909Joumnal ofNeurology, Neurosurger, anid Psychiatry 1996;61:90-92

SHORT REPORT J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.61.1.90 on 1 July 1996. Downloaded from

Simple partial status epilepticus: causes, treatment, and outcome in 47 patients

Frans B Scholtes, Willy 0 Renier, Harry Meinardi

Abstract Somatomotor SPSE is synonymous with A retrospective case note review was con- EPC when myoclonic jerks are continuously ducted of 47 patients of 15 years and older present in the same body parts affected by the who had sustained simple partial status somatomotor seizures.2 ' Most case studies or epilepticus (SPSE) in The Netherlands reviews concern EPC; other types of SPSE between 1980 and 1987. In 46 patients the have only rarely been described.' As part of a type of SPSE was somatomotor (in four study on status epilepticus in The Netherlands adversive), and in one aphasic with visual we were able to study retrospectively 47 adult and auditory hallucinations. SPSE was patients with SPSE. We were particularly more common over the age of 50. Six of 27 interested in the various types of SPSE, the patients with previous epilepsy had an causes, EEG findings, treatment, and out- acute symptomatic cause. In 20 patients come. without previous epilepsy stroke was the most frequent cause (75%). Outcome was determined by the underlying cause. In Methods one patient the continuing epileptic activ- The cooperation of neurologists in 50 different ity may have caused neuronal damage. hospitals in The Netherlands was requested. Fourteen agreed to cooperate, but two were (3 Neurol Neurosurg Psychiatry 1 996;61 :90-92) unable to provide adequate documentation of admissions and discharges. In addition data from two of the three epilepsy centres in The Keywords: simple partial status epiepticus; causes; Netherlands were included. We restricted this treatment; outcome retrospective study to adult patients (older than 15 years). Simple partial status epilepticus (SPSE) is During the period 1980-87 we gathered a http://jnnp.bmj.com/ characterised by partial seizures without total of 576 admissions caused by various impairment of consciousness or secondary types of SE. We visited every hospital and generalisation, and with preserved neurovege- inspected the patient files. tative regulation.' The clinical expression For SPSE, only patients with preserved depends on the region of the brain where the consciousness and with a minimum seizure seizures originate.The most frequent type of duration of 30 minutes were included.

SPSE is somatomotor. Other types include Patients with hemi-grand mal SE, asymmetric on September 30, 2021 by guest. Protected copyright. aphasic and somatosensory SPSE. Partial generalised tonic-clonic SE, or patients with seizures with preserved consciousness are successive secondary generalised tonic-clonic called SPSE when continuous clinical and seizures with persistent clonic jerks of one side EEG seizures are present for at least 30 min- of the body and impaired level of conscious- utes.2 ness were excluded. In general neurological practice two groups When a single tonic-clonic seizure preceded of patients with SPSE are encountered most or occurred at the end of the SPSE, the patient Dr Hans Berger often: was included. Morbidity was defined as all Clinic, Breda, The a succession of new neurological signs occurring during the Netherlands (1) Those with simple par- FB Scholtes tial seizures with Jacksonian march, without period of SE and was calculated per event. University Hospital, persistent segmental myoclonus (somatomotor The causes of morbidity were classified as (a) Nijmegen, The SPSE). the underlying cause, (b) the seizures them- Netherlands (2) Those with persistent myoclonus in a selves and the ensuing medical complications, W 0 Renier H Meinardi limited area of the body, present for weeks or and (c) unknown (when the contribution of months and sometimes in combination with the underlying cause or the seizures them- Correspondence to: Dr F Scholtes, Dr Hans somatomotor or tonic-clonic seizures (epilep- selves could not exactly be established). The Berger Clinic, PO Box causes of have been divided in the 90108, 4800 RA Breda, sia partialis continua, EPC). Consciousness mortality The Netherlands. may be normal but can also be impaired. same way. Received 8 December 1995 Both types are usually caused by a sympto- Outcome has been described as the neuro- and in final revised form 13 March 1996 matic brain lesion, but may also occur in logical condition at discharge from the hospi- Accepted 20 March 1996 patients with a history of epilepsy. tal. Simlple partial status epilepticus: causes, treatm2ent, and outcome in 47 patients 91

