a case report cinefilm, and - bmj

Journal ofNeurology, Neurosurgery, and Psychiatry, 1975, 38, 11-17

Ictal laughter: a case report with clinical, cinefilm,and EEG observations

FRANCES R. AMES AND OSCAR ENDERSTEIN

From the Department of Neurology, Groote Schuur Hospital, Capetown, South Africa

SYNOPSIS Seizures with laughter, verbalization, and repetitive abduction-adduction movementsof the upper limbs are described in a 13 year old male of normal intelligence. Cinefilm and EEGrecords illustrate some of the points made in the text.

It is just over 100 years since Trousseau (1873)first described laughter as an ictal phenomenon.The subject has recently been reviewed by severalauthors (Gumpert et al., 1970; Loiseau et al.,1971; Gascon and Lombroso, 1971; Swash,1972; and Chen and Forster, 1973). Rey-Pias(1972) states that there are 'today approximately120 reported cases although in almost one-halfof them there is not sufficient information tojustify their inclusion in this group'. Their rarityis emphasized by Chen and Forster (1973) who,in reviewing 5,000 consecutive cases of 'com-plex', and therefore selected epilepsies, found anincidence of only 0.20% of 'gelastic' epilepsy.The introduction of the term gelastic epilepsy byDaly and Mulder (1957) may have resulted inless precision in diagnosis. Mirth (gelos) doesnot necessarily include laughter. Smiling may bevoluntary or barely perceptible, whereas thelaryngeal and respiratory components oflaughterare more likely to be involuntary and are definite.Inappropriate laughter is often due to diffusecerebral disease. If, in addition, it is episodic andthe patient has amnesia for it, it is likely to beictal.We report a patient with ictal laughter, not

only because well-documented cases are un-common, but also because cinefilm recordingsduring seizures revealed unusual movements ofthe upper extremities; tape recording during theseizure confirmed the clinical observation ofstereotyped verbalization (the content of whichseemed to indicate remembrance of past attacks)and on two occasions electroencephalographic(EEG) recording coincided with ictal laughter.(Accepted 11 July 1974).

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CASE HISTORY

A white male of 13 years presented with unusual epi-sodic behaviour for two months. His teacher re-ported that on several occasions 'he cleared histhroat loudly in class'. This was interpreted as 'anattempt to gain attention', especially as his schoolwork had deteriorated during the preceding term.About a week later his mother noticed that, severaltimes a day, he would suddenly stare and 'be blank'for a few seconds, On many occasions this disturb-ance of consciousness would be accompanied by'clearing of the throat and laughing in an embarras-sed manner'. After 'laughing' he often said, 'Herewe go again', put his hands together and 'wentrigid'. These episodes lasted 10 to 15 seconds and hehad no recollection of them. At night his parentsoften heard him making the same noise in his sleep-'it sounds like a laugh and a whimper'. After one ofthese attacks in the early morning he fell out of bedand was found unconscious on the floor with his legsextended. After this he slept deeply for about anhour. At this stage his parents sought medical help,having dismissed the previous episodes as 'psycho-logical and perhaps related to masturbation'. Thepatient said that he himself could not see any con-nection between masturbation and his attacks butsuggested, 'I think the laughing attacks came aboutbecause I listened to a radio programme in which oneof the actors had a rather peculiar laugh. I thought itamusing and used to imitate the laugh when I wasfooling around. This laugh must have just "comeout" when I had an attack.'He was the youngest of six children. Birth and

developmental milestones were normal. For someyears he had occasionally complained of generalizedheadaches that were diagnosed as 'migraine'. He hada minor head injury without loss of consciousness a

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week before his attacks manifested themselves.There was no family history of epilepsy.

General and neurological examination was normal.He was right-handed. Verbal IQ was 134 and per-formance IQ 100. He appeared to have some im-pairment of short-term visual retention. Cerebro-spinal fluid was normal. Lumbar pneumoencephalo-gram was normal. Two brain scans (24 September1973 and 1 April 1974) were normal.

EEG The resting record showed a dominant fre-quency of 9 Hz. This was symmetrical and blockedwell. Occasional irregular sharp waves appeared inthe right frontal leads but the most striking abnormal-ity was episodic slow and sharp activity of increasedamplitude on the left especially in the anteriortemporal area (Fig. 1). This was accentuated byhyperventilation, which also provoked generalizedhigh amplitude slowing. This persisted during inter-mittent photic stimulation. On one occasion he had a

laughing attack during EEG recording. This lastedfive seconds and, during it, initial general de-synchronization was followed by generalized slowingmost prominent frontally (Fig. 2). On anotheroccasion, after a routine recording, the machine wasswitched off when he suddenly pushed back his chair,abducted his arms, and laughed for 60 seconds. Thelatter part of the attack was recorded and showedgeneralized slowing (2 to 3 Hz) of high amplitude(Fig. 3).On one occasion we cinefilmed and tape-recorded

attacks during an hour of vigorous hyperventilation.Two laughing attacks occurred, the one lasting 20and the other five seconds. A selected sequence of thefive second attack is depicted (Fig. 4). The patientstopped hyperventilating and began to make clonicexpiratory noises resembling laughter. This was fol-lowed by the clearly enunciated words, 'Here goes',and then a moan. As the noise began he flexed hiselbows so that his hands, which had been resting on

