Canad. M. A. J.June 1954, voL 70

SYMONDS: CATAPLEXY 621

CATAPLEXY AND OTHERRELATED FORMS OF SEIZURE*

SIR CHARLES SYMONDS, M.D., F.R.C.P.,London, Eng.

IN 1880 GELINEAU published his classic paper

describing a number of patients presenting two

kinds of attack. The one took the form of sudden,overpowering drowsiness and sleep during theday, usually of brief duration. This kind of attackhad been previously described and called narco-

lepsy. The other was an attack of weakness or

paralysis precipitated by emotion, again suddenand invariably brief. To this second variety ofseizure the name cataplexy was given. Laterwriters have, as it appears to me, created con-

fusion by subsuming both varieties of attackunder one heading and using the term narco-

lepsy to describe Gelineau's syndrome. They takenarcolepsy to include both sleep attacks andcataplectic attacks. This is inaccurate and mis-leading for, as has been pointed out by many

others before, either form of seizure may occur

alone. Narcolepsy is a correct and convenientterm for the sleep attacks, and cataplexy for theothers; it is in this sense that I shall use them,referring to the association of the two as

Gelineau's syndrome.Cataplexy is, of course, far more often ob-

served in Gelineau's syndrome than in any othercontext, and cases of this syndrome are com-

monplace in neurological practice. I shall never-

theless venture to remind you of its main fea-tures, which can be stated simply. The patient,who is otherwise in good health, is liable toattacks of overpowering drowsiness leading tosleep during the day. These are most likely tooccur during inactivity, or after a meal, butmay occur at any time, for instance, during con-

versation or while eating. The duration of sleepis variable-from ten minutes to an hour or two.Nocturnal sleep is normal. The other complaintis of sudden loss of power caused by vivid emo-

tion1, and especially by laughter. The duration ofthese attacks is always biief. They last from a

few moments to a minute or two. Their severityvaries from a sense of weakness to completecollapse, so that the patient falls if standing.Apart from laughter or amusement, other pleasur-able emotions are usually the most effective

*Address given to the Academy of -Medicine, Tor onto,September, 1953.

precipitants-the excitement associated with awinning stroke at tennis, or raising a gun for agood shot at a bird. One of my patients, a youngand active man, said he had had to give upplaying all games because any thrill would makehim "go flop". For laughter to be effective theremust be genuine amuserrient. A patient said tome that when he told a funny story and laughedhe always had an attack, but not always whenhe laughed at someone else's story. Of the non-pleasurable emotions, annoyance is most oftenblamed. It must be a sudden surge of feehng. Apatient of mine ran to catch a bus in the rain,atid as he almost reached it the conductor rangthe bell and it moved off. He fell helpless in themuddy road, overcome by his emotion. Of allthe emotions, however, laughter is the most effec-tive, and in some of my own cases it has beenthe only precipitant of attacks. This specificityof trigger mechanism is remarkable, and I willallude to it again later.

Rarely cataplexy may occur without the co-existence of narcolepsy, but in these cases, so faras I am aware, it is not laughter, but some otheremotion which is the usual precipitant. One ofthe best examples is that recorded by GordonHolmes (1927), in a patient whom I was privi-leged to see on several occasions while he wasin the National Hospital, Queen Square, London.This was a boy of 14, who since the age offive had been subject to falling on noise or sur-prise. Any loud sound, such as a tire bursting,a motor hooting, or a door slamming, mightcause him to fall; surprise had a similar effect.For example, being overtaken silently by acyclist when walking, a cat jumping on to thetable, or being touched from behind might causehim to fall, sometimes so abruptly that he hadsuffered many fractures of his limbs. There wasnever any degree of loss or disturbance of con-sciousness, no change of colour, and no vertigo.At first while he was in the ward it was easy toproduce a fall by a sudden noise, but he becameexpectant and, the element of surprise beingremoved, the stimulus was no longer effective.There had, it seemed, to be a certain affectivequality in the stimulus. While in hospital he hada single epileptic attack in his sleep. In thishe was cyanosed, his limbs were convulsed andhis eyes deviated to the right. This case at onceraises the question of the relationship of cata-plexy to epilepsy, which has been much disputed.Adie (1926) considered Gelineau's syndrome to

622 SYMONDS: CATAPLEXY

be a disease sui generis, having nothing to dowith epilepsy. Wilson (1928), from his own ob-servations, arrived at the opposite conclusion,and confirmed this opinion in his textbook (1940).Before giving my own views on this subject Ishall present some further observations.

