Journal of the neurological Sciences, 1975, 26:377-393

377

c ElsevierScientific Publishing Company, Amsterdam - Printed in The Netherlands

Altered States of Consciousness in Disorders of Daytime Sleepiness CHRISTIAN GUILLEMINAULT, MICHEL BILLIARD*, JACQUES M O N T P L A I S I R * * AND W I L L I A M C. D E M E N T Sleep Disorders Clinic and Laboratory Stanford University School o/ Medicine, Stan/ord, Cal. 94305 (U.S.A.)

(Received 8 April, 1975) INTRODUCTION We have seen at our clinic 190 patients complaining of "excessive daytime sleepiness" since January 1972. Sixty-five percent were narcxrleptics and 220//0 presented a sleep-induced apnea syndrome. Narcolepsy was diagnosed by the presence of recurring episodes of daytime sleep, the existence of cataplectic attacks a n d / o r sleep paralysis, and polygraphic demonstration of a sleep-onset R E M (rapid eye movement) period. Sleep-induced apnea has been reported in a variety of neurological conditions including high cervical cordotomy, kyphoscoliosis, Arnold-Chiari syndrome, bulbar poliomyelitis, brain-stem tumors, and brain-stem infarction with lesions usually involving the lateral medullary tegmental area or the paramedian reticular formation. Diagnosis of sleep-induced apnea syndrome was made only after these conditions had been ruled out. Both obese, Pickwickian-type patients and non-obese patients were classified as presenting a sleep apnea syndrome. In addition to the complaint of hypersomnia, 9 5 % of this patient population presented what might best be described as "altered states of consciousness" that the patients do not identify as sleep. These states impair the patients' professional, social and familial lives and sometimes led to severe accidents. Despite the incidence and

This research was supported by National Institute of Neurological Diseases and Stroke Grant NS 10727; Research Scientist Development Award MH 05804 to Dr. Dement, and a grant from the Grant Foundation. Dr. Billiard was supported by a fellowship from Minist~re des affaires 6trang~res fran~ais; Dr. Montplaisir was supported by a fellowship from Conseil de Recherches Mrdicales Canadien. * Current address: Service de Mrdecine Exprrimentale, Facult6 de Mrdecine, Montpellier, France. ** Current address: Facult6 de Mrdecine, Universit6 de Montreal, Montreal, Canada. Information and reprint requests should be addressed to Sleep Disorders Clinic and Laboratory (Dr. C. Guilleminault), Stanford University School of Medicine, Stanford, California 94305, U.S.A.

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c. GUILLEMINAULT,M. BILLIARD, J. MONTPLAISIR,W. C. DEMENT

severity of these experiences, these states generally were n o t i n c l u d e d as part of the clinical s y m p t o m a t o l o g y reported by referring physicians, a n d we have b e c o m e aware of the frequency of this p r o b l e m as our clinical experience with hypersomniacs has increased. By appropriately questioning o u r patients and t h r o u g h the use of c o n t i n u o u s polygraphic recording techniques we have been able both to d o c u m e n t the o c c u r r e n c e of these altered states of consciousness and to describe more precisely what these states are a n d w h e n they most c o m m o n l y occur in the different types of h y p e r s o m n i a c disorders. I. AN ALTERED STATE OF CONSCIOUSNESS: THE AUTOMATIC BEHAVIOR SYNDROME OF HYPERSOMNIACS Case Histories Patient A: "I left Reno alone at 10:00 a.m. en route to Tahoe, a 70 mile drive. I remember

perfectly what happened up to Carson City (30 miles away). After that, I had a complete blackout. I found myself at the reception desk of a hotel in Tahoe, knocking on the desk, and a receptionist asking me what he could do to help. I suddenly 'came back', I could not remember why I was in that hotel, where I had parked my car, and what had happened in the past 90 minutes. Since I sometimes suddenly fall down while laughing or under excitement, and being conscious but suddenly unable to move for a short period of time, I have been considered an epileptic. However, I had several EEG's, one even after sleep deprivation, and performed with naso-pharyngeal leads. All of them were considered normal, except for a tendency to fall asleep very quickly. I have been under Dilantin 1.5 g for six months without any relief from my problem". Patient B: "I was going to do the dishes. It was just after dinner. I remember walking in the kitchen and when I 'woke up' about 30 minutes later, the kitchen was a complete mess. I had put all the plates in the clothes dryer and turned it on!". Patient C: "I usually leave the plant near 3:30 p.m., drive 30 miles back home, and frequently miss the turn from the freeway. I find myself sometimes up to 50 miles away from home without knowing what has happened while I was driving. The most embarrassing situation happened recently. I found myself two miles away from home in someone else's driveway. I was blowing my horn and yelling at the same time in front of the garage door. The lady of the house was frightened and nearly called the police, and the worse, I could not remember how that happened!". Patient D: "I am on the verge of being fired from my job. I am a computer programmer. My last mistake was to run a completely inappropriate program for three hours which could have cost the company $25,000. During that period of time I had to do a certain number of tape rewindings, etc., which I did. It seems that I even talked appropriately to one of my assistants. However, I do not remember what happened. This happens more and more often, particularly between 11:30 a.m. and 3:00 p.m.".

