Complex Partial Status Epilepticus Presenting as Gelastic Seizures: A Case Report Jaga Nath Glassman, M.D. zyxwvutsrqponmlkjihgfedcbaZYXWVUTSRQPONMLKJIHGFEDCBA Resident in Psychiatry, Brown University Affiliated Hospitals Donna Dryer, B.S. Brown University Medical School, Providence, Rhode Island James R. McCartney, M.D. Psychiatrist-in-Chief and Assistant Professor, Department of Psychiatry, The Miriam Hospital, Providence, Rhode Island zyxwvutsrqponmlkjihgfedcbaZYXWVUTSRQPONMLKJIHGFEDCBA Abstract: A middle-aged man, who presented to the emergency increasing interest in complex partial status epilep- room because of bizarre outbursts of laughter, was found to be in ticus [l-4]. There can be a broad spectrum of partial complex status epilepticus. His seizure disorder had been clinical behavior that may mimic psychotic behavmisdiagnosed, at various times, as a variety of “ ‘ functional” ior. psychiatric disorders. Despite proper diagnosis and aggressive These behavioral manifestations must be distreatment, management was difficult, being complicated by tinguished from those of other diagnostic entities. postictal agitation and confusion, postictal psychosis, and interDissociative disorders such as prolonged psychoictal compulsive and paranoid personality features. This case is genic fugues, derealization, and depersonalization described, and issues of diagnosis and management in partial can present with parallel symptoms, as can hyscomplex epilepsy are briefly discussed. The importance of not terical pseudoseizures or even malingering. The overlooking organic and especially epileptic factors, despite the behavior in complex partial seizures is more sterpresence of prior psychiatric illness, psychologic contributors, eotypic and repetitive than in these conditions. and environmental stressors, is emphasized. zyxwvutsrqponmlkjihgfedcbaZYXWVUTSRQPONMLKJIHGFEDCBA A case of partial complex status epilepticus, presenting as gelastic seizures, illustrated quite vividly some important clinical features of complex partial epilepsy. Complex partial status epilepticus is often difficult to diagnose. It can be mistaken for a variety of other psychiatric illnesses. The patient to be described had been diagnosed at various times as suffering from a Bipolar Affective Disorder-manic phase, brief, Reactive Psychosis, and Conversion Disorder, as well as a paranoid personality disorder. In addition, the difficulties in the clinical management of the varied features of both complex partial status and complex partial epilepsy in general were well illustrated in this case. There has been General Hospital Psychiatry 8,614X 1986 0 1986 Elsevier Science Publishing Co., Inc. 52 Vanderbilt Avenue, New York, NY 10017 One type of bizarre behavior rarely seen is epileptic laughter (gelastic epilepsy). Gelastic episodes have been known to accompany the automatisms, fugues, confusion, and psychotic behavior observed during complex partial status [5-71. Case Report: M r. B M r. B is a 55-year-old, right-handed, married, Caucasian male, currently on disability. He presented initially in the emergency room late in the afternoon on 5/l/84, brought by his wife because of strange outbursts of raucous laughter. These outbursts, very disturbing to his wife and daughter, seemed unrelated to any external stimuli. The first such laughing episode had occurred earlier that day while the patient was attending an art class at a local college. For no apparent 61 ISSN 0163~8343/86/$3.50 J. N. Glassman, D. Dryer, and J. R. McCartney reason, he had started a deep, raucous laughing accompanied with a motion of pounding the desk with his right fist, in a movement akin to a “slapping of the thigh” often associated with laughter, this was quite disruptive to the class, and Mrs. B. had to drive him home. At home, the laughter episodes continued intermittently, typically lasting for lo-30 seconds. Despite Mrs. B’s repeated imploring of him to “tell me what the joke is, so I can laugh too,” he remained strangely non-communicative, with only rare verbal responses. The authors were called to consult in the emergency room after medical screening and neurologic physical examination proved to be unremarkable. The nurses indicated that “he won’t talk but only laughs.” He was lying supine on the gurney, a swarthy, grayhaired, bearded, middle-aged man with eyes closed and a slight smile. Initially, he did not respond to questioning at all; as we gently, but firmly, encouraged him to