Althouigh the constituent features of Gelineau'ssyndrome are remarkably constant in nearly allcases, there are exceptions which deserve care-ful consideration. Thus in some instances, other-wise typical of the syndrome, attacks of power-lessness may occur without any precipitatingcause, being in all other respects like the usualcataplectic attacks. On account of this spontane-ous character they need a separate name, but forconvenience of discussion I shall refer to themas spontaneous cataplexy. The following caseprovides an illustration.CASE 1A married woman aged 27 had begun a year previ-

ously to have attacks of sudden, unaccountable drowsi-ness in the day so that she would fall asleep even whileeating a meal. A little later she began to have suddenfalls. Without warning, her legs wouTd give way and shewould flop and have to stay on the floor for half aminute. If sitting, she would be quite unable to move orspeak and her eyes-would close, though the attacks wereso brief that they often passed unnoticed by others. Theymight also occur in bed. There was no disturbance ofconsciousness, and she had a clear appreciation of hersurroundings throughout the attack. She was emphaticthat these attacks usually occurred without any cause sofar as she could determine, but on one or two occasionsshe had noticed an emotional precipitant. The examplesshe gave were, sudden apprehension, if she saw some-thing unpleasant about to happen, or if she were excitedat an unexpected meeting with an old friend. Such oc-casions, however, were exceptional. As a rule the attackswere spontaneous.

Examples of spontaneous cataplectic attackshave been recorded by others (Wilson 1928,Rothfeld 19t38). Wilson (1928) was also the firstto draw attention to the resemblances of cata-plexy to sleep paralysis. This condition was firstdescribed by Weir Mitchell (1897), who calledit nocturnal paralysis; but, as attacks may alsooccur in the day, sleep paralysis is the betterterm. They may happen either at the moment ofgoing to sleep, or during sleep, and have ac-cordingly been divided by Levin (1933) intopre-dormitial and post-dormitial paralysis; butsleep paralysis covers both forms. In these at-tacks the subject is perfectly conscious, but un-able to move, the paralysis lasting for a minuteor two. The paralysis is as a rule complete, thepatient being unable to speak. This uncommonaffliction is not as a rule associated with othersymptoms, but may occur in association with

Canad. M. A. J.June 1954, vol. 70

vides an example and at the same time introducesanother rare feature of the syndrome, forms ofattack which are intermediate between -narco-lepsy and cataplexy.

CASE 2The patient was a serving officer aged 29, seen during

the war. He had had no illness of note before the presentillness began. The first episode occurred during coitus,when at the moment of orgasm he experienced a suddencollapse. All his muscles went completely limp, his jawdropped, his head fell forward, and he was unable tospeak. The whole episode lasted about a quarter of aminute and he then felt quite well.Two days later he was just going off to sleep and

suddenly found himself paralyzed. He was unable tomove any part of his body. The attempt to speak re-sulted in an inarticulate sound. The attack was over inless than a minute. Subsequently he continued to havethese attacks at frequent intervals just as he was goingoff to sleep, either at night or if he dozed during the day.He next developed a typical Gelineau's syndrome-attacksof irresistible sleep during the day and a liability to col-lapse on excitement. Joking and laughing in the messwould bring these on, "especially if I make the joke ratherthan others". He had once fallen on a tennis court whenabout to deliver a smashing volley. He still occasionallyhad a collapse in coitus. He would also have collapsesfor no apparent reason. He found, moreover, that if hewas feeling tired and sleepy and gave way to this feeling,he might have a collapse instead of going to sleep. Eventhe thought of relaxing might cause this. He gave anexample. Going on leave after a long walk through streetshe entered a lift in a hotel, in which there was a seat.The thouight of relaxing and sitting down at once madehim cruimple up. There was no sleepiness.

