ICTAL BEHAVIOR DISTURBANCE AS THE ONLY MANIFESTATION OF SEIZURE DISORDER: CASE REPORT ROBERT JAFFE, M.D? There is a certain group of patients in whom convulsive disorder is present, but who may show manifestations limited to the sphere of mental function. The manifesta- tions are characteristically episodic, but the duration of the episodes may vary from minutes to days. The degree and nature of the disturbance in thinking and general behavior is also extremely variable (10). At times the epileptic origin of the behavior disturbance is not immediately apparent, and electroencephalographic evidence, as well as clinical observation, is necessary that proper diagnosis and treatment may be achieved. The patient reported here was a 75-year- old woman who was first seen by one of the consulting psychiatrists of the Home for the Aged and Infirm Hebrews when she was referred for psychiatric evaluation, prior to her admission to the Home, because of “epi- sodes of memory loss” which had been re- ported by her family physician. After re- viewing her social history and personal history, his report goes as follows: ‘Patient remained in the family ail her life, took care of various sick members of her family and always felt that she was being a good person and thereby worthy of love herself because of this devotion. She now feels that she has done her duty and would like to be taken care of as a reward in the family here at the . Home. She is in good health and denies any major emotional symptoms. She tends to be somewhat tense and excitable, with very + Department of Psychiatry and Neurology, The Home for Aged and Infirm Hebrews of New York, 121 West 105th Street, New York, N. Y. The author wishes to express appreciation to Frederic D. Zeman, M.D., Chief of Medical Services, and to Alvin I. Goldfarb, M.D., Chief of Department of Psychiatry and Neurology at The Home for Aged and Infirm Hebrews of New York, for their assistance and cooperation. mild mood lability. The most striking path- ological manifestation involves her ‘spells.’ These have occurred on the average of once every year or two and have lasted for pe- roids ranging from a few hours to a day or two. She describes these spells as a reaction to feeling that she cannot take any more. For example, when her sick sister and sister- in-law would demand constant attention from her all day and all night and she would become increasingly tense and fa- tigued, things would finally come to a cli- max in a spell in which she would just go to bed and withdraw from everybody and everything. During these periods she pays no attention to people although she is aware of everything that is going on around her, no longer feels driven to do things, is no longer anxious, has no feelings at all. There is no memory loss during these periods, or disturbances of the level of consciousness. She has never acted in any disturbed or bizarre fashion during these periods and comes out of these without treatment. “Diagnosis: Passive-dependent person- ality. The patient’s “spells” certainly do not sound organic. I would characterize them as an emergency response to increased anxiety by a transient period of regressive withdrawal”. Two months later the writer first saw the patient when she was referred for neurologic examination because of an episode of altered responsivity which had occurred five days earlier. This episode was said to have oc- curred following the receipt of the news of the death of her sister-in-law. The patient stated that this was one of her usual “black- outs.” The only unusual feature was that it had lasted only a few hours, instead of its usual duration of one to two days. She denied having lost consciousness, stating 470 ICTAL BEHAVIOR DISTURBANCE that she had been aware of her surroundings at all times, but that she was unable to initiate any activity, or to speak. The in- cident had occurred in the morning, ap- parently beginning after she had dressed for the day’s activities. Her roommate noticed that she stopped speaking and moving about, and called the doctor. The staff doctor stated that the patient had been able to walk and move normally, but did not speak or respond to questions. Upon being fed, she proceeded to feed herself, but “seemed to be doing it automatically.” Upon questioning as to whether she had ever actually lost consciousness, the patient stated that she had done so on rare occa- sions. On one occasion during a “blackout” she