Episodes Of Convulsive Status Epilepticus Research Articles

Seizure control during pregnancy and postpartum period in women with epilepsy: an Egyptian prospective study

BackgroundProper seizure control during pregnancy and postpartum is essential to optimize the outcome of women with epilepsy (WWE). The current work aimed to address factors related to seizure occurrence during pregnancy and postpartum.MethodsOne hundred twenty-five WWE, compliant with their anti-seizure medications (ASMs) regimen, were prospectively evaluated for seizure control and ASMs changes all through the pregnancy up to 4 weeks postpartum.ResultsMost of the patients, 73 (58.4%), completed their pregnancy without seizures, while 52 (41.6%) had seizures. Only one case developed one episode of convulsive status epilepticus in the third trimester. Due to breakthrough seizures, the ASM dose was increased from the first to the third trimester in 19.2% of pregnancies, while another ASM was added in 8 pregnancies. Uncontrolled seizures during the six months before pregnancy were associated with a four-fold increase in the risk of seizures during pregnancy (95% CI 2.476–6.695). The latter nearly doubled the risk of seizures during the postpartum period (RR 1.978) (95% CI 1.44 -2.717). Furthermore, genetic etiology would increase the risk of seizures during the postpartum period by 2.7 times more than the unknown etiology (RR 2.778, 95%CI 1.156–6.679).ConclusionWomen with epilepsy should be counselled that proper seizure control six months before pregnancy is necessary to pass their pregnancy and the postpartum period without seizures.

Long-term prognosis after childhood convulsive status epilepticus: a prospective cohort study

The prognosis of convulsive status epilepticus (CSE), a common childhood medical neurological emergency, is not well characterised. We aimed to investigate the long-term outcomes in a cohort of participants who previously had CSE. In this prospective study, we followed up a population-based childhood CSE cohort from north London, UK (the north London convulsive status epilepticus surveillance study cohort; NLSTEPSS). We collected data from structured clinical neurological assessment, neurocognitive assessment (Wechsler Abbreviated Scale of Intelligence), brain MRI, medical records, and structured interviews with participants and their parents to determine neurological outcomes, with adverse outcome defined as presence of one or more of epilepsy (active or in remission), motor disability, intellectual disability, or statement of special educational needs. We applied multiple imputation to address missing data and performed binary logistic regression analyses on complete-case and imputed datasets to investigate sociodemographic and CSE factors associated with adverse outcomes. Of 203 survivors (90% of inception cohort), 134 (66%) were assessed at a median follow-up of 8·9 years (IQR 8·2-9·5). The cumulative incidence of epilepsy was 24·7% (95% CI 16·2-35·6), with most (89%) emerging within 18 months after CSE. The cumulative incidence of epilepsy was lower in patients with prolonged febrile seizures (14·3%, 6·3-29·4) and survivors of acute symptomatic CSE (13·3%, 3·7-37·9) than in those of remote symptomatic CSE (45·5%, 21·3-72·0) and unclassified CSE (50·0%, 25·4-74·6). One participant (2·9%, 0·5-14·5) in the prolonged febrile seizures group developed temporal lobe epilepsy with mesial temporal sclerosis. The absence of fever at CSE was the only predictor of incident epilepsy (odds ratio [OR] 7·5, 95% CI 2·25-25·1). Motor and intellectual disability was seen predominantly in participants who had idiopathic and cryptogenic CSE (seven [36·8%, 95% CI 19·1-59·0] and 16 [84·2%, 62·4-94·5] of 19, respectively) and remote symptomatic CSE (33 [62·3%, 48·8-74·1] and 40 [75·5%, 62·4-85·1] of 53), and most of these participants had pre-existing disabilities. Pre-existing epilepsy was the only predictor of intellectual disability (OR 8·0, 95% CI 1·1-59·6). 51·5% (95% CI 43·1-59·8) of those followed up had a statement of special educational needs. Childhood CSE is associated with substantial long-term neurological morbidity, but primarily in those who have epilepsy, neurological abnormalities, or both before the episode of CSE. Survivors without neurological abnormalities before CSE have favourable outcomes. BUPA Foundation, The Academy of Medical Sciences, Wellcome Trust, National Institute for Health Research, and Young Epilepsy.