N u m e r o u s anecdotes of this type have b e e n o b t a i n e d from o u r patients. T o m a k e a s a n d w i c h with a sponge and eat it as o n e of the patients r e p o r t e d m a y seem h u m o r o u s , b u t several of o u r patients were b r o u g h t to the hospital, h a v i n g risked their lives or the lives of others d u r i n g these episodes. O n e patient scalded her h a n d while c o o k i n g in such an a b n o r m a l state of alertness. A n o t h e r o n e r e m e m b e r e d that, while driving, he saw the gate of a railway crossing going d o w n a n d the red light flashing 300 yards in front of him. Seconds later, he "woke up',i smashing the gate. A third patient, a house painter, fell from a roof d u r i n g one of these episodes.

ALTERED STATES OF CONSCIOUSNESS

379

Our records disclosed that 75 narcoleptic-cataplectic patients, 20 sleep apneic patients and 7 other patients complaining of daytime "sleepiness reported this automatic behavior syndrome. The incidence of it would almost certainly have been larger if a systematic study had been done since the beginning. Unfortunately these clinical data were not systematically gathered during the first years of our clinic operation. We only started to document this problem two years ago, after we became aware of the high frequency with which it appeared. This automatic behavior syndrome appears nearly daily in each patient. The duration is variable from a few seconds to several hours. The episodes typically involve the continuation of an activity which usually does not require extensive skill. If skill is needed during the abnormal state of consciousness, the patient will usually make frequent mistakes. The patient will continue to perform, sometimes for several hours, without being awaN of his errors, wasting time and money, and sometimes jeopardizing his life or the lives of others. In narcoleptic-cataplectic patients these episodes usually do not occur in the early morning upon waking from sleep, although patients presenting other diagnoses-particularly sleep apnea-most frequently experience episodes at this time. This state will develop more easily if the patient is doing a monotonous task, alone, in a warm, closed room. An example of this is driving an automobile for more than a few miles. All of our patients reported here have experienced altered states while driving. However, the state can develop anywhere, and all our patients also have experienced it in social situations. During these episodes simple questions are answered appropriately, but if the conversation requires complex answers, the inappropriate or meaningless sentences the patient makes in response are quite evident. A common feature is a sudden burst of words, sometimes without any meaning, and always without any relation to what the patient might just have been saying. All of our patient's expressed embarrassment and concern about these abrupt and meaningless intrusions in their conversation. If we summarize the reports of all our patients, this syndrome can be described clinically in the following way: the patient first feels that he is not as "awake" as before, and usually fights against a feeling of drowsiness. Then he becomes less aware of his actions and his performance deteriorates. The ability of the patient to express himself in a coherent way is also impaired. Simple answers to simple questions may not indicate the abnormal state of consciousness but attempts at complex answers are abortive and inappropriate. Actions which do not require skill will be performed satisfactorily albeit in a semi-automatic way; however, if a sudden and well-planned decision is required, the pa'tient will be unable to adapt appropriately "to the new demand. Amnesia is a very common characteristic of this syndrome. A patient cannot remember what has happened during these episodes though he may have some images like in a "broken movie". One of our patients commuted from the South San Francisco Bay area, returning home at about 5:00 p.m. One day he found himself 50 miles to the north of his home in the Golden Gate Park of San Francisco at the end of a dirt road. It was 8:00 p.m. and his engine was still running. His remembrance was that he made a turn on a dirt road. He could still see himself on a very short portion of that dirt road, but had no

380

c. GUILLEMINAULT,M. BILLIARD,J. MONTPLAISIR,W. C. DEMENT

notion where it was or at what time he went on it. This isolated image was the only facet he could remember of a 3 hr period. The notion o] t i m e is completely annihilated during these episodes, and patients may think that very few seconds or minutes have elapsed when sometimes several hours have passed. When patients realize this problem, some question their sanity and seek medical help. Twenty of our patients had had clinical electroencephalographic (EEG) recordings. Seven of them were tested after sleep deprivation with nasopharyngeal leads. None were given a positive diagnosis. Ten patients were given anti-epileptic medication (primidione and/or diphenylhydantoin) without any improvement of their symptomatology, sometimes even with a secondary increase in drowsiness. When seen in our clinic, none of these patients presented any clinical neurologic abnormality. All the patients were diagnosed as having a specific "disorder of sleep" by means of all-night or 24 hr polygraphic monitoring. While studying a sample of 5 patients presenting an undescribed "hyper-somnia" syndrome (Guilleminault, Phillips, Dement 1975a) by means of several continuous 24 hr polygraphic recordings, we found that the "automatic behavior" syndrome could be related to repetitive "micro-sleep" episodes. We then decided to investigate this syndrome in a larger sample of hypersomniacs using continuous polygraphic recordings and repetitive performance tasks which were monotonous and likely to induce sleep. Experimental procedures

Twenty REM narcoleptic-cataplectic patients and one sleep apneic hypersomniac were continuously monitored for a minimum of 58 hours (7 REM narcoleptic and 1 sleep apneic hypersomniac had four 24-hr period monitoring). All patients were drug-free for a minimum of 15 days before the recording, The ages ranged from 38 to 62 yr. Twelve "normal" controls, aged 40 to 60 yr, were recruited through press and radio advertisements and were recorded simultaneously with the patients. Three different protocols were tried on our sample - protocols which were designed to give insight about the narcolepsy syndrome (Billiard 1975) and at the same time provide information about the "automatic behavior syndrome" presented during the daytime by our patients. Protocol A

One patient (a sleep apneic hypersomniac), aged 38 yr, was hospitalized for 4 24-hr periods. EEG ( C J A . , - C4/A 1 - 01/A 2 - OJA1)*, electro-oculogram (EOG) and chin electromyogram (EMG) were continuously monitored except for 1 hr every day spent in personal care. Respiration was monitored by means of 2 abdominal and thoracic strain gauges and 2 nose and mouth thermistors for 48 hr. During the first 48-hr period, the patient was put under a strict nocturnal sleep schedule with bedtime scheduled from 23.00 until 07.30 hr. During the daytime, the patient was asked to perform 2 different tests (each of them 1 hr long), repeated * Standard derivations for sleep monitoring as described in A Manual o/ Standardized Terminology, - Techniques and Scoring System [or Sleep Stages o[ Human Subjects (Reehtschaffen and Kales 1968).

