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 L'histoire des neurosciences à La Pitié et à La Salpêtrière J Poirier
The history of neurosciences at La Pitié and La Salpêtrière J Poirier 

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8 novembre 2007
Oxford Medical Publications
von economo
Encephalitis lethargica
Its sequelae and treatment
Constantin Von Economo
von economo
Parkinsonism and its most frequent accompanying symptoms


von economo
p 26-27
The somnolent ophthalmoplegic form.
The prodromal phenomena, which generally make an abrupt appearance a few days or, very rarely, a few weeks before the onset of the disease, consist of general discomfort, lassitude, seediness, shivering, headache, occasionally vertigo and vomiting. The temperature is generally only a little raised to slightly above 98.6° F., but in some cases may rise above 1O2.2° F. Graver catarrhal affections of the airpassages are practically never present, though c1oe examination reveals in most cases a slight pharyigitis. Very soon this, influenza-like prodromal stage is succeeded by a certain somnolence; the patient is dazed and confused, and may (but not invariably) show at the same time phenomena of slight meningeal irritation, such as sensitiveness of the skull to percussion, a trace of stiffness of the neck and Kernig's sign, slight pains on compression of the eyeballs, frequent yawning, and sometimes also trismus and singultus. This slight meningism is used by the virus, and can be anatomically proved to be a very slight inflammatory irritation of the meninges by the infectious process. The headache may in rare cases be more intense, and pains in the limbs may also occur. These prodromal phenomena are usually of very moderate degree in the somnolent-ophthalmoplegic form and very rarely reach an alarming intensity. This is more commonly met with in the hyperkinetic syndrome, as we shall see in discussing the latter.
Within the next few days somnolence begins to predominate. If ,slight signs of meningeal irritation have existed, they disappear gradually or at least remain stationary, so as to play a subsidiary role compared with the new symptoms which now become more prominent and frequently lead to a revision of the mistaken diagnosis of an incipient meningitis. In the now increasing somnolence one often observes that the patients, left to themselves, fall asleep in the act of sitting and standing, and even while walking, or during meals, with all signs of tiredness and yawning, go to sleep with food actually in the mouth. They have the appearance of being in a light slumber or sometimes in deep sleep, with typical respiration, even snoring. If aroused, they wake up quickly and completely, are orientated and fully conscious, and can reply sensibly to questioning; they are fully aware of the situation, carry out all requests promptly, get up if told to do so and walk about, but, left to themselves, soon drop back to sleep.
This kind of sleep therefore bears a deceptive resemblance to normal sleep. In the mildest cases of the disease, which soon recover spontaneously, this symptom soon tends to disappear; a quite slight encephalitis of such nature is often mistaken for a physiological reaction to an actual or imaginary preceding over-exertion. In severe cases, however, this somnolence, which recurs for periods of hours, days, or even longer, gradually progresses towards more or less permanent sleep, out of which at first it is still possible to rouse the patients, for instance for meals or other necessary functions. Sleep in this form may last for weeks or even months, but frequently deepens to a state of most intense sopor or even a comatose condition, which may terminate fatally after some days or weeks. In case of recovery, an increased need of sleep or occasionally some other disturbance of the sleep function often persists for months after the acute affection itself. One might be inclined to look upon the somnolence as a result of the rise of temperature or of the toxic component (as in influenza), or of cerebral pressure, were it not that experience teaches us that somnolence may be present in encephalitis lethargica without rise of temperature and without cerebral pressure, and is repeatedly found, in quite slight caes as the only well-marked symptom. The consequence is that we must look for other causes of this somnolence.
p 50-52
Monosymptomatic form.
I have just indicated when discussing the neuritic forms how it is practically impossible in considering an individual case, especially at the time of an epidemic, to exclude encephalitis as the source of origin. This applies also to other isolated pathological manifestations. During the encephalitis epidemic I repeatedly saw cases which showed no other symptom than, for instance, trismus, which at first suggested tetanus, but, on account of its transitory character, had to be interpreted as probably monosymptomatic encephalitis, especially as trismus in some other cases at that time was associated with definite encephalitic symptoms. Similarly, sporadic chorea or hemithorea without accompanying symptoms may represent a monosymptomatic encephalitis On investigating the previous history of possibly post-encephalitic-diseases it is therefore always advisable to inquire very closely indeed iiffo such possibilities and signs of a masked primary affection. During epidemics of encephalitis we were able to observe different monosymptomatic disturbances in otherwise healthy people, such as varied respiratory complaints, dyspnoea, tachypnoea, asymmetrical respiration, &c.; besides pathological yawning, compulsive masticating and clucking of lips and tongue, some clonic phenomena, &c.
