Journal ofNeurology, Neurosurgery, and Psychiatry 1992;55:45-48 45

Stormy onset with prolonged loss of J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.55.1.45 on 1 January 1992. Downloaded from consciousness in benign childhood with occipital paroxysms

Sara Kivity, Pinchas Lerman

Abstract describing the clinical features of 16 children In nine of 62 children with benign with occipital spike-wave complexes suppres- occipital epilepsy (BOE) the onset was sed by eye opening, stressed the association of stormy and alarming. The first and often this EEG pattern with a wide range of clinical only was characterised by disorders, including basilar artery , prolonged loss of consciousness lasting BOE, and lesional epilepsy. up to 12 hours, suggesting an acute In a previous report6 presented at the 17th cerebral insult. In all but one case there International Epilepsy Congress in 1987 we was a tonic adversion either of eyes alone summarised the seizure patterns in 62 chil- or of both head and eyes which was dren fulfilling the electroclinical criteria of interpreted as conjugate deviation. The BOE formulated by Gastaut,' finding visual other accompanying ictal motor phen- symptoms in 18 adversive (almost all omena were either partial or generalised contraversive) in 22, in 24, and . In five patients the seizure vomiting in 15. Prolonged alarming loss of was heralded by a headache, and in five consciousness lasting up to 12 hours was the cases was accompanied by vomiting. The presenting symptom of the first seizure in nine seizure began with visual symptoms in patients who we describe here. only one patient. The seizure occurred while awake in seven and during sleep in two. The age at onset was from 3+ to 10 Patients and results years. Interictal EEGs showed occipital During the years 1970-85, 62 children were discharges typical of BOE, and the clin- diagnosed as having BOE at the seizure clinic ical course was benign. In four cases a and the EEG Laboratory of the Beilinson few partial or complex partial seizures medical center. The seizure patterns in these recurred during subsequent anticonvul- children have been briefly described else- sant therapy, but in five cases seizures where.6 Data from seven boys and two girls never recurred. were who had their first seizure with prolonged loss discontinued in five patients who of consciousness are presented here (table 1). remained free from seizures for one to The age at onset ranged from 3+ to 10 years. Ili years after withdrawal of treatment. In all cases there was no family history of Sudden coma in a child associated with epilepsy or migraine. One patient had suffered focal features such as tonic deviation of a simple febrile at the age of 21 http://jnnp.bmj.com/ the head or eyes or both may represent a years and had a family history of febrile con- benign seizure disorder. vulsions. Five patients suffered from headache before the seizure: in three it lasted less than 20 minutes, in two it was prolonged, lasting Benign occipital epilepsy has been defined by from 12 to 48 hours and fluctuating in severity, Gastaut" as another form of benign partial and in one patient it was accompanied by epilepsy in children. He described seizures vomiting. Loss of consciousness, whether which included visual symptoms often fol- preceded or not by headache, was sudden and on October 3, 2021 by guest. Protected copyright. lowed by motor or psychomotor manifesta- prolonged, lasting from four to 12 hours and tions and sometimes terminating with post- accompanied in all but one by a tonic devi- ictal migrainous symptoms such as headache, ation either of eyes or both head and eyes nausea, and vomiting. The typical interictal followed by bilateral clonic jerks in two, gen- Pediatric Seizure EEGs consisted of high voltage occipital eralised tonic clonic convulsions in two, left Clinic and EEG spike-wave paroxysms attenuated by eye hemiclonic in one, right tonic clonic in one, Laboratory, Beilinson Medical Center, Petah opening. The diagnostic criteria included a and right brachiofacial in one. The patient Tiqva, and Sackler normal neurological state and no abnormal (case 6, see table 2) whose initial seizure was Faculty of Medicine, neuroradiological findings. The course is non-convulsive later experienced complex Tel Aviv University, Israel benign and seizures cease in most patients partial seizures. Vomiting occurred in four S Kivity before adulthood. The clinical and EEG patients during the adversive stage and in one P Lerman features of BOE have been reported by in association with the headache preceding the Correspondence to: others.'9 Camfield et al'0 reported four adoles- seizure. In only one patient did the seizure Dr Kivity, Pediatric Seizure cents with Clinic, Beilinson Medical basilar migraine, infrequent begin with visual symptoms followed by loss Center, 49 100 Petah Tiqva, cerebral seizures, and the same interictal EEG of consciousness and convulsions. Israel. findings as in BOE with a benign clinical Received 14 March 1990 course. In and in revised form 1980 Panayiotopoulos" described a EEGfindings 23 April 1991. similar case arguing in favour of the epileptic In one patient (case 8) EEG was performed Accepted 2 May 1991 nature of the syndrome. Newton et al4 in during the prolonged seizure, and it showed 46 Kivity, Lerman

