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 16 octobre 2005
Respiratory disorders in epidemic encephalitis
Aldren Turner, Macdonald Critchley
National Hospital for the Paralysed and Epileptic, Queen Square, London.


Epidemic encephalitis is now regarded as one of the most disabling diseases of the nervous system. Its late effects have been studied and described with great care and an extensive literature has arisen. Amongst the sequel, disorders of the respiratory mechanism have been regarded as rare after-effects. A study of the most recent monographs (Hal, Lévy) has shown, however, that respiratory disabilities take quite a large and important share in the general post-encephalitic picture.
The more frequent occurrence of these disabilities has received confirmation from our own recent experience, as we have observed the existence of minor respiratory symptoms to be not uncommon if looked for in association with Parkinsonism and other well-recognized effects of this disease. (...)
Clinical manifestations.
For descriptive purposes the respiratory disorders following epidemic encephalitis may be classified as follows
(1) Disorders of the respiratory rate (tachypnœa and bradypnœa).
(2) Dysrhythmias, or disorders of the respiratory rhythm (CheyneStokes breathing, breath-holding spells, sighs, forqed or noisy expiration, inversion of the inspiration-expiration ratio).
(3) Respiratory "tics. "-(Yawning, hiccough, spasmodic cough, sniffing.)
It must be realized, however, that any combination of the above types may co-exist with or without the other sequelie of encephalitis.
Before proceeding to a description of respiratory manifestations it is advisable to define the terms we propose to use.
Breathing which is faster than normal we shall speak of as "tachypnœa." The word "bradypnœa" we shall apply to slow respirations. Respiratory movements which are shallower than normal may be named "hypopnoea." The term "hyperpnœa" should strictly speaking be limited to those of abnormal depth. It may be pointed out here that tachypucea is, as a rule, accompanied by some degree of compensatory hypopuœa. In this article we propose to discard the word "polypnœa" altogether.
(1) Disorders of the Respiratory Rate.
Tachypncea constitutes one of the commonest of the respiratory manifestations. The increased respiration rate may be either continuous) in which case it bears no relationship to the output of work, or paroxysmal. The patient usually suffers no inconvenience and cyanosis is rare ; in the majority of cases there is no accompanying tachycardia. The tachypnœa may occur during sleep only, or during both sleeping and waking states. On the other hand the respiration rate may fall during sleep or even when the patient is recumbent. Physical examination of the chest usually gives negative results, but in some cases, particularly in the presence of the Parkinsonian syndrome, there may be a relative immobility of part of the thorax or of the diaphragm. The amplitude of each expiration is usually shallower than normal and may be saccadé or jerky in character.
 parkinson encephalite
Paroxysmal tachypnœa may arise in attacks occurring three to four times daily or several times in the course of an hour. It is sometimes observed that the paroxysms tend to come on towards nightfall, each one lasting anything from a minute to several hours. The patient is usually conscious of the increased respiration rate and is able voluntarily to control it for a short time. When questioned as to why he breathes more rapidly no very clear reply is given, but complaint may be made of a sensation of thoracic constriction or pulsation. Farquhar Buzzard has pointed out that in many cases of post-encephalitis acts which are normally automatic in nature (swallowing, speaking, breathing, &c.) become purely voluntary actions. This statement is true for certain of these paroxysmal tachypnœas. The intensity and frequency of the attacks are increased by emotional stimuli, and conversely become less marked when attention is distracted. Frequently the act of deglutition temporarily inhibits the tachypnœa.
Of the complications of tachypnœa, tetany is the most important, and occurs in those cases in which the usual compensatory hypopnœa is replaced by respirations of full amplitude. The experiments of Collip and Backus, Grant and Goldman, Rosett and many others have demonstrated that tetany is a physiological accompaniment of "over ventilation of the lungs" due to forced respiration, the causative mechanism arising through the altered H'/OH' balance. Vasomotor disturbances may also appear in the course of the tachypnœa, giving rise to lowered arterial tension and cyanosis of the extremities.
Children in particular are liable to display tachypnœa. This manifestation may arise during the acute phase, during convalescence, or as a remote sequel. When arising as a late result of encephalitis it is rare not to find some other abnormality, physical or psychical. Reference has been made to the association of tachypncea with synchronous myoclonic contractions of the biceps muscle.
Bradypnœa, a much rarer phenomenon, may be continuous or paroxysmal. The respiration rate may fall to as low as six per minute, and pressure on the eyeballs sometimes reduces it yet further. The amplitude of each respiration is usually increased, and expiration tends to become prolonged and noisy.
(2) Dysrhythmias, or Disorders of the Respiratory Rhythm.
Sighs: This is one of the commonest manifestations. The patient in the course of normal breathing may suddenly give a deep sigh, which is followed by a short period of compensatory expiratory apnœa. These sighs may occur only during sleep, or, on the other hand, may come on after some slight exertion. In certain instances the patient at the same time may exhibit a tic-like grimace or movement, such as opening of the mouth or shrugging the shoulders. Occasionally the expiratory component is broken or "cogwheel" in character. At times a regular periodicity may be detected in the order of their appearance, each sign occurring after a definite number of respirations.
Apnœic pauses: Intervals in which no breathing occurs may take place as a result of sighing inspiration, during a spell of breath-holding, or spontaneously in the course of normal respiration. These pauses are particularly prone to arise during sleep. The resumption of respiration, unlike what is seen in Cheyne-Stokes breathing, is not "crescendo."