The opposite occurrence, attacks of sleep pre-

cipitated by emotional excitement, has also beenrecorded (Wilson 1940), though I have notobserved it personally.The resemblance of spontaneous cataplexy to

a certain variety of epilepsy, so-called akineticepilepsy, is obvious. Akinetic epilepsy is a termnow used a good deal for seizures in which theoutstanding event is a fall, and it is indeed worthenquiring why some patients in minor attacksfall and others do not. The fall does not appear

to depend upon loss of consciousness. Some pa-

tients in their attacks are unresponsive and ap-

parently unconscious without falling: others fallwith only a momentary arrest of thought, andothers again (so far as can be observed) withoutany loss of counsciousness at all. Falling in minorepilepsy may, of course, result from widespreadmuscular spasm, and this may be so brief thatit is observed with difficulty. Natter and Gastaut(1950) suggest that this is the true interpretationof the so-called akinetic attacks which Lennox(1951) and others include in their symptomatictriad of juvenile petit mal. According to thisview, there is a sudden, momentary, generalized,

Gelineau's syndrome. The following case pro- myoclonic shock which throws the patient to-the

Canad. M. A. J.June 1954, vol. 70 SYMONDS: CATAPLEXY 623

ground. This interpretation may be correct, butit is certain that in some epileptic seizures fallingis due to momentary loss of muscular action.Whether there is interruption of consciousnessin attacks of this kind is difficult to ascertain,but from the introspection of patients I amsatisfied that falling with no disturbance of thesenses does occur. Ramsay Hunt (1922) deservesthe credit of recognizing such attacks, and ofcoining for them the descriptive term "dropseizures". My own impression is that drop seizuresare rare, that they occur not in association withfrank epilepsy, but as an isolated phenomenon,that they are usually met with in women pastmiddle-age and that a family history of the com-plaint is not uncommon. I have notes only offour cases in my private records, but have cer-tainly seen an equal number in hospital practice.The story of the attacks in each instance has beenthe same and may be illustrated by the follow-ing account given me by a woman aged 62.

CASE 3The first of her attacks had occurred in her early

thirties (my impression being that this is an unusuallyearly age of onset). Thereafter she had had them in-frequently until recently. She might be free for severalmonths and then have two or three in the course of afortnight. There had been five in the five weeks beforeI saw her. The onset was sudden and unexpected, herlegs gave way and she fell forward on to her hands andknees. She was certain that there was no disturbance ofconsciousness. She was able to put her hands out to saveherself, but wouild occasionally bruise her knees. Therewas no giddiness. She was able to pick herself up im-mediately. Her husband, who had witnessed many at-tacks, confirmed her account, being certain that therewas no jerking or twitching of the limbs, nor any lossof consciousness. The attacks occurred only when shewas walking. They were never precipitated by laughter orsudden emotion. The patient clearly remembered that hermother at the age of 55 began to have exactly similarattacks and continued to have them occasionally until shedied 10 years later. The patient had witnessed theseattacks, but not until after she herself had had them.A sister of the patient, two years older, had similar at-tacks, the age of onset in her case being uncertain.

