Stormy Onset with Prolonged Loss of Consciousness in Benign Childhood Epilepsy with Occipital Paroxysms 47
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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 epilepsy 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 seizure was characterised by disorders, including basilar artery migraine, 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 seizures (almost all omena were either partial or generalised contraversive) in 22, headache in 24, and convulsions. 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. Anticonvulsants 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 convulsion 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 anticonvulsant 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.