continuous bilateral spike and wave com- His course is detailed in the case report given J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.55.1.45 on 1 January 1992. Downloaded from plexes, more prominent on the left side. In below. None of the nine patients had a seven patients EEG was obtained within 24 recurrence of severe prolonged seizures com- hours after the initial seizure and in one (case parable to the first one. 1) after five days. In three of them occipital spikes were seen in this first record whereas in Illustrative case reports the five others whose first EEG showed Case 1 This 9 year old boy, some 30 minutes occipital slow activity occipital spikes were after he had fallen asleep was found one night recorded in subsequent EEGs obtained be- in a "coma", gargling and vomiting, with his tween two days to six months later. These eyes and head turned to the right. There were waking interictal EEG recordings showed fine clonic jerks of the hands and feet, slight high voltage spike-waves or sharp and slow cyanosis, and a loss of sphincter control. On waves in the posterior regions, in runs or transfer to our hospital he was unconscious almost continuously, attenuated by eye open- with right hemiparesis and a right Babinksi ing. In five the discharges were unilateral, in sign. CT scan showed a narrow left ventricle four bilateral with predominance of one side. due to oedema of the left hemisphere. He was In two patients (cases 2 and 9, table 1) a few intubated and given intravenous dexameth- brief generalised spike-wave discharges were asone, mannitol, and diazepam. Several hours recorded in some of the later tracings in addi- later his body temperature was 38-5°C, and tion to the occipital focus, and in one case acyclovir was started with the suspicion of (case 5) sporadic centro-temporal spikes were encephalitis. The child regained conscious- seen in addition to the occipital focus three ness after 10 hours with no neurological years after the initial EEG. deficit. Five days later a repeat CT scan was normal, and the antiviral treatment was CTfindings stopped; two EEG recordings made on the CT scans performed during the initial seizure fifth and seventh days demonstrated a slow in seven patients were normal in five; in the wave focus over the left occipital area. Four other two (cases 1 and 2) narrowing of one or months later he experienced two brief noctur- both lateral ventricles was demonstrated. The nal seizures, half an hour apart, with right course was benign in all nine patients, despite tonic deviation of head and eyes, vomiting, the stormy onset. In five of eight patients who and loss of consciousness. EEGs obtained at received therapy after the first 12 hours and one week later showed repetitive seizure there was no recurrence of seizures. spike and slow wave discharges over the left Four of them (cases 3, 7, 8, 9, table 1) are no occipital region, inhibited by eye opening. longer taking medication (for 3 5, 8-5, 5 0 and Treatment was begun with 90 mg phenobar- 11-5 years respectively). Among the three bitone daily. One and a half and two months remaining patients one (case 5, table 2) had later he had two additional adversive diurnal two brief adversive seizures with vomiting and seizures without vomiting. In the former the loss of consciousness on waking after reduc- loss of consciousness lasted 10 minutes with tion of medication six and 24 months later. gradual return to full consciousness over 20 One of these patients (case 6, table 2) had two minutes, and in the latter it lasted for five brief complex partial seizures after eight and minutes. Both seizures were associated with

14 months. The course of the third patient headache which preceded the seizure and con- http://jnnp.bmj.com/ (case 2) is also presented in table 2. In the one tinued post-ictally for eight and four hours patient who did not receive any medication respectively. EEG recordings made two, six, after the initial seizure (case 1, table 2) a brief 12, and 16 months after the cessation of second seizure occurred four months later. seizures showed the same abnormalities, but