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his thighs, touched each other. He then began toabduct and adduct his arms proximally, successivelyincreasing the degree of abduction to about 45degrees. Simultaneously he flexed and extended hiswrists, turned his head to the left and his face becamepale. His eyes remained open and he smiled slightly,but the poverty of facial movement contrastedstrikingly with the vigour of the upper limb move-ments and the immobility of the lower limbs. He hadcomplete amnesia for the whole attack.During his stay in the ward the staff reported hear-

ing him laugh during the day or in his sleep on manyoccasions. He was discharged on diphenylhydantoin100 mg and diazepam 2 mg each three times daily.His parents and the school staff reported frequentattacks, during one of which he behaved 'auto-matically' for about 30 minutes. His school fellowsmocked him for being 'mad'. Carbamazepine 100 mgand diazepam 2 mg thrice daily did not improvehis condition but when he was placed on carbamaze-

pine 200 mg thrice daily and ethosuximide 250mg twice daily his attacks decreased in frequency andnow (eight months after their onset) his laughingattacks have disappeared but he still gets an occa-sional 'blank' lasting a few seconds. A recent EEGshowed generalized slowing with virtually con-tinuous 3-4 Hz slow and spike wave activity bi-frontally.

DISCUSSION

In addition to the laughter, the unusual clinicalfeatures in this patient are the type of verbaliza-tion and the repetitive abduction-adductionmovements of the upper extremities.As far as the laughing is concerned, the ex-

planation volunteered by him of his attentionhaving been arrested by a particular contrivedlaugh, his repeated imitation of it, and its later

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incorporation into the seizure pattern is notaltogether fanciful. It is certainly less fancifulthan the extraordinary suggestion that theattacks were related to masturbation. This latterview is advanced even by Martin (1950) who, indiscussing epileptic laughter, states 'Some ofthese cases have very peculiar features and inparticular in a number of them the attacks areaccompanied by "orgasmolepsy", a fact whichlends supports to the suggestion that the motorcentres for laughter and for certain sexual motoractivity may be neighbours'. No details of the'orgasmolepsy' are given, nor whether it occur-red more frequently in those patients whose ictallaughter and precocious puberty were related tohypothalamic pathology.

The verbalization was always a stereotypedrepetition of a sentence or part of the sentence'Here we go again'. This seems to indicate aremembrance of past attacks for which, however,he was completely amnesic. It suggests activationof speech and memory mechanisms and thusinvolvement of temporal lobe. Ictal verbalizationwas reported by Serafetinides and Falconer (1963)in 15 of 100 patients submitted to anteriortemporal lobectomy. 'Recurrent, irrelevant andemotional utterances' occurred in 11 patientswith right-sided lesions as opposed to four withleft-sided lesions. Schmidt and Wilder (1969)agree that speech automatisms, for which thepatient is amnesic, are more commonly oftemporal lobe origin but have no lateralizing

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significance and 'may sometimes accompanypetit mal automatisms' which they describe as' vocalization or crude speech'. 'Crude speech' isnot defined. Psychomotor automatisms are notonly rare but are notoriously difficult to separateclinically from partial seizures of temporal lobeorigin. Money and Hosta (1967) cite Foerster andGagel (1933) as evoking hilarity and speech in aconscious patient during craniotomy by stimula-ting the floor of the third ventricle. However,this type of response was not ictal but related toan induced emotional state.One patient with gelastic epilepsy in whom

verbalization resembled that of our patient, wasreported in a boy aged 11 years by Daly andMulder (1957). After one of his laughing attacks,he sighed deeply and said 'Oh dear'. At opera-tion a left temporo-occipital 'mixed oligo-ependymoma' was removed. Gascon and Lom-broso (1971) reported a 10 year old boy with arounded filling defect in the posterior portion ofthe third ventricle whose laughing attacks'would occasionally end with automaticallyrepeated sentences'. Unfortunately, the detailsare not given. In our patient, as well as these twocases, verbalization always followed the laughter.The repetitive abduction-adduction move-

ments that occurred as virtually an isolatedmotor phenomenon in our patient are not thosethat sometimes accompany hearty laughter.Indeed, in the only other patient with identicalmovements and ictal laughter that we have beenable to trace in the literature, the laughter andmovements occurred independently. Gascon andLombroso (1971) reported a 5 year old child witha hypothalamic astrocytoma. Preoperative seiz-ures took this form-

'the eyes rolled up, the head flexed, then the armsflexed at the elbows and beat symmetrically at theside like "butterfly wings". The seizure ended withthe head turned to the left with a strange twistedsmile. She maintained some degree of consciousnessduring these episodes, which occurred several timesdaily.' [After excision of the astrocytoma she had]'seizures that consisted of a giggle that was partiallya laugh and partially a cry. Occasionally there wasleft facial twitching but no other movements'.

