Subject(s)
Anti-Inflammatory Agents/therapeutic use , Antibodies, Monoclonal/therapeutic use , Dermatologic Agents/therapeutic use , Psoriasis/therapy , Adolescent , Anti-Inflammatory Agents/adverse effects , Antibodies, Monoclonal/adverse effects , Biological Therapy/methods , Child , Child, Preschool , Dermatologic Agents/adverse effects , Dermatology/methods , Drug Therapy, Combination , Female , Humans , Male , Pregnancy , Psoriasis/complications , Tumor Necrosis Factor-alpha/antagonists & inhibitors , Virus Diseases/complications , Virus Diseases/drug therapyABSTRACT
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.
Subject(s)
Spasms, Infantile/diagnosis , Tuberous Sclerosis/diagnosis , Adolescent , Adrenal Cortex Hormones/therapeutic use , Anticonvulsants/therapeutic use , Brain/pathology , Child , Child, Preschool , Electroencephalography/drug effects , Female , Follow-Up Studies , Humans , Infant , Magnetic Resonance Imaging , Male , Spasms, Infantile/drug therapy , Spasms, Infantile/mortality , Survival Rate , Treatment Outcome , Tuberous Sclerosis/drug therapy , Tuberous Sclerosis/mortalityABSTRACT
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.
Subject(s)
Electroencephalography , Epilepsy/complications , Epilepsy/diagnosis , Tuberous Sclerosis/complications , Tuberous Sclerosis/diagnosis , Brain Diseases/complications , Brain Diseases/diagnosis , Child , Child, Preschool , Cognition Disorders/complications , Cognition Disorders/diagnosis , Female , Hamartoma/complications , Hamartoma/diagnosis , Humans , Infant , Magnetic Resonance Imaging , Male , Predictive Value of Tests , Prospective Studies , Severity of Illness IndexABSTRACT
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.