Hans Förstl


Alois Alzheimer

Die Seelenstörungen auf arteriosklerotischer Grundlage.

Allgemeine Zeitschrift für Psychiatrie 59:695-710

Mental Disturbances of Arteriosclerotic Origin

   . . . Binswanger and I first described arteriosclerotic brain atrophy in detail in 1894 at a meeting of  German psychiatrists  and we pronounced the need to distinguish it from paralysis (Griesinger, 1876). On the same occasion Binswanger separated encephalitis subcorticalis chronica diffusa from arteriosclerotic brain atrophy. He stated that it also exhibited a severe arteriosclerosis of the brain vessels. This would suggest that the subcortical loss of myelin fibers is due to trophic disturbances caused by arteriosclerosis. Later on, I described two other types of disease caused by arteriosclerosis: perivascular sclerosis and senile cortical wasting. In view of the anatomica appearance on examination, Binswanger's subcortical encephalitis, senile cortical wasting, and perivascular sclerosis must be considered as subtypes of arteriosclerotic brain atrophy. Conditions in the human brain are different from other organs. It is without clinica1 importance whether half a kidney perishes, or whether a thousand miliary foci infiltrate the renal tissue, as long as the same number of glomeruli and epithelia remain intact. In the comp1icated physiologica1 and anatomical relations of the brain, however, vessels may be affected by arteriosclerosis in different locations, and the resu1ting clinica1 conditions bear so little resemblance to each other that it is difficult to give a summary of the symptoms of cerebral arteriosclerosis., . .
The basal ganglia and the internal capsule show a predilection for atheromatous disease and for bleeding. This may give rise to a peculiar disorder, dementia post apoplexiam (post-stroke dementia). Histologica1 examination demonstrates that dementia is not caused by stroke as such, but by arteriosclerotic areas in the hemispheres. This interpretation is underlined by the histological findings and by those cases in which dementia was evident before a stroke.. . .

The arteriosclerotic disorders mentioned so far are of a decisively progressive nature. There are other rather exceptiona1 cases, which are also undoubtedly caused by arteriosclerosis, but which exhibit only slight progression and usually only mild symptoms, that one could call 'nervous.' They are usually not admitted for inpatient treatment, but are quite frequently seen in general practice.. . . The histological findings in brain arteriosclerosis are quite specific and peculiar, and the clinical symptoms are so well-characterized that the diagnosis can be established during lifetime in the majority of cases. … The mildest form of arteriosclerosis, which we call the 'nervous' form ... is principally characterized by mental and physical fatigue, memory impairment, headache, and attacks of vertigo.

This disorder can be observed as soon in life as the early forties; most of my patients were between 50 and 65 years old. Myocardial enlargement and kidney wasting of substantial extent are often missing. The patients often become irritable and unable to sustain their work or to continue with their profession. Mental activity is restricted to stereotyped patterns; mental productivity is paralyzed. Conversation, calculation, and registration show the patients' fatigue immediately. This is often quite annoying for the patient himself.
The same is true for the memory impairment.

Sometimes only the patient complains about it, and there is no way of demonstrating it with ordinary methods. In these cases it is merely a subjective feeling of difficulty in retrieving certain ideas, and not a true deficit. Nevertheless a prolongation of reaction time can often be measured. Sometimes the patient succeeds by approaching the ideas in a roundabout way. Memory for names and numbers, which are least secure in our memory, is most impaired, especially with respect to registration of new information, but also with retrieval of old material.

Headache is usually frontal and only rarely parietal or occipital. It is described as oppressive and of tormenting intensity. It is often constant. In one case it vanished regularly during daytime. One patient complained of a regular increase in the headache during defecation.

Attacks of vertigo occur spontaneously or with a sudden move, when leaving bed, or with physical or mental strain. Another frequent complaint is flickering vision and sometimes tinnitus…..

Repeated examinations of the patient show striking variation in the intensity of symptoms. Sometimes I have also noticed an impaired retrieval of rather remote names, e.g., on looking at illustrated books, as a transient symptom. Similarly there was a striking impairment of word comprehension (mental hypacusis). I will not continue here with the enumeration of single symptoms, whose clinical importance will have to be studied in further cases.

A clear awareness of the disease and a definitive fear of becoming demented are always present. The symptoms appear to be largely reversible, but often remain stable for years. Death is finally caused by stroke, coronary artery sclerosis, or intercurrent diseases, and quite rarely by transition into a severe progressive form ....

