CASE OF MUSICOGENIC EPiLEPSY - BMJ · records were played (a Tosca aria and a noisy jazz record). The patient became tearful and emotional, but noattack wasinduced, norwasanychange

CASE OF MUSICOGENIC EPiLEPSYBY

DAVID SHAWSutton Emergency Hospital

and

DENIS HILLTeaching and Research Laboratory, Maudsley Hospital, London

(RECEIVED 13TH DECEMBER, 1946)

DEFINITE and unequivocal examples of musicogenicepilepsy (epilepsy induced by musical sounds) areclinical rarities. In the most comprehensive review,Macdonald Critchley (1937) described eleven casespersonally known to him and referred to a furthernine culled from the literature. Some of the latterwere by no means established, since only shortanecdotal accounts were given of them. The onsetof symptoms was noted as occurring later than isusual in idiopathic epilepsy. Further, many of thepatients displayed an unusual knowledge and a keenappreciation of classical music. The musicalstimulus might be of a specific kind, but the natureof the epileptic phenomena varied considerably;in some only minor attacks were induced, and inothers only major attacks. The importance of theemotional state was stressed by Critchley, partic-ularly in the period between first hearing the musicand the onset of the attack. Speculations aboutthe pathogenesis of this unusual form of epilepsywere made. It was accepted from the evidenceand description of these cases that hysteria is noexplanation, and an opinion was expressed that aconditioned reflex to certain musical sounds may bepresent. Critchley in 1942 briefly reported two fur-ther cases. In one an emotional disturbance set upby classical music was closely related to the actualseizure, and in the second-it was only certain notesor tones that induced an epileptiform fit. In thislatter case there was a history of closed head injurynine years previously. Taylor (1942) reported 'afurther case, in which a head injury occurred sixyears before the occurrence of occasional epilepti-form attacks. These attacks bore a close relationto music, and finally both major and minor attackswere readily induced by it.

Case ReportThe patient, a married woman of 44, of German origin,

came to this country when Hitler's succession to powerin 1932 seemed imminent. She comes of a middle-classfamily in Saxony. Her sister is reported to have died of"fits " as a child. The patient developed normally anddid very well at school, which she left at the age of 15.She then did clerical work, and later became a secretaryto a large German Film Company and travelled widely.She was cosmopolitan in outlook, and politically stronglydemocratic and hence bitterly opposed to the rapidlygrowing Nazi philosophy. In England she workedregularly as a secretary until just after the outbreakof the war. In 1935 she had a liaison with a marriedman, who died suddenly, and after she heard the newsshe had an amnesic and confused episode lasting severalhours, during which she wandered aimlessly around thestreets. She married some four years ago, her husbandserving for much of this time in the Army overseas. Sheis happy with her husband, who is very considerate andsympathetic. Her menses are normal and regular. Shehad an appendicectomy eleven years ago. There is nohistory of any convulsions in early life, but she had anervous breakdown in 1929. At this time she wasdepressed, but recovered after a rest in the country.There is no history of head injury. She is ordinarilyvivacious and energetic, prides herself on her efficiency,and is sociable and very popular, artistic, and sensitiveto " atmosphere." She seeks her own way and is ratherargumentative. She is very keen on good music, whichat any time will move her very much, and is herself amoderately good pianist.

In February, 1941, she had a period of automatismwhile speaking on the telephone. She had no recollectionof events during the attack and, in fact, put through thecall again five or ten minutes later and repeated hermessage. Similar momentary " blank attacks " recurredon the average of one every two or three weeks. Theyoccurred at any time, although much more frequentlyduring the day. On two or three occasions she was

107

Protected by copyright.

