biographies de neurologues
Nouvelle Iconographie de La Salpêtrière
 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

 mise à jour du
30 septembre 2007
Oxford Medical Publications
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Encephalitis lethargica, its sequelae and treatment
Baron Constantin Von Economo
Cinquante ans de sommeil O Sacks
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p 26-27 The somnolent ophthalmoplegic form
p 50-52 Monosymptomatic form
Parkinsonism and its most frequent accompanying symptoms.
As Parkinsonism I describe here the chronic form of the amyostatic complex of symptoms of encephalitic origin. It was in France (Sicard and Paraf) that attention was first definitely directed to the frequency of this sequel, and that it was so named because of its similarity to paralysis agitans (Parkinson's disease). Parkinsonism may develop both in immediate sequence after the amyostatic phenomena of acute encephalitis lethargica and in the akinetic or somnolent stage of its hyperkinetic form, as well as after an apparently complete recovery from acute encephalitis lethargica following on a shorter or longer interval of freedom from symptoms. The gradual and progressive development of Parkinsonism may also ensue even although no amyostatic symptom has appeared during the acute phase. With regard to its course, it may develop as a chronic form, or as a relapse with 'intermittent progressions', or as a genuine sequel. It happens that quite a number of cases of Parkinsonism have been seen in places where there had been no very serious encephalitis lethargica epidemic, as, for instance, in Buenos Ayres (Dasso).
The sequelae often begin with weakness in one arm or leg, or with tremor, or with a peculiar, somewhat stiff posture. On closer examination it is noticed that the weaker arm or both arms no longer participate in the finer pendulous movements coordinated with the act of walking. These disturbances of motility, as a first symptom, may prompt the patient to consult the doctor. The patient complains of physical and mental exhaustion, decrease of mental agility; everything seems difficult. Frequently these complaints are wrongly thought to be purely functional. Close objective examination, however, furnishes even at this stage some isolated slight symptoms which enable a diagnosis to be made: the manner of speaking gives an impression of some effort and is dragging; the swinging movements of the arms in walking are perhaps already absent, posture and gait are no longer as free and easy as before. Gradually in the course of months the stiffness in one or other extremities as well increases, and soon extends to the other extremities as well. The face, too, may become motionless; all expression is lacking, the space between the upper and lower lids appears wide, the range of movement of the lids is slight, and the staring gaze of the eyes gives to the patients an expression of stupid bewilderment. As a result of abnormally free secretion of the sebaceous glands the face looks shiny ('greasy face' (Cohn)); the speech is jerky; generally there is salivation. Genuine compulsive laughing or compulsive crying I cannot remember to have seen in these patients, but it has been described. The posture comes to be distinctly bent; the neck is inclined, back, pelvic, and shoulder girdles are carried stiffly as though the joints did not exist. The gait is short-stepped and hesitating, there is propulsion and retropulsion, as in genuine paralysis agitans, that is to say, the patient, as a result of deficiency of the finer movements of the vertebral column and the extremities, once he is moved forwards or backwards, cannot stop spontaneously, but moves on with gathering speed as far as the next obstacle, where he comes to a passive halt. It often happens that he loses his equilibrium and falls, because, with the slowness of his movements, he cannot keep pace with the momentum of his body.
Sometimes, although rarely, a hyperextension of the vertebral column backwards or sideways may develop instead of the bent posture, when the head or one of the extremities may also be fixed in a peculiar extreme position (La Torre). Sometimes, but much more rarely than in paralysis agitans, tremor may be present, tremor of the tongue as well as of the extremities; genuine Parkinson-tremor ('pill-rolling movement') is rather rare in Parkinsonism. The peculiar stiffness which seems to hold the patient imprisoned consists of two elements, paucity of movement and rigidity, which occur often separately but more commonly together; it is advisable, however, to keep these elements in mind on account of their separate occurrence. The paucity of movement is clearly shown by the lack of mimic expression mentioned above and also by the absence of the other affective co-movements of trunk and extremities, the so-called associated movements (Souques). The more minute expressive movements of the hands are likewise wanting, and even the coarser movements of the limbs are less frequent. The patients generally sit motionless if left to themselves, even for a period of hours; when they seize an object the movements are executed with excessive slowness. If they look round at all, they do so generally only with the eyes, without turning the head or adjusting the trunk.