The cases of Severe progressive arteriosclerotic brain degeneration will be summarized in the second group.

The ordinary form of arteriosclerotic brain atrophy may start with headache, attacks of vertigo, and memory impairment, quite similar to the nervous form. If the disease has not started with severe mental symptoms, then they will soon occur. Sometimes a morose, tearful mood, sometimes tantrums of distemper, unwilling stubbornness, or perplexed restlessness occur. Quite frequently these states are followed by a striking lassitude and dull apathetic behavior. A closer examination reveals that there are hardly any real deficits. The cause of the apathetic behavior is an extraordinary impairment of comprehension, reasoning, and retrieval. The condition shows marked fluctuations. The patient's sudden striking remarks about his person, situation, and surroundings are quite surprising. The fast variations are dazzling. Gradually, however, the deficits become more and more profound. appears severely disturbed. Large islands of remote memories are preserved, but sometimes their shores have to be reached by circumstantial questioning. Thereby the severe weariness becomes obvious. The patient's interests wander. Sometimes, however, a visit by relatives may arouse ideas and emotions which were deemed long forgotten. The patients' mood is mostly dull. I have never seen euphoria, but often tearful depression. Hallucinations and delusions occur mostly during transient states of excitement. Unlike Klippel, I have never seen ideas of grandiosity. Gradually a state of more profound and torpid mental impairment develops. Even in this profound state of dementia, the decay of personality is not uniform. Individual parts of the personality may remain strikingly preserved for a long time.

The course of disease is usually interrupted by frequent attacks, which may appear in quite extraordinary variations. Sometimes there are only attacks of vertigo, sometimes mild or severe epileptiform or apoplectic attacks. After these attacks meticulous examination may reveal indications offocal symptoms, such as asymbolic behavior, deficits of language, or visual field and cortical movement disorders. The attacks may also consist of purely mental disturbances, such as transient states of mild confusion, perplexity, states of hallucinatory excitement or confusion with excited raving.

An awareness of disease is often preserved for a strikingly long time. When asked, one of my patients used to respond, with tears in his eyes "I have become quite silly." I have also seen patients who are unable to speak and who put their fingers to the forehead to explain their inability to understand and to respond.

In these patients I have often observed a severe melancholic dysphoria and violent anxiety states which continued during the whole course of the disease, even if the anxiety symptoms appeared less emotional and more stereotyped during the last stage of disease. Sometimes a long and purely melancholic syndrome preceded the later, quite typical arteriosclerotic disease. I would not like to have to decide whether we should consider this illness a complication of presenile or senile melancholia with arteriosclerotic cerebral degeneration, or whether these depressive states are only a symptom of the course of cerebral arteriosclerosis.

The pupillary reactions are rarely lost. Language is often disturbed, more in the apoplectic than in the paralytic cases. Hemipareses are often the consequences of small capsular foci.
The ages of my patients were between 52 and 64 years, and the duration of disease between 1 and 6 years. Death occurred from cerebral palsy, apoplexia, myocardial paresis, kidney wasting, pneumonia, or diabetic coma ….

As I have already mentioned, clinically and anatomically different forms of cerebral arteriosclerosis may be caused by specific localizations of arteriosclerosis. One of the most typical of these is Binswanger's encephalitis subcorticalis chronica. This is an exceptionally severe arteriosclerotic degeneration of the long vessels to the hemispheric white matter, which often shows a high degree of atrophy. In particularly typical cases, the cortex and the immediate subcortical short association fibers are not affected, whereas the total remaining white matter is severely diseased. Thus, we could almost call it a system disease.. . .
The striking difficulties in associative reasoning  appear as one of the earliest and most obvious clinical symptoms. So on thereafter, slowly and gradually increasing language deficits are noted. Often, difficulties of verbal association precede the real defects. Soon the disease is complicated by attacks: attacks of vertigo, complete epileptic attacks, and apoplectiform insults. These are sometimes followed by excitement and confusion, often also by focal cerebral symptoms, restrictions of the visual field, asymbolia, motor and sensory aphasia, agraphia and monoplegia. These symptoms may largely vanish, recur, and finally remain stable. Sometimes they develop gradually, and sometimes suddenly, without mental, language, or motor disturbances. The focal phenomena often represent exceptionally isolated, limited defects, and their investigation will certainly permit interesting conclusions. Often there is a convulsive weeping, now and then stereotyped yelling. After a longer time various focal cortical symptoms are quite characteristic for this form of disease; they do, however, generally indicate only partial disturbances in the respective cortical area, and they often change in intensity. Sometimes one finds motor and sensory language disturbances, cortical pareses, or visual field defects. I have also observed a disturbance of depth perception, as described by Pick, in 2 cases.