on January 22, 2021 by guest.http://jnnp.bm

j.com/

J Neurol N

eurosurg Psychiatry: first published as 10.1136/jnnp.10.3.107 on 1 A

ugust 1947. Dow

nloaded from

http://jnnp.bmj.com/

DAVID SHAW AND DENIS HILLincontinent of urine. Her memory, previously very igood, became unreliable; embarrassing mistakes anddifficulties in connexion with her secretarial workoccurred, and she herselfgave up thejob. The " blanks "continued. She became depressed, sleepless, andworried by the situation in which she found herself. Shewas treated with small doses of phenobarbitone andpsychotherapy, but with no relief to her symptoms. Sheobtained night work as a packer in a food factory, sinceher " blanks " were much less frequent at night. In acommunication to her friend and subsequent employer,she complained at this time about her bad memory andgrumbled about the incessant radio programmes at thefactory where she worked. In April, 1943, she was sentfor a period of convalescence at the Lady ChichesterHospital, Hove. There the connexion with music firstbecame apparent. A typical attack occurred when shewas in the hospital Chapel listening to the organ. Dr.F. Reitman observed that music induced " a dreamy statelasting about five minutes, followed by a severe general-ized headache." Two major attacks were induced bymusic. They commenced with conjugate deviation ofthe eyes and a turning of the body to the right; tonic andclonic phases followed, and, after a brief period ofunconsciousness, a transient confused state. Cornealreflexes were present but bilateral extensor responseswere noted.At this time the EEG was done on two occasions by

Dr. Cobb at Haywards Heath E.M.S. Hospital. Onthe first occasion the report was as follows:

"Resting: Low voltage record consisting of aboutequal proportions of 2 rhythms-I c/sec. and about20 c/sec. up to 25 microvolts. In addition there aremany slower waves, single and in short bursts at about6 c/sec. of about the same voltage. On overbreathing(3 minutes) there is a slight increase in the number andamplitude of the slow waves, persisting for some time."Sedatives were discontinued and she "enjoyed"

music, and after fourteen days a major attack occurred.A second EEG was done and reported on as follows:

" Dominant rhythm at 12 c/sec., rather irregular andnot very persistent, voltage up to- 50 microvolts andblocked by eye opening. There were other rhythmspresent. (1) Continuous 20 c/sec. rhythm, mostly oflow voltage, but occasionally comparable with that ofdominant rhythm. (2) Much 4-6 c/sec. at about sameamplitude as dominant rhythm. (3) One short burstof high voltage 3 c/sec. waves. On overbreathing (3minutes) there was a great increase in slow activitvpersisting for a considerable time and including severalshort bursts of high voltage 3 c/sec. wave and spikecomplexes."The conclusion was that the response to overbreathing

was now quite typically epileptic.She was transferred to Sutton E.M.S. Hospital for

further investigation in August, 1943, coming under thecare of one of us (D.S.). Physical examination showedher to be a well-nourished, heavily-built woman. Bloodpressure was 110!70 mm./Hg. No -significant abnorm-ality was elicited in the centra*l nervous or other svstems.Cerebrospinal fluid pressure was 240 mm. C.S.F. and the

Queckenstedt test was qualitatively normal. TheWassermann reaction was negative in blood and cerebro-spinal fluid. A radiograph of the skull showed noabnormality. During an EEG examination gramophonerecords were played (a Tosca aria and a noisy jazzrecord). The patient became tearful and emotional,but no attack was induced, nor was any change noted inher EEG. Two brief petit-mal attacks were observedwhile she was in hospital. They were not related tomusic. She left hospital after a month and continued toattend the Maudsley Hospital as an out patient. Shemanaged to carry on in a secretarial job as an employeeof a former acquaintance and fellow-countryman, butboth minor and major turns persisted and were nowbecoming more easily " fired off" by musical stimuli.They occurred by day and by night, and in the case ofthe latter were often connected with a dream aboutnmusic. Life was now becoming very difficult for thepatient. She avoided music, as she said, " like theplague," and the situation had reached absurd lengths.If they went out together her husband would make apreliminary reconnaissance to find a cafe witbout awireless or gramophone, and even the street musiciansnear her place of work were bribed by her employerto keep away from the vicinity. She had been treatedduring this period as an out patient with Caps." epanutin," gr. 14 b.d. and later t.d.s., and Tab.phenobarbitone, gr. 1 daily, without any demonstrableeffect on the frequency of the seizures. During thisperiod she put on nearly 3 stone in weight, reaching 15stone, and was treated with Tab. thyroideum siccum gr.14 b.d.She was readmitted to Sutton E.M.S. Hospital on

