Clinical, EEG, and quantitative MRI differences in pediatric frontal and temporal lobe epilepsy.

OBJECTIVE: To examine the clinical, electrographic, and quantitative MRI differences between frontal lobe (FLE) and mesial temporal lobe epilepsy (MTLE) in children. METHODS: The population included children who underwent video-EEG monitoring between 1995 and 2000 who were classified as either FLE (n = 39) or MTLE (n = 17) according to the criteria of the International League Against Epilepsy. Clinical, EEG, and quantitative MRI data (including frontal cortical volumes) were compared between the two syndromes and a control group (n = 42). RESULTS: In FLE, seizures were significantly briefer, more frequent, and predominantly from sleep, and had differing motor characteristics. The rates of bilateral epileptiform interictal and ictal EEG abnormalities were significantly higher in FLE. A nonlesional MRI was significantly more common in FLE. Mean frontal cortical volume in FLE was significantly lower than MTLE and controls. Seizure freedom after surgery was lower in FLE. CONCLUSIONS: The clinical syndrome of FLE is clearly distinct from MTLE. The etiology of this disorder is unknown in the majority of cases despite extensive investigation. Because of a lack of a clearly defined etiology and frequent nonlateralizing EEG changes, few of these children are considered optimal surgical candidates. The demonstration of bilateral frontal cortical volume loss and bilateral EEG abnormalities suggests that FLE is a bilateral disease in a high proportion of patients. The outcome in those patients who were deemed surgical candidates was significantly worse than the MTLE cases.

Evaluation of SPECT in the assessment and treatment of intractable childhood epilepsy.

The authors retrospectively examined the role of SPECT in 65 children undergoing video-EEG telemetry. SPECT was concordant in most children whose lesions were already localized by MRI and epilepsy syndrome and provided localizing data in more than half not localized by these modalities. Ictal SPECT provided no additional prognostic benefit in patients undergoing epilepsy surgery (n = 23) who have a localized MRI lesion. In patients without lesions, however, ictal SPECT provides useful additional localization that may be used as a guide to intracranial implantation.

Staring episodes in children analyzed by telemetry.

Staring episodes in children may be ictal or nonictal, and telemetry helps make this distinction. Twenty-seven children referred to our service for elucidating the nature of their staring spells were studied by telemetry. No staring events were recorded in four children. The staring events were not associated with electroencephalographic (EEG) changes in 12 children. In 11 children, the staring events had EEG accompaniments: four had generalized spike-and-wave changes; three had focal or asymmetrical changes; and four had generalized decrement (desynchronization), which has not been described before as an electrical correlate of staring. EEG video telemetry in this group of patients led to accurate diagnosis and appropriate medical, surgical or behavioral management.

Neonate with benign familial neonatal convulsions: recorded generalized and focal seizures.

A neonate with benign familial neonatal convulsions is presented. Ictal electroencephalography demonstrated a seizure of right frontal onset with generalization and one of right frontal onset which remained confined to that hemisphere. Very few ictal recordings of this entity exist. In this patient a partial seizure and a seizure with generalization were recorded, emphasizing the difficulties with the present classification.

Outcome of neonates with electrographically identified seizures, or at risk of seizures.

A prospective study was conducted to investigate survival at 1 month and survival and developmental outcome at 1 year in a cohort of 53 neonates either suspected of or at risk of having seizures. For all patients, presence of seizures, diagnoses, and structural abnormalities were identified. If seizures were present, seizure variables were quantified. Correlations between neonatal parameters and subsequent outcome were investigated. Forty-three patients survived the first month of life. Background EEG was the only significant predictor of survival at 1 month. Three patients died after 1 month, and 2 of the three had extremely depressed interictal EEGs. Development outcome at 1 year was determined for all available surviving patients. Abnormal findings from brain imaging studies and number of independent electrographic seizure foci were correlated with some aspects of outcome at 1 year. No other correlations were identified between neonatal parameters and outcome.

The health-related quality of life of childhood epilepsy syndromes.

