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Biomedical subjects

J Maytal

Publications and source records attributed to J Maytal.

At least 19 recordsLinked to original sources

Short-term outcomes of children with febrile status epilepticus.

Febrile status epilepticus (SE) represents the extreme end of the complex febrile seizure spectrum. If there are significant sequelae to febrile seizures, they should be more common in this group. We have prospectively identified 180 children aged 1 month to 10 years who presented with febrile SE over a 10-year period in Bronx, New York, and Richmond, Virginia. They were compared with 244 children who presented with their first febrile seizure (not SE) in a prospective study done in the Bronx. The mean age of the children with febrile SE was 1.92 years, and of the comparison group, 1.85 years. Duration of SE was 30-59 min in 103 (58%), 60-119 min in 43 (24%), and > or =120 min in 34 (18%). Focal features were present in 64 (35%) of cases. There were no deaths and no cases of new cognitive or motor handicap. Children with febrile SE were more likely to be neurologically abnormal (20% vs. 5%; p < 0.001), to have a history of neonatal seizures (3% vs. 0; p = 0.006) and a family history of epilepsy (11% vs. 5%; p = 0.05) and less likely to have a family history of febrile seizures (15% vs. 27%; p = 0.01) than were children in the comparison group. The short-term morbidity and mortality of febrile SE are low. There are differences in the types of children who have febrile SE compared with those who experience briefer febrile seizures. Long-term follow-up of this cohort may provide insight into the relationship of prolonged febrile seizures and subsequent mesial temporal sclerosis.

Age Distribution↗

Biphasic course of infant botulism.

The syndrome of infant botulism, characterized by constipation, poor feeding, hypotonia, poor head control, and bulbar involvement, is typically a monophasic disease. We describe a 7-month-old infant with a recurrence of illness 13 days after resolution of the presenting signs. The source of infection was unknown and the only potential risk factors were exclusive breastfeeding and decreased bowel movements, which by themselves cannot explain the recurrence. Although treatment with botulism immunoglobulin is now suggested for the acute phase of infantile botulism, its use for recurrence is controversial.

Botulism↗

Headaches in a pediatric emergency department: etiology, imaging, and treatment.

OBJECTIVE: To assess the spectrum of diagnoses, the use of CT scans of the brain, and pharmacological treatments in patients presenting to a pediatric emergency department with headaches as the chief complaint. METHODS: A 1-year retrospective chart review of all children who presented to the emergency department with a headache as the chief complaint. RESULTS: One hundred thirty patients (0.7% of all pediatric emergency department visits, mean age = 9.3 years) were included in the study. Primary headaches included 11 migraine (8.5%) and 2 tension headaches (1.5%). Most of the secondary nonneurological headaches were associated with viral and respiratory illnesses (n=37, 28.5%), while the majority of the secondary neurological headaches included 26 posttraumatic (20%), 15 possible ventriculoperitoneal shunt malfunctions (11.5%), and 3 cases of aseptic meningitis (2.3%). The neurological etiology in 9 of these children (6.9%) was found to be serious (subdural hematoma, epidural hematoma, proven ventriculoperitoneal shunt malfunction, brain abscess, pseudotumor cerebri, and aseptic meningitis). Fifteen patients could not be etiologically classified, either because no specific etiology was found or their discharge diagnoses could not clearly explain the headache. Approximately 10% (5/53) of the CT scans of the head that were performed showed new abnormal findings including hydrocephalus secondary to ventriculoperitoneal shunt malfunction (2), subdural hematoma (1), epidural hematoma (1), and skull fracture (1). Forty-two patients (32%) were treated pharmacologically. Thirty-nine of the 42 treated patients (93%) were given over-the-counter analgesics, and 9 (21%) were given prescribed analgesics. CONCLUSIONS: The majority of the headaches in the pediatric emergency department were secondary to concurrent illness and minor head trauma, and required no pharmacological treatment or only treatment with minor analgesics. In a small minority of patients, headaches were secondary to serious neurological conditions, which required immediate medical attention. Computed tomography scans showed new abnormalities in a minority of patients and should be reserved for those with neurological diagnoses such as head trauma and ventriculoperitoneal shunt, as well as for those patients with recent onset of headaches with no clear etiological explanation, and for those with high-risk medical conditions, such as hypocoagulabilities. Future prospective studies are needed to assess the efficacy of the various pharmacological treatments in this population.