Table 1 Clinical datafor nine children with benign childhood epilepsy with occipital paroxysms on October 3, 2021 by guest. Protected copyright. Clinicalfeatures ofseizures Seizures Duration Age when Duration of of at awake (W) Other loss of drug Recurrence Follow Case onset or motor consciousness Interictal therapy of up No (years) Sex asleep (S) Headache Vomiting Adversion features (hours) EEG (years) seizures (years) 1 9 M S No Yes Right head & eyes Clonic jerks (all 10 Left occipital Pb/3 Yes 4 limbs) 2 34 M W Yes No Left eyes Clonic jerks (all 12 Right > left PHT/4 Yes 4 limbs) occipital & generalised S/W 3 8 M W Yes Yes Left eyes Left hemi-clonic 6 Right occipital PHT/2 No 54 4 4 M S No Yes Left head&eyes Generalised tonic- 4 Right > left CBZ/31 No 34 clonic occipital 5 5 M w Yes Yes Left head & eyes Generalised tonic- 4 Right > left CBZ/31 Yes 34 clonic occipital 6 8 M W Yes No None None 5 Left occipital CBZ/4 Yes 4 7* 9 F W No No Right head & eyes Right tonic-clonic 4 Left > right PHT/3 No 114 occipital 8 10 M W Yes No Right head & eyes Right brachiofacial 12 Left occipital CBZ/24 No 74 9 4 F W No Yes Left head & eyes None 4 Right occipital & CBZ/2 No 134 generalised S/W *Seizure began with visual symptoms. Pb: phenobarbitone; PHT: phenytoin; CBZ: carbamazepine; S/W: spike and wave. Stormy onset with prolonged loss of consciousness in benign childhood epilepsy with occipital paroxysms 47

Table 2 Frequency and clinical manifestations of subsequent seizures in four children with recurrence J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.55.1.45 on 1 January 1992. Downloaded from Ictal manifestations Case No Time afterfirst seizure On waking During sleep 1 4 Months Brief tonic adversion of head and eyes, vomiting, loss of consciousness twice within 30 minutes 5i Months, 6 months Brief tonic adversion of head and eyes, loss of consciousness, preceded and followed by headache 2 6 Months (during medication) Onset not witnessed. Loss of consciousness (30 minutes), on arousal, headache and vomiting 14 Months Brief generalised tonic, loss of consciousness 22 Months Twitching of left mouth corner and clonic movement of left upper limb with loss of consciousness (45 minutes) 5 6 Months, 24 months (during reduced dose) Brief tonic adversion ofhead and eyes, vomiting, loss of consciousness 6 8 Months, 14 months Brief complex partial seizure, post-ictal headache (10 minutes)

within three years the EEG normalised and consequently subjected to emergency inves- eventually the medication was reduced and tigations, including lumbar puncture and CT finally discontinued. ofthe brain, and was admitted as critically ill to Case 8 This 10 year old boy, generally in a paediatric or neurosurgical intensive care good health, complained of a sudden severe unit. Case 1 was first diagnosed as having headache while in school. Some 20 minutes oedema of the left hemisphere, was initially later he became confused and shortly there- treated with iv steroids and mannitol and after lost consciousness. On examination in received acyclovir several hours later when the paediatric ward two hours later he was still focal encephalitis was considered to be the unconscious with deviation of the head and probable diagnosis. In case 2 iv dexamethasone eyes to the right and fine clonic jerks of the was given with the diagnosis of brain oedema. right mouth angle and right upper extremity. In two cases (5 and 6) toxic was An ictal EEG showed continuous bilateral also considered in the differential diagnosis. In slow spike and wave complexes, predomin- no case, however, was the possibility of an antly on the left. During EEG the spike idiopathic epilepsy even considered. In all activity disappeared completely after intra- these cases the rapid recovery occurring within venous administration of 7 mg diazepam, with less than 24 hours and then the finding of an the appearance of drug-induced beta activity occipital epileptic focus came as a surprise, over the right hemisphere and post-ictal delta ruling out the possibility of an acute cerebral waves on the left, more prominent in the insult. The finding of epileptic activity either posterior region. The convulsions stopped, during the episode (case 8) or within 24 hours but the child remained unconscious. There thereafter (cases 3, 4, 6) revealed the true were no signs of increased intracranial pres- aetiology ofthe event. Hence the importance of sure. CT scan of the brain was normal. an urgent EEG during the ictus in such cases. Twelve hours after onset of this episode the EEGs recorded shortly after the ictal events,