fell out of her bed and injured her right shoulder. A detailed review of her history revealed that she had had her first attack approxi- mately 30 years earlier. She specifically re- called that this had occurred on washday, and that she had been preparing the wash. She knew that she had behaved in an ir- rational manner, since she had mixed the white and colored clothes together. At that time she lost consciousness and fell to the floor. She stated that she did not injure herself, and was not seen to have any con- vulsive movements. Her relatives picked her up and put her to bed, and she recovered consciousness in a few hours. Following this she had spells quite frequently, as often as once or twice a week. This condition per- sisted for a few months, following which the spells became less and less frequent. They ultimately decreased to an incidence of one every year or two. When these spells were occurring rarely, they were often as- sociated with emotional crises. Additional information was obtained from one of the members of the staff of the Home, who had been the patient’s next-door neighbor for many years. This staff member stated that the patient invariably had one of her attacks following the receipt of either very good or very bad news. The family 471 distinctly made this association, and had instructed this neighbor in how to handle the attacks if they occurred. The patient was said to invariably be found in a dazed and relatively unresponsive condition, but usually would be able to feed herself and respond to some degree. This dazed condi- tion sometimes lasted as many as four to six days. At the time of this examination the pa- tient’s mental status was normal, and the neurological examination was entirely nega- tive. On November 23, 1959, an EEG was per- formed, and revealed definite abnormalities. There were diffuse delta bursts, strongly accentuated in the left fronto-temporal re- gion, and left fronto-temporal focal spike activity. The patient was given phenobarbital 15 mg., and diphenylhydantoin 0.1 gm. t.i.d. She continued to have episodes of a similar nature, at irregular intervals. On January 10, 1960, the patient was invited to go downtown for lunch and to the theatre on January 13th. This was an un- usual and exciting event in her life. At 7:00 am. of January 12, she was found in her room in an unresponsive state. When first seen by the staff physician she turned her head in response to a greeting, but did not speak or obey commands. She did not feed herself, or chew food put in her mouth. She was brought to the 105th St. branch of the Home, from Kingsbridge House in the Bronx, by ambulance. When first seen in the EEG laboratory at 10:00 a.m., she was lying quietly on a stretcher, with her eyes open. There was no response to questions or com- mands. She did not respond to the applica- tion of needle electrodes. By 11:50 a.m, she still kept her eyes open but responded to some simple commands. She did not know right and left. Upon being asked her own name she stated, “I don’t know.” There was complete extinction of hand stimuli, on the Face-Hand test (3). EEG recording performed between 10:30 472 and 11:50 showed diffuse bilateral spike- wave activity, with slow waves at a fre- quency of 14% to 2 cps. The spike activity was generally bisynchronous but showed higher voltage on the left side in frontal and anterior temporal leads, and a fairly con- sistent presence of double spikes and multi- ple spikes in the same leads (Figure 1A). At 12 o’clock an intravenous injection of 5 per cent amobarbital sodium was started. After 150 mg. had been given, the EEG showed almost complete absence of the bi- synchronous spike and spike-wave activity, but persistence of high-voltage spikes at the left frontal electrode, with synchronous low voltage spikes at the left anterior temporal ROBERT JAFFE electrode. At this point she gave her name in answer to questioning. By twelve minutes after twelve, 250 mg. of amytal had been given, and the patient was very drowsy, had nystagmus and dys- arthria. With arousal she gave her name, and said she was at “1738 Andrews Ave” (her prior home address). At this time the EEG showed minimal diffuse slowing, con- sistent with her drowsy state, and low voltage spikes in the left frontal lead (Fig- ure 1B, C). By 12:16 the spike activity had again become diffuse and bisynchronous, with progressively increasing amplitude, until 12:36 (Figure 1D, E). At