Jan. 28, 1946. She was now voluble and rather moretearful and emotionally unstable than formerly. Alwaysco-operative, she would nevertheless quickly get put outand become irritable. She demanded that all allowancesbe made for her susceptibility to music. There was noevidence of a general intellectual deterioration, but testsshowed an impairment of retentive memory. She wasintensely self-centred. Firstly she worried over herfailing memory and the consequent difficulties at work,and secondly, her German origin-which she did herbest to hide-was a source of considerable distress to her.She was afraid of being considered a Nazi sympathizerin view of her Aryan descent. On the other hand, sheexpressed fears that Nazi organizations in London wouldforce her to join their ranks under the threat of harmingher relatives in Germany. The fate of her parents wascausing her considerable anxiety. During the heightof the 1940-1941 blitz on London she had been at firstfortified by music. She had made a practice of puttingon her radio as loud as possible to drown the noise ofthe air-raids. But it now seemed to her, that music was"distorted " in some way and invariably inducedunpleasant emotions.Minor attacks were now almost invariably induced by

music. In these she suddenly felt " dead," and regainedconsciousness feeling drowsy and muddled for abouthalf-an-hour. These attacks were frequently followedby a period of automatism lasting from twenty minutesto an hour, and in these episodes, of which she had no

108



j.com/

J Neurol N


ugust 1947. Dow

nloaded from


MUSICOGENIC EPILEPSY 109

-000~~~~ ~ ~ ~ ~ ~ ~ ~ ~ ~ 0

0. 0~~~~~~~~~~~~~~~~~~~~~~~~~~.>

(0

Ca 0 ci

'.4

000

o~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~

0.0~~~~~~~~~~~~~~~~~~~~~

LU~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~C CZ

0) 00'~~~~r

E~~~~~~~C

E 0



j.com/

J Neurol N


ugust 1947. Dow

nloaded from


110 DAVID SHAW AND DENIS HILL

recollection, she was reported to have pulled back the room. There was no recollection for any of the con-blackout curtains on one occasion, and on another to versation during the period just before the fit.have wandered off from her office in'a dazed state. ,She A series of electro-encephalographic investigationsstated she had even done some shopping or eaten her meal were carried out at the Maudsley Hospital. With thein her usual caf6 with no memory at all of having done so. patient lying at rest, records were taken with a Grass 3Characteristically, there was no warning of the attacks, channel apparatus. Bipolar leads were used. Thewhich averaged three or four a month. . alpha rhythm was of low voltage, irregular in form andThe major attacks occurred less frequently, one in varied in frequency from 10-13 c/sec.; the distribution

every two or three months, and were also usually induced was symmetrical, the rhythm being seen best in the lowerby music, but not invariably. They occurred both by part of the hemispheres with antero-posteriorly placednight and by day, without aura. She had bitten her bipolar leads, but better still with transverse electrodetongue and micturated during the attacks on several positions. Blocking to visual stimuli was normal.occasions. - Continuous medium voltage fast activity at 20-22 c/sec.Again no significant abnormality in the central nervous was seen in the fronto-central regions. With all trans-:

system was elicited on clinical examination, but the blood verse electrode placements irregular moderate voltagepressure was now 160/110 mm./Hg.' The urine was normal. (50-70 ,uv.) bilaterally synchronous-rhythmic potentialsOn lumbar puncture the cerebrospinal fluid pressure was were seen. Among these, three rhythms could be200 mm. C.S.F., and the C.S.F. was qualitatively normal. identified having slightly different amplitudes, viz.,Radiographs of the skull showed no bony abnormality, (1) 3 c/sec. at 60-70 [tv.-irregular complex wave formsand when further radiographs were taken following air in bursts of 2-3 waves, coming chiefly from the postreplacement by the cisternal route by Mr. McKissock, central areas, (2) 5-6 c/sec. potentials at 40-50 Iv-. havingno distortion'ofthe ventricular system was found. Later, relatively sinusoidal appearance in the lower central areasfollowing the injection of an opaque substance into both (tfieta rhythm), and (3) 4 c/sec. potentials at 50,60 ,Lv.carotid arteries, x-ray pictures of the head revealed a rather irregular in form, tending to lateralize to the leftnormal vascular appearance. Aural investigations by side in the anterior temporal region. No focal locationMiss E. Whetnall revealed no abnormality in the ears. for the potentials of this discharge could, however, beThe hearing tested by the audiogram was normal, and no obtained with any constancy.subjective or objective response occurred to any tones The EEG was then recorded while music was played.except to 512 c/min., which, when varied in intensity and No significant change in the rhythms was seen before theiniterrupted, was unpleasant to the patient. She became onset of the fit. During this phase, which lasted fromemotional but no fit ensued. She was similarly irritated three to five minutes, signs of emotion, however, wereby the Barany noise box. When the patient watched a evident. The onset of the fit on each of four occasionsrevolving drum the direction of which was reversed, was heralded by an abrupt cessation of respiration,the optokinetic nystagmus obtained to the left side was coincident with tonic, generalized muscular contractiongreater than that obtained' to the right. This finding which was evident in the EEG record as fast irregularsuggested a central lesion on the left side. activity, conducted probably from neck muscles. The