speak with us, he answered some questions appropriately with nods of the head, then opened his eyes, looked directly at us, and began to answer some questions with brief verbal responses. He would not answer simple mental status questions. When asked if something disturbing had happened at the art class, he seemed to become sad and averted his eyes. The past history is interesting and relevant. Although he was described as a somewhat suspicious, compulsive, and rigid man, he had had no formal psychiatric or neurologic history until 1980, when he fell from an office chair, striking his forehead on the desk and injuring his lower back. Four months later, he was hospitalized at a local private psychiatric hospital for what was thought to be a manic episode. Increasingly restless and irritable while at home recuperating from his back injury, he was hospitalized when he began screaming semicoherently in his yard and threatening his neighbors. He was treated with chlorpromazine and gradually calmed. He had had some bizarre laughing episodes during this hospitalization and an EEG was performed, showing some occasional simple sharp waves in both temporal areas with occasional two-to-three per second spikes in the left mid to anterior temporal region. CT scan of the head, with contrast, was normal. He was started on carbamazepine and phenytoin and responded well. All anticonvulsants were gradually stopped. Subsequently, three EEG’s-all with nasopharyngeal leads-were read as normal, although one showed “rare theta activity in the left mid-temporal area.” Over the 4 years following his psychiatric hospitalization, he had had no further major psychiatric or medical problems, except for the development of COPD. It was unclear whether his behavior in the ER was seizure activity, or possibly an unusual adjustment reaction or brief psychotic episode in response to some stressful situation, in a man with a somewhat para62 noid and compulsive premorbid personality. Encouraged by the fact that he seemed to respond to psychotherapeutic intervention, with reduction in his laughter outbursts, we suggested that the patient go home and return to the office the next day for followup. The patient agreed with nods. Early the next morning, his wife brought him back to the emergency room. At 5 zyxwvutsrqponmlkjihgfedcbaZ A.M., he had walked into his teenage daughter’s room and, seemingly in anger, swept all the objects from her dresser with his arm, and then proceeded to throw her stuffed animals at the wall. On evaluation this second time, he seemed more withdrawn, less able to be engaged, and angry. He often stared at us, seeming to glower, and would burst into his by now familiar raucous laughter in bursts lasting lo-30 seconds, every 20-40 minutes. At times, he would have two to four bursts of laughter in succession. There is no psychiatric or neurologic ward or service per se in the authors’ general medical and surgical adult hospital. The Medicine Department was persuaded to admit the patient despite skepticism about admitting such an obviously bizarre psychiatric patient. They were assured that Psychiatry would assist in management. An EEG with nasopharyngeal leads was done and showed a grossly abnormal record with “a nearly continuous discharge” of seizure activity over both anterior temporal regions with occasional lateralization to the right anterior temporal region. The patient was presumed to have been in partial complex status epilepticus since his initial presentation to the emergency room. The patient was given intravenous phenytoin. In 12 hours, after receiving 900 mg of phenytoin in divided doses, his status had markedly changed. The laughter outbursts stopped, but he had become agitated, yelling loudly, with apparent auditory and/or visual hallucinations. He was given 10 mg of IV diazepam, and within 5 minutes was calm, alert, and answering questions appropriately. Thirty minutes later, however, he talked incoherently, seemed unaware of his surroundings, and began to manipulate the television switches, the nurse call button, and tubing in a repetitive nongoal-directed manner. He tried to get out of bed repeatedly and did not respond at all to verbal intervention. He did not respond to further diazepam and soon began swinging his arms at the nurses. After security forcibly restrained him in bed, he calmed and eventually slept for about 2 hours. On awakening, he continued with this semi-purposeful, confused, and occasionally