this time she RF ao in AYN t { RE aa yieyalic RF “aun rpg te LF -to RM -ra YAY LM -urd WAY WY, pe RE AW UWA) Hlryellmvalenfod LAT-LPT D 112/60 10:30 AM RE. RAT “yoann oP RAT-RF YUAN AM iy th A RP -RO LP -LO RPT-RE LF -Lo Na AMYTAL STARTED LF -LM AN ee LE =LM Wyte erpinteny may A YM RP -RO NAAM Nar AT aaa A en Asmat Na AMYTAL,225mg. etn ny eA AN p LP -Lo fe RPT-RE LPT-LE ined anton feta LPTHLE wanda enadteninged RMT-RAT et alee Aah RMT-RAT eer LMT~LAT toflvefla( feet hett LM TELAT mearnsii, V12/60 (NSN RF -LF Healy, RM -at wy yt a yy LF LAT et LM LMT bao 12:01PM 112/60 12:10PM toonv| ISEc, vaT-Le ANY Sly SRP -RE shan iti uur) RMT-RM ANIA aint LP LE al hy tonr ny tapng LM Lai ge ai Yada 1412/60 12:16 RAT-RPT sail ogi Sued LAT=LPT pelle afte 1712/60 12:35 Fic. 1. Tracings performed on first day of seizure, showing changes induced by intravenous administration of amobarbital sodium. ICTAL BEHAVIOR DISTURBANCE was in about the same mental state as prior to the barbiturate injection. She denied knowing her name, confused right and left, but obeyed some simple commands, She was also able to identify simple objects. The patient was then sent to a room, where she slept for a few hours, and then was able to cat a meal. On examination at 10:00 a.m. of January 13 she was much more alert, spoke slowly, but more rapidly than on the previous day. She knew that she was in an attack, said that it had started “yesterday morning.” On this day she greeted the writer by name, said that she recalled having come in an ambulance, recalled having seen me on the previous day but did not recognize me or know my name. She recalled on this morn- ing that she had been planning to go out for lunch and to the theatre. On examination of the mental status, she showed some delay in identifying the loca- tion. She was oriented for month and year, but not for day and date. She knew right and left, crossed the midline on command. She was unable to perform serial subtrac- tion of 7s, showed skipping and reversals on serial subtraction of 2s. She read the news- paper quite readily, but with poor compre- hension. The responses to Face-Hand test were normal. At this time the EEG showed long bursts and runs of bisynchronous spike and spike- wave activity, with the same characteris- tics as on the previous day. There were, however, occasional brief periods of normal activity (Figure 2A). The figures show that the spike activity is most marked at the left frontal electrode, and in some runs phase reversals at this electrode were dem- onstrated. A further attempt at localization was made by placing additional electrodes midway between the level of the nasion and the frontal electrode. In a transverse run, along a horizontal line from the right to the left mid-temporal electrode, it can be seen that the spike activity is of highest voltage, with phase reversal, about the left pre- 473 frontal electrode, less about the left anterior temporal electrode (Figure 2C). During the remainder of this day she behaved in what appeared to be a normal fashion, in the opinion of those attending her. On examination at nine o’clock on the morning of January 14, she was quite alert, she gave the day, date and place correctly and without hesitation. Calculation was much better, but still not perfect. The EEG at this time was perfectly normal. Although it is imposible to say without continuous EEG monitoring, it is my estimate that this episode lasted approximately 36 hours. Primidone 0.25 Gm. t.id. was added to the patient’s medication, and she had no further seizures during a 15-month period of observation. DISCUSSION Clinically, this case most closely resem- bles some which have been described in the literature under the name “petit mal status” (4, 6, 9). These patients have periods of altered consciousness lasting from hours to days. The alteration may be so mild as to constitute only a slight “mental slowing,” with complete ability to perform the per- son’s normal daily tasks, or may be so severe as to induce almost complete with- drawal and immobility. In a number of cases there has been continuous twitching of the eyelids, or hands, similar to that seen momentarily with petit mal lapses (9). All reported instances of petit mal status with EEGs performed during the attack, have shown almost continuous diffuse and sym- metrical spike-wave activity. The clinical manifestations in this type of condition, however, show marked varia- tion, and it