Spontaneous seizure remission following status epilepticus in drug-resistant epilepsy due to focal cortical dysplasia

We describe a patient with chronic pharmacoresistant epilepsy related to right parietal focal cortical dysplasia (FCD), who became seizure-free following an episode of convulsive status epilepticus (SE). Magnetic resonance imaging (MRI) and fludeoxyglucose positron emission tomography (FDG-PET) were performed before and after SE. Longitudinal MRI scans showed a stable appearance of the FCD with no new signal change. However, diffusion tensor imaging showed altered white matter fiber tract orientation in posterior cortices, especially in proximity to the lesion, at 3 years post-SE. FDG-PET showed more widespread hypometabolism 3 years after SE. The unusual occurrence of spontaneous seizure remission following SE in the context of FCD-related epilepsy, in association with neuroimaging evolution, suggests possible cerebral reorganization triggered by SE as a mechanism in this case.

P48 – 1793 Treatment of convulsive status epilepticus in the UMCG: a retrospective, observational study

Objectives: Little is known about clinical practice with respect to the application of guidelines in the treatment of Convulsive Status Epilepticus (CSE). This retrospective, observational study evaluated treatment of episodes of CSE in children at the University Medical Centre Groningen (UMCG). Material and methods: Episodes of CSE were derived from the UMCG database of children with epilepsy aged 1 month. Treatments used in the UMCG were compared with recommendations from two most recent Dutch treatment guidelines for CSE in children aged >1 month. Results: 269 episodes of CSE occurring in 69 children (53.6% male, median age 4.3 years; range 0.1-16.3 years) were included; 219 (81.4%) of the episodes started outside the hospital, 118 (43.9%) had remote symptomatic aetiology, 96 (35.7%) were accompanied by fever. Half of the CSE episodes lasted

Early Developmental Outcome Following Convulsive Status Epilepticus

Investigators at the Developmental Cognitive Neurosciences Unit, Institute of Child Health, London, other centers in the UK, and Dartmouth School of Medicine, NH, USA, prospectively recruited children aged between 1 and 42 months from North London who had at least one episode of convulsive status epilepticus (CSE) and classified them as prolonged febrile seizures (PFS) or nonfebrile CSE.

Early developmental outcomes in children following convulsive status epilepticus: A longitudinal study

Convulsive status epilepticus (CSE) is the most common pediatric neurologic emergency and is often associated with unfavorable neurodevelopmental outcomes. The early developmental trajectory of children following CSE has not been previously investigated, leaving a gap in our understanding of how these adverse long-term outcomes emerge. We prospectively recruited children aged between 1 and 42 months from a predefined geographic region of North London who had at least one episode of CSE and classified them as prolonged febrile seizures (PFS) or nonfebrile CSE. Neuropsychological and imaging investigations were conducted within 6 weeks of CSE (baseline) and were repeated a year later (follow-up). Neurodevelopment was assessed using the Bayley Scales of Infant Development III and compared to normally developing children. Predictors of neurodevelopmental scores at baseline and follow-up were investigated using regression analyses. Of the 54 children that underwent investigations a mean of 38 days following CSE, 27 had PFS (mean age 18.4 months) and 27 had nonfebrile CSE (mean age 15.5 months). In addition, 17 healthy controls were assessed (mean age 20.49 months). Children with nonfebrile CSE had a worse developmental outcome than children with PFS (p < 0.002), despite there being no differences in seizure characteristics. In contrast to expectations, the PFS group had a worse developmental outcome than controls (p = 0.002). There were no significant differences in performance from baseline to 1-year follow-up for the 70.4% of children who provided data. Seizure characteristics were not shown to be significant predictors of performance. CSE is associated with developmental impairments within 6 weeks of the acute event that continue to be present a year onward. This is also true of PFS cases that under-perform relative to controls despite mean scores within the clinically normal range. The absence of a change in performance from baseline to follow-up as well as the lack of a relationship between seizure characteristics and developmental outcomes supports the notion that premorbid abilities may be overshadowing any direct effects of CSE itself on outcome.