The desynchronization and generalized slowingof the EEG while laughing is similar to that de-scribed by Gumpert et al. (1970), although otherauthors (Weil et al., 1958; Roger et al., 1967;

Chen and Forster, 1973) recorded focal temporalabnormalities in some of their cases.

Ictal laughter is generally regarded assymptomatic', but patients in whom localizedcerebral pathology has been demonstrated atoperation or necropsy are rare. The site ofpathology in these cases has either been temporallobe (Daly and Mulder, 1957; Loiseau et al.,1971); frontal lobe (Loiseau et al., 1971); orhypothalamus (Dott, 1938; Martin, 1950; Listet al., 1958; Money and Hosta, 1967; andGascon and Lombroso, 1971). However, in manyof these cases pathological changes were alsopresent in surrounding structures. Laughter is acomplex neurophysiological event and clearlyinvolves many neural structures. In most patientswith ictal laughter analysis of the seizure patterndoes not enable the clinician to localize a singlelesion. We experience this difficulty with ourpatient but feel that the absence of clinical signsof hypothalamic involvement, the normalpneumoencephalogram, the type of verbaliza-tion, the age of onset, and the initial interictalEEG may favour primary left temporal lobeinvolvement, although the laughter and move-ments clearly involve midline structures.

ADDENDUM

When last seen in September 1974, after etho-suximide 250 mg three times a day had beenadded to his medication, the patient reportedthat he had not had a 'laughing' attack for fivemonths but about twice a month he wouldexperience a 'feeling as though my head swimsand I remember an incident-it is always thesame but I cannot remember it afterwards. It islike a dream-it only lasts a few seconds'. HisEEG was normal apart from occasional episodicleft temporal abnormality.

REFERENCES

Chen, R.-C., and Forster, F. M. (1973). Cursive epilepsy andgelastic epilepsy. Neurology (Minneap.), 23, 1019-1029.

Daly, D. D., and Mulder, D. W. (1957). Gelastic epilepsy.Neurology (Minneap.), 7, 189-192.

Dott, N. M. (1938). Surgical aspects of the hypothalamus. InThe Hypothalamus, p. 179. Edited by W. F. Clark, Le G.Beattie, J. Riddoch, and N. M. Dott. Oliver and Boyd:Edinburgh.

Foerster, O., and Gagel, 0. (1933). Ein Fall von Ependymcystedes III. Ventrikels. Zeitschrift fiir die gesamte NeurologieundPsychiatrie, 149, 312-344.

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Ictal laughter: a case report with clinical, cinifilm, and EEG observations

Gascon, G. G., and Lombroso, C. T. (1971). Epileptic(gelastic) laughter. Epilepsia, 12, 63-76.

Gumpert, J., Hansotia, P., and Upton, A. (1970). Gelasticepilepsy. Journal of Neurology, Neurosurgery, and Psy-chiatry, 33, 479-483.

Ironside, R. (1956). Disorders of laughter due to brainlesions. Brain, 79, 589-609.

List, C. F., Dowman, C. E., Bagchi, B. K., and Bebin, J.(1958). Posterior hypothalamic hamartomas and ganglio-gliomas causing precocious puberty. Neurology (Minneap.),8, 164-174.

Loiseau, P., Cohadon, F., and Cohadon, S. (1971). Gelasticepilepsy. A review and report of five cases. Epilepsia, 12,313-323.

Martin, J. P. (1950). Fits of laughter (sham mirth) in organiccerebral disease. Brain, 73, 453-464.

Money, J., and Hosta, G. (1967). Laughing seizures withsexual precocity. Report of two cases. Johns HopkinsMedical Journal, 120, 326-336.

Rey-Pias, J. M. (1972). Gelastic epilepsy (laughing seizures).

Schweizer Archiv fur Neurologie, Neurochirurgie undPsychiatrie, 111, 29-35.

Roger, J., Lob, H., Waltregny, A., and Gastaut, H. (1967).Attacks of epileptic laughter; on 5 cases. Electroencephalo-graphy and Clinical Neurophysiology, 22, 279.

Serafetinides, E. A., and Falconer, M. A. (1963). Speechdisturbances in temporal lobe seizures: a study in 100epileptic patients submitted to anterior temporal lobec-tomy. Brain, 86, 333-346.

Schmidt, R. P., and Wilder, B. J. (1968). Epilepsy, pp. 14 and28. Davis: Philadelphia.

Swash, M. (1972). Released involuntary laughter aftertemporal lobe infarction. Journal of Neurology, Neuro-surgery, and Psychiatry, 35, 108-113.

Trousseau, A. (1873). Clinique Medicale de l'Hotel-Dieu deParis, 4th edn, p. 409. Bailliere: Paris.

Weil, A. A., Nosik, W. A., and Demmy, N. (1958). Electro-encephalographic correlation of laughing fits. AmericanJournal of Medical Science, 235, 301-308.

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