Every movement, every action is strikingly slowed down; even to speak takes the patients a long time; their reaction-times, in spite of intact consciousness, are enormously prolonged; this is described as bradyphrenia (Bianchi, Naville). They masticate a piece of food for hours with long pauses and are hardly able to direct it into the pharynx to swallow it (dysphagia). Their speech is low-voiced, jerky, strikingly monotonous, without any modulation or accompanying facial expression. This 'paucity of movement' or akinesis also occasionally occurs, as I have repeatedly emphasized, without any rigidity (Gerstmann and Schilder, Bychowski, Verger and Hesnard). We shall revert to this circumstance on discussing the mental symptoms. Compare also the section on acute amyostatic states (vide Mayer-Gross).
Generally, however, this akinesis is combined with genuine muscular rigidity, so that repeated attempts have been made to explain the akinesis, erroneously, as a result of the hypertonia (Hauptmann).
On examining the patient we find an increase of muscular tone, an increase of the normal elastic resistance against passive movements. This stiffness of the musculature is more marked when dealing with the large proximal joints than the distal, smaller ones; it is, for instance, more marked in the shoulder-joint than in the wrist and the fingerjoints, thus differing from the so-called pyramidal increase of tone, in which, as it occurs, for instance, in hemiplegias through lesion of the pyramidal tract (volitional tract), rigidity is more particularly marked as time advances in the distal joints. Further, as a characteristic difference, there is no exaggeration of tendon-reflexes. The patient himself feels this stiffness very distinctly. Sudden passive movements overcome the increased tonus more easily, whereas moderately slow ones make it more obvious. In the opening stages of the disease we can satisfy ourselves that the tonus is a static postural tonus-increase of the static postural reflexes (Stertz, Jakob, Froment)-that is to say, when, for instance, the patient leans against something, is supported or sits, the rigidity, which could be observed before in arm or hand or when standing without support, vanishes. In the further course of the disease, however, the tonus becomes permanent, and in patients confined to bed we find arms and legs in a state of continuous tonus, the head, in consequence of rigidity of the musculature of back and neck, lifted from its support and carried freely in the air. But the character of the rigidity varies in different cases. The general characteristics which distinguish it from the rigor of pyramidal paralyses are:
1. The rigidity, generally also present during rest, lessens in most cases on intention of movement (pyramidal rigidity increases).
2. The big proximal joints are generally more affected by the rigidity than the distal small ones (in pyramidal rigidity the reverse obtains).
3. Flexors and extensors are equally affected by the rigidity (in pyramidal rigidity the flexors are more affected in the upper extremities, the extensors more in the lower).
4. Reflex-disturbances are absent (in pyramidal rigidity there is an increase of reflexes).
Now the rigidity of Parkinsonism may sometimes deviate considerably from these rules, and in accordance with this we distinguish with Gerstmann and Schilder: (a) the plastic type, which displays the tension already well marked during rest. Passive movements do not cause any considerable increase in tone, but there is a tendency to retain any posture imparted or spontaneously assumed by muscular action (flexibilitas cerea); (b) the proprio-reactive type with a slight degree of 'rest-form' tonicity ('Ruheformtonus') and no flexibilitas cerea, where passive movements, particularly if repeated, increase the rigidity; (c) the reactive type with hardly any' rest-form' tonicity and no flexibibitas cerea; hence passive movements suddenly (though only at times) provoke the tonus, stimulation of the skin producing the same result; in this type active movements do not, as under (a) and (b), decrease the tonus, but rather evoke it; and finally (d) the reflectory type, in which though active movement removes tension, the slightest passive movement provokes at once a maximal counter-spasm. It is important to add that types (a) and (b) are more irquent and typical, whereas (c) and (d) already, approach more closely to the pyramidal type; furthermore, the types may occur together in the same patient, different limbs being affected by different types.