OBJECTIVE: There is increasing awareness of the importance of assessing physical, psychological, social and behavioural well-being in chronic disease. The aim of this study was to examine the health-related quality of life (HRQoL) of children with common epilepsy syndromes and to explore if there are HRQoL differences between those syndromes. METHODS: Each child had their epilepsy syndrome defined according to the International League Against Epilepsy classification. Epilepsy syndromes included symptomatic frontal, temporal, parietal/occipital lobe and partial unlocalized epilepsy, and two idiopathic epilepsies, childhood absence epilepsy (CAE) and benign rolandic epilepsy (BRE). Seizure semiology and ictal/interictal electroencephalogram (EEG) were determined for symptomatic partial epilepsy syndromes by video-EEG monitoring. HRQoL was evaluated with an epilepsy-specific instrument, the Quality of Life in Childhood Epilepsy Questionnaire, and two generic instruments, the Child Health Questionnaire and Child Behavior Checklist. RESULTS: Children with symptomatic partial epilepsy syndromes were affected by epilepsy in a similar way and did not have unique HRQoL profiles. However, these children had significantly lower HRQoL scores compared to those with CAE or BRE. All children with epilepsy regardless of syndrome had a higher frequency of behavioural problems compared to normative data. CONCLUSION: These results indicate that children with epilepsy regardless of syndrome require evaluation of the psychosocial implications. There is a greater impact on HRQoL in symptomatic epilepsy compared to idiopathic epilepsy. Specific symptomatic partial syndromes did not differ in the degree they affect HRQoL. These findings have important implications for clinicians caring for children with epilepsy.

The effects of morphine and midazolam on EEGs in neonates.

We investigated the effect of morphine, midazolam and their active metabolites on background electroencephalogram (EEG) in 6 neonates undergoing extracorporeal membrane oxygenation (ECMO) by conducting simultaneous EEGs and serum drug levels. Despite serum levels that were sufficient to produce adequate sedation, no patients had burstsuppressed or flat EEG backgrounds. We did, however, note that scalp oedema caused by prolonged immobility led to artefactual attenuation of EEG background. We conclude that an EEG prior to sedation will provide important baseline information that allows subsequent comparison and an awareness that scalp oedema after prolonged sedation and immobility will reduce misinterpretation of artefactual EEG attenuation.

The impact of epilepsy surgery on quality of life in children.

OBJECTIVE: To determine if epilepsy surgery is effective in improving the quality of life (QOL) of children with intractable seizures using the Quality of Life in Childhood Epilepsy Questionnaire (QOLCE). METHODS: The authors conducted a prospective study of the families of 35 children with intractable epilepsy who underwent epilepsy surgery. Parents completed the QOLCE preoperatively and again 6 to 18 months after surgery. At both assessment dates parents indicated the severity of their child's seizures during the past 6 months and the frequency of their child's seizures during the past 4 weeks on Likert-type scales. Children were split into two groups according to surgery outcome: seizure free vs persistent seizures. Statistical analyses were conducted to determine if children rendered seizure free showed a greater improvement in QOL compared to those with persistent seizures postoperatively. RESULTS: Greater improvement in QOL was documented for children rendered seizure free vs children with persistent seizures. This was significant for the overall QOLCE QOL score and subscales assessing cognitive, social, emotional, behavioral, and physical domains of life. CONCLUSIONS: Epilepsy surgery improves the quality of life of children rendered seizure free. Families can be counseled preoperatively of the potential benefits of surgery beyond seizure reduction.

Cramps following exercise.

A 13-year old boy presented with a 10-year history of severe muscle cramps experienced an hour after prolonged exercise. There was no history of exercise intolerance or myoglobinuria. A muscle biopsy showed a lipid myopathy and a deficiency of muscle carnitine palmityl transferase. He has responded to a high carbohydrate, low fat diet with added carbohydrate intake preceding extensive exercise. Diagnosis of this entity before an episode of rhabdomyolysis is unusual.

Complex partial seizures in young children.

We retrospectively analyzed the clinical manifestations of complex partial seizures (CPS) in children aged < or = 10 years using video and EEG telemetry and evaluated their course, investigations, management, and seizure status at follow-up. Seventeen patients with CPS were studied at the Prince of Wales Children's Hospital (POWCH) and Prince Henry Hospital (PHH) between 1987 and 1992. Because 15 of the 17 patients had intractable seizures, the population was selective. Mean age was 6.5 years; 4 patients were aged < or = 2 years. Clinical features were normal or mild intellectual handicap (13); hemiplegia (5); and infantile spasms preceding CPS (4); of these, 2 also had simple partial motor seizures. Structural abnormalities were noted on scanning in 9 patients. Eighty-seven seizures were reviewed. Mean duration of each clinical seizure was 59.7 s (total population), 108 s (subgroup 1, aged < or = 2 years), and 48.5 s (subgroup 2, aged > 2 years). Major ictal manifestations were auras (9), staring (9), autonomic changes (6), and automatisms (17). In subgroup 1, automatisms were simple and mainly oroalimentary and gestural. Two patients had no change in surface ictal recordings, and 2 had normal interictal EEGs. At follow-up, 8 patients were seizure-free for 6 months, 1 was partially controlled (more than two seizures a month), and 8 had intractable seizures (two or more seizures a month). Seven patients underwent operation for intractable epilepsy, and 4 achieved a class 1A outcome (Engel classification).