Treatment was regarded as inadequate During SPSE EEG investigation was made J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.61.1.90 on 1 July 1996. Downloaded from when an insufficient dose was given, when the in only 13 patients and showed abnormal find- route of treatment was wrong (for example, ings corresponding to the clinical expression in intramuscular injection of diazepam), when 12 of them. However, specific epileptic dis- unnecessary delay was present (for example, charges were present in only five patients. waiting for more than an hour after diazepam Brain CT was performed in all; MRI, PET, or injection while the seizure continued before SPECT in none. giving a second agent), when mechanical venti- lation was not started despite signs of respira- CAUSES OF SPSE tory failure or the presence of various other In 27 patients with previous epilepsy six devel- medical complications, or when EEG moni- oped SPSE because of a new neurological toring was not done on patients treated with problem: metastases (two), stroke (one), antiepileptic drugs together with curarisation. pneumococcal meningitis (one), osteomyelitis of the skull (one), and hypocalcaemia (one). Compliance problems (antiepileptic drugs) Results were only present in three patients. In three The Netherlands has a population of nearly 15 patients a systemic infection was considered to million people. About 100 000 have epilepsy. be the precipitating factor. In 15 patients the The admissions we retrieved covering a period cause remained unknown. of eight years concerned 47 patients; 27 were In 20 patients without previous epilepsy known to have a history of epilepsy. The stroke seemed the most prominent cause (14). causes of previous epilepsy were symptomatic In three patients a brain tumour had been in 18, whereas in nine patients the cause found, whereas in another three patients a remained unknown. In five patients SPSE cause could not be established. ended with a generalised tonic-clonic seizure, in seven a tonic-clonic seizure preceded SPSE. DURATION OF SPSE In our material a preponderance of 30 Duration was less than two hours in 13 women versus 17 men was found. There was patients, between two and four hours in 12, an increase of SPSE with age. Only 16 of our between four and 20 hours in four, and longer patients were younger than 50; two were than 20 hours in 18. Half of these had a dura- younger than 30. tion of one to three days, the other half between three and 14 days. An exceptionally TYPES OF SPSE long duration (seven weeks or more than one Apart from one patient with aphasic SE all year) was seen in three patients with continu- showed somatomotor SPSE; four of these had ous focal myoclonic jerks (EPC). adversive SPSE. The facial musculature was involved in 38 OUTCOME OF SPSE patients, alone (eight) or in combination with Four patients died, all due to cerebral infarc- the eyes (one), an arm (seven), platysma tion. (two), and an arm and a leg (20). In four Morbidity after SPSE was present in 10 patients jerking was limited to an arm (one), a patients and consisted of paresis (four), aphasia http://jnnp.bmj.com/ hand (two), or the abdominal wall (one). (two), psychiatric disturbance (one), cognitive The patient with aphasic SPSE was a woman impairment (two), and persistent impairment aged 67, known to have had previous epilepsy of consciousness (one). Morbidity was mainly (caused by stroke), who developed SPSE with a due to the underlying cause (six patients); in duration of five days. The clinical presentation three patients the exact cause of morbidity consisted of aphasia, acoustic hallucinations could not be established. In one patient mor- (strange sounds, music, voices), left sided bidity was attributed to SE itself.

headache, and visual hallucinations (red balls in In the six patients with sequelae because of on September 30, 2021 by guest. Protected copyright. the front of the right eye). She remained alert the underlying cause, four had had a cerebral and showed recurrent epileptic seizures on the infarction, one a subdural haematoma, and EEG, starting in the left occipital region with a one an intracerebral haematoma. The patients 13 Hz sharp wave activity, changing to spike with sequelae of unknown cause had all had wave activity (3-4 Hz), and spreading over the previous epilepsy. left hemisphere. Sometimes a start was seen in the left paracentral region. Further investiga- TREATMENT tions could not establish a cause. When the The last treatment, given just before SPSE EEG showed that the SPSE had been arrested ended or given as a last attempt consisted the patient continued to exhibit persistent word mainly of benzodiazepines, in particular finding problems and a slight verbal memory (40%) clonazepam or clonazepam with pheny- deficit. toin. Other drugs were intravenous phenytoin, In 26 of 46 patients with somatomotor diazepam, or lidocaine, etomidate infusion, SPSE, frank seizures were evident without doc- thiopentone infusion, carbamazepine, and umented continuous myoclonus. The remain- intravenous calcium in a case of hypocal- ing 20, showed more or less continuous clonic caemia. In this last case calcium was immedi- or myoclonic jerks, corresponding to EPC. ately successful, whereas diazepam and When we compared the patients with EPC with clonazepam failed. those without continuous myoclonus, no differ- When outcome was related to quality of ences were found for outcome, number of treatment we found in patients with good out- patients with previous epilepsy, or causes. come that treatment was inadequate in 21 %; 92 Scholtes, Renier, Meinardi