It is a very interesting fact that akinesis and hypertonia are not unconquerable symptoms. Sometimes patients who can walk only with difficulty may dance quite well to musical accompaniment, or may in a state of excitement run after and jump on board a moving vehicle. A musical female patient, who on account of her rigidity could no longer write, was able to play waltzes and other melodies quite well on the piano. Moreover energetic commands may, at least temporarily, break through the akinesis. On discussing the mental condition we shall again return to these phenomena. The fact, repeatedly observed by us, that high fever (even artificially produced) may in some cases abolish rigidity and akinesis, although unfortunately only for a few days, after which the previous condition re-establishes itself, may prove of possible importance for future therapy.
Other disturbances of motility also occur in Parkinsonism, if not regularly yet occasionally, as, for instance, pareses of one or other extremity; but these are not paralyses with reflex-differences but diminished motility, not as a result of rigidity, but of inability to execute more powerful movements. Also hemipareses are repeatedly observed. Genuine paralyses with reflex-disturbances, however, occur only in exceptional cases. I have also repeatedly seen flaccid pareses of the muscles which keep the head in position, so that it dropped on the chest. Such pareses are usually transitory; probably they are rather to be looked upon as atonias, belonging to the group of the atonie paralyses of chorea, than as motor paralyses of a possibly neuritic nature. The reflexes in such states of weakness of the musculature may be diminished; generally they are unchanged and only very rarely exaggerated-hardly ever to such a degree as in pyramidal lesions, though occasionally Babinski's sign may be seen. In the latter case a combination of centrally caused motor and tonic disturbances is likely.
The muscles feel often peculiarly inelastic, 'doughy'. The mechanical muscular irritability is in general distinctly increased, a phenomenon which is probably caused by general disturbances, perhaps disturbances of metabolism; also the formation of an idiomuscular wheal is repeatedly found. Mechanical hyperexcitability of nerves, particularly Ohvostek's sign in the field of the facial nerve, is not infrequently witnessed. The galvanic excitability is often, though not always, distinctly increased, in particular increase of the anodal excitability should be looked for (Stern). Sometimes a peculiar faradic after-contraction in a muscle electrically stimulated, which may even last for more than half a minute; has been seen (Vincent, Jarkowsky).
Parkinsonism is not generally associated with tremor, so that we are faced by the picture of paralysis agitans sine tremore. Though this is the rule, tremor is not infrequently found in any of its varieties. The pillrolling movement, and tremor during rest which ceases on intended movement, are hardly ever seen; all other kinds of tremor are more frequently met with, for instance violent shaking of the extremities which may spread over the whole body. In other cases again tremor occurs only on the initiation of movements or on mental excitement.
Repeatedly one sees attacks of tremor, tremor of the jaw, of the lower lip, of the muscles of mastication, less often of the head or any other kind of trembling in general. Clonic spasms and fascicular twitches do not occur in the chronic forms.
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 Parkinsonism 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, mimetic tics, tics of clucking and hissing, also torticollis and tics of the upper and lower, extremities, but we find also fits of yelling and yawning, &c. Often these tics may be influenced by the position of the body, for instance thày cease on lying down; but not infrequently they may be influenced bysuggestion, 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 for 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 proximal 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 disturbance 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 patients. The neurotic aspect of these patients is then still further enhanced by the apparent possibility, mentioned above, of influencing or even suppressing their tics with their associated mental (primary as well as secondary) symptoms by suggestion.
Baron Constantin von Economo1876 - 1931

Sleep as a problem of localisation von Economo 1930 - pdf

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