Attacks Deliberately Induced by Music.-Throughout muscle contraction was not sufficient to result in sig-her stay in hospital no spontaneous seizures occurred, nificant change of posture, although slight flexion of thebut attacks were successfully induced by music on each head and trunk was evident on one occasion. Dunrngof the nine occasions attempted. Various kinds of the initial few seconds automatic rotatory movements of-music, from a musical comedy song to a Wagnerian the right arm were noticed, with pouting of the pursedOverture, have all produced varying degrees of emotional lips. Six to eight seconds after the cessation ofbreathing,agitation and tearfulness followed by a fit. The pattern low voltage (30-40 uv.) 6-7 c/sec. potentials appearedof the fit was repeated on subsequent occasions and the synchronously and with equal amplitude on the twovesical incontinence occurred twice. hemispheres. During the succeeding 72-112 seconds,On hearing music she became agitated immediately, until respiration recommenced, the potentials showed a

and talked rapidly, repeating various inconsequential steadily progressive increase of amplitude, with progres-comments in an effort apparently to distract her attention. sive decline in frequency, and finally developed aShe seemed to talk for the sake of talking. After ten rhythmical high voltage (150 uv.) 2J-3 c/sec. bilaterallyminutes her right hand began to make pawing movements synchronous discharge. When respiration was re--which widened in range. Then head and shoulders established, this discharge lost its rhythmical sinusoidalflexed, and her right hand slowly went up to her nose appearance, the waves becoming irregular in outline,which she gripped firmly. She stiffened in a tonic spasm, slower (1-2 c/sec.), and of lower voltage (Fig. 1). Apursed her lips markedly, held her breath and became gradual return to consciousness was now evident, thecyanotic, staring ahead with fixed congested eyes. This patient behaving in a dazed manner for which there wasphase lasted altogether from two to three minutes. No subsequent amnesia. This period lasted variously fromabnormal neurological signs were elicited. She then got ten to twenty minutes.up from her chair, fumbled about in a confused way, While music was played the patient showed overt signsmuttering and evidently looking for her handbag. of emotion. Fig. 2 is a record of the respiratoryContact -was gradually re-established in spite of the excursions, recorded on the drum of a Sandborn B.M.R.continuing music. She c-omplained of headache and apparatus from -which the lime had been removed.asked repeatedly how she had managed to get into the Fig. 3 demonstrates the changes of heart rate during the



j.com/

J Neurol N


ugust 1947. Dow

nloaded from


111MUSICOGENIC EPILEPSY

111ll.

F-

I. 1JI

RESPIRATION DURING MUSIC

I 1111

h ii FIT

Ill- . I112 MINUTES

FIG. 2.-Record of the respiratory excursions recorded on the drum of a SandbornB.M.R. apparatus from which the lime had been removed.

I

LII' I

I i t

w



j.com/

J Neurol N


ugust 1947. Dow

nloaded from


112DAVID SHAW AND DENIS HILL:DAVID SHAW AND

UW)m-

o 2 3 4M I N UTES

U-U-0

D 0 0E: E ( ui

-122 ::z

_115 X7S107U6

uJ

4

wI I I t---I 183 15- 6 7 8

FIG. 3.-Shows changes in heart rate during the pre-fit period of musical

2 CO2

stimulation.

07 Dzzcr

CZ

w

w75 r

MINUTESFIG. 4.-Similar record of the heart rate changes during a period of musical stimulation, while the

patient breathed oxygen containing 5 per cent. carbon dioxide.