ALTERED STATES OF CONSCIOUSNESS

381

3 times throughout the day [the Wilkinson Addition Test - WAT (Wilkinson 1968), given at 11.00, 15.30, and 20.00, and the Light Stimulus Vigilance Test - LSVT (Morrell 1966), administered at 09.00, 13.30, and 20.00 hr]. During the following 48 hr the patient was on an ad libitum sleep schedule and did not perform any test. When awake he would watch TV, read, play cards or talk with physicians and technicians. If the patient fell asleep during the first 48-hr period at any time during the day (testing or non-testing period) he was permitted to sleep for 3 min - the time being controlled by polygraphic monitoring - then awakened. This same protocol had previously been followed on our first 5 patients with an undescribed "hypersomnia" syndrome (Guilleminault et al. 1975a). Four normal controls of the same age group underwent the same protocol. Protocols B and C were simple variants of Protocol A. Protocol B

Five narcoleptic-cataplectic patients followed this protocol. During the first 24-hr period, after 1 adaptation night, patients were continuously monitored on ad libitum sleep schedules but were asked to perform a 1-hr WAT after each nap between 08.00 and 21.00 hr. If a sleep episode continued longer than 3 min during the test, the patient was awakened by experimenters. During the second 24-hr period, patients were allowed to fall asleep ad libitum, but were systematically awakened after a lO-min sleep period and were asked to perform a 1-hr WAT. Protocol C

Fifteen narcoleptics followed this protocol. After one adaptation night patients were continuously monitored for the next 58 hr. During Day 1 from 07.00 till 22.30 patients were kept awake. They were asked to perform WAT and a serial counting task (the Serial Alternation Task - SAT) (Lubin, Moses, Johnson and Naitoh 1974) in a slightly different schedule than under Protocol B. Each test was broken into two 30-min periods with a 15-min break between each 30 min. WAT was given at 09.30 and 10.15, 14.00 and 14.45, 18.30 and 19.15; SAT was performed C3/A2 po~

Fig. 1. Example of repetitive micro-sleep episodes in the polygraphic recording of a narcoleptic patient performing a serial counting task (Serial Alternation Task). Micro-sleep episodes are underlined. Behaviorally the patient had his eyes open, staring straight ahead. The recording shows the existence of slow rolling eye movements during the microsleep episodes contrasting with the fast ones recorded during alertness. During these episodes the patient stopped pressing the counter appropriately.

382

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Fig. 2. Examples of repetitive micro-sleep episodes in the continuous polygraphic monitoring of a narcoleptic patient while he was talking to a technician. A and B present 3 micro-sleep episodes which occurred in close cluster (A and B were continuous). The patient first presented a burst of "synchronous theta rhythm" which lasted for 10 see (A). Then (B) 2 new m]crosleeps were recorded: one was a low amplitude, mixed frequency burst with a normal high amplitude EMG; the second (at the right side of the graph) was a very short E M G inhibition associated with rapid eye movement and a slow alpha characteristic of a "micro-REM sleep" The patient, behaviorally, had a "glaring" type of look with no eye movement, and spoke out a few meaningless words.

ALTERED STATES OF CONSCIOUSNESS

383

at 11.45 and 12.30, 16.15 and 17.00 hr. During Day 2 patients were allowed and even encouraged to sleep during each 15 rain break between tests (from 10.00 to 10.15, 12.15 to 12.30, 14.30 to 14.45, 16.45 to 17.00, 19.00 to 19.15 hr). During the testing periods on Day 1 and 2 patients were systematically awakened after 2 min of recorded sleep. Eight normal controls of similar age (35 - 60 yr) were asked to undergo the same protocols as our patients. Results

The performance of control subjects during each of the testing sessions was normal; the error range on the WAT was approximately 6% and performance on the SAT and LSVT was continuous and uneventful. By contrast, patient performance after the first 10 min of a testing session deteriorated. During the WAT administration patients suddenly would begin to perform inadequately, sometimes doing the same addition again and again, other times showing an increasing amount of errors (up to 30°/0), or simply jumping over 1 series of additions. During the SAT they would sometimes stop pressing the counter or press it inappropriately. During this time patients' eyes might be open but with a staring, empty look. Some patients (see Figs. 1, 2A and 2B) presented this abnormal behavior while talking to the technician. They might say something that bore no relation to any preceding conversation. Such statements were grammatically correct but contextually inappropriate. During these periods of inadequate performance and inappropriate speech, repetitive "micro-sleep" episodes were noted on the polygraph. We define "micro-sleep" as a sudden, short-lasting burst of typical stage 1, slow-wave sleep as described by Rechtschaffen and Kales (1968) and/or a short burst of "synchronous theta activity" recorded in central monopolar derivations (C3/A2, C4/A~, O2/A1). These short bursts are usually repetitive in close clusters (see Fig. 2A). In REM narcoleptic patients we recognized some patterns which could be classified as "micro-REM sleep" with a very short drop of muscle tone and rapid eye movements. This pattern has not been seen in patients presenting any other "hypersomnia" syndrome. One patient was accurately completing a questionnaire when he experienced some short, repetitive "micro-sleep" episodes, and during that time wrote the sentence shown on Fig. 3. He had no idea of what it meant nor could he give any reason for writing it. Comment