Inversion of the inspiration/expiration formula:
inspiration is shorter than expiration with the proportion of 5:8; during sleep the converse is true. In certain post-encephalitic patients the normal ratio is reversed so that the respiration during the waking state resembles that of normal sleep.
Cheyne-Stokes respiration. This is a rare manifestation; Pierre Marie affirming that he has never seen a genuine case. Goldflam, Leroy and Dupouy, and Naville have, however, recorded cases of abnormal breathing conforming to this type; tachypnœa constituted a marked symptom in their patients.
Bigeminal and trigeminal respiration.-In certain cases, a graphic record of the respiration reveals a regular arhythmia in the amplitude of each effort; for example, breaths may be alternately deep and shallow (bigeminal or alternating respiration) ; or a deep breath may be succeeded by two of smaller amplitude (trigeminal respiration). These abnormal types of breathing are frequently associated with the occurrence of sighs or apnœic pauses.
Breath-holding spells.-This subclass includes a great variety of interesting manifestations which differ from the simple apnœic pauses in that the breath is held in almost full inspiration. It is into this class that one must place the first and third cases recorded by us.
The paroxysms occur at irregular intervals and may or may not be interposed with periods of normal breathing. They tend to come on especially towards nightfall and in some cases (as in our third case) may be present only during sleep. In other instances (as in our first case) the paroxysms occur during the waking state only. The attacks, like those of simple tachypnœa, are to a limited extent under the patient's control and may be temporarily inhibited by voluntary effort, by deglutition or when attention is forcefully distracted. Similarly, the attacks increase in number and frequency when the patient is under emotional stress or is the object of marked. attention.
The paroxysms are ushered in by a series of deep, forced inspiratory efforts with noisy expirations; the patient's subjective sensations at this stage frequently take the form of a feeling of dizziness, or of "something in the chest interfering with full inspiration." A deep inspiration is then taken and maintained for a period of ten, twenty or thirty seconds. At the same time choreo-athetotic movements of the limbs, bizarre attitudes or peculiar grimaces are common. Sometimes the patient stands up and throws back his head; others run their fingers through their hair. Roch and Schmidt quote a patient who would try to run away and conceal himself. Cyanosis is unusual, but may occur. In the severe cases, consciousness may be lost for a moment or two, as in our first and fifth cases. The inspiratory apnœa is terminated bya forced and often noisy expiration which is followed by a short period of compensating expiratory apnoea. Breathing is then resumed, each breath being of normal amplitude.
(3) Respiratory Tics.
(a) Hiccough is mentioned merely because discussion of its relationship with epidemic encephalitis would be beyond the scope of this paper.
(b) Yawning. Involuntary opening of the mouth is not an uncommon feature in the post-encephalitic Parkinsonian syndrome: during the last three months, three patients out of twenty-five observed by ourselves showed this symptom. Occasionally "mouth opening" occurs in paroxysms, is sustained for a few seconds and amounts in degree to a wide gape. Such a manifestation is tantamount to a yawn and may be included amongst the respiratory tics of post-encephalitic nature. An extreme instance is recorded in our third case and is illustrated by photograph. Other cases have been reported by Sicard and Paraf, Pardee, Hinds Howell, Abrahamson and Farquhar Buzzard.
(c) "Soufflement." The French writers have used this word to denote tic-like expiration of air through the nose as though in order to dislodge a foreign body. (Babinski and Charpentier.)
(d) Spasmodic cough. One of the most distressing post-encephalitic "tics" consists in a constant, short, hacking, dry cough which persists during the daytime and often throughout the night. Pierre Marie gives the term "toux coqueluchoïde "to this cough. There are no pulmonary physical signs and examination of the larynx and pharynx is negative. This cough may be succeeded later by some other respiratory disorder as in Case 1 of our series.
These respiratory tics are particularly common in children after encephalitis. They tend to persist through the daytime, but show an increased frequency towards nightfall.
From the study of the recorded cases it is apparent that respiratory phenomena may arise at any stage of epidemic encephalitis. It does not seem possible to trace any association between the symptomatology of the original attack and the subsequent onset of respiratory disorders. Thus cases which were originally characterized by insomnia or delirium do not tend to develop subsequent respiratory disorders any more than cases showing lethargy. The myoclonic variety is known also to be followed occasionally by disorders of breathing.
Although respiratory disorders may occasionally constitute the sole late manifestations of encephalitis, it is more usual for other psychical and nervous after-effects to be present; association with inversion of the sleep rhythm is particularly common. The nature of the co-existing sequelie does not appear to exer6ise any essential bearing on the nature or severity of the respiratory abnôrmality. It is difficult to gauge the frequency of respiratory disorders during and after encephalitis. It is probable that the slighter dysrhythmias are not uncommon features, and it is only by careful observation of the respiration during sleep as well as in the waking state that many of them are discovered. Miss Anderson, in her study of forty children recovered from encephalitis, records "hyperpnœic" breathing in "many." Hinds Howell mentions three cases in his series of twenty-eight. Mlle. Lévy found ten instances of respiratory disorders out of 129 cases. On the other hand, out of 423 surviving cases of encephalitis recorded by Price, Bing and Staehelin, Palitzsch, Duncan, Shrubsall, Paterson and Spence, and Edgeworth, no mention whatever is made of respiratory upsets; we do not know, however, whether any such changes were looked for.
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