Adolescent↗

The value of early postictal EEG in children with complex febrile seizures.

PURPOSE: To assess the usefulness of an early postictal EEG in neurologically normal children with complex febrile seizures. METHODS: We conducted a retrospective chart review of all neurologically normal children who were hospitalized over a period of 2.5 years after complex febrile seizures, and had an EEG up to 1 week after the seizure. RESULTS: Thirty-three patients (mean age, 17.8 months) qualified for inclusion into the study. Twenty-four patients were qualified as complex cases based on one factor (prolonged in 9, repetitive in 13, and focal in 2). Nine other patients had two complex factors: in six patients, the seizures were long and repetitive; in two patients, the seizures were focal and repetitive; and in one patient, the seizures were long, focal, and repetitive. Thirteen (39%) patients experienced prior febrile seizures. All 33 patients had a normal postictal sleep EEG. Our results indicate with a 95% probability that the true rate of abnormalities in an early postictal EEG performed on otherwise normal children with complex febrile seizures is 8.6% or less. CONCLUSIONS: The yield of abnormalities of an early postictal EEG in this population is low and similar to the reported rate of abnormalities in children with simple febrile seizures. The routine practice of obtaining an early EEG in neurologically normal children with complex febrile seizures is not justified.

Child, Preschool↗

The role of brain computed tomography in evaluating children with new onset of seizures in the emergency department.

BACKGROUND: The purpose of neuroimaging of a patient with new onset of seizures is to demonstrate cause and explore the prognosis. It was recently recommended that emergency brain computed tomography (CT) be performed only in adult seizure patients with an increased likelihood of life-threatening lesions, i.e., those with new focal deficits, persistent altered mental status, fever, recent trauma, persistent headaches, history of cancer, history of anticoagulation, or suspicion of acquired immunodeficiency syndrome. The objective of this study was to determine the diagnostic utility of emergency brain CT in children who present to the emergency department with new onset of seizures. METHODS: A 1-year retrospective chart review of all children who presented to the emergency department of the Schneider Children's Hospital with a new onset of seizures and who underwent CT of the brain, excluding children with simple febrile seizures. RESULTS: Sixty-six patients, 34 boys and 32 girls with a mean age of 4.9 years, qualified for inclusion in the study. Fifty-two patients (78.8%) had normal CT results and 14 patients (21.2%) had abnormal CT results. Seizure cause was considered cryptogenic in 33 patients, of whom 2 (6%) had abnormal CT results; neither patient required intervention. Seizure cause was considered symptomatic in 20 patients, of whom 12 (60%) had abnormal CT results (p < 0.0001). In two patients with abnormal CT scans (both acute symptomatic), the imaging findings were of immediate therapeutic significance and were predictable from the clinical history and the physical examination. None of the 13 patients with complex febrile seizure cause had an abnormal CT scan. Patients with partial convulsive seizures were more likely to have abnormal CT scans than patients with generalized convulsive seizures, but the difference was not statistically significant. CONCLUSIONS: The routine practice in many pediatric emergency departments of obtaining brain CT scans for all patients with new onset of nonfebrile seizures is unjustified. History and physical examination are sufficient to identify those patients for whom such studies are likely to be appropriate. Emergent CT is not indicated for patients with no known seizure risk factors, normal neurological examinations, no acute symptomatic cause other than fever, and reliable neurological follow-up. For these patients, referral to a pediatric neurologist for further workup, including electroencephalography and the more diagnostically valuable magnetic resonance imaging, would be more appropriate.

Adolescent↗

Midazolam coma for refractory status epilepticus in children.