Spatial and temporal characteristics of neonatal seizures.

Thirty-two neonates (26 term and 6 premature) having seizures were prospectively recruited and studied. Using prolonged video/EEG monitoring, we quantified seizure variables (electrographic and clinical seizure durations, interictal periods and electrographic seizure spread) for all 1,420 seizures recorded. The effects of time and antiepileptic drug (AED) therapy were analyzed statistically. Seizures were generally frequent, with limited electrographic spread. However, some neonates had consistently longer interictal periods and 13% had mean interictal periods > 60 min. Seizure variables were relatively stable over time, but they changed with AED therapy. There was a trend to decreased seizure duration, increased length of interictal periods, and decreased electrographic spread. Furthermore, there was evidence of reduced clinical features after sequential AED infusions. Seizures ceased during the monitoring period in 22 neonates. Eighty-five percent of all seizures had no clinical manifestations. Among neonates with clear clinical correlates, clinical observations underestimated electrographic seizures in individual neonates by a mean of 54% (range 0-95%). Seizures generally had limited electrographic spread. Use of only four recording electrodes, characteristic of some portable EEG systems, underestimated seizures in 19 neonates, and missed all seizures in 2.

Video EEG analysis of non-ictal events in children.

Over a 3 year period 186 children aged 3 weeks to 17 years were studied by telemetry (prolonged video and EEG monitoring) at the Prince of Wales Children's Hospital: 74 had events considered at referral to possibly represent seizures but which were shown by clinical analysis and telemetry to be non-ictal. Nine such patients were developmentally delayed, one was neurologically impaired and 16 were both developmentally delayed and neurologically impaired. A specific diagnosis of the non-ictal events was reached in 24 subjects-postures of spasticity in children with neurological impairment (6), Münchausen-by-proxy (5), pseudoseizures (3), breathholding (2), masturbation (2), reflux (2), shudder (1), movement disorder (1), motor tic (1) and pertussis (1). Specific descriptive patterns were assigned to the remaining 51 events. These included staring (20) and jerks (16) or unusual behaviour (15). 49 inter-event EEGs were normal; 7 displayed abnormal background rhythms and 19 showed epileptiform activity. We discourage use of the term 'pseudoseizure' for the majority of the events described and prefer that a specific diagnosis be made or a descriptive analysis be provided. The events seen illustrate the wide spectrum of childhood behaviour and on occasions suggest the need for telemetry to determine their true nature.

Single photon emission computed tomography in intractable infantile seizures.

We aimed to determine the site of ictal foci and the pathogenesis of seizures in 4 infants with intractable seizures. The patients were studied using simultaneous video and electroencephalographic (EEG) monitoring, structural studies and ictal and interictal single photon emission computed tomography (SPECT). Ictal neurophysiology showed multifocal seizure propagation in Patients 1 and 2 and generalised abnormal electrical patterns in Patients 2, 3 and 4. Magnetic resonance imaging (MRI) demonstrated a focal abnormality in Patient 4. SPECT studies showed focal or multifocal increased uptake in 3 subjects (Patients 1,3,4) and increased uptake in the thalamic and basal ganglia regions of 2 subjects (Patients 2,3). SPECT studies contributed to an understanding of the pathogenesis of seizure initiation and propagation in the 4 patients studied.

Non-accidental head injury in children.

AIMS: To analyse the clinical features, social circumstances, investigations and outcome of 49 children with significant non-accidental head injury admitted to The Prince of Wales Children's Hospital between 1979 and 1993. CLINICAL FEATURES: Age ranged from one month to eight years, with 30 children less than one year old. There were 28 boys and 21 girls. Thirty-three were in the care of a parent. Eleven of these were known to the Department of Community Services and 22 were from families with stressed socioeconomic circumstances characterised by deprivation. In 44 cases the history was incompatible with the clinical findings. On admission, 21 had marked depression of awareness, with a Glasgow Coma Scale score (GCS) of 9 or less. Eleven of this group were fitting. Seizures were present in a further 13 children who were semi-purposeful (GCS > 9). Twenty-eight had retinal haemorrhages and 35 had soft tissue scalp contusions, 23 in association with skull fracture. Twenty-six had associated injuries. Seven children died. Of the 42 survivors at the time of discharge, 19 had severe motor disability associated with other deficits. Twenty-four were returned to the care of their families. CONCLUSIONS: Retinal haemorrhage and unconsciousness on admission (GCS < or = 9) were positively associated with a poor outcome (P < 0.001 and 0.002, respectively). Cerebral oedema found on computerised tomography was positively associated with severe motor disability on discharge (P < 0.001). Lowest socioeconomic circumstances appeared to be a risk factor for occurrence of injury: 22 of 49 children came from circumstances of deprivation.