This "automatic behavior" syndrome is a cause of great anxiety for all our patients. The syndrome has put their lives in jeopardy, impaired their ability to work adequately and has led to professional and marital failures. The patients' inability to perceive the alteration of consciousness and the complete amnesia associated with the "automatic" activity had resulted in medical diagnoses ranging from "temporal lobe epilepsy" or "possible onset of pre-senile dementia" to "hysteria." Recently a great deal of attention (Colquhoun 1972) has been given to the effect of sleep loss on human efficiency in normals. Johnson, Naitoh and Moses (1974) have clearly demonstrated, in normal subjects with selective sleep deprivation, that the amount of sleep loss is much more important than the type of sleep

384

C. G U I L L E M I N A U L T , M. B I L L I A R D , J. M O N T P L A I S I R , W . C. D E M E N T 3.

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Fig. 3. A narcoleptic patient was filling out a questionnaire. He answered most of the questions appropriately. Then suddenly he presented repetitive bursts of "micro-sleep" and wrote "'on or about 85". This sentence had no relation to the question and the patient could not explain why he wrote it nor what it meant.

lost. It seems that without any doubt human performances can be impaired by severe lack of sleep. Our patients are apparently different; they present a sleep pathology which urges them to take repetitive naps during the daytime. Some may have slight nocturnal sleep deprivation, but their total amount of sleep during a 24-hr period is at least equivalent to the total sleep time of normals. Even if patients were allowed to sleep before performing, they still would present symptoms of automatic behavior. This syndrome is related to excessive drowsiness. It is usually incompletely controlled by amphetamine or methylphenidate; levodopa or tricyclic medications have no effect on the syndrome. Symptoms are always intensified by anti-epileptic medication from phenobarbital to hydantoin, primidone or benzodiazepine. Although there is no effective treatment for this syndrome, recognition of the disorder as part of the patient's sleep pathology can help prevent misdiagnoses and assist the patient in avoiding life-threatening situations. II: AN A L T E R E D S T A T E O F C O N S C I O U S N E S S :

THE CATAPLECTIC ATTACK

Fifty catapleetic attacks in 20 narcoleptic patients ranging from 33 to 60 yr of age were monitored during 3 hr afternoon recording sessions. We monitored continuously EEG, EMG from the elfin and various upper and lower limb muscles, EOG

ALTERED STATES OF CONSCIOUSNESS and

H-reflex

following

the

technique

of

Guilleminault,

385 Wilson

and

Dement

( 1 9 7 4 c ) . T w e n t y of t h e s e c a t a p l e c t i c a t t a c k s w e r e r e c o r d e d o n F M t a p e . M o s t o f t h e m w e r e of a s h o r t d u r a t i o n a l t h o u g h s o m e l a s t e d s e v e r a l m i n u t e s - - t h e

longest

r e c o r d e d w a s o f 15 m i n d u r a t i o n . D u r i n g t h e s e e p i s o d e s p a t i e n t s w e r e a s k e d t o t r y to move different parts of their body. Orders were complex, for example, "try to m o v e t h e t h u m b o f y o u r left h a n d " , " t r y t o e x t e n d t h e f i f t h t o e o f y o u r r i g h t f o o t " . A t t h e e n d of a C a t a p l e c t i c a t t a c k p a t i e n t s w e r e a s k e d t o r e p o r t t h e i r f e e l i n g s d u r i n g the attack and any specific imagery.

Results Short cataplectic attacks were usually not associated with any abnormal state of consciousness; patients remained in normal contact with their surroundings. However, when the attack lasted several minutes very different reports were obtained. Three representative cases have been selected from our recorded tapes:

Patient S Q A

Q A Q A

Q A

Q A

Mr. S, could you explain what happened during your cataplectic attack and exactly what you felt? When I did have the attack fully I was out in the street somewhere and I could see the concrete. I was bent over, head between my legs and oh, questions were asked, about movement, if I could move my fingers or my feet. Also I could see the concrete and I could tell when you asked me to move. Could you see us? No, I had my eyes closed when you were talking to me, also this was a dream-type that I knew was unreal. What gave you the clue that it was unreal? I really can't tell you. I can't explain it to you myself how I knew that. I could feel the shock going through (H-reflex stimulus) and I was getting very uncomfortable with my head down. Now whether it was down or not I don't know. Did you feel like you were sitting down? No. I was sitting in this chair and at the same time I was out on the street. My head was going over. I was looking down. I wasn't looking up. I could see concrete, what to me was concrete and curbing. I could see feet moving by occasionally which would be on the street. I thought I could see and hear tires moving to the side of me; whether they were automobile tires or not I don't recall, I just thought we better get out of the street instead of . . . Well, I couldn't do anything about it. I didn't know whether you were going to help me out or not. At the same time you heard me asking you to do things? (Yes.) Do you remember specifically what I asked you to do? Yes. Move fingers on the left hand. I did. I moved one and five. Right hand went the same way, moved one finger at a time. Tried to move my right thumb. I remember doing that and at the same time I was on the street, I leaned over . . . D u r i n g t h e e n t i r e c a t a p l e c t i c a t t a c k t h e p a t i e n t w a s s e a t e d in a c o m f o r t a b l e a r m -

c h a i r , h a d h i s h e a d t o o n e side, r e s t i n g o n h i s s h o u l d e r , w i t h h i s e y e s h a l f c l o s e d , t r y i n g t o o b e y all o r d e r s w h i c h w e r e c o n t i n u o u s l y g i v e n t o h i m (see Fig. 4).