DENIS HILL112



j.com/

J Neurol N


ugust 1947. Dow

nloaded from


MUSICOGENIC EPILEPSY

w 115

z 107

cr

L- 94_

CYi_ 83Lr

w

I

75

180

170

160

150

140

130 ,I

120 EE

110

100

90

80

TIME-0 70,{

TIME-+2 V miinn.s.6A080 SECS

0-0-SYSTOLIC -ECG INTERVALS*-w DIASTOLIC

FIG. 5.-Composite record of the heart rate and blood-pressure changes (taken by sphygmomanometerfrom the arm) during a further investigation.

DORYL 0-375 mg s.c.

180

170

160

150

140

130CJI

120EE

110

100

90

80

7C)

20 min. after inj: APNOEA 6087 SECS

FIG. 6.-Heart rate and blood-pressure changes after injection of I c.cm. of " doryl "intravenously.

113

i-F



j.com/

J Neurol N


ugust 1947. Dow

nloaded from


114DAVIDSHAWANDDENI Lt..

DA--VID - SHAW:AND DE:NIS'HILL

OBR-AIR

.F(n

(I0-Lf-on)

3 4

1-

z5-

107 cmLLU0-UJ

94 t

w83 Er

M I N U T E SFIG. 7.-Record of the heart rate taken during a period of three minutes' voluntary overbreathing.

pre-fit period of musical stimulation. This was obtainedby recording the arm-to-arm electrocardiogram on onechannel of the Grass equipment (the other two channelsrecording the EEG). With a constant paper speed,variations in the distance between five successive R-wavesof the electrocardiogram, measured in millimetres, givean accurate account of the heart rate. Fig. 4 is a similarrecord of the heart-rate changes during a period of musicstimulation, while the patient breathed oxygen containing5 per cent. carbon dioxide. Fig. 5 is a composite recordof the heart-rate and blood-pressure changes (taken bysphygmomanometer from the arm) during a furtherinvestigation. Fig. 7 is a record of the heart rate takenduring a period of three minutes' voluntary overbreathing.Thee investigations demonstrate increased depth andfrequency of respiration, significant increase of heartrate, and a rise in systolic and diastolic blood pressurewhile music was played. A further increase of heartrate and blood pressure is observed at the onset of the fit.Fig. 4 illustrates that breathing 5 per cent. carbondioxide facilitated the increase of heart rate and did notprevent the silbsequent fit. In this case, therefore, themechanism of fit precipitation was not alkalosis fromoverbreathing. It should also be recorded that voluntaryoverbreathing of much greater depth for three minuteshad not induced a fit, nor equivalent increase ofheart rate.

In view of the increased heart rate, blood pressure, andrespiration rate during the playing of music, it wasdecided to observe the effects of a drug having an acetyl-choline-like action. " Doryl," 1 c.cm., was injectedintramuscularly and the procedure repeated. This didnot prevent the devetopment of a fit, but in the opinionof observers the objective signs of emotion were less

evident while music was played. This observation gainssome support from the heart rate and blood-presstLrechanges, both of which were less on this occasion(Fig. 6).

DiscussionIt seems clear that we are dealing with a case of

epilepsy. For the following reasons the suggestionthat the musicogenic phenomena are a manifestationof hysteria can be disposed of, at any rate as far asthis case is concerned. There is a family historyof epilepsy. Urinary incontinence has occurred inthe attacks on several occasions, and the fitsobserved have frequently been followed by a periodof automatism. Major attacks have also beenreliably witnessed and have been accompanied bytongue-biting and urinary Incontinence. Further,the EEG examination made in 1943 showed thepresence on overbreathing of the wave and spikecomplex discharge, and this would suggest anidiopathic aetiology. However, minor fits beganat the age of41 and at first bore no relation to music.As Critchley observed in his cases, the time of onsetin this patient was later than is usual in cases ofidiopatiic epilepsy. Like the cases reported, fu4lneurological investigations have failed to demon-strate a lesion in our patient. However, theevidence suggests a local onset for the fit in this case.In the deliberately induced- fits it was repeatedlynoticed that the moyement first began in Jacksonian

114



j.com/

J Neurol N


ugust 1947. Dow

nloaded from



fashion in the right- hand and arm, with adversiveturning of the head and eyes to the right. Thissuggests a focal epileptogenic area in the left hemi-sphere, probably the left temporal lobe. This isfurther. supported by the results of the optokinetictest, and to a certain extent by the EEG. But ofthe nature of the focus we have no knowledge.The air studies and arteriogram were normal.