OBJECTIVE: To implement and retrospectively evaluate a therapeutic algorithm for the treatment of refractory status epilepticus with midazolam coma. METHODS: Eight consecutive patients with refractory status epilepticus were mechanically ventilated. Their arterial and central venous blood pressures were continuously monitored by indwelling vascular catheters. These patients were also continuously monitored by a 16-channel video electroencephalogram (EEG). A midazolam bolus of 0.15 mg/kg was administered, and a continuous infusion of 1-2 microg/kg/min was started. If seizures continued, the infusion was increased every 15 mins by 1-2 microg/kg/min. If seizures stopped and/or burst suppression was achieved, the patients continued to receive that dose for 48 hrs and were then weaned by decrements of 1-2 microg/kg/min every 15 mins. RESULTS: The patients' ages ranged from 17 days to 16 yrs, and they had various underlying diseases. In five of the eight patients, cessation of seizures occurred before achieving burst suppression on EEG, in two patients, cessation occurred during burst suppression, and in one patient, no response before or during burst suppression was encountered. The maximal midazolam doses required to achieve cessation of seizures and/or burst suppression, whichever came first, ranged from 4-24 microg/kg/min, with a mean of 14 +/- 6 microg/kg/min. The patients maintained stable cardiovascular function while receiving the maximal dose of midazolam and did not require inotropic support. CONCLUSION: Midazolam infusion, as per our described algorithm, is effective in terminating refractory status epilepticus. This treatment is not associated with cardiovascular instability, even at doses resulting in burst suppression. In the majority of cases, cessation of seizures occur before burst suppression is achieved on EEG.

Adolescent↗

Risk of excessive weight gain in epileptic children treated with valproate.

We sought to identify factors associated with excessive weight gain in children treated with valproate, excluding patients fed by gastrostomy or treated with medications known to affect appetite (eg, stimulants). Weight and height were recorded before treatment and at the time of follow-up; a measure of adiposity, body mass index, was computed and expressed in kg/m2, and weight and height for age were converted to Z-score. Putative risk factors included sex, age at start of treatment, monotherapy at start of treatment, duration of follow-up, mental retardation, seizure type (generalized or partial), etiology (idiopathic or cryptogenic versus remote symptomatic), and dose of valproate. Fifty-five children (30 girls, 25 boys), ranging in age at the start of therapy from 1.8 to 16.9 years were followed for 8.6 to 33.8 months. Forty-three patients had primarily generalized seizures, 34 had idiopathic or cryptogenic epilepsy (including 30 with generalized idiopathic epilepsy), and 18 had mental retardation. Valproate was the first antiepileptic drug for 21 patients, and 43 were on monotherapy at the time of follow-up. Height Z-score decreased significantly in girls but was stable in boys. There was a significant increase in body mass index and in weight Z-score. Body mass index was greater than the 90th percentile for age in 14 patients at the start of treatment and in 20 patients at follow-up. Decrease in height Z-score was significantly correlated with female sex and duration of follow-up. Changes in weight Z-score and body mass index were significantly correlated with initial weight Z-score and initial body mass index, respectively, but not with age at start of therapy, duration of follow-up, sex, seizure type, etiology, dose of valproate, or monotherapy.

Adolescent↗

The value of head ultrasound in infants with macrocephaly.

OBJECTIVE: To assess the value of head ultrasound (HUS) in neurologically normal infants with macrocephaly and to develop a rational approach to radiological investigation of macrocephalic infants. MATERIALS AND METHODS: A retrospective analysis of infants with macrocephaly (seen by one pediatric neurologist at this institution) was conducted. Infants with normal neurological examinations, no evidence of raised intracranial pressure and who had undergone a HUS were included. RESULTS: Twenty-seven infants were included in the analysis. Mean age at first assessment was 4.8 months. In 12 of 27 (44%), HUS was reported as normal. The remaining 15 had clinically insignificant abnormalities on HUS; 7 had prominent ventricles and 7 had increased subarachnoid fluid, with 1 infant having both. Seven infants had CT or MRI in addition to HUS. No clinically significant abnormalities were seen on CT or MRI scans that were not evident on HUS. Four infants had mild developmental abnormalities on initial evaluation. Mean duration of follow-up was 13.1 months. No infant developed neurological abnormalities during the follow-up period. CONCLUSION: In infants with macrocephaly with a normal neurological examination and no signs of raised intracranial pressure, HUS in conjunction with close neurological follow-up is reliable for the detection or exclusion of relevant intracranial pathology.

Abnormalities, Multiple↗

Pseudotumor cerebri in Lyme disease: a case report and literature review.