Patient N Q A

What happened? (The cataplectic attack was induced by a joke.) It was a good joke. I could not fight against it. It just took me by surprise. I tried to move my feet as you asked but I really could not do it. Then I saw Molly (a friend of the patient who was not present). I could not figure out what she was doing in this mess (patient's description of the experimental situation). She was with a pretty blonde.

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Q A Q A

I tried to call her, but you were asking these questions. 1 had to move my right thumb, and then I tried to grab her but she would not let me do it. I did not have the strength for it. I was much smaller than her. W h a t do you m e a n by "smaller?" I don't know. I had the feeling o f being crushed down and she was really taller than myself. I could not reach her; it was like looking up at her from below. Did you have your eyes open? Yes, but I h a d a blurred vision. I could see the machine a n d the paper a n d : M o l l y at the same time.

Patient B Q A Q A Q A

Q A

Q A

What happened? I was trying to cooperate with you folks. A t the start of it, someone asked me to open my eyes, and I did open them, and they seemed to be a wee bit crossed. Could you see us? Yes. You were blurred. Double image? No. They weren't double images. My eyes were focusing in farther than they should. There was no double vision whatsoever. But I was floating. I was really up in the air. I thought it was ridiculous because I was seated in that chair with these shocks (H-reflex). Had you a "feeling" of floating or were you actually in the air as in an airplane? I cannot explain it. I knew it was not real, that I was here with you, but my body was up in the air, like one of those yoga tricks. I was kind of dissociated, no weight feeling. It was very uncomfortable. No feeling of position. I could hear you, but I had wind in my ears. you know, same as when you speed on a motorcycle. Did you see anything special? No, I didn't have any hallucination, just you were not real anymore. 1 was way up in the air, but also in that chair.

Comment Cataplexy

is u s u a l l y a s h o r t , s u d d e n d e c r e a s e i n o r a b r u p t loss of m u s c l e t o n e

w h i c h r e s o l v e s as q u i c k l y as it a p p e a r s ( G u i l l e m i n a u l t et al. 1 9 7 4 c ) . D u r i n g t h e s h o r t e p i s o d e s , l a s t i n g 1 m i n o r less, w e h a v e n e v e r o b t a i n e d r e p o r t s o f a l t e r e d s t a t e s . But when the attacks have a longer duration, this combination perception"

integrated into the reality of the surroundings

of "dream-type

of

is f r e q u e n t , P a t i e n t s

ALTERED STATES OF CONSCIOUSNESS

387

often reported distortion of sense of position during long cataplectic attacks. The feeling of "floating" in the air is very common and raises the possibility that transmission and/or processing of proprioceptive impulses may be altered during a cataplectic attack, and that this lack of sensory information may explain the feeling of weightlessness, change of size and what could be called a "Gulliver syndrome" which frequently were reported. Another interesting point is the meshing of reality and hallucinatory-dreamy type sensations experienced and reported by the patients during some long-lasting cataplectic episodes. The patient simultaneously obeys - - o r tries to obey--orders given by the experimenter, perceives and integrates messages, and at the same time, is in a dream situation that he knows is unreal but which is intense enough to make him call out a name or try to run away from a frightening scene. A close analysis of the EEG during these episodes does not show any striking difference compared to wakefulness; however, for very short periods of time, usually lasting less than 10 seconds, it is impossible to differentiate the recording obtained during the attack from one obtained on the same subject during a REM period. The problem of the possible interrelation between hallucinations, sleep and dreaming sleep has been questioned for years. Is REM sleep really a "third" state different from wakefulness and sleep as Jouvet has postulated (Jouvet 1974), or is REM sleep just a melange of reality intruding on a sleepy brain, resulting in visual misperception, temporal disorientation, cognitive disorganization, tactual misperception and disorientation for space, objects and persons in a way similar to that observed by Williams, Morris and Lubin (1962) after sleep deprivation in normals? The cataplectic attack as an experimental "preparation" has been neglected for years; however, in our view it is an approach which could eventually give us more insight into the fascinating problems of how endogenous hallucinatory input is integrated with reality. III. AN ALTERED STATE OF CONSCIOUSNESS: SLEEP-INDUCED APNEA

The Pickwickian syndrome described by Burwell, Robin, Whaley and Bikelman (1956) is a well-known entity. In recent years it has been demonstrated that a nonobese patient could present the same respiratory abnormality as an obese one (Lugaresi, Coccagna, Montovani, Berti-Ceroni and Pazzaglia 1969; Guilleminault, Eldridge and Dement 1972, 1974a), and that different types of apnea can be associated with sleep in this population. An "obstructive sleep apnea" is predominant in obese Pickwickian-type hypersomniac patients and in non-obese hypersomniacs (Guilleminault, Raynal and Dement 1974b). The term "obstructive" should not be misconstrued since it describes what is actually happening during the sleep of the patient, i.e., an upper airway obstruction. It does not mean that there is an external cause of the obstruction. In fact, it appears that a neurologic dysfunction is induced or increased during sleep which leads to an active hypercontraction at the level of the larynx and/or a sudden relaxation involving the pharyngo-laryngeal muscles. A central nervous defect is suspected. Recent studies on cats using micro-electrodes have shown that for some cells in regions which