Psychological examination showed that, althoughsuperficially well adjusted, the patient was neurotic.There is an earlier history of a nervous breakdowninduced by stress, and she has shown a tendencyto react explosively to emotional stimuli; shebecame amnesic and confused after hearing of herlover's sudden death. Was this an earlier mani-festation of epilepsy ? In general, the musicalstimulus is of a non-specific kind, although it istrue that a pure tone of 512 c/min. when variedproduced an emotional response, but no fit. Fitsare induced by any kind of music,' seemingly. Thepatient has always been keenly appreciative ofmusic (perhaps over-emotional to it), and onlylatterly has found that musical sounds are distortedand produce an unpleasant emotional response.Critchley has discussed two different views of, thepathogenesis of musicogenic epilepsy-the physio-genic and the psychogenic. In the first it is sup-posed that music itself induces the fit by causingincreased cerebral blood flow. Records taken ofthe heart rate and blood pressure during the stimu-lation period and also throughout the fit showthat there is a marked adrenaline-like responsecommencing immediately the stimulation is givenand continuing throughout the initial stages ofthe seizure. It has also been suggested thatthe hearing of music might induce - overbreath-ing and thus precipitate a fit by alkalosisin a latent epileptic. Records of respiration doindeed show overbreathing, and of the typecommonly associated with emotion, that is, ofgreater depth and increased frequency, and withirregularity of both depth and frequency. How-ever, voluntary overbreathing for three minutesproduced less increase of the heart rate than whenlistening to music at rest, and no fit was ever pro-duced by this procedure. Further, when thepatient breathed 5 per cent. carbon dioxide whilelistening to music a fit supervened in the usual way.The possibility that alkalosis from overbreathingis the cause of the fit in this case can, therefore, beexcluded.The heart rate and blood-pressure changes are of

particular interest in a case of musicogenic epilepsybecause of the association of such changes withaudiogenic fits in rats (Lindsley aAd others, 1942).

The signs of emotion, restlessness, lacrimation, andoverbreathing were invariably observed in thispatient when she listened to music, and this associa-tion has been emphasized in other cases (Critchley,1937). On the four occasions when records weremade, similar heart rate and EEG changes wereobserved, indicating a temporal sequence of events.When the musical stimulus was given, and some-times during the brief anticipatory period precedingit, a sharp rise in heart rate and systolic blood-pressure occurred. After thirty or forty secondsthe heart rate and blood pressure ceased to increaseand remained steady, the normal variations ofheart rate being less evident.The type of EEG discharge during the fit is that

commonly seen in epileptic psychomotor seizures(Gibbs and others, 1936; Jasper, 1941) and theinitial frequency of the discharge, together-with itsbilaterally synchronous occurrence, is in favour ofa basal origin for the cerebral dysfunction (Jasper,1941; Cobb, 1944; Walter and Dovey, -1944).Tlhe progressive facilitation of the dischargeindicates a focal 'origin, recruiting progressivelymore and more neurone groups.

It is relevant to consider the similarities anddifferences which this case shows with the audiogenicfits in rats investigated by many workers (Maier,1939; Morgan and Morgan, 1939; Maier andGlaser, 1940; Lindsley and others, 1942). Incertain strains of rats, though not carried as a simplemendelian dominant, a susceptibility to abnormalbehaviour culminating in convulsions is found asa response to high-pitched sounds (whistle, key-jingling, etc.). The abnormal behaviour in responseto sound stimuli shows two phases, bearing resem-blance to the case reported here. In the first phase,seen as violent, undirected running and jumpingending in tonic-clonic convulsions, there are markedchanges in heart rate and blood pressure, usuallyan adrenaline-like effect (Lindsley and others, 1942).The second phase is one of inactivity, the animalbeing comatose, showing no response to sensorystimulation. The reflexes and righting responsesare absent and the animal can be moulded intovarious positions. There is a gradual return tonormal. During this phase the heart rate fallssharply and then gradually returns to normal.Lindsley and others (1942) found that, if their ratswere restrained, no. fits occurred. In the unre-strained rats, the heart rate usually showed a sharpincrease of rate of considerable magnitude andusually a decrease just before the attack. In othercases there was an initial fall in the heart rate. Inour patient increase in the heart rate was invariable.The temporal sequence of events in the audiogenic

115



j.com/

J Neurol N


ugust 1947. Dow

nloaded from


1DAVID SHAW AND DENIS HILL

seizure is emphasized by these writers. Thisconsists of (1) increase of heart rate, (2) a markedelectrocortical discharge in the EEG at the onsetof the attack. In the present case the EEGcharacters are clearly different from those in the rats,though it is doubtful whether the two may be com-pared. In the rat the discharge-slow waves andmixed spikes and waves-is maximal at the onset ofthe fit and does not show the progressive build-upevident in our patient.