Pseudotumor cerebri is an unusual presentation of Lyme disease. The case of an 8-year-old girl with pseudotumor cerebri secondary to acute neuroborreliosis is reported. She presented with acute onset of headache, papilledema, sixth nerve palsy, increased intracranial pressure, and cerebrospinal fluid pleocytosis. Serum and cerebrospinal fluid Lyme antibodies were positive. Twelve reported cases that mostly presented with systemic findings and signs of Lyme disease before development of pseudotumor cerebri were reviewed. We conclude that acute neuroborreliosis can present with pseudotumor cerebri as an initial manifestation. It is important to include Lyme disease in the differential diagnosis of pseudotumor cerebri in an area endemic for Lyme disease.

Abducens Nerve↗

In whom does status epilepticus occur: age-related differences in children.

PURPOSE: Status epilepticus (SE) is an uncommon but potentially life-threatening seizure. It is most common in children. Little is known about the differences within the pediatric age group in terms of the type of patient seen with SE. METHODS: We analyzed the records of 394 children aged 1 month to 16 years who were part of two large studies of pediatric SE conducted in Bronx, New York, and Richmond, Virginia. The 394 children had a mean age of 4.4 years and included 349 (89%) with an initial episode of SE. RESULTS: Status epilepticus was most common in younger children with >40% of cases occurring in those younger than 2 years. The distribution of causes was highly age dependent. More than 80% of children younger than 2 years had SE of febrile or acute symptomatic origin, whereas cryptogenic and remote symptomatic causes were most common in older children (p < 0.001). One hundred fifty-eight (40%) of the cases were known to be previously neurologically abnormal, including 35 (21%) of 169 younger than age 2 years and 123 (55%) of 225 older than 2 years (p < 0.001). One hundred seventy-seven (45%) children had a history of seizures including 142 (41%) of the 349 children with a first episode of SE. A history of seizures was present in 34 (20%) of those younger than 2 years and 143 (64%) of those older than 2 years (p < 0.001). The effect of age remained significant even when the analysis was limited to those with SE of cryptogenic or remote symptomatic origin. CONCLUSIONS: There is a strong effect of age on the frequency and etiology of SE, as well as on the type of child who has SE. In young children, SE occurs primarily in children who are neurologically normal and with no history of unprovoked seizures. In older children, SE occurs primarily in those who are known to have prior unprovoked seizures and who are often also neurologically abnormal.

Adolescent↗

The incidence of acute and remote seizures in children with intraventricular hemorrhage.

Seizures are a well-known complication of intraventricular hemorrhage (IVH) in premature infants; however, the rate at which they occur is not known. The authors decided, therefore, to investigate both the incidence of acute and remote seizures in infants with IVH and the association with the grade of hemorrhage. One hundred and three infants with IVH were identified and their records were reviewed for acute seizures, remote seizures, and associated morbidity and mortality. The average gestational age of these infants was 29 weeks (range, 23-40 weeks). Of the 103 infants, 32 (31%) developed grade 4 IVH; 19 (18%), grade 3 IVH; and 52 (50%), grades 1 and 2 IVH. Seventeen (17%) patients had acute seizures during their first month of life. Six of the 61 patients (10%) who survived the neonatal period and for whom follow-up data were available had remote seizures. Infants with grade 4 IVH had significantly more acute seizures than infants with grades 1 and 2. In this cohort, only infants with grades 3 and 4 IVH developed remote seizures. Furthermore, among infants with grade 4 IVH acute seizures were a significant risk factor for development of remote seizures. The use of long-term antiepileptic drug therapy in neonates with a history of acute seizures is not established. These results suggest that antiepileptic drug therapy beyond the neonatal period should be reserved for infants with grade 4 IVH with history of acute seizures.

Acute Disease↗

Pediatric migraine and the International Headache Society (IHS) criteria.