in patients with morbidity in 40%, and in azepam intravenously. Next the patient should J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.61.1.90 on 1 July 1996. Downloaded from patients with fatal outcome in 50%. be transferred to the intensive care unit because of the threat of respiratory depression. Continue with 1 mg clonazepam every one or Discussion two minutes intravenously until seizures stop The symptom SPSE is relatively rare and most or respiratory depression becomes evident. If case reports and reviews concern EPC, also clonazepam fails after 10 minutes, start pheny- named somatomotor SPSE.4 We did not find toin-sodium intravenously (15-18 mg/kg with specific differences between patients with con- a rate of 50 mg/min). For those who continue tinuous clonic or myoclonic activity and with seizures start with midazolam-infusion at a patients with partial motor seizures for cause, rate of 0-3-0 6 mg/kg/24 h after a bolus injec- outcome, and the presence of previous tion of 0-1-0-3 mg/kg. We also have good epilepsy. Our findings differ from those pub- results with propofol infusion. In most patients lished for the frequency of previous epilepsy in with SPSE thiopentone infusion is not neces- patients with SPSE. Duration of SPSE in our sary. series was generally shorter than mentioned in During treatment adequate medical care the medical literature and we differ in having a with special attention to respiration is essen- higher prevalence of female patients. tial. The cause of SPSE should be established The cause of SPSE in our 20 patients with- as soon as possible, maintenance treatment out previous epilepsy was stroke in 70%, a with antiepileptic drugs should be continued brain tumour in 15%, and unknown in or started. In patients resistant to drug therapy another 15%. Two of the latter patients had a surgical treatment may be considered. 14 chronic psychiatric disease and one a remote symptomatic cause (subarachnoid haemor- We thank Mrs G van Etten for preparing the manuscript. rhage some years earlier). 1 Gastaut H. Classification of status epilepticus. In: Delgado- In patients with previous epilepsy an acute Escueta A, Treiman D, Wasterlain C, Porter R, eds. neurological problem seemed to be a promi- Status epileptncus. Advances in neurology. Vol 34. New York: Raven Press, 1983:15-47. nent cause (22%). 2 Delgado-Escueta A, Treiman D. Focal status epilepticus: No correlation could be established modern concepts. In: Luders H, Lesser R, eds. Epilepsy: electroclinical syndromes. New York: Springer-Verlag, between outcome and duration. Outcome was 1987:347-93. mainly determined by the underlying cause. In 3 Thomas J, Reagan T, Klass D. Epilepsia partialis continua. Arch Neuroll 977; 34:266-75. three patients the cause of morbidity remained 4 Schomer D. Focal status epilepticus and epilepsia partialis unknown. All three had previous epilepsy; one continua in adults and children. Epilepsia 1993;34(suppl 1) :S29-36. was suspected to have a brain lesion, but the 5 Lohler J, Peters U. Epilepsia partialis continua exact nature was not known; another was (Kozevnikov-Epilepsie). Fortschr Neurol Psychiatr 1974; 42: 65-212. mentally retarded and the third had epilepsy 6 Juul-Jensen P, Denny-Brown D. Epilepsia partialis con- because of stroke and a new cerebrovascular tinua. Arch Neurol 1966;15,563-86. 7 Meienberg 0 and Karbowski K. Die Epilepsia partialis accident was suggested but this could not be continua Kozevnikov. Dtsch Med Wochenschr 1977;102; determined with certainty. Only in one case 781-4. 8 Andermann F, Lugaresi E, Dvorkin G, Montagna P. SPSE itself as a cause of morbidity was likely. Malignant migraine: the syndrome of prolonged classical Continuing seizure activity as a cause of migraine, epilepsia partialis continua, and repeated strokes; a clinically characteristic disorder probable due http://jnnp.bmj.com/ morbidity in PSE has been established in to mitochondrial encephalopathy. Funct Neurol 1986;1; complex partial SE,9 and SPSE as a cause of 481-6. 9 Engel J, Ludwid B, Fetell M. Prolonged partial complex morbidity has been suggested in some other status epilepticus: EEG and behavioral observations. patients.'° " Neurology 1978;28:863-9. 10 Knopman D, Margolis G, Reeves A. Prolonged focal In SPSE, as in GCSE12 we found that treat- epilepsy and hypoxemia as a case of focal brain damage: a ment was more often inadequate in patients case study. Ann Neurol 1977;1: 195-8. 11 Duncan R, Patterson J, Hadley D, Bone I. Unilateral cere- with less good outcome, but the number of bellar damage in focal epilepsy. _7 Neurol Neurosurg

patients with mortality or morbidity was too Psychiatry 1990;53:436-7. on September 30, 2021 by guest. Protected copyright. 12 Scholtes F, Renier W, Meinardi H. Generalized convulsive low to draw conclusions. status epilepticus: causes, therapy and outcome in 346 Treatment of SE, irrespective of type, patients. Epilepsia 1994;35: 1104-12. 13 Scholtes F, Reni W, Meinardi H. Generalized convulsive should be according to protocol.3 We advise status epilepticus. Pathophysiology and treatment. starting with rectal diazepam solution, 20-30 Pharmacy World and Science 1993;15:17-28. 14 Bancaud J, Bonis A, Trottier S, Talairach J, Dulac 0. mg in adult patients. When seizures persist for L'epilepsie partielle continue: syndrome et maladie. Rev 10 minutes after the diazepam give 1 mg clon- Neurol (Paris) 1982;138:803-14.