Lindsley and others (1942) found that physicalrestraint prevented the occurrence of seizures intheir rats. Partial curarization had a similar-effect.Following vagotomy the seizures were allo preventedand no heart-rate changes occurred to sound stimuli.Since in the present patient the heart rate and bloodpress§1re preceding the fit invariably showed an

adrenaline-like response, an attempt to balancethis with a drug having an acetylcholine-like actionwas made. Doryl injected intramuscularly resultedin less heart rate and blood-pressure change tomusic, and the overt signs of emotion-as judgedby observers-were less during the stimulationphase, but the fit was not prevented. If the resultof this experiment is considered significant, it maybe regarded a's evidence that the peripheral or

cerebral vascular effects of emotional over-activityare not of major importance in the production offits in this patient.The earlier workers on audiogenic seizures in

rats had likened the phenomena to the " experi-mental neurosis." Maier (1939) induced fits by a

conflict situation in which the rats were forced torespond to, a physical stimulus which they hadpreviously been conditioned to avoid. Maier andGlaser (1940) found that " barriers to response"to stimuli increased the frequency and susceptibilityto fits. On the other hand, Lindsley and others(1942) prevented fits by restraining their rats. Inthe rats who were not restrained but who did notdevelop fits during auditory stimulation, some formof " substitute behaviour " was noticed, such as

nose or ear rubbing and teeth cha,ttering. It was

observed that our patient would talk rapidly andbecome agitated when music was played. Shewould repeat various inconsequential comments inan effort apparently to distract her attention.However, she could not prevent the fit by thesemeans. It has been noted already that Critchley(1937) considered the possibility that a conditionedreflex to musical sounds might have been createdin these cases. In our patient's case, fits, bothmajor and minor, had occurred before any relationto musical stimuli became apparent, and the evidencesuggests that she is an idiopathic epileptic. The

relationship to musical precipitation started duringthe London blitz, when no doubt emotional stresswas associated with hearing music. After this theassociation with fit precipitation became progres-sively more complete. In the case of the rats,Lindsley and others (1942) conclude from their datathat auditory stimulation rather than conflictneurotic reaction is the chief component in theproduction of seizures, and these workers suggestthat the auditory'stimulus excites autonomic centresin the diencephalon by direct spread. In theparticular case reported here, there is much tosuggest a conditioned process. Such a conditionedreflex cannot, however, be explained as a simplepattern involving the acoustic nuclei and the dien-cephalon. Masserman (1943) in an extensivereview of his own and others' experiments, concludes'that the hypothalamus behaves not as an afferentexperience-mediating organ, but as a way-stationon efferent sympathetic and motor pathways, whichcannot be conditioned by direct stimulation (Gantt,1937; Gantt and Loucks, 1938). Attempts tocondition "hypothalamic responses " to soundstimuli in lightly etherized animals failed, and suchattempts left no change in the action potentialsfrom the hypothalamus. The establishment of aconditioned response is dependent upon the" meaning." value of the stimulus, and from this itmay be inferred that the function of the cerebralcortex is involved. This is supported by the factthat no fits could be produced by exposing thepatient to pure tones, although she showed signsof emotion with 512. The dependence on harmonicsounds would suggest a cortical receptor level andthe difference between " music" and pure tonesfor, experience is one largely of "meaning." Thefit pattern, the optokinetic responses, and theresting EEG pattern all suggest an epileptogeniczone in the left temporal lobe. The fits bearresemblance to the " dreamy states " seen in thetemporal lobe epilepsy. However Beach andWeaver (1943) found that in the rat an intact cortexis not essential to the production of the fits. Infact, almost complete decortication increased thesusceptibility to fits and the intensity and durationof them.Although there are similarities in the temporal

course of events between the epilepsy of this patientand the 'characteristic audiogenic seizures of rats,it would be unsafe to argue from the case of thelatter as to site of origin of the epileptic dischargein the human. Against the clinical evidenceindicating a focal discharge arising in the lefttemporal cortex is the evidence that no new patternwas observed in-the EEG until six or eight seconds

