Wernicke’s take on Wernicke’s encephalopathy

When I read this case report, I had to smile a bit. Not that it is not worth publishing, but you would expect the authors to look up Wernicke’s original description of what we nowadays call his encephalopathy (mind you, he was of sound mind). We use this trigger to read the excerpt of Wernicke’s Neurology textbook as available online here in his Lehrbuch der Gehirnkrankheiten (vol 2/3, p. 232, for those of you who don’t like to read german, try this translation), where he describes in 3 cases of his own and one french case the precise clinical appearance as well as the pathological peculiarities of the disease, of which he could not know the etiology. In his summary he writes

Es handelt sich um eine selbstständige, entzündliche, acute Kernerkrankung im Gebiete der Augenmuskelnerven, die in der Zeit von 10-14 Tagen zum Tode führt. Die Herdsymptome bestehen in associirten Augenmuskellähmungen, die rasch entstehen, fortschreiten und schliesslich zu einer fast totalen Lähmung der Augenmuskulatur führen; doch bleiben selbst dann noch gewisse Muskeln davon ausgenommen, wie der Sphincter iridis oder Levator palpebrarum. Der Gang der Kranken wird taumelnd und zeigt eine Combination von Steifheit mit Ataxie, die am meisten an die Ataxie der Alkoholiker erinnert. Die Allgemeineerscheinungen sind sehr auffallender Natur und bestehen in Störungen des Bewusstseins, und zwar entweder von Anfang an Somnolenz, oder einem Schlussstadium der Somnolenz, das durch ein länger dauerndes der Agitation eingeleitet wird. Ausserdem war allen 3 Fällen eine Theilnahme der Sehnerven und zwar entzündliche Veränderungen der Papillen eingenthümlich. Stets waren schwere Schädlichkeiten dem Ausbruch der Krankheit vorangegangen, das eine Mal eine Schwerfelsäurevergiftung, die mit Stenose des Pylorus geheilt war, in den beiden anderen Fällen ein Alcoholmissbrauch ungewöhnlich starken Grades. Die Frage, ob in diesen letzteren die Erscheingungen des Delirium potatorum als Complication oder als selbstständige, dieser Krankheit zukommende Allgemeinerscheinung aufzufassen sei, kann wohl aufgeworfen, aber nicht entscheiden werden, jedenfalls lag nicht ein gewöhnliches, sondern mindestens ein mit den Symptomen der Poliencephalitis complicirtes Delirium tremens vor. Doch mag darauf hingewiesen werden, dass die characteristische Unorientirtheit auch in dem ersten Falle constatirt wurde, dessen Erscheinungsweise von der des Delirium möglichst weit entfernt war. Nachdem die erste Beobachtung bei Lebzeiten ganz unverstanden geblieben war, gelang es mir bei der zweiten und dritten trotz der Abweichung in den Allgemeinerscheinungen die Diagnose zu stellen. Daraus folgt wohl die Berechtigung diese Fälle als ein besonderes Krankheitsbild hervorzuheben.

This would be translated as

What is present is an independent, inflammatory, acute nuclear disease in the area of the nerves of the eye muscles,
leading to death in a period of 10–14 days. The focal symptoms consist of associated paralyses of the eye muscles, which rapidly arise, progress and finally lead to almost total paralysis of the eye muscles; but even then with the exception of certain muscles, such as the sphincter iridis or levator palpebrarum. The patient begins to stagger when walking and his gait shows a combination of stiffness and ataxia, which is most reminiscent of the ataxia of alcoholics. The general signs are very striking and consist of alterations of consciousness, either somnolence from the outset or a final phase of somnolence preceded by a longer phase of agitation. In addition, in all three cases the visual nerves were affected, with inflammatory lesions of the papillae. In every case the onset of the disease had been preceded by taking harmful substances, in the one case sulphuric acid poisoning, which had healed [leaving?] pyloric stenosis, in the other two cases an uncommonly high level of alcohol abuse. The question of whether in these latter cases the signs of delirium potatorum should be regarded as a complication or as a separate general manifestation pertaining to this disease may be posed but cannot be decided; at any rate what was present was not an instance of common delirium tremens but, at the least, delirium tremens complicated by the symptoms
of poliencephalitis. However, it should be pointed out that the characteristic disorientation was also observed in the first case, the features of which were totally different from those of delirium [tremens]. Whereas the first observation had not been at all understood while the patient was still alive, in the second and third cases I was able to make the diagnosis despite the difference in the general signs. I think that this provides a justification for drawing attention to these cases as [representing] a particular syndrome.

What can be learned from that? See one new disease and be able to recognize it in the next patient in order to diagnose it? Write up detailled descriptions of your patients and their examination? Read old textbooks, because they are written well and very instructive?

I think that all of that is true.

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