In their paroxysmal character and stereotypedpattern, these drop seizures clearly resembleepilepsy. The fact that there is no disturbanceof consciousness is, of course, no objection totheir inclusion in this category, for focal dis-charge vithout disturbance of consciousness is awell known occurrence. Indeed, momentaryparalysis of a more obviously focal characterwithout any attendant disturbance of the sensesis well recognized. A man at present under mycare with a right parietal tumour has momentaryattacks in which his left arm goes limp and hedrops whatever he may be holding in the hand.There is no twitching and no parnesthesik, and

between attacks he has little disability in thelimb. These inhibitory motor seizures have beendescribed by Holmes (1927) and others. Therarity of their occurrence requires explanation,but they may be reasonably accepted as epi-leptic; in this connection it is noteworthy that incataplexy, in the attacks which I have calledspontaneoiis cataplexy, in sleep paralysis, and indrop seizures the paralysis has been observed attimes to be partial or asymmetrical. It wouldseem, therefore, that cataplectic attacks mayrepresent only a wider distribution of the focalinhibitory motor epilepsy which may be observedin a single limb.There is another point of resemblance between

cataplexy and epilepsy-the reflex nature of theattacks. Reflex epilepsy, that is to say seizuresclinically recognizable as epileptic and inducedby specific stimuli, has been long known andmuch discussed. Noise, startle, sexual orgasm,and the act of micturition are among the stimuliknown to precipitate attacks in certain subjects.The act of coughing may also induce attacks.This has been questioned owing to the fact thatviolent and prolonged coughing may cause un-consciousness and clonic convulsions fromcerebral anoxia (Baker 1949). This doubtless ac-counts for most of the cases of so-called laryngealepilepsy reported in the past, but there are somein which the cough is no more than a tickle andcould not cause cerebral anoxia. I mention thepoint because it is relevant to the interpretationof a case to be described presently.Of the precipitants mentioned, startle and

noise are the most common, and sometimes areeffective only in combiniation. But startle alonemay induce attacks in some persons, and thenquite often. Thus one epileptic patient under mycare would have attacks induced by stubbing histoe against an obstruction when walking in thedark, another under the same circumstances bythe sudden and unexpected passage of a cyclistfrom behind. Cases have also been observed ofepilepsy precipitated by laughter. Thus there isa considerable body of circumstantial evidencefor a clinical affinity between cataplexy and epi-lepsy. I have two unrecorded cases to presentwhich add to this evidence:

CASE 4

A service man, aged 40, was referred for specialistexamination for dozing- off to sleep at odd times andattacks of paralysis.A maternal uncle was epileptic. He himself had had

no illness apart from a tuberculous hip in boyhood and

624 SYWIONDS: CATAPLEXY

the synmptom11s n1ow to be described, which fall into threedistinct phases. In his childhood he was subject to whatwere called fainting attacks if he hurt himself (for ex-ample, if he cut his finger) or if he were upset (forexample, if he saw a dog run over) or if he lost histemper. Tkings went black, and he would fall to theground unconscious. He was not told of any convulsivemovement. This liability continued up to the age of 16.At 15, while standing to watch a procession, wit-out anyemotional precipitant, he had a faint in which a com-panion observed jerking movements of the limbs, andin which he wet his trousers. This was the first phase.The story is that of frequent fainting on emotion, endingwith a fit; this sequence has often been observed anddiscussed without any explanation other than that theexcessive liability to fainting and the liability to epilepsyare separate manifestations of an inferior nervous system.The second phase lasted from the age of 16 to 35,

during which period the only occasions on which helost his senses were when he got up at night to passwater. There would usually be some urgency about this,but the faint would not occur until he had got to thelavatory and commenced to pass water. Things would goblack, and he would lose his senses and fall. He hadoccasionally hurt himself in falling. After one of thesefaints he would have a headache and feel shaky. Attacksof this kind are uncommon; but I have collected a dozencases, all in males, with exactly the same story, none ofthem, however, with any other symptoms. I have re-garded them as a species of reflex epilepsy.The third phase began when he was .35; while washing

in the morning he suddenly fell to the ground withoutany blacking out of vision or loss of senses. His mouthdropped open: he could not move his limbs: he couldopen his eyes only with a great effort. This lasted onlya few seconds, after which he picked himself up andfelt quite well again. Eighteen months passed withoutincident and he then began to have typical attacks ofcataplexy, provoked by amusement, anger, pleasurableexcitement or sudden noise such as gunfire. The liabilityincreased so that the attacks would occur with littleprovocation, and he would have as many as six in a day.He noticed at the onset quivering of the jaw. An attackwas witnessed by a nurse and described as follows. Hewas talking normally when a comrade threw a box ofmatches to him. He then seemed to lose all use of hislimbs and fell with chin sagging loosely. His eyes werepartly closed as if he were trying to open them, but hecould not manage it. At the onset his face generallytwitched.