is sometimes very difficult to make a specific diagnosis on clinical grounds alone. Cases of post-ictal confusion, and even pre-ictal mental slowing, may show features indistinguishable from some cases of petit mal status (5, 8). Marked similari- ties may also be seen in some eases of the 474 ROBERT JAFFE o natal | MUAY ype RF -RO { RM. RMT Wu Weal ratianiy omen /U/ WI! yin nr LM -LMT Weare wetness iy RP —RE Wrarranynunnnnnnoayoa alpina hanes LP KLE Naa auawanlna nny yy A hamish RAT-RPT Muflutndnentntintn ayn ny) /a/ a4 (ayers LAT-LPT Minoan olfalilindi lilioagannrw ray 1713 460 9:30AM B. C. RE -RATA\ heer talc tel RMT-RAT myn A ey RAT-RF Af att yo th aad fll RAT- _ppeN htt ad vt RF rR ene AN nt pPe-LeF tits LPFALE penta! LAT LMT Nil AA LF “La py ier 1713/60 Koiniininnled LAT-LE } RF -LF tad faeintinfy roomv|_ 1712/60 Fa. 2. Tracing performed on second day of seizure. B and C show the most pronounced focal spike activity, with phase reversal, at left prefrontal and anterior temporal electrodes. “Getal automatisms” seen with focal tempo- orientation and abstract thinking. Since ral lobe lesions (5, 10). the general behavior may appear normal, it The cases of verified petit mal status tend is often necesary to elicit these defects by to have some common features, the most specific testing of the mental status. These prominent of which are definite defects in “organic” deficits may coexist with, and ICTAL BEHAVIOR DISTURBANCE be partially masked by, disturbances in thinking which show elements suggesting conversion hysteria and even malingering (1, 6). More severely affected patients may suggest, in their behavior, catatonic stupor or depressive withdrawal. These cases must be differentiated from those described by Weil (13), Hill (7), and by Ervin et al. (2) in whom episodie disturbances of affect and mentation occurred in the presence of EEG abnormalities which were not diffuse or continuous. The transient improvement with associ- ated elimination of EEG abnormality, after injection of amobarbital sodium, was previ- ously described in a case of petit mal status by Bornstein et al. (1). The beneficial effect of intravenous barbiturates on obviously convulsive behavior is well known. The results of such injections in ictal behavior disturbances must be borne in mind, how- ever, in view of superficially similar effects which may be obtained in cases of catatonic stupor or hysterical fugue states. In many instances the final diagnostic distinction can only be made with the aid of an EEG. In the present case the EEG was particularly revealing. Although a fronto-temporal spike focus had been dem- onstrated during a seizure-free interval, one could not define the probable electro- physiological basis of her periods of altered consciousness on clinical grounds alone. The tracing during the episode, however, did show continuous diffuse bisynchronous spike-wave activity. This focally mduced bisynchronous activity was described by Tukel and Jasper (12) and called “second- ary bilateral synchrony.” Although the clin- ical data in their series did not include any case similar to the one presented here, the EEG findings are essentially similar. It seems probable that, as in their surgically verified cases, there is a focal epileptogenic lesion in the frontal parasagittal region, which under certain circumstances induces bisynchronous convulsive discharges in the 475 deeper structures, with concomitant gross behavior disturbances. In view of the absence of typical clinical evidence of petit mal lapses, and in the absence of bisynchronous 3/second spike- wave activity, it seems unjustifiable to make a definite diagnosis of “petit mal status” in this case, in spite of the striking clinical similarities. It seems more appro- priate to use a more inclusive term, such as ‘Setal twilight state” (10), since this case does not fall within any of the more spe- cifically defined categories. SUMMARY A case is presented of a 75-year-old woman with a 30-year history of infrequent two- to six-day episodes of withdrawn be- havior. One such episode was observed, with serial EEG tracings showing continuous spike-wave abnormalities, apparently aris- ing from a frontal parasagittal focus. The differentiation of ictal twilight states from other epileptic and non-epileptic epi- sodic behavior disturbances is discussed. REFERENCES 1. Bornstern, M., Connon, D. anp Sone, S. 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