Daniel K. Miles

Diagnostic Value of Pediatric Outpatient Video-EEG

Outpatient video-electroencephalography (OVEEG) was performed in 100 infants, children, and adolescents with diagnosed (group I, n = 64) or suspected (group II, n = 36) epilepsy. Median monitoring duration was 4 hours. Indications for OVEEG in group I were classification of seizures, reported seizure exacerbation, or onset of new signs. OVEEG indications in group II were repetitive paroxysmal and stereotyped signs of myoclonic movements, fixed gaze, abnormal behavior, or nonmyoclonic motor activity. In group I patients, symptomatic events were recorded in 89%, half of which were seizures. Among group II patients, events were recorded in 67% and were seizures in 22%. Overall, OVEEG was successful in 83% of patients. Compared to a 24-hour inpatient admission for video-EEG monitoring, OVEEG represented cost reductions of 55-80% per patient. We conclude that OVEEG is a cost-effective, useful alternative to continuous inpatient video-EEG monitoring in the investigation of selected infants, children, and adolescents with diagnosed or suspected epilepsy.

Epilepsy, atherosclerosis, myocardial infarction, and carbamazepine.

Epileptic patients receiving medications such as phenytoin or phenobarbital have been noted to have an exceedingly low incidence of myocardial infarction, but children treated with carbamazepine have shown alteration of their serum lipid profile that could predispose them to atherosclerosis. This report seems to corroborate this point and describes the findings in an 11-year-old boy who died following a major seizure, rhabdomyolysis, and renal failure. The autopsy demonstrated that he had marked coronary atherosclerosis and myocardial infarction. The child had been treated with carbamazepine for more than 10 years. (J Child Neurol 2003;18: 150—151).

Long-Term Computer-Assisted Outpatient Electroencephalogram Monitoring in Children and Adolescents

The aims of this study were (1) to define the role of long-term computer-assisted outpatient electroencephalographic monitoring (COEEG) in children and adolescents with known or suspected epilepsy, and (2) to compare COEEG data with routine interictal electroencephalograms (EEG). We performed 18-channel COEEG in 84 children and adolescents with diagnosed (group 1, n = 49) or suspected (group 2, n = 35) epilepsy. Mean recording time was 1.4 days. Overall, COEEG was useful in 87% of patients. In group 1, events were recorded in 73% of patients and were electrographic seizures in 45%. In group 2, events were detected in 86% of patients and were electrographic seizures in 17%. Nocturnal and partial seizures predominated. Seizure diagnosis and classification by COEEG was concordant with interictal EEG findings in 19% and discordant in 63% of patients. COEEG is a useful technique for the diagnosis of epileptic and nonepileptic events among selected children and adolescents. When compared to routine interictal EEG, COEEG could offer additional accuracy in the classification of seizures in pediatric patients. (J Child Neurol 2000;15:49-55).

West syndrome in tuberous sclerosis complex

West syndrome occurs commonly in children with tuberous sclerosis complex and is associated with a grave prognosis for cognitive and seizure outcomes. We sought to determine the epilepsy outcome of children with tuberous sclerosis complex and West syndrome and whether EEG, MRI, or steroid therapy duration were different in those whose epilepsy improved compared with those with intractable seizures. Seventeen patients with tuberous sclerosis complex and West syndrome were identified. For each patient, two sets of clinical evaluations, EEG and MRI data, and treatment information separated by at least 12 months were obtained. The patients were divided into two seizure outcome groups. EEG, MRI, and treatment data were compared between the groups. The intellectual deficiency was either severe (76%) or moderate (24%). Seizure control improved in 10 and worsened in seven, without mortality (follow-up range = 12-216 months). No significant differences in EEG background, MRI findings, or steroid treatment duration were evident between the groups. The difference in EEG-sleep approached statistical significance (P = 0.06). Our findings did not confirm reports of high mortality and poor epilepsy outcome in intellectually deficient children with West syndrome and tuberous sclerosis complex. EEG sleep was the best indicator of seizure control and approached statistical significance. The duration of steroid therapy had no influence on seizure control.