Death within 8 years after childhood convulsive status epilepticus: a population-based study

The risk of long-term mortality and its predictors following convulsive status epilepticus in childhood are uncertain. We report mortality within 8 years after an episode of convulsive status epilepticus, and investigate its predictors from a paediatric, prospective, population-based study from north London, UK. In the current study, we followed-up a cohort previously ascertained during a surveillance study of convulsive status epilepticus in childhood. After determining the survival status of the cohort members, we defined cause of death as that listed on their death certificates. We estimated a standardized mortality ratio to compare mortality in our cohort with that expected in the reference population. Multivariable Cox regression analysis was used to investigate any association between the clinical and demographic factors at the time of status epilepticus and subsequent risk of death. The overall case fatality was 11% (95% confidence interval 7.5-16.2%); seven children died within 30 days of their episode of convulsive status epilepticus and 16 during follow-up. The overall mortality in our cohort was 46 times greater than expected in the reference population, and was predominantly due to higher mortality in children who had pre-existing clinically significant neurological impairments when they had their acute episode of convulsive status epilepticus. Children without prior neurological impairment who survived their acute episode of convulsive status epilepticus were not at a significantly increased risk of death during follow-up. There were no deaths in children following prolonged febrile convulsions and idiopathic convulsive status epilepticus. A quarter of deaths during follow-up were associated with intractable seizures/convulsive status epilepticus, and the rest died as a complication of their underlying medical condition. On regression analysis, presence of clinically significant neurological impairments prior to convulsive status epilepticus was the only independent risk factor for mortality. In conclusion, there is a high risk of death within 8 years following childhood convulsive status epilepticus but most deaths are not seizure related. Presence of pre-existing clinically significant neurological impairments at the time of convulsive status epilepticus is the main risk factor for mortality within 8 years after the acute episode. The attributable role of convulsive status epilepticus on mortality remains uncertain, but appears less than is generally perceived.

Dravet syndrome with an exceptionally good seizure outcome in two adolescents

We present two children who exhibited the characteristics of Dravet syndrome during infancy and young childhood, with SCN1A mutation, but nevertheless achieved seizure freedom for at least four years during adolescence. These patients had no episodes of convulsive status epilepticus with a duration of more than 30 minutes and their overall favourable seizure outcome may be related to the prevention of convulsive status epilepticus.

A long-term follow-up study of Dravet syndrome up to adulthood

Summary Purpose: We intended to elucidate the whole clinical course of Dravet syndrome (DS) comprehensively, from infancy through adulthood. Methods: Subjects were 31 patients with DS (14 with typical DS, and 17 with borderline DS) who were followed from childhood to at least 18 years of age. Their seizures, abilities, and electroencephalography (EEG) findings were investigated and statistically analyzed. Results: The clinical findings of the patients with typical DS and those with borderline DS became largely similar in adolescence and adulthood. Seizures were intractable in childhood in all patients, but suppressed in five (16.1%) during follow-up. Thirty-five (87.5%) of the 40 apparently generalized convulsive seizures that were captured by ictal EEG recording at 7 years of age or later were of focal origin. The seizure-free outcomes were significantly correlated with the experience of <3 episodes of convulsive status epilepticus, and also with disappearance of spikes on the follow-up EEGs. Mental outcomes involving less severe intellectual disability were correlated with the presence of occipital alpha rhythms in the background activity of the follow-up EEGs. Mean age at the recording of the follow-up EEGs was 23.8 years. Discussion: Prevention of the occurrence of convulsive status epilepticus was indicated to be critically important for the improvement of seizure prognosis in DS.