agitated behavior that did not respond to phenytoin, diazepem, lorazpam or haloperidol. A second EEG, again with nasopharyngeal leads during this phase, showed a much changed pattern of slow-wave activity anteriorly with some generalized slowing as well. The ongoing seizure process had stopped although there was some persistent left temporal sharp wave activity. Status Epilepticus Presenting as Gelastic Seizures By the next morning, he was calm, coherent, but puzzled and suspicious. He knew he was in the hospital but had no idea why, could not remember anything of the last 3 days, and did not know the day of the week or the date. He admitted to one of the examiners, in complete confidence, that he was convinced that staff in the hospital were plotting against him, as part tween two behavioral phases: 1) a continuous “ twilight state” with partial and amnesic responsiveness, interrupted by 2) an unresponsive, speech-arrested state, with temporal lobe discharges on the EEG that are different from discharges during the “ twilight” state. Status can then be defined as continuously recurring cycles of these of a global plot. two phases, without recovery between seizures, By the following day, the suspiciousness had reand without residual deficits. In fact, Weiser resolved as well. In fact, he remembered nothing of ported the transition from discrete seizures, during being suspicious, and was astounded by the assertion which the patient remained fully conscious and that he had indeed felt that there were conspiracies. Both he and his wife felt that he was back to his usual oriented, to a continuous epileptic clouded state self. He had continued on phenytoin and was dis[2]. In 1979, Weiser also was able to elucidate the charged on 300 mg of phenytoin b.i.d. zyxwvutsrqponmlkjihgfedcbaZYXWVUTSRQPONMLKJIHGFEDCBA complex relationship between clinical signs and electrical findings, using surface and deep electrode tracings to correlate EEG findings with interictal Discussion periods, ictal events, and prolonged local status. Shuka et al. described an interictal confusional psyThis case exemplified the difficulties in diagnosis chosis in those with temporal lobe epilepsy (TLE) and management of complex partial status epilepthat seemed to be precipitated by status epilepticus, ticus. The patient’s psychiatric hospitalization for a and was characterized by confusion, clouding of “ manic episode” was, in retrospect, actually due to sensorium, disorientation, and bizarre behavior [S]. partial complex seizure disorder. Upon his presenOnce the proper diagnosis was made, appropritation to the emergency room, we attributed his ate treatment could be instituted. Still, manageunusual behavioral disturbance to functional ment over the next 3 days was often difficult, with causes, hypothesizing that the patient, with a rigid, potential for physical harm to the patient and/ or compulsive, and suspicious personality style, had staff. The EEG seizure activity ceased after the padecompensated under some stress. A Brief, Reactient received phenytoin, and the gelastic episodes tive Psychosis or Conversion Disorder seemed to be likewise ended. The patient, however, became conthe most likely diagnostic possibilities. Several asfused, agitated, and combative, necessitating repects of his presentation contributed to the initia1 straints. Although it is known that efforts to reconsideration of a functional, nonorganic diagstrain a patient during the postictal state can nosis: 1) He walked into the ER voluntarily and provoke aggression, the patient’s aggressive beseemed to follow simple directions. 2) He was able havior preceded staff efforts to restrain him. As is to engage in intermittent conversation and at times often the case, the restraints seemed to actually his responses seemed coherent and relevant. 