Technetium 99mTc-HMPAO SPECT in Children and Adolescents With Neurologic Disorders:

We evaluated regional cerebral blood flow with technetium 99mTc hexamethylpropyleneamineoxime single photon emission computed tomography (SPECT) in 20 children and adolescents with neurologic dysfunction of varied etiology and abnormal electroencephalograms (EEGs). All patients were also examined with computed tomography (CT) and magnetic resonance imaging (MRI). Abnormal perfusion was found in 17 (85%) of 20 SPECT scans. Abnormal CT or MRI scans were noted in nine (45%) and in 10 (50%) of 20 cases, respectively. In eight (73%) of 11 cases with normal CT scans and in seven (70%) of 10 with normal MRI scans, the SPECT scan was abnormal. Abnormal regional cerebral blood flow on SPECT scans correlated better with EEG abnormalities than with neurologic examination or CT or MRI scan findings. We conclude that in children and adolescents with a spectrum of neurologic diseases and abnormal EEGS, abnormalities of brain structure or function are more likely to be documented by SPECT than by CT or MRI scans. SPECT findings correlate well with the location and type of EEG abnormality. (J Child Neurol 1993;8:227-234).

Tuberous Sclerosis Complex and Epilepsy: Prognostic Significance of Electroencephalography and Magnetic Resonance Imaging

Tuberous sclerosis complex is a disease that affects many organs, including the central nervous system. Nervous system involvement in the form of hamartomas often results in seizures. In this study we wanted to determine the outcome of epilepsy in tuberous sclerosis complex and determine whether interictal electroencephalograms (EEGs) and hamartoma burden as seen with magnetic resonance imaging (MRI) are predictive of degree of seizure control. The study population consisted of 30 patients. For each patient two sets of EEG and MRI data, separated by at least 12 months, and information on seizure frequency at time of data collection were obtained. Sensitivity, specificity, and positive and negative predictive values of various EEG and MRI findings were determined. Seizure control improved in 20 and worsened in 10 patients. In relation to seizure control, the specificity of an abnormal sleep EEG and the positive predictive value of normal sleep EEG were 100%. MRI and EEG background were neither sensitive nor specific for predicting seizure control. A majority of children with tuberous sclerosis complex can achieve good seizure control. The sleep EEG is helpful in predicting eventual seizure control. (J Child Neurol 2000;15:81-83).

Episodic Epileptic Verbal Auditory Agnosia in Landau Kleffner Syndrome Treated With Combination Diazepam and Corticosteroids

We report 2 pediatric patients who presented initially with seizures followed by subacute language regression characterized by a verbal auditory agnosia. These previously normal children had no evidence of expressive aphasia during their symptomatic periods. Further, in both cases, auditory agnosia was associated with sleep-activated electroencephalographic (EEG) epileptiform activity, consistent with Landau-Kleffner syndrome. However, both cases are unique since the episodic auditory agnosia and sleep-activated EEG epileptiform activity rapidly responded to combination therapy with pulse benzodiazepine and corticosteroids. Further, in each case, recurrences were characterized by similar symptoms, EEG findings, and beneficial responses to the pulse benzodiazepine and corticosteroid therapy. These observations suggest that pulse combination high-dose corticosteroid and benzodiazepine therapy may be especially effective in Landau-Kleffner syndrome.

Neonatal adrenoleukodystrophy presenting as infantile progressive spinal muscular atrophy

Two siblings with neonatal adrenoleukodystrophy are described. The signs and laboratory data documenting infantile progressive spinal muscular atrophy included the initial presentation of 1 sibling with neonatal adrenoleukodystrophy. These patients indicate that neonatal adrenoleukodystrophy should be considered in the differential diagnosis of infantile progressive spinal muscular atrophy.

Somatosensory Evoked Potential Abnormalities in HIV-Infected Children are More Frequent Than in HIV-Exposed Children, Reflecting the Degree of HIV-Related Neurological Disease

Somatosensory Evoked Potential Abnormalities in HIV-Infected Children are More Frequent Than in HIV-Exposed Children, Reflecting the Degree of HIV-Related Neurological Disease