A long‐term follow‐up study of Dravet syndrome up to adulthood

We intended to elucidate the whole clinical course of Dravet syndrome (DS) comprehensively, from infancy through adulthood. Subjects were 31 patients with DS (14 with typical DS, and 17 with borderline DS) who were followed from childhood to at least 18 years of age. Their seizures, abilities, and electroencephalography (EEG) findings were investigated and statistically analyzed. The clinical findings of the patients with typical DS and those with borderline DS became largely similar in adolescence and adulthood. Seizures were intractable in childhood in all patients, but suppressed in five (16.1%) during follow-up. Thirty-five (87.5%) of the 40 apparently generalized convulsive seizures that were captured by ictal EEG recording at 7 years of age or later were of focal origin. The seizure-free outcomes were significantly correlated with the experience of <3 episodes of convulsive status epilepticus, and also with disappearance of spikes on the follow-up EEGs. Mental outcomes involving less severe intellectual disability were correlated with the presence of occipital alpha rhythms in the background activity of the follow-up EEGs. Mean age at the recording of the follow-up EEGs was 23.8 years. Prevention of the occurrence of convulsive status epilepticus was indicated to be critically important for the improvement of seizure prognosis in DS.

Convulsive status epilepticus in children

Convulsive status epilepticus is the most urgent neurological medical emergency in children. Generalized convulsive status epilepticus is the most common and life-threatening type of status epilepticus. It is not a syndrome in the same sense as febrile convulsions, benign rolandic epilepsy, and infantile polymorphic epilepsy. These latter disorders have a tight age frame, seizure semiology, and a reasonably predictable outcome. Episodes of convulsive status epilepticus can occur in each: occasionally in symptomatic and febrile convulsions, and Lennox Gastaut syndrome, rarely in benign rolandic epilepsy, and West syndrome. Status epilepticus has many causes, which vary depending on the age and patient population. Convulsive status epileptucus continues to be associated with significant neurological morbidity and mortality, with different hazards and outcome. Although the outcome is dependent on etiology, it is known that appropriate early management may reduce mortality and some of the morbidity associated with convulsive status epilepticus. Status epilepticus is a disorder in which the mechanisms attempting at terminating the seizure fail. Continued convulsive activity in convulsive status epilepticus results in decompensation of all organs and systems, thus being life threatening. Seizure activity in convulsive status epilepticus is associated with neuronal damage. The aim should be to halt this activity urgently, using, ideally, a 100% effective drug, administered quickly, without compromising the consciousness level or producing other negative effects on cardiovascular, respiratory function or other unexpected effects.

Analysis of Convulsive Status Epilepticus in Children of Taiwan

Convulsive status epilepticus is a medical emergency with significantly associated mortality and morbidity. The demographic data and outcomes of convulsive status epilepticus in children were collected for descriptive analysis. We retrospectively reviewed cases of convulsive status epilepticus in the Pediatric Intensive Care Unit of Chang Gung Children's Hospital between 1999 and 2006. We enrolled 141 patients with 198 episodes of convulsive status epilepticus, aged 2 months to 18 years: 24.8% of first episodes developed convulsive status epilepticus, with a duration of over 60 minutes. First episodes of convulsive status epilepticus were most often evidenced in febrile status during acute central nerve system infections (48.2%), and in nonfebrile status during acute noncentral nerve system illness in previously epileptic children (28.4%). Before their first episode, 63.8% of children were neurologically healthy, and 12.2% exhibited a prolonged febrile seizure. The most common etiology of mortality was acute central nervous system infection. The immediate mortality rate was 7.1%. Convulsive status epilepticus in childhood is more common, with a different range of causes and a lower risk of death, than convulsive status epilepticus in adults. Acute central nervous system infections appear to be markers for morbidity and mortality.