BACKGROUND: The optimal criteria for the diagnosis of migraine without aura in children are controversial. One strategy for assessing the validity of diagnostic criteria is to compare them with expert clinical diagnoses. OBJECTIVE: To study the agreement between clinical headache diagnoses assigned by pediatric neurologists and symptom-based diagnoses using the International Headache Society (IHS) criteria as well as alternative case definitions. METHODS: We reviewed the records of 253 children and adolescents consecutively evaluated by pediatric neurologists at the Montefiore Headache Unit. Clinical diagnoses assigned by the physicians were used as the gold standard in evaluating the validity of the IHS criteria for the diagnosis of migraine without aura. Alternative symptom-based diagnoses were compared with the clinical gold standard. RESULTS: Detailed headache histories were abstracted from charts of 253 children; 167 children had complete data on all features required for IHS diagnosis. Eighty-eight (52.7%) children received a diagnosis of migraine without aura. Using the clinical diagnosis as the gold standard, the IHS criteria had a sensitivity of 27.3% and a specificity of 92.4%. The poor sensitivity of the IHS definition is a consequence of the rarity of certain features in children clinically diagnosed with migraine: duration of 2 hours or longer (55.7%), unilateral pain (34.1%), vomiting (47.7%), and phonophobia (27.3%). Based on these findings we suggested a definition for pediatric migraine headache without aura that is less complex, more sensitive (71.6%), and almost as specific as the IHS criteria. CONCLUSIONS: The IHS criteria for migraine without aura have poor sensitivity but high specificity using a clinical diagnosis as the gold standard. The IHS criteria should be modified to better reflect current pediatric clinical practice.

Adolescent↗

Risk factors for status epilepticus in children with symptomatic epilepsy.

The purpose of this study was to determine risk factors for status epilepticus (SE) in children with symptomatic epilepsy through a retrospective case-control study. Patients (44 children with a prior diagnosis of symptomatic epilepsy experiencing one or more episodes of SE between January 1, 1991, and June 1, 1995) were matched for age at follow-up to controls (88 children with symptomatic epilepsy without SE during that interval) and medical records were reviewed. Patients and controls did not differ in etiology or in age at epilepsy onset (1 year 5 months [SD, 2 years 3 months] versus 1 year 3 months [SD, 1 year 5 months]). Univariate analysis revealed significant associations between SE and several factors, including history of a first seizure as SE, partial epilepsy, partial seizures with secondary generalization, focal paroxysmal abnormalities and focal background abnormalities on EEG, and generalized abnormalities on neuroimaging. Groups did not differ in the proportion of patients on polypharmacy or with subtherapeutic serum antiepileptic drug levels, but there was a trend for greater use of phenobarbital in patients (57% versus 38%, odds ratio (OR) = 2.6, p = 0.057). With multiple logistic regression, four factors emerged as independent predictors of SE risk: focal background EEG abnormalities (OR = 6.51, p = 0.0005), partial seizures with secondary generalization (OR = 4.61, p = 0.0021), first seizure as SE (OR = 3.99, p = 0.034), and generalized abnormalities on neuroimaging (OR = 2.85, p = 0.034). These four factors are indicators of a higher risk of SE in children with symptomatic epilepsy.

Case-Control Studies↗

Subdural empyema in 12-year-old girl: the value of magnetic resonance imaging.

A 12-year-old girl presented with an acute history of fever, headaches, and focal neurological deficits. An initial computed tomography (CT) scan of the head was nondiagnostic whereas plain and gadolinium-enhanced magnetic resonance imaging (MRI) detected an extensive subdural empyema. The report emphasizes the hazard of relying on a nondiagnostic CT scan in a septic patient with deteriorating neurological function, and the need of MRI with contrast enhancement if subdural empyema is a serious clinical concern.

Child↗

Immunoglobulin M immunoblot for diagnosis of Borrelia burgdorferi infection in patients with acute facial palsy.

We used immunoblotting to improve the specificity of the serologic diagnosis of Lyme borreliosis in cases of acute facial palsy. Twelve of 15 patients (80%) with suspected Lyme borreliosis, versus 0 of 10 controls, were positive by immunoglobulin M immunoblotting of acute-phase sera and 3 were negative, including 2 with borderline enzyme immunoassay results. Immunoglobulin M immunoblotting is a useful test to confirm Borrelia burgdorferi infection in patients with acute facial palsy and a positive enzyme immunoassay result.