The most common of these monosymptomatic forms is singultus or hiccup. I have already said that it may be accidentally met with as an accompanying symptom in the most varied forms of encephalitis lethargica. In the hyperkinetic forms it has been interpreted as myoclonus of the diaphragm; whether this view applies also to these sporadic cases, where there are no other myoclonisms present or whether we should not look in this instance on the hiccup as the result of a state of phrenic nuclear stimulation, remains an open question for the present. Sporadically, singultus has sometimes been observed as an incipient symptom of encephalitis lethargica. A few weeks before the outbreak of the serious hyperkinetic epidemic of encephalitis in Vienna in 1920 there happened to be a small epidemic of singultus in that city, which attacked a great number of people and which, after a troublesome duration of hours and sometimes of days, without any other symptoms, vanished as suddenly as it had appeared. Such singultus epidemics have also been observed at various times in Paris and at other places during periods of encephalitis lethargica and influenza epidemics. MacNalty described an epidemic of hiccup in England of as recent appearance as the first two months of 1929. (Rosenow has cultivated a streptococcus from the nasal and pharyngeal mucous discharges of such patients and claimed to have obtained similar diaphragmatic convulsions in animals on inoculation with that germ.)
It is still doubtful whether these singultus epidemics may be regarded as abortive and rudimentary epidemics of encephalitis (formes frustes). It has properly been pointed out (Sicard) that of the great number of people attacked during these epidemics of singultus none has been observed-apart from exceptional cases (Neel)- to suffer afterwards from a post-encephalitic disease. Measured by the frequent occurrence of sequelae this circumstance makes the identity of the two affections in some degree doubtful. On the other hand this should not be regarded as an absolute criterion; I would merely recall that the first epidemic of encephalitis lethargica which I described was practically free from sequelae. Also, if one should feel inclined to postulate a different virus as the cause of the singultus, the fact of the synchronization of outbreaks of encephalitis and of epidemic singultus and their simultaneous disappearance should be borne in mind. This points to a condition of epidemic 'neighbourhood'. If a separate virus for these two diseases be assumed, one should at least postulate a similarity or identity of those secondary external conditions which, in the opinion of Dorr, are instrumental with regard to the outbreak of an epidemic. The literature of singultus epiderniø is fairly voluminous. Sicard described it as paraencephalitis. Redlich is certainly right in assuming that during these last years, from reasons unknown, we have witnessed the outbreak of quite a series of neurotropic germs, and that it is a mistake to reckon all these cases under the heading of encephalitis. I share this opinion and hold that Cruchet commits this mistake in his last publication, when he tries to refer to his old observations as cases of encephalitis lethargica, which for the most part they are not.
p 113-120
Parkinsonism and its most frequent accompanying symptoms
Since the discovery of lenticular degeneration by Wilson we attribute this kind of static rigidity, tremor, and the whole of the complex of symptoms surrounding paralysis agitans, the choreiform and athetotic movements, and the so-called extra-pyramidal disturbances of locomotion, such as we find so well developed in Parkinsonism, to affections of the basal ganglia, the nucleus caudatus, and nucleus lentiformis, in more recent times also of the subthalamic nuclei and the substantia nigra. We speak also of the striatal or, better, with regard to the last-named localizations, extrapyramidal complex of symptoms. Post-mortem examination of the cases which die of Parinsonism tells largely in favour of this supposition. In this region of the basal grey cerebral masses there is probably located a series of centres which exercise an influence on progression of movements, rhythm, volume of progressive movements, timing, and many other processes, quite independently of the question whether these movements are of a mainly automatic nature or are originally volitional and executed under the control of attention and have only later become partly automatic.