Status epilepticus in resource‐poor countries

Status epilepticus is common in patients admitted to hospitals in resource poor countries (RPC). However, there appear to be differences in the epidemiology, aetiology and outcome of status epilepticus in these regions compared to the West, although there is little data from the former regions. RPC countries are those classified by the World Bank as low income or middle-low income countries encompass those situated in the tropical and subtropical regions. The International League Against Epilepsy (ILAE) definition of status epilepticus (ILAE, 1993) is problematic in these areas, since patients often present to health facilities, without adequate documentation of the duration of convulsion. Thus more pragmatic definitions have been developed (Sadarangani et al., 2008). Most studies on status epilepticus in RPC describe convulsive status epilepticus (CSE) (Misra et al., 2008;Sadarangani et al., 2008), since non-convulsive epilepticus is rarely detected in these regions. Although one study non-convulsive status epilepticus was detected in 11% of adults with altered mental status (Narayanan and Murthy, 2007). The incidence of convulsive status epilepticus appears to be higher in RPC than the West, but there has been only one epidemiological study conducted. In this study, the incidence of children fulfilling the ILAE definition of CSE who presented to a Kenyan District General Hospital was 35/100,000/year, with 268/100,000/year in children aged 1-11 months old. The incidence is higher if those with probable epilepsy are also included (35/100,000/year, with 268/100,000/year). These figures are likely to be an underestimate; since many children with CSE are not admitted hospital, either because convulsions are thought to be caused by traditional causes or the children die before they reach hospital. Thus the rate in this area of Kenya is 2-5 times that of similar study in London, United Kingdom (Chin et al., 2006). CSE appears to be more common in children than adults; mainly because acute symptomatic seizures or febrile status epilepticus are more common, particularly in RPC(Sadarangani et al., 2008;Tabarki et al., 2001). A significant proportion of people with epilepsy in RPC have an episode of CSE, usually in childhood and often as the first reported seizure. Thus in a cross-sectional study of active convulsive epilepsy in Kenya, 35% of those identified with active convulsive epilepsy had a history of prolonged seizures (probable CSE), of which 75% were associated with a febrile illness(Edwards et al., 2008). Thus, the high incidence of acute symptomatic epilepsy in many RPC may account for the increased incidence and prevalence of epilepsy in these regions. Most CSE is symptomatic, with the increased incidence attributed to the increased incidence of infections (Misra et al., 2008;Sadarangani et al., 2008). In malaria endemic areas, malaria is an important cause of CSE in children (Sadarangani et al., 2008), whist bacterial meningitis and viral encephalitis are important causes in other areas (Murthy et al., 2007). Patients appear to be in status for longer periods, although there is little reliable data on the duration of CSE in patients presenting to hospital. The increase duration may be due to the lack of treatment prior to admission to hospital, inadequate treatment on admission to hospital and/or a reduction in the responsiveness to the benzodiazepines. The mortality associated with CSE in RPC is greater (11-15%), both in adults (Murthy et al., 2007) and children (Sadarangani et al., 2008); but the long-term outcome in terms of premature mortality and neurocognitive sequelae is undetermined. Risk factors for mortality i.e. young age (< 1 years), aetiology (particularly bacterial meningitis), focal seizures and duration of seizure, are similar to the West. The neuro-cognitive outcome of CSE appears worse with 36% of Tunisian children having intellectual disability or epilepsy (Tabarki et al., 2001). The risk factors for neuro-cognitive impairment include young age (<1 year), focal seizures. Many hospitals in RPC, particularly those in rural areas have few drugs to treat CSE, and many hospitals do not have intensive care facilities such as ventilators or sufficient staff to provide optimal management. The antiepileptic drugs are limited to benzodiazepines, particularly Diazepam and Phenobarbital. However, the response to diazepam appears poorer in many areas, either because the patients present with seizures lasting many hours or possibly the aetiology such as malaria makes them more resistant to these compounds. The treatment of CSE in RPC is further complicated by the lack of rectal preparations of diazepam (parenteral preparation is often used, but has erratic absorption (Ogutu et al., 2002)) and lack of supply of parenteral Phenobarbital (Wilmshurst and Newton, 2005). These issues may affect the outcome. Thus, CSE appears to be more common, with a worse outcome in RPC than in the West; but at present, there is little data on which to base the burden and recommendations of treatment in these countries.