Acute Disease↗

Status epilepticus in children with epilepsy: the role of antiepileptic drug levels in prevention.

OBJECTIVES: To determine the association between subtherapeutic antiepileptic drug (AED) levels or AED withdrawal and status epilepticus (SE) in children with epilepsy. METHODS: We studied the AED levels at the time of SE in 51 consecutive children with epilepsy. Information about prior AED levels, possible etiology of seizures, and acute precipitants was extracted from medical records. RESULTS: The mean age at the time of SE was 5.7 years (range, 3 months through 18 years). Forty-three patients had history of remote insult, five had history of progressive encephalopathy, and three patients were classified as idiopathic. At the time of SE all AED levels were therapeutic in 34 (66%) patients and at least one level was therapeutic in 42 (82%) patients. All levels were subtherapeutic in 9 (18%) patients. Four patients had their AED reduced or discontinued less than 1 week before SE. Twelve patients with therapeutic AED levels on their most recent clinic visit had at least one subtherapeutic level at the time of SE. Eight (16%) patients were febrile and one was hyponatremic. Of the 51 patients, 31 (61%) had no obvious explanation for the development of SE, as all known AEDs were therapeutic and there were no known acute insults. CONCLUSIONS: Neurologically abnormal children with preexisting epilepsy are at high risk for development of SE despite having therapeutic AED levels at that time. Acute precipitants of SE, such as fever or AED withdrawal, may play a role in inducing SE only in a minority of patients.

Adolescent↗

Prognosis and treatment of seizures in children with acute lymphoblastic leukemia.

We reviewed the records of 127 consecutive pediatric patients with acute lymphoblastic leukemia (ALL) to determine the incidence, timing, etiologies, and recurrence rate of seizures in this population. Patients with ALL and seizures were identified retrospectively by review of the records of all pediatric ALL patients who were diagnosed and treated during the years 1983 through March 1993 in a large tertiary-care hospital. Seventeen patients (13%) developed one or more seizures. In 16 patients, seizures occurred during antileukemic treatment, and in almost all of them seizures were related to intrathecal methotrexate (IT MTX) or subcutaneous L-asparaginase treatment. One patient who developed a seizure while not receiving chemotherapy had a history of cerebral infarctions. In 8 patients, (47%), the initial seizure episode was associated with a cerebral lesion. One or more seizures recurred in 6 patients. Four of these patients had an isolated recurrence, in 3 patients < or = 3 months and in 1 patient < or = 6 months after the initial event. Two patients (12%) with static encephalopathy and neurological deficits developed a chronic seizure disorder. There is a significant risk of acute symptomatic seizures in pediatric ALL patients. Most seizures in these patients occur during the acute treatment phase and are most frequently related to side effects of chemotherapy. The long-term recurrence risk is low; recurrence occurs most often in patients with evidence of cerebral structural lesions and neurological deficits. Long-term antiepileptic drug (AED) therapy should be restricted to such patients.

Adolescent↗

How long to treat childhood onset absence epilepsy.

The relation between the duration of treatment and achievement of seizure-free state after stopping medication in childhood onset absence epilepsy (COAE) has not been established, and children are usually treated for periods of 2-4 years. We hypothesized that a group of patients defined by prompt total clinical and electrographic remission, verified by 24-hour ambulatory EEG, could be withdrawn from treatment without recurrence. Three patients with new onset of COAE were treated. Ethosuximide (ESM) was used as first line drug. Dosages correlated with blood levels were gradually increased until a clinical and electrographic seizure-free state was achieved. The three responded to ESM promptly at therapeutic blood levels (75-90 mcg/ml). The effects of a 5- to 6-week period of withdrawing ESM after 6 months of treatment were as follows: a 10-year-old boy remained seizure-free with recurrence of inter-ictal 3/second spike-wave bursts; 5- and 10-year-old girls remained seizure-free and their 24-hour ambulatory EEG burst-free. Our findings suggest that optimization of treatment in COAE, aimed at achieving an electroclinical and electrographic seizure-free state, may identify patients in whom therapy can be successfully ended much sooner than has been usual. Thus the same good prognosis may be reached in a shorter period of treatment with minimal risk of recurrence of absence seizures.

Anticonvulsants↗