child regained consciousness, without any however, may show focal occipital slowing and http://jnnp.bmj.com/ neurological deficit. Carbamazepine therapy, spikes. This, with the additional poverty offast 600 mg per day, was instituted. EEG record- rhythms in the same focal areas, may encourage ings made one day and then one week later the diagnosis of underlying structural lesion. still showed slow activity (theta and delta) Subsequent development of occipital parox- over the left hemisphere, predominantly in ysms clarifies the diagnosis. In case 1 the the occipital region. In consecutive EEGs per- diagnosis of BOE was made only after four formed six weeks later and then twice a year months when the child had a second seizure there were rhythmic sharp and slow wave and the EEG performed at that time revealed on October 3, 2021 by guest. Protected copyright. discharges in the left posterior region, the occipital spike-focus; indeed the course was inhibited by eye opening. Two and a half benign. All the patients were eventually con- years later there was normalisation of trolled by standard anticonvulsants. In five the EEG, and treatment was discontinued cases there was no recurrence whatsoever, and gradually. He has remained free of seizures in the other four (cases 1, 2, 5, 6) there were after withdrawal of medication for more than subsequently a few seizures, none of them five years. severe. In five cases (1, 3, 7, 8, 9) treatment has already been discontinued with no recurrence. The seizures in BOE are often brief, Discussion especially when occurring on waking and The alarming feature in these nine children was sometimes even without loss of conscious- the prolonged loss of consciousness with tonic ness.27 Prolonged seizures lasting from 45 deviation ofeither ofeyes alone or ofboth head minutes to three hours have been mentioned in and eyes either preceded by headache or some reports.2412 Panayiotopoulos9 found in 16 accompanied by vomiting in a previously heal- patients that "consciousness was preserved thy child. In all these cases an acute cerebral throughout the ictus in four patients. In all the insult-for example, increased intracranial other children consciousness was impaired or pressure, cerebrovascular accident, or focal lost either from the onset or during the course encephalitis-was suspected, and the child was of the fits. Seizures lasted only a few minutes 48 Kivity, Lerman