Recurrent Takotsubo Cardiomyopathy Triggered by Convulsive Status Epilepticus

Takotsubo cardiomyopathy can complicate several conditions including neurological emergencies. A few recurrent cases associated with seizures have been reported, but none of the patients had status epilepticus. The pathophysiology of takotsubo syndrome, although debated, may involve stunning of the myocardium by a catecholamine storm triggered by stress. Patients with epilepsy may be at increased risk for takotsubo syndrome, which may occur repeatedly. We report on a postmenopausal woman with symptomatic epilepsy who experienced recurrent takotsubo cardiomyopathy triggered by convulsive status epilepticus. Brief seizures were not associated with takotsubo syndrome. The relevant literature was reviewed. Over a 1-year period, she experienced two episodes of convulsive status epilepticus with complete neurological recovery after treatment. Echocardiography showed latero-septo-apical hypokinesia and apical ballooning. The cardiac abnormalities resolved fully and she recovered her baseline level of self-sufficiency. During the same period, she experienced several brief seizures, with no cardiac manifestations. The occurrence of takotsubo cardiomyopathy in association with convulsive status epilepticus, but not with brief seizures, supports neurogenically mediated myocardial stunning related to direct toxicity of endogenous catecholamines. Neuro-intensivists must be aware of this potentially fatal but fully reversible cardiac complication, which may be among the causes of death in patients with status epilepticus.

Incidence, cause, and short-term outcome of convulsive status epilepticus in childhood: prospective population-based study

Convulsive status epilepticus is the most common childhood medical neurological emergency, and is associated with significant morbidity and mortality. Most data for this disorder are from mainly adult populations and might not be relevant to childhood. Thus we undertook the North London Status Epilepticus in Childhood Surveillance Study (NLSTEPSS): a prospective, population-based study of convulsive status epilepticus in childhood, to obtain a uniquely paediatric perspective. Clinical and demographic data for episodes of childhood convulsive status epilepticus that took place in north London were obtained through a clinical network that covered the target population. We obtained these data from anonymised copies of a standardised admission proforma; accident and emergency, nursing, ambulance, and intensive-care unit notes; and interviews with parents, medical, nursing, and paramedic staff. We investigated ascertainment using capture-recapture modelling. Of 226 children enrolled, 176 had a first ever episode of convulsive status epilepticus. We estimated that ascertainment was between 62% and 84%. The ascertainment-adjusted incidence was between 17 and 23 episodes per 100,000 per year. 98 (56%, 95% CI 48-63) children were neurologically healthy before their first ever episode and 56 (57%, 47-66) of those children had a prolonged febrile seizure. 11 (12%, 6-18) of children with first ever febrile convulsive status epilepticus had acute bacterial meningitis. Conservative estimation of 1-year recurrence of convulsive status epilepticus was 16% (10-24%). Case fatality was 3% (2-7%). Convulsive status epilepticus in childhood is more common, has a different range of causes, and a lower risk of death than that in adults. These paediatric data will help inform management of convulsive status epilepticus and appropriate allocation of resources to reduce the effects of this disorder in childhood.