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have been defined as respiratory (Orem, Montplaisir and Dement 1974) sleep disrupts the association of neuronal activity with respiration. This implies a reduction in central respiratory drive during sleep in cats and may eventually be of great help in understanding the pathophysiology of sleep apnea. All of our 45 sleep apneic patients (adults and children) present abnormal states of consciousness. Very shortly after sleep onset repetitive apneic episodes occurred. The sleep polygraphic monitoring of patients appeared very disturbed and sleep stages were difficult to identify. During the apneic episode slow waves of high voltage often occur, associated with a sudden chin electromyographic inhibition. Then when patients resumed breathing the E E G ( C J A , 2 - C4/A 1 standard derivations for sleep monitoring) usually switched from a theta rhythm to a slow alpha which looked like stage 1 non-rapid eye movement sleep. Sometimes patients presented "alpha arousal" at the end of an apnea. The polygraphic recording indicated that patients then were in a "light" sleep. They very seldom present a stage 3 or 4 ("deep") sleep. During this period of EEG-defined light sleep these patients are behaviorally in a state of consciousness which appears to be far removed from their environment. Patients cannot be "'awakened" easily, even with painful stimulation. It is often necessary to shake them violently for several minutes before obtaining a response. A patient may even open his eyes, appear to look around, then present another apneic episode, even as stimulation is continued. The sleep of these patients is very agitated with frequent abnormal movements before respiration resumes. These movements may either be simple movements of the feet a n d / o r the hands, or may involve the whole body. Such massive movements can be dangerous for the spouse. One of our patients, a 195 cm, 124 kg Causasian male, unknowingly slaps and kicks his wife. She has never been successful in waking him during this state and has had to leave the bed at least twice a week during the past 10 months to avoid injury. Patients may also suddenly sit up in their bed. They seem to be half awake behaviorally, but unfortunately the amount of movement artifacts on the EEG's did not allow a good analysis of the polygraphic recordings obtained during these episodes. Patients do nol respond to questions and after 30 sec or as much as 2 min they fall back asleep. Sometimes patients try to get out of bed. We have actually seen such action in only 1 patient but have obtained similar information from spouses or mothers of 10 other patients. On occasion patients will stand up, even try to walk and suddenly fall on the floor in front of them. They sometimes will then sleep the rest of the night on the floor. sometimes in very abnormal positions. Several patients have bruised themselves severely during these episodes. There was never any evidence of epileptic activity in any of our recordings and neither we nor the families ever noticed convulsive movements. Ear oxymetric curves and direct measurement of arterial blood gases by means of a brachial arterial catheter (in 12 cases) showed that abnormal movements always started during apneic episodes with concomitant hypoxemia, hypercapnia and acidosis (Guilleminault, Eldridge, Simmons and Dement ] 975c). However, during the episodes of ambulation or "half awake" states patients lind normal respiration and showed no evidence of hypoxemia or hypercapnia. Such reversion to normal blood gas status usually o c c u r s after one or two breaths.

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All of our children and 2 adults also presented repetitive enuretic episodes during their sleep. Enuresis appeared in the evolution of the disease after the onset of daytime sleepiness and was practically an every night problem. It was considered a very annoying disorder by all our patients and they had tried every possible means to avoid it. The enuretic episodes were always recorded in a "stage 2 N R E M sleep" after more than 30 min of repetitive apneic episodes. Two enuretic episodes could occur during the same night in a given subject at various times of night. In the morning, for various lengths of time which could last from 3 to 30 min, patients appeared slightly disoriented, giving inappropriate answers to questions, having difficulty remembering events which happened the previous day. Most patients described this state as having a "foggy mind". In our children, particularly 8 and 9 yr old males, this abnormal state was usually associated with very frightening hallucinations. Hypnagogic hallucinations were reported by 15 of our adult subjects during the daytime when uncontrollable urges to sleep occurred. These patients might suddenly see "a tree in the road", "a flashing red light", "a dog driving a car" for a very short period of time. They quickly recognize the hallucination and correct the distortion; however, it will be real enough at the time of the vision to make them brake abruptly while driving, which led to a car accident in one case. The children reported slightly different hallucinations after awakening. Frankie is 8 years old and lives in a world where Superman, Frankenstein and other monsters continuously interact with him. These "monsters" appear to participate in Frankie's life in an abnormal way (in contrast to the fantasies of the other children) and he was referred for psychiatric care, primarily becouse of his "monsters". Frankie has a sleep apnea-hypersomnia syndrome and has great difficulty waking up. He is in a continuously sleepy state in the shower, at the breakfast table, or just standing up in his room. It is during these periods that Frankie has contact with his monster friends. He may see them around him and the visual hallucinations seem so very real that later on during the day the child has difficulty discriminating reality from his morning hallucinations. He realizes that something is not "real" but at the same time his "foggy" morning awakenings with their abnormal imagery leads to a hyper-excitatory state. He is fearful of falling asleep and tries to fight against it as much as possible.