Simultaneously there are often hemipareses, which are caused by capsular or pontine foci. In 3 cases I have found a striking difficulty in the coordination of eye movements without evident paresis. The pupils often react promptly until death, but I have also seen some cases of early light and accommodation paresis. The language is often strikingly slowed and monotonous with monosyllabic speech.

Also in these cases the awareness of disease is retained for a long time. In spite of his apparently profound mental decay, the patient may occasionally make remarks which disclose a certain recognition of his sad condition. It all ends, however, in a state of dementia reminiscent of decerebrated laboratory animals, as Binswanger has correctly reported. This state may last for years. In single cases it may also lead to death within a few months…..
The possibility of a clinical distinction [between paralysis and senile dementia, translator's note] is founded on the anatomical differences, as Binswanger has already mentioned. There can be no doubt that the different mental diseases which lead to dementia, i.e., to permanent mental defects-paralysis, senile dementia, epilepsy, some depressive states, and juvenile dementing psychoses-each forms a peculiarly characteristic mental impairment in such a way that in every disease the mental capacities are disturbed to a different degree and in a different combination.

The accurate investigation of these different forms of dementia is an important and unresolved task, which will perhaps reveal many valuable indicators for diagnosis. . . .
We can only comment on some particularly characteristic features [of arteriosclerotic dementia, translator's note]. The striking slowing of and difficulty in reasoning, the impairment of association, can be considered as such a symptom, which is noticeable early and which can be followed into the late stages of disease. The patient is aware of this deficiency and he makes great efforts to get over it. A genuinely depressed affect is usually not combined with it, but there is some feeling of helplessness and perplexity. The twilight or oneiroid features, which accompany similar states of paralysis and senile dementia, are completely missing in these cases. Similar to paralysis and dementia senilis, the interests of the patient are soon restricted by the disease, but the core of personal¬ity and correct judgment are preserved for much longer. This may explain the long awareness of dis¬ease. The affective life becomes dull, but we tend to find normal affect, apart from transient states of ex¬citement.

The mental deficits [in arteriosclerotic dementia, translator's note] although isolated, are often pro¬found, while they are more generalized in dementia senilis and paralytica, ifless marked at the beginning.

Moreover, arteriosclerotic brain atrophy is-apart from the attacks of excitement-in essence restricted to individual deficits, while signs of excitement, de¬pressed and elated affect, delusional ideas and real psychotic elements frequently contribute to dementia senilis and paralytica.

Therefore the patient with arteriosclerotic demen¬tia always appears as a patient with organic brain dis¬ease, whereas a patient with paralysis and senile de¬mentia appears to suffer from a mental disorder. Fur¬thermore there are the various peculiar attacks mentioned above, and finally the differences in physi¬cal and mental phenomena, which permit a differen¬tial diagnosis….

I would emphasize once again just how differently arteriosclerotic degeneration may start in the brain, and how many different clinical features it may cause. I hope to have proved that arteriosclerotic brain atro¬phy is a disorder which can definitely be distinguished from other brain diseases.

I also express my hope to have demonstrated that it was the combination of clinical and anatomical inves¬tigation which brought progress. One may well expect that this way will lead us one step further in a difficult territory, just as pathological anatomy and clinical observation walking hand in hand have gathered the most prosperous fruits in somatic medicine.

If that takes longer in psychiatry than in organic medicine, this is because of the much larger obstacles presented by the complicated histological conditions and clinical manifestations. Such difficulties, how¬ever, should serve only to stimulate us.

(translated by Hans Förstl, Robert Howard and Raymond Levy. A commented version was published in “Neuropsychiatry, Neuropsychology, and Behavioral Neurology” 1992)

Summary: Alzheimer wrote this paper in 1902 as a review of previous c1inical and neuropathologic work. He had himself contributed several original papers of seminal importance about various aspects of cerebrovascular disease. This abbreviated translation concentrates on the clinical parts of his paper in which he characterized and distinguished different subtypes of vascular dementia: post-stroke dementia, mild 'subc1inical' cerebral arteriosc1erosis, severe progressive arteriosc1erotic brain degeneration, Binswanger's encephalitis subcorticalis chronica, perivascular gliosis, and mixed forms of degenerative brain disease. Some of his clinical characterizations foreshadowed modern diagnostic criteria and standards of c1assification.