In the past year or two he had also had attacks onwaking from sleep, either in the night or day, identicalwith the attacks of paralysis during waking hours. Andfinally, for four years he had had an increasing liabilityto drop off to sleep in the day time. The description ofthis was characteristic of narcolepsy. The sleep attackswere of sudden onset, and one had occurred when hewas driving a car, his wife saving him from disaster bytaking the wheel.

I have found no parallel for this case eitherin my own experience or in the literature, butpresent it as suggesting a link between cataplexvand reflex epilepsy.The next case is also importanit in this con-

nection.

CASE SA quartermaster-sergeant aged 35, a regular soldier

with 13 years' service, came with the symptoms andsigns of syringomyelia, which had been present for aboutfour years; for the same period he had been subject tothe attacks which will be described. It should be recordedthat his mother was an undoubted epileptic, and that hispaternal grandfather and grandmother had both diedinsane.

(Canad. M. A. J.June 1954, vol. 70

conscious without warning and recovered his senses 20minutes later, having bruised the back of his head. Sub-sequently he found himself liable to attacks of a peculiarkind if he laughed with real amusement, or was startledby a sudden noise. There was also a particular liabilityto attacks at the moment of going off to sleep if his wifespoke to him or anyone came into the room. In mostof these attacks he lost his senses and his wife told himhe was convulsed, though there was no tongue biting orincontinence. But in some of the attacks precipitated bylaughter or sudden noise he might collapse, and if un-supported fall, without loss or disturbance of conscious-ness. He appeared to have some warning of an affectivekind and stated that if at this moment le exerted con-trol he could prevent loss of consciousness. When he didlose consciousness he was told that he was always con-vulsed. He also had attacks which began with a coughand ended in a fit. In the past eight months, in coitus,after ejaculation he had had an attack of coughing whichwent on to a fit, this sequence being invariable.

There were no attacks of narcolepsy or sleep paralysisin this case.

Here, therefore, we have a patient whose firstsymptom was what appears to have been aspontaneous epileptic attack, and who subse-quently had attacks which might take the formeither of cataplexy or reflex epilepsy, their formbeing determined by the opportunity of exertingthat volitional control which we know to be ofteneffective in preventing the spread of epileptic dis-charge. From these observations I conclude thatcataplexy and the other related forms of seizure(spontaneous cataplectic attacks, drop seizures,falling on noise or surprise and sleep paralysis)may be considered as epileptic variants, havinga common focal region of discharge. Lookingfor some comparable epileptic manifestation, Ibelieve we may find an analogy in myoclonicseizures. These, as is well known, may be pre-cipitated by sensory stimuli. A visual stimulusin the form of flicker is the most effective, butone may observe patients in whom a touch ornoise will suffice to provoke a myoclonic spasm,if it is unexpected. I have found a patient whocould invariably be thrown into a spasm by asurreptitious kick against the foot of his bed.There is one group of myoclonics in which thereis another curious resemblance to G6lineau's syn-drome. These are patients who are subject tomyoclonic jerks just as they are dozing off tosleep, or even if they relax during the day. Theyare then roused by a sudden involuntary jerk, re-peated when they doze off again, and such repe-tition may continue to an extent which preventssleep for an hour or two. In these patients myo-clonus may also occur during sleep and awakethem, but they very rarely have attacks whichgo on to loss of consciousness or convulsions. Wefind, therefore, in the analysis of myoclonic epi-lepsv features which in some respects resembleThe first of, his attacks was spontaneotis. He fell 1111-,