Unfavourable outcome of Hashimoto encephalopathy due to status epilepticus

Sirs: A 27-year-old woman with a history of Hashimoto’s thyroiditis presented with psychotic symptoms and myoclonus. Four months earlier, her relatives had noticed myoclonic jerks in the four limbs and depression. During the following weeks, the patient also manifested visual hallucinations and generalized tonic-clonic seizures. In addition, progressive cognitive deterioration and slowed psychomotor activity became evident. One month later, she experienced two episodes of convulsive status epilepticus lasting over 50 minutes that resolved after intravenous lorazepam and phenobarbital had been given. On admission to our department, neurological examination revealed disorientation, fine finger tremor and spontaneous and stimulus-induced myoclonus at the four limbs. Neuropsychological evaluation showed Mini-Mental Status Examination’s score of 23/30, verbal memory deficit, frontal dysfunction and impaired attention. The patient was taking L-thyroxine, 100 μg/day, valproate, 750 mg/day and phenobarbital, 100 mg/day. Laboratory analysis demonstrated high titres of thyroid microsomal antibodies (1781 IU/ml; normal < 60) antithyreoglobulin antibodies (127.5 IU/ml; normal < 28.7) and TSH (40.9 μU/ml; normal < 4.2 μU/ml). FT3 and FT4 were within the normal limits. Antinuclear and antiDNA antibodies were normal. Serological test for syphilis and HIV were negative. CSF examination showed slightly elevated protein levels (55 mg/dl, normal < 40) and two oligoclonal bands. 14-3-3 protein was absent and there was no evidence of bacterial or viral infection. EEG showed diffuse slowing of background activity and bifrontal theta activity. Photic stimulation induced massive myoclonus in the four limbs (Figure). Brain MRI and 99Tc SPECT were unremarkable. Genetic study excluded Unverricht-Lundborg and Lafora disease, and MERRF. As diagnosis of Hashimoto encephalopathy (HE) was made, intravenous treatment with methylprednisolone 1 g followed by prednisolone 40 mg/day was started. No significant improvement was observed and, 6 days later, the patient manifested progressively increasing massive myoclonus, culminating in convulsive status epilepticus. Intravenous benzodiazepines failed to interrupt status epilepticus and pentothal anesthesia was initiated. Ictal activity was never fully suppressed at EEG prolonged monitoring. The patient died after a prolonged comatose state from cardiac failure two weeks later. Autopsy was performed. Microscopic examination revealed focal infiltration of thyroid by lymphocytes. Fixed brain tissue was paraffin-processed, and 10 μm sections were obtained. Gross examination revealed few hemorrhagic areas in the pons. Immunohistochemical staining by using the avidin-biotinperoxidase complex method for assessment of astrocytes (glial fibrillary acid protein), microglia (HLA-DR) and T cells (CD3, CD4, CD8, CD19, CD45), did not show lymphocytic infiltrates. PrPSc staining was negative and no evidence of PAS+ inclusion bodies was found in the cerebral parenchyma. The clinical picture of our patient suggested the diagnosis of diffuse (type II) HE [1, 2]. HE is defined by the association of encephalopathy with the presence of antithyroid antibodies [1, 2]. Steroid treatment is usually effective but refractory cases have been reported [2]. Accordingly, clinical LETTER TO THE EDITORS

Convulsive status epilepticus in children with intractable epilepsy is frequently focal in origin.

Convulsive status epilepticus (CSE) is a common neurological emergency. Our objectives were to study children with recurrent nonfebrile CSE to assess the evidence for focal origin. Series of 18 children with recurrent CSE and intractable epilepsy were identified by chart review. Clinical, radiological, and EEG data were reviewed. Focal structural abnormalities were identified on MRI and CT images by one neuroradiologist who was unaware of the clinical details. The patient's ages ranged between 6-22 years (mean 15.3, SD 4), and 67% were males. Most children (89%) had a severe cognitive and / or behavioural disorder. Most patients (89%) had multiple seizure types and 95% of these were partial seizures. Twelve (67%) children had at least one episode of CSE with focal features identified clinically. Focal brain abnormalities were detected on 18% and 55% of CT and MRI films respectively. Overall, 53% had a focal abnormality on structural neuroimaging. Interictal EEG revealed focal or multifocal abnormalities on at least one occasion in 94% and 22% of patients respectively. Overall, 17 patients had focal features on at least one EEG. Thirteen ictal EEGs were recorded on 11 (61%) patients. Ten (91%) of these recordings revealed a focal onset. Many handicapped children with recurrent CSE have focal clinical, radiological, or electrographic features. This supports a focal origin for CSE in most children with intractable epilepsy.