116



j.com/

J Neurol N


ugust 1947. Dow

nloaded from



after the start of the fit, and the fact that when suchchanges appeared they were equal and synchronouson the two hemispheres. It is possible that theelectrodes were placed too far away from the siteof origin of the discharge, with the result that bythe time it reached them the rhythm had developedbilateral synchrony. This is known to occur intemnporal-lobe discharges (Jasper, 1941). On theother hand there is evidence (Walter and Dovey,1944; Cobb, 1944) that rhythms with the frequencyseen in their case, having bilateral synchrony, arefound in lesions in the neighbourhood of the thirdventricle. The extremely slow " build up-" of therhythms during the fit demonstrates that thedischarge is progressing in the brain very slowly.It seems reasonable to suggest that the site ofongin of the discharge in this patient is deep in theleft temporal lobe, that it spreads predominantlytowards the basal brain, and that it only involvesthe cortex at a later stage in the development ofthe fit.

Summary1. The development of a specific musical

stimulus to fit production is described in an epi-leptic woman.

2. Extensive clinical investigations failed toreveal an acquired cerebral lesion, although the fitpattern, optokinetic response, and inter-seizureEEG indicate an epileptogenic area in the lefttemporal lpbe.

3. Increases of blood pressure, heart rate, andrespiration while listening to music were observedin this patient. These were associated with overtsigns of emotion.

4. Fits could not be induced by pure tones,although the patient felt emotional to a tone of 512,

which persisted and was varied in loudness.Different kinds of music were invariably followedby a fit within five minutes.

5. Electroencephalographic studies showed aconstant pattern for the development of the fit. Nochange of rhythm was seen while music was playedbefore the fit began. The cortical discharge begansix or seven seconds after the clinical onset. Thisdischarge consisted of 6 c/sec. low voltage potentialshaving bilateral synchrony, steadily increasing involtage and falling progressively in frequency toreach 22-3 c/sec.

6. The possible mechanisms of fit productionare discussed.

REFERENCESBeach, F. A., and Weaver, T. (1943). J. comp. Neurol.,

79, 379.Cobb, W. A. (1944). J. Neurol., Neurosurg. Psychiat.,

7, 96.Critchlcy, Macdonald, (1937). Brain, 60, 13.--(1942). J. roy. Nav. Med. Serv., 28, 182.

Gantt, W. H. (1937). Amer. J. Physiol., 119, 313.-and Loucks, R. B. (1938). Ibid., 123, 74.Gibbs, F. A., Lennox, W. G., and Gibbs, E. L. (1936).

Arch. Neurol. Psychiat. Chicago, 36, 1225.Jasper, H. H. (1941). In W. Penfield and T. C. Erickson:

"'Epilepsy and Cerebral Localization." Springfield.C. Thomas. p. 380.

Lindsley, D. B., Finger, F. W., and Henry, C. E. (1942).J. Neurophysiol., 5, 185.

Maier, N. R. F. (1939). "Studies of AbnormalBehaviour in the Rat." New York. Harper Bros.

-and Glaser, N. M. (1940). Comp. Psychol. Monogr./16, 1.

Masserman, J. (1943). " Behaviour and Neurosis."Chicago. Univ. Press.

Morgan, C. T., and Morgan, J. D. (1939). J. Comp.Ps~vchol., 27, 505.

Taylor, S. (1942). J. roy. Nav. Med. Serv., 28, 394.Walter, W. G., and Dovey, V. J. (1944). J. Neurol.,

Neurosurg., Psychiat., 7, 57.

117P

rotected by copyright. on January 22, 2021 by guest.

http://jnnp.bmj.com

/J N

eurol Neurosurg P

sychiatry: first published as 10.1136/jnnp.10.3.107 on 1 August 1947. D

ownloaded from


Documents

CASE OF MUSICOGENIC EPiLEPSY - BMJ · records were played (a Tosca aria and a noisy jazz record). The patient became tearful and emotional, but noattack wasinduced, norwasanychange