Comment Many questions are still unanswered in the hypersomnia/sleep-induced apnea syndrome. For example: What are the roles of hypoxemia and hypercapnia in the causation and maintenance of the sleep apneic's abnormal sleep and of his socalled "half awake/foggy" state? Repetitive sleep apnea is associated with recurrent hypoxemia, hypercapnia and acidosis; however, when the patient, even if not behaviorally wide awake, presents an "alpha arousal" on the polygraphic monitor, respiration resumes and blood gas values compare to baseline while awake within a few seconds. One can question the cerebrospinal fluid (CSF) changes induced by hypercapnia and hypoxemia and their possible interaction on the different central receptors or surrounding brain-stem structures. Unfortunately no studies were ever done on the amplitude and duration of CSF changes after hypoxia and hypercapnia in humans and at this time there is no good explanation for the altered states of consciousness. Some points, however, must be emphasized. The abnormal movements seen during sleep apneic episodes are a very common feature--we observed

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c. GU1LLEMINAULT~ M. BILLIARD, J. MONTPLAISIR, W. C. DEMEN g

them in all our patients. Their intensity is variable and dependent on several factors: an obvious factor is the level of oxygen desaturation but there were also important individual differences. For the same reading of hypoxemia and hypercapnia simple movements of flexion and extension of feet and hands can be observed in one case, typical "flapping tremor" in another, and large movements of four limbs in a third one. Of course when patients awaken completely there is no sign of any abnormal movement and neurologic examinations are always normal.

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Fig. 5. Recording obtained from a sleep apneic patient. The definition of sleep is difficult. As seen here the chin E M G decreased markedly during the apneic episode. The definition of the stages of sleep using criteria defined in normals doesn't seem appropriate. Low amplitude, mixed frequency E E G with sudden bursts of high amplitude theta or delta waves are usually recorded during the repetitive apneic episode. In the case of this patient. 620 apneic episodes occurred during a 7-1/2 hour polygraphic monitoring and his polygraphic recording, except for typical R E M sleep defined by the burst of rapid eye movements on the eye movement channel, was similar throughout the night to the one on the figure.

A second problem is the definition of sleep stages in these patients. Non-rapid eye movement (NREM) sleep has been subdivided into 4 basic stages. We are dealing here with patients and it must be emphasized that they present an abnormal, pathological sleep recording (see Fig. 5). Sleep researchers and clinicians still try to classify these pathological sleep recordings using standardized criteria already described (Guilleminault e t al. 1974a). But obviously, a "stage 2 NREM sleep" in sleep apneic patients is a very ambiguous and misleading term. There is still an overall analogy with a stage 2 NREM sleep seen in normals but the use of this term only emphasizes the normal aspect of the recording and completely ignores the actual pathological phenomena. Unfortunately, the "scoring manual for sleep pathologies" has yet to be written. Sleep apneic patients have an "abnormal" sleep. They present enuretic episodes and their polygraphic recordings

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usually show low voltage, mixed frequency activity with sudden polyphasic high voltage, slow waves which occur paroxysmally. Sleep spindles are rare--waves similar to K complexes occur more frequently. With a mean of every 45 sec, at the end of an apneic episode, a sudden change in the polygraphic recording occurs which very often involves EEG, chin E M G and EOG. One could question why enuretic episodes are recorded in that stage and not in stage 4 as is often observed in enuretic children. Also of interest is why patients appear to be in such "deep" sleep during recording of stage 1 or 2 N R E M sleep. We believe that our patients have their altered states during their sleep. For us then, normal sleep is a normal state of consciousness and the behavioral symptoms noted during cataplexy and repetitive sleep apnea are the signature of an altered state of consciousness. These altered states during sleep and the "half awake/foggy" state may interact with the personality of the individual. The spouse of one of our patients summarized perfectly the common reports on personality changes noted by the family in the following way: " D has a rather high I.Q. and has had a wide range of interests. H e could converse intelligently on myriad topics and had a quick wit and a good sense of humor. N o w it seems that his interests have diminished greatly and he does not seem to converse easily. It is as if he is just too tired to think and in fact he has told m e that much of the time he simply doesn't think about anything. If I was u n a w a r e of his sleeping and breathing p r o b l e m s (our patient's spouse m a d e an exact diagnosis before we saw the patient!), I would believe that he is suffering from a deep psychological disturbance. However, in considering his overall condition, I believe that it may result f r o m a longstanding physical p r o b l e m . . . . "

Understanding of the underlying mechanism behind the hallucinations seen in adults and children needs much more documentation. Obviously the long-term impact of the "after awakening" hallucinations in children does not seem to be similar to the hypnagogic hallucinations of our adult patients when an urge to sleep occurs. The effect of these hallucinations on children can be dramatic and may lead to very important and distressful personality changes. Hypnagogic hallucinations of narcolepsy, when occurring in patients between 12 and 15 years of age, have led some of them to question their sanity and into major depressive states. Hallucinations induced by the sleep apneic syndrome may lead to even more severe personality changes. Our group of children ranged from 6 to 12 years of age (Guilleminault, Eldridge, Simmons and Dement 1975b) and the interaction of the altered state of consciousness with the surrounding world can eventually lead to marked secondary psychological disorganization. CONCI~,USION

Daytime sleepiness has never been considered a serious disease. More than 500/0 of our patients were ashamed of their disease: "People consider us as 'lazy', physicians don't think that 'too much sleep' can impair our capabilities or our lives", was the general comment obtained from our patients. A study performed on the first 50 narcoleptics seen in our clinic showed that a mean of 15 years existed between the onset of symptoms and an exact medical diagnosis, despite the fact that numerous medical consultations had been sought.