The association between seizure clustering and convulsive status epilepticus in patients with intractable complex partial seizures.

We examined the association between seizure clustering and convulsive status epilepticus (SE) in patients with intractable complex partial seizures, to identify whether patients whose seizures typically cluster are at high risk for convulsive SE (CSE). Seventy-six patients with intractable complex partial epilepsy who underwent presurgical evaluation in the Montefiore Epilepsy Management Unit from 1993 to 1997 were contacted and interviewed about typical seizure frequency and distribution and history of CSE. Seizure clustering was defined as three or more complex partial seizures within a 24-h period, with return to baseline between seizures. Of the 76 patients contacted, 21 (28%) had experienced at least one episode of CSE, and 36 (47%) typically experienced clustered seizures. SE occurred in 16 (44%) of 36 patients with clustered seizures, and in five (12.5%) of 40 patients with nonclustered seizures (p < 0.002). Of 53 patients with temporal lobe epilepsy, CSE occurred in 13 (50%) of 26 patients with clustered seizures, and four (14.8%) of 27 patients with nonclustered seizures (p < 0.006). Patients with intractable complex partial or localization-related epilepsy who typically experience seizure clustering are at a significantly higher risk for CSE than are patients with nonclustered seizures.

Bilateral occipital calcification, epilepsy and coeliac disease: clinical and neuroimaging features of a new syndrome.

Twenty patients affected by bilateral occipital cortical-subcortical calcification (BOC) are described, 19 (95%) had epilepsy. In 8 of 16 cases studied, intestinal biopsy revealed coeliac disease. Fourteen patients had occipital partial epilepsy with a relatively benign outcome, while 4 patients were affected by a severe form of epilepsy, with very frequent, drug-resistant, generalised and partial seizures with mental deterioration. One patient had a single episode of convulsive status epilepticus at four months of age. The neurological examination was normal in all patients. CT showed flocculo-nodular, cortico-subcortical BOC, without enhancement and without lobar or hemispheric atrophy. MRI was normal. The clinical and neuroimaging features of these patients are different therefore from those with the Sturge-Weber Syndrome. The study confirms a high prevalence of coliac disease in patients with BOC, but the relationship between these two pathologies still needs to be clarified.

Status epilepticus in children: etiology, clinical features, and outcome.

Between August 1984 and September 1986, data were gathered prospectively on 114 episodes of convulsive status epilepticus, defined as seizure duration longer than 30 minutes, affecting 97 children. Status epilepticus was symptomatic in 72% (chronic 59%, acute 13%) and idiopathic or febrile in 28%. We identified precipitating factors in 63% of episodes. The most common factors were inadequate blood levels of anticonvulsants (32 of 60 episodes in children with prior seizures) and febrile illnesses, excluding meningitis or encephalitis (38 of 114 episodes). There was an elevated peripheral white blood cell count in 60%, acidosis with a pH of less than 7.0 in 12.5%, and cerebrospinal fluid pleocytosis not due to meningitis or encephalitis in 8 of 64 episodes. Eight children died, three with severe pre-existing brain damage, two with meningitis, and two with a poorly defined encephalopathy. Eighteen children developed a new neurologic deficit. Outcome was associated with the etiology and duration of status epilepticus, with age at the time of status a minor factor. A permanent deficit occurred in only five children with idiopathic or febrile status epilepticus.