This points to some direct influence acting upon the course (so far as it is automatic) of volitional acts and in its further interpretation to a possible indirect action on purely mental processes. Under the heading of this group of disturbances I should feel inclined to classify quite a number of phenomena which, as post-encephalitic phenomena, are generally associated with other signs of Parkinsonism or accompany a fully developed state of Parkinsonism, but which occasionally may occur as a separate syndrome of sequelae by themselves or in common with other quite different post-encephalitic symptoms even without Parkinsonism. One might describe them as disturbance of the automatisms. Many years ago I proved the existence in the substantia nigra of an important shuntingstation for automatic rhythm of movement and progression of acts of mastication and swallowing. Now in post-encephalitic disturbances we repeatedly find a rhythmic repetition or tic-like recurrence of movemente of mastication and swallowing or convulsive yawning and sounds of clucking and sucking. But other tics and compulsive movements occur as well, particularly in those groups of muscles in which the nonencephalitic tics locate themselves by preference and not, as might be expected, in those muscles which are generally affected by the acute encephalitic hyperkinesis (Wagner y. Jauregg). We see therefore blepharospasms, mimetictics, tics of clucking and hissing, also torticollis and tics of the upper and lower extremities, but we find also fits of selling and yawning, &c. Often these tics may be influenced by the position of the body, for instance they cease on lying down; but not infrequently they may be influenced by suggestion, for instance by hypnosis, or may be suppressed for some time by will-power. But unfortunately these improvements by suggestion or education do not last for long. To this group probably belong also the respiratory disturbances which we often find in post-encephalitic patients (Vincent and Bernard, Babinski and Charpentier, van Bogaert, S. E. Jelliffe, W. A. Smith, Westphal, Stem, Hess). The respiratory disturbances of the acute phase have already been discussed on p. 50.
Relatively speaking, the most frequent disturbances are attacks of hyperpnoea with forced expiration. Often these attacks lead to quite grotesque paroxysms with the oddest accompanying movements, which the patients feel to be compulsive. A patient in this condition opened his mouth wide, twisted his trunk and arms backwards in the most extreme fashion, stamped at the same time with his feet, and uttered a snorting sound. On being questioned he did not perhaps give dyspnoea as the reason f these weird acts, but said: 'Well, I must twist my arms that way.' As a matter of fact there is actually as a rule no dyspnoea present. Sometimes there seems to be a diminished amount of bicarbonate in the blood-serum (Happ and Mason), but at other times this is not observed. Quite occasionally, however, genuine dyspnoeic attacks occur (Bériel). But this seems rather a disturbance of rhythm and 'progression of movement' and volume of movement in proxintai centres, whereby the automatism of the distal respiratory centre loses part of its proper regulation through the absence of central inhibition. These attacks may be released by emotion (Insabato), but at other times they may themselves cause states of fear (Kennedy).
Hyperpnoea may also exist as a condition of some duration, sometimes in conjunction with peculiar accompanying movements, for instance, contractions of the biceps with every breath. The hyperpnoea may be accompanied by tetanoid symptoms as a result of hyperventilation (Barker and Sprunt), also bradypnoea, apnoea, irregular respirations, nervous cough, spastic cough, sighing, tics of respiration, &c., occur. These purely nervous functional disturbances are primarily caused by organic lesions. They can bring sensations or complexes of sensations and emotions in their wake, which on the one hand may be primary, that is, of the same generic order as the accompanying movements so frequently seen and caused by the same disturbance as the disturbanèe of motility itself, or on the other hand possibly secondary. Thus, for instance, appreciation of the position in which he is placed may cause emotions of fear or embarrassment in a patient. Certain mental fixations may therefore lastingly associate themselves with these motor disturbances and lend them a neurotic imprint; this neurotic element asserts itself (Jelliffe) in dreams or otherwise in the behaviour of the patiénts. The neurotic aspect of these patients is then still further enhanced by the apparent possibility, mentioned above, of influencing Or even suppresing.tieir tics with their associated mental (primary as well as secondary) symptoms by suggestion.
Not only is the order of the movements of respiration repeatedly disturbed, but one finds repeatedly in post-encephalitics, when the attention is directed to it, more intricate motor disturbances, as, for instance, disturbances of writing reminiscent of writer's cramp, and also micrographia (Bériel, Souques, Gerstmann and Schilder), that is to say, the patients begin to write in the normal way, then the characters become gradually smaller, tifi in the end they become minute and indecipherable and the hand comes to a cramp-like standstill. On making patients read with a loud voice similar odd disturbances are found; thus one of our patients, after a few minutes' reading, began to increase the speed gradually to such a degree that articulation altogether, failed and ended suddenly in an indistinct mutter. A certain resemblance to pro- and retropulsion in walking is obvious. Sometimes also the last syllables of a sentence are repeated, chorus-like (so-called logoclonia). A pathological disturbance of an opposite nature may also occur. Sometimes patients are unable to iterrupt an action once begun; when, for instance, they read, they carry on till one actually takes the book away from them; a passive movement is frequently repeated for a long time (Mayer-Gross and Steiner).
Baron Constantin von Economo1876 - 1931

Sleep as a problem of localisation von Economo 1930 - pdf