(nine patients) or were prolonged for several (35%) of the patients previously reported.6 hours (seven children)." A review of the eight Indeed, contraversive seizures were the most J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.55.1.45 on 1 January 1992. Downloaded from cases described by Panayiotopoulos"2 reveals prominent motor manifestation in this group of that the "duration of the seizure varies from 62 patients. The clinician facing a child who seven minutes up to three hours, but usually has suddenly lapsed into a coma associated with lasts from 10 to 15 minutes." Among the seven focal features such as tonic deviation of either cases reported in detail by Gastautl the seventh ofeyes alone or ofboth head and eyes should be case had, at the age of 4 years, a nocturnal aware that besides an acute cerebral insult such seizure starting with adversion to the right, symptoms may represent an occipital seizure followed by nausea, vomiting, and diarrhoea disorder which may prove to be benign. The before losing consciousness for over one hour. early performance of an EEG, preferably dur- Newton and Aicardi4 reported among their 16 ing the seizure, may clarify the issue. The cases one 8 year old girl who "had bouts of loss finding of an occipital focus in such cases ofconsciousness lasting between a few minutes indicates a favourable outcome. to two hours, most about 30 minutes." Bickerstaff'3 has described impairment of consciousness in several patients with basilar artery migraine, but this was of slow onset and 1 Gastaut H. Anew type ofepilepsy: benign partial epilepsy of short duration and never sufficiently profound childhood with occipital spike waves. Clin Electro- encephalogr 1982;13:13-22. to render the patient unarousable. Further- 2 Gastaut H. Benign epilepsy of childhood with occipital more, these patients had no seizures, and no paroxysms. In: Roger J, Dravet C, Bureau M, Dreifuss FE, Wolf P, eds. Epileptic syndromes in infancy, childhood EEG abnormalities were mentioned. Pro- and adolescence. John Libbey Eurotext Ltd, 1985:159-70. longed confusional states associated with dizzi- 3 Beaumanoir A. Infantile epilepsy with occipital focus and good prognosis. Eur 1983;22:43-52. ness and disturbed sensorium with varying 4 Newton R, Aicardi J. Clinical findings in children with degrees ofagitation, simulating toxic metabolic occipital spike-wave complexes suppressed by eye-open- ing. Neurology 1983;33:1526-9. psychosis, encephalitis, or acute toxic enceph- 5 Herranz Tanaro FJ, Lope ES, Sassot SC. La pointe onde alopathy were described by Gascon and occipitale avec et sans epilepsie benigne chez l'enfant. Rev EEG Neurophysiol 1984;14:1-7. Barlow'4 as a presenting feature in four patients 6 Kivity S, Lerman P. Benign partial epilepsy of childhood with juvenile migraine, none of whom had with occipital discharges. In: Manelis J, Bental E, Loeber JN, Dreifuss FE, eds. Advances in epileptology. XVIIth epileptic seizures. EEG recordings obtained Epilepsy Intemational Symposium. New York: Raven shortly after these episodes were either within Press, 1989:371-3. 7 Terasaki T, Yamatogi Y, Ohtahara S. Electroclinical normal limits or showed posterior or diffuse delineation of occipital lobe epilepsy in childhood. In: slow wave activity. None ofthe children repor- Andermann F, Lugaresi E, eds. Migraine and epilepsy. London: Butterworth, 1987:61-9. ted as suffering from basilar artery migraine5 16 8 Fois A, Malandrini F, Tomaccini D. Clinical findings in showed protracted loss of consciousness but children with occipital paroxysmal discharges. Epilepsia 1988j29:620-23. rather suffered from confusional states. Furth- 9 Panayiotopoulos CP. Benign childhood epilepsy with ermore, these attacks were not associated with occipital paroxysms: A 15-year prospective study. Ann Neurol 1989;26:51-6. convulsive seizures nor with occipital spike- 10 Camfield P, Metrakos K, Andermann F. Basilar migraine, waves. With regard to the other accompanying seizures and severe epileptiform EEG abnormalities. Neurology 1978;28:584-8. symptoms, in basilar artery migraine the 11 Panayiotopoulos CP. Basilar migraine ? seizures and severe headache supervenes after the visual and EEG abnormalities. Neurology 1980;30:1122-5. 12 Panayiotopoulos CP. Benign nocturnal childhood occipital the other symptoms of brain stem dysfunction epilepsy: a new syndrome with nocturnal seizures, tonic the and J Child Neurol whereas in our cases it heralded the seizures. deviation of eyes vomiting. http://jnnp.bmj.com/ 1989;4:43-8. Prodromal headache preceding epileptic 13 Bickerstaff ER. Impairment of consciousness in migraine. seizures had been described by Penfield and Lancet 1961b;ii:1057-9. 14 Gascon G, Barlow C. Juvenile migraine presenting as an Jasper.'7 acute confusional state. Pediatrics 1970;45:628-35. Ictal vomiting may be common in BOE'2 18; 15 Lee CH, Lance JW. Migraine and stupor. Headache 1977; 17:32-8. interestingly in four of our nine cases vomiting 16 Swanson JW, Vick NA. Basilar artery migraine: 12 patients occurred during the adversive phase of the with an attack recorded electroencephalographically. Neurology 1978;28:782-6. seizure. Panayiotopoulos9 12 has recently 17 Penfield W, Jasper H. Epilepsy and thefunctonal anatomy of emphasised that adversion is common in BOE, the human brain. Boston: Little Brown, 1954:34. on October 3, 2021 by guest. Protected copyright. 18 Panayiotopoulos CP. Vomiting as an ictal manifestation of particularly in nocturnal seizures. In all but one epileptic seizures and syndromes. J Neurol Neurosurg of our nine patients it was present and in 22 Psychiatry 1988;51:1448-51.