3) His help him stay calmer and in control. This was follaughter, interspersed with apparently hostile lowed by a brief period of suspiciousness and wellglowering, reinforced our speculation regarding organized paranoid delusions. The EEG with the existence of an affect-laden stressor. As the nasopharyngeal leads during this period showed cyclicity of the pattern continued with a “ twilight only some generalized and focal slowing. These state” of partial responsiveness alternating with symptoms did not respond well to a variety of bizarre behavior with verbal unresponsiveness, the pharmacologic interventions, and physical rediagnosis of partial complex seizure was suspected straints remained necessary. and confirmed by an EEG with nasopharygneal The occurrence of schizophrenic-like, interictal leads. A normal EEG does not exclude complex states in those with temporal lobe epilepsy has been partial seizures. Activation techniques, such as described previously [9- zyxwvutsrqponmlkjihgfedcbaZYXWV 111.Stoudemire and colsleep deprivation, a sleep EEG, and use of leagues hypothesize that there may be a reciprocal nasopharyngeal leads can increase the diagnostic relationship between dopamine levels in the limbic sensitivity. structures and temporal lobe seizure activity and Treeman and Degado-Escueta [2] have stated recommend combined treatment with carbamazethat the essential distinguishing feature of complex pine and haloperidol in these patients [3]. With this partial status that allows differentiation from funcpatient and others with temporal lobe epilepsy, the tional psychiatric disorders is the “ cyclicity of agitated behavior and psychotic symptoms during clinical behavior” 111, involving a fluctuation be63 J. N. Glassman, D. Dryer, and J. R. McCartney derstanding and appreciation of the varied pheinterictal and postictal phases often do not respond nomenology of partial complex seizures should well to haloperidol or any other pharmacologic lead to more sophisticated diagnosis and treatment. treatment. The patient’s symptoms abated seemingly independently of any treatment save the ablation of his seizure activity. He then returned to his References baseline personality, which included obsessive and 1. Trimble MR: Disorders of the limbic system. Integr paranoid features that predated the onset of his Psychiatry 1:96-106, 1984 seizure disorder. 2. Treeman DM, De&ado-Escueta A: Complex partial The relationship of temporal lobe epilepsy, and status epilepticus. In Delgado-Escueta A, et al. (Eds), epilepsy in general, with personality has been Advances in Neurology, Status Epilepticus. New much debated. Bear reports the development of York. Raven, 1983 3. Stoudemire A, Nelson A, Houpt J: Interictal schizopersonality changes in those with temporal lobe phrenia-like psychoses in temporal lobe epilepsy. epilepsy but only after years of ongoing seizure Psychosomatics 24:331-339, 1983 disorder [9]. Shuka also found a positive rela4. Weiser, HC: Temporal lobe or psychomotor status tionship between epilepsy and personality epilepticus: A case report. Electroencephalogr Clini changes, related more to severity of epilepsy than Neurophysiol48:558-572, 1980 Black D: Pathological laughter. J Neurosis Ment Dis 2, to locus [8]. Reynolds, in contrast, refutes these 1982. views. In a retrospective review of 666 patients with Gasion GC, Lombrosco CT: Epileptic (gelastic laughtemporal lobe epilepsy, he found that only 6% had ter). Epilepsid 12:63-76, 1971 severe psychosocial disorders [12]. It will be inDaly D, Mulder D: Gelastic Epilep NeurolI:189-192, teresting to follow this patient’s progress over time. 1957. Shuka GD, Strivastava ON, Kateyar BC, Joshi V, He has had a temporal lobe seizure disorder for 4 Mohan PK: Psychiatric manifestations in temporal years, with an ongoing rigid, perfectionistic, and lobe epilepsy: A controlled study. Br J Psychiatry suspicious personality style, but without apprecia135:411-417, 1979 ble changes in personality thus far. zyxwvutsrqponmlkjihgfedcbaZYXWVUTSRQPONMLKJIHGFEDCBA 9. Bear D: Qunatitative analysis of interictal behavior in Conclusion As patients present in confused psychotic states, one must be aware of the possibility of an organic and epileptic basis for the presentation. One wonders how many “ non-phenothiazine-responding and “ non-lithium-responding” schizophrenic” manic patients might prove to have abnormal EEGs with nasopharyngeal leads. Unfortunately, unless something different or bizarre arouses suspicion, tests are not performed, diagnosis is not made, and proper treatment is not instituted. Increasing un- temporal lobe epilepsy. Arch Neurol 34:454-467, 1977 10. Slater E, Beard AW: Schizophrenia-like psychoses of epilepsy. Br J Psychiatry X%95-105, I963 11. Pavinsky 0, Bear D: Varieties of aggressive behavior in temporal lobe epilepsy. Am J Psychiatry 141:5, 1984 12. Reynolds EH: Interictal behavior in temporal lobe epilepsy. Br Med J 286: 918-919, 1983 Direct zyxwvutsrqponmlkjihgfedcbaZYXWVUTSRQPONMLKJIH reprinf requesfs to: James R. McCartney, M.D. Department of Psychiatry, The Miriam Hospital 164 Summit Avenue Providence, RI 02906