June1954a sol. 70 SYMIONDS: CATAPLEXy 625

attacks of the cataplectic and narcoleptic type,and we may in this respect regard paralysis andmyoclonus as corresponding opposites.The close association of narcolepsy and cata-

plexy, and the observation of intermediate forms,are of some physiological interest. Flaccid im-mobility and normal sleep are naturally associ--ated phenomena. It would not be surprising,therefore, if the functional centres controllingthese two phenomena were closely associated inthe brain. The patient, whom I have mentionedas having paralytic attacks when he becamedrowsy and relaxed, remarked that it seemed tohim as if his body went to sleep without hismind. Another patient with sleep paralysis saidthat his mind appeared to wake up without hisbody.One of the striking things about patients with

cataplexy is that they do not lose consciousnessand are not convulsed. The case I have recorded,it is true, provides an exception to this rule, butit is a rule that is almost absolute. If cataplexy isan epileptic variant the discharge is one that doesnot spread. Little is as yet known of the physio-logical factors which inhibit or facilitate thespread of epileptic discharge, but the occurrenceof circumscribed discharge is well recognized inexperimentally induced seizures. It may dependuipon anatomical factors. If cataplexy is the clini-cal expression of an epileptic discharge arisingin deep-lying structures, for example, in the peri-aqueductal grey matter, with little opportunitvfor outflow to the cortex we might expect tofind consciousness intact, absence of convulsivemovement and a normal electroencephalogram,as in fact we do.

I have mentioned the reticular formation ofthe brain stem as a possible site for the dis-charge responsible for flaccid immobility. Thisspeculation is not new, being derived from theobservation of Gelineau's syndrome as a sequelof encephalitis lethargica, in which the mainincidence of the disease is in this region. Thereis evidence also from cases of tumour support-ing the existence of functional localization in thisarea for control 'of sleep and waking, and formaintenance of postural tone. Kelly (1951) inhis analysis of the symptoms in 29 cases of colloidcyst of the third ventricle found six in whichthere were attacks of sudden falling describedthus: "There is a sudden paresis of both lowerlimbs apparently without any interference withthe state of consciousness. The patient suddenly

collapses to the ground and is usually unableto rise again for a few seconds." I have observedsimilar attacks in two cases of suprasellar tumour.

Ethelberg (1950) has described, under thetitle symptomatic cataplexy, attacks of the samekind in two cases of depressed fracture with pres-sure upon the lateral surface of the frontal lobe,and has drawn attention to other cases in whichthe lesion was supratentorial. These, as he re-marks, are to be distinguished from attacks inwhich flaccid immobility is confined to a singlelimb, yet the underlying mechanism may be thesame. If there are centres, in the Jacksoniansense, in the brain stem for the maintenance ofmotor activity, one would expect this functionto be re-represented at cortical level and in rela-tion to different parts of the body. In other casesof generalized cataplectic attacks Ethelberg hasnoted cortical lesions affecting the mesial surfaceof both frontal lobes. In all these cases the cata-plexy was of the spontaneous kind. The attackswere in fact drop seizures. Stoupel (1950) statesthat in three patients with true akinetic epilepsyhe has observed electroencephalographic signsof focal discharge on the mesial surface of onehemisphere.To sum up,_ clinical observation suggests that

there is no fundamental distinction between epi-lepsy and cataplexy, and that other types ofseizure related to cataplexy are also epilepticvariants. There is reason for supposing a naturalphysiological association between flaccid im-mobility and sleep which might explain the fre-quent occurrence of cataplexy and narcolepsy inthe same patient. Such evidence as is providedby pathology suggests that the discharging lesioniresulting in the symptom of transient flaccid im-mobility is usually in the brain stem. There isfurther evidence suggesting that transient flaccidimmobility, either of a generalized or partialcharacter, may result from epileptic discharge inthe cerebral cortex.

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