr. Alzheimer's paper appears in the book, The Early Story of Alzheimer's Disease, edited by Katherine Bick, Luigi Amaducci and Giancarlo Pepeu. It is reprinted here with permission of the Distributor, RavenPress, New York.
A Characteristic Disease of the Cerebral Cortex
Alzheimer reports the case of a patient who was kept under close observation during institutionalization in Frankfurt am Main and whose central nervous system was examined by Director Sioli.
The patient showed early clinical symptoms which deviated from the common ones and could not be classified under any well-known clinical patterns. The anatomical findings were also different from those of the usual disease processes.
One of the first disease symptoms of a 51-year-old woman was a strong feeling of jealousy towards her husband. Very soon she showed rapidly increasing memory impairments; she could not find her way about her home, she dragged objects to and fro, hid herself, or sometimes thought that people were out to kill her, then she would start to scream loudly.
During institutionalization her gestures showed a complete helplessness. She was disoriented as to time and place. From time to time she would state that she did not understand anything, that she felt confused and totally lost. Sometimes she considered the coming of the doctor as an official visit and apologized for not having finished her work, but other times she would start to yell in the fear that the doctor wanted to operate on her; or there were times that she would send him away in complete indignation, uttering phrases that indicated her fear that the doctor wanted to damage her woman's honour. From time to time she was completely delirious, dragging her blankets and sheets to and fro, calling for her husband and daughter, and seeming to have auditory hallucinations. Often she would scream for hours and hours in a horrible voice.
As she was unable to understand any particular situation, she got upset any time a doctor wanted to examine her. Only after several efforts was it possible to obtain any data.
She suffered from serious perception disorders. When the doctor showed her some objects she first gave the right name for each one, but immediately afterwards she had already forgotten everything. While reading she would omit sentences, she would spell every word or read without intonation. In a writing test she often repeated the same syllables, omitting others, and became confused and absent-minded. In her conversation she often used confused phrases, single paraphrasic expressions (milk-jug instead of cup), sometimes she would suddenly stop talking completely. She evidently did not understand many questions. She did not remember the use of particular objects. She still walked normally and had full use of her hands. There were no motoric disturbances in her gait or use of her hands. Her pupils reacted normally. She had somewhat rigid radial arteries, no cardiac hypertrophy, no albumen.
During the course of the disease symptoms appeared which could be considered focal symptoms; sometimes these were very prominent and sometimes quite faint. But they were always very mild. Mental regression advanced quite steadily. After four and a half years of illness the patient died. She was completely apathetic in the end, and was confined to bed in a fetal position (with legs drawn up), was incontinent and in spite of all the care and attention given to her she suffered from decubitus.
The autopsy showed an evenly affected atrophic brain without macroscopic foci. The larger cerebral vessels showed arteriosclerotic changes.
The Bielschowsky silver preparation showed very characteristic changes in the neurofibrils. However, inside an apparently normal-looking cell, one or more single fibers could be observed that became prominent through their striking thickness and specific impregnability. At a more advanced stage, many fibrils arranged parallel showed the same changes. Then they accumulated forming dense bundles and gradually advanced to the surface of the cell. Eventually the nucleus and cytoplasm disappeared, and only a tangled bundle of fibrils indicated the site where once the neuron had been located.
As these fibrils can be stained with dyes different from the normal neurofibrils, a chemical transformation of the fibril substance must have taken place. This might be the reason why the fibrils survived the destruction of the cell. It seems that the transformation of the fibrils goes hand in hand with the storage of an as yet not closely examined pathological product of the metabolism in the neuron. About one-quarter to one-third of all the neurons of the cerebral cortex showed such alterations. Numerous neurons, especially in the upper cell layers, had totally disappeared.
Dispersed over the entire cortex, and in large numbers especially in the upper layers, miliary foci could be found which represented the sites of deposition of a peculiar substance in the cerebral cortex. It was even possible to recognize these without staining, but they were much more evident once stained.
The glia had abundant formed fibers; in addition, many glia cells showed large deposits.
There was no infiltration of the vessels. Against this, focal lesions in the endothelium could be observed, and in some sites new vessel formation could also be seen.
On the whole, it is evident that we are dealing with a peculiar, little-known disease process. In recent years these particular disease-processes have been detected in great numbers. This fact should stimulate us to further study and analysis of this particular disease. We must not be satisfied to force it into the existing group of well-known disease patterns. It is clear that there exist many more mental diseases than our text books indicate. In many such cases, a further histological examination must be effected to determine the characteristics of each single case. We must reach the stage in which the vast well-known disease groups must be subdivided into many smaller groups, each one with its own clinical and anatomical characteristics.
Copyright Liviana Editrice, 1987.