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Narcolepsy and sleep-induced apnea-hypersomnia are two different entities which involve a very different physiopathology; however, they both head to a similar impairment of the social and professional capability of our patients. Observers may not even be aware of the short, continuous interruptions of alertness which afflict this group. Patients themselves sometimes do not realize the frequency of their micro-sleep episodes or altered states of consciousness. They usually are only aware of them when a major error or accident occurs. Our continuous polygraphic recordings have shown that, in fact, they may ignore most of thc~e episodes which can lead not only to eventual accident but also important changes in personality. One can only stress the importance of an early, positive diagnosis in such cases. The possibility of sleep pathology should be considered when diagnosing neuropsychiatric problems. ACKNOWLEDGEMENT

We wish to thank Dr. Merrill M. Mitler for his help in editing the manuscript. SUMMARY

Patients with daytime sleepiness present altered states of consciousness. The occurrence of these stetes impairs their professional, social and familial activities and may threaten life itself. The automatic behavior syndrome is characterized by continuation of mechanical activity and complete amnesia. Episodes lasting from a few seconds to several hours are correlated with repetitive micro-sleep periods. During cataplectic attacks, patients may have a meshing of reality with hallucinatory dream contents. Sleep-induced apnea may lead to abnormal movement and abnormal ambulation during sleep as well as hallucinations in the early morning. These altered states of consciousness must be considered as diagnostic indexes in differentiating epileptic syndromes from syndromes of daytime sleepiness. REFERENCES BILLIARD, M. (1975) Rapid eye movement sleep versus non-rapid eye movement sleep in nar-

coleptic patients. In: C. GUILEEMINAULT.W. C. DEMENTANDP. PASSOUANT(Eds.), Narcolepsy An International Meeting, Spectrum Publications, New York. N.Y., In press. BURWELL,C., E. ROBIN,R. WHALEYAND m. BIKELMAN(1956) Extreme obesity associated with alveolar hypoventilation - A Pickwickian syndrome. Amer. J. Med., 21: 811-812. COLQUHOUN,W. P. (1972) Aspects o[ Human Efficiency, The English Universities Press. Ltd.. 344 pp. GUILLEMINAULT, C., F. L. ELDRIDGE. W. C. DEMENT ~1972) I n s o m n i a . n a r c o l e p s y a n d sleep

apneas. Bull. physiol. Path. Resp., 8: 1127-1139. GUILLEMINAULT, C., F. L. ELDRIDGE. W. C. DEMENT 11974a) H u m a n p a t h o l o g y -- Sleep-induced

apneas and cardio-vascutar changes, Bull. physiol. Path. Resp., 10: 244-247. GUILLEMINAULT,C., R. PHILLIPSAND W. C. DEMENT(1975a) A syndrome of hypersomnia with automatic behavior syndrome. Electroenceph. clin. Neurophysiol., 38: 403---413. GUILLEMINAULT,C., D. RAYNALAND W. C. DEMENT(1974b) Comparison between Pickwickian and non-obese sleep apneic patients, Sleep Research, 3: 138. GUILLEMINAULT, C., R. WILSON AND W. C. DEMENT (1974C) A s t u d y on cataplexy. Arch.

Neurol. (Chic.), 31: 255-261. GUILLEMINAULT, C., F. t . ELDRIDGE. E. B. SIMMONS AND W. C. DEMENT t1975b/Seven children

with sleep apnea, Pediatrics. Submitted for publication.

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GUILLEMINAULT, C., F. L. ELDRIDGE, F. B. SIMMONS AND W. C. DEMENT (1975C) Une anomalie du systbme nerveux central peut-elle ~tre responsable de troubles h6modynamiques? Nouv. Pres. M6d., 4: 1343-1348. JOHNSON, L. C., P. NAITOH AND J. M. MOSES (1974) Deprivation with total sleep loss - Experiment 2, Psychophysiol., 11: 147-159. JOUVET, M. (1974) Le R~ve, La Recherche, 5: 515-527. LUBIN, A., J. M. MOSES, L. C. JOHNSON AND P. NAITOH (1974) The recuperative effects of REM sleep and stage 4 sleep on human performance after complete sleep loss - Experiment 1, Psychophysiol., 11: 133-146. LUGARESI, E., G. COCCAGNA, M. MONTOVANI, G. BERTI-CERONI AND P. PAZZAGLIA (1969) lpersonnie essenziali associate a respirazione periodica, Sist. nerv., 21: 18-27. MORREL, L. (1966) Some characteristics of stimulus-provoked alpha activity, Electroenceph. clin. Neurophysiol.. 21:551 561. OREM, J., J. MONJPtAISIR AND W. C. DEMENT (1974) Changes in the activity of respiratory neurons during sleep, Brain Res., 82: 309-315. RECHTSCHAFVEN, A. AND A. KALES (1968) A Manual o/ Standardized Terminology - Techniques and Scoring System /or Sleep Stages o/ Human Subjects, U.S. Government Printing Office, U.S. Public Health Service, Washington, D.C. WILKINSON, R. (1968) Sleep deprivation - Performance tests for partial and selective sleep deprivation. In: L. ABT AYD B. RIESS (EDS.), Progress in Clinical Psychology, Vol. 8, Grune and Stratton, New York, N.Y., pp. 28-43. WILLIAMS, H. L., G. O. MORRIS AND A. LUBIN (1962) Illusions, hallucinations and sleep loss. In: L. J. WEST, (Ed.), Hallucinations, Grune and Stratton, New York, N.Y., p. 295.

Altered states of consciousness in disorders of daytime sleepiness.

Patients with daytime sleepiness present altered states of consciousness. The occurrence of these states impairs their professional, social and famili...
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