Prolactin and seizure activity.

Prolactin secretion after tonic-clonic seizures (10 patients), complex partial seizures (five) and non-epileptic attacks (three) was studied in a group of children aged between 0.3 and 14 years. Seven patients with other subcategories of seizure disorders were also studied. Eight children with tonic-clonic seizures exhibited post ictal concentrations of prolactin greater than 500 mU/l. One of the children, who responded on one occasion, did not do so on another. Three children with complex partial seizures had post ictal prolactin concentrations greater than 500 mU/l, while in two the increased values were more modest (390 mU/l and 420 mU/l). The timing of the peak post ictal prolactin concentration varied from less than 20 minutes to a prolonged plateau for three hours. Other seizure types--simple partial with motor signs (2), absence seizure (1), myoclonic seizure (1), minor epileptic status (3) (with one exception), and non-epileptic attacks (3) were not associated with post ictal concentrations greater than 500 mU/l.

Transient zinc deficiency in a full-term breast-fed infant of normal birth weight.

A full-term male infant was seen at age 5 months with symptomatic zinc deficiency. He was breast fed and the mother's milk zinc levels were low. The infant responded to oral zinc supplements and has continued to be asymptomatic for 12 months after their withdrawal. This is the first report of transient zinc deficiency in an otherwise healthy, breast-fed, full-term infant of normal birth weight.

Multiple sclerosis in childhood: a new look.

Five patients (four female, one male) with multiple sclerosis were managed at the Hospital for Sick Children, London, between January 1978 and June 1983. The age at which first symptoms occurred varied from three to 14 years. Focal seizures occurred in three cases and an encephalopathic process in three cases, and there was evidence of progressive intellectual deterioration in all five children. Computed tomography showed low-density lesions in the white matter of all five. Electroencephalograms were abnormal in all cases, and in two the disturbance was severe. Oligoclonal bands were present in the cerebrospinal fluid of two children. Four of the five children had abnormal responses to pattern-reversal stimulus.

Epilepsy surgery in Sturge-Weber syndrome.

A patient with Sturge-Weber syndrome developed seizures at the age of 4 years. At 13 years of age, she had intractable complex partial seizures with marked visual symptomatology. Interictal encephalograms showed bilateral slow activity, more marked over the right hemisphere with epileptogenic activity maximal in the right temporal region. Serial computerized axial tomography scans demonstrated evolution of bilateral occipital lesions with calcification and adjacent low density areas that were more marked on the right. Magnetic resonance imaging, angiography and neuropsychological evaluations were performed. An extensive resection of the right occipital lobe was achieved. One year after surgery, the patient has had seven brief seizures. Delineation of an epileptogenic focus and surgical removal of the lesion in patients with intractable seizures can now be considered in selected patients with bilateral central nervous system pathology.

Clinical features of neonatal seizures.

OBJECTIVE: Identification of seizures in neonates is difficult. This study analyses the clinical features of seizures in a cohort of neonates. METHODOLOGY: The clinical events of 41 neonates referred for investigation of suspected seizures were studied with prolonged video/electroencephalographic (EEG) telemetry. RESULTS: Sixteen neonates had no seizures recorded; 25 had confirmed seizures, 13 with clinical correlates. Each neonate with electroclinical seizures had paroxysms of abnormal movements in stereotyped patterns. These patterns were consistently found to have electrical correlates. Focal clonic movements were seen most frequently. Multiple clinical features characterized the seizure repertoire in six neonates. In five neonates the clinical features became less evident during monitoring and these seizures were difficult to recognize. This change was associated with anticonvulsant administration in three cases. CONCLUSIONS: Electroclinical seizures are characterized by abnormal paroxysmal stereotyped behaviour, often with multiple clinical features. Recommendations for the management of abnormal neonatal events are proposed.

The obesity hypoventilation syndrome and the Prader-Willi syndrome.

Fourteen children with the Prader-Willi syndrome have been managed at the Royal Alexandra Hospital for Children between the years 1964-1980--twelve male, two female. Six male children developed features of the obesity hypoventilation syndrome. The age of onset of this complication ranged from 4.0 to 12.6 years. With one exception those children with the obesity hypoventilation syndrome were more obese than those without it. At the time of onset of the syndrome, five of six patients had weights greater than or equal to 6.5 standard deviations above ideal body weight. Those children without the obesity hypoventilation syndrome had a range of standard deviations 1.0 to 4.2 above the ideal body weight. In four of six cases weight reduction and a cardiac failure regimen resulted in reversal of the obesity hypoventilation syndrome. With two of the six children there had been cardiomegaly and increased pulmonary venous vascularity on x-ray at a chronological age of three months. Two of the six children died.