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Query: UMLS:C0085584 (encephalopathy)
 18,178 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Within the last years five new antiepileptics have become available in Germany. Vigabatrin is a second choice drug against partial seizures, West syndrome and epilepsies in infant encephalopathy syndromes. Lamotrigine and Gabapentin can be used as add-on therapy in partial seizures in children above 12 years of age Felbamate has a high incidence of severe side-effects like aplastic anemia and liver failure. Therefore it should be restricted to the treatment of Lennox-Gastaut syndrome. Oxcarbazepine is not yet on the German market, but is available by import from Austria. Its therapeutic range is similar to carbamazepine with less side-effects. The new antiepileptics discussed have turned out to be useful additional therapeutics, especially in focal epilepsies. There is, however, still limited experience with these drugs in children. So none can as yet be considered a drug of first choice in any epileptic childhood disorder. The classical antiepileptic drugs remain essential in antiepileptic therapy.
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PMID:[Value of the new anticonvulsants in pediatrics]. 952 99

Topiramate (TPM), a new antiepileptic medication, is efficacious as adjunctive therapy in adults with partial onset seizures. Its efficacy as adjunctive therapy in children was evaluated in two randomized double-blind placebo-controlled trials involving childhood epileptic encephalopathy (the Lennox-Gastaut syndrome) and partial onset seizures. In these studies, topiramate adjunctive therapy resulted in a significant reduction in drop attacks (tonic or atonic seizures) in patients with the Lennox Gastaut syndrome and a significant reduction in partial onset seizures in children with refractory partial epilepsy. In both trials, TPM's efficacy improved as the dose escalated from the double-blind phase to the open-label portion. The minimally effective topiramate dose for adjunctive therapy in children with refractory epilepsy appears to be 6 mg/kg/day. Topiramate was well tolerated with mild or moderate side effects, predominantly related to the central nervous system. Practical tips are provided that may increase the chance that topiramate will be effective and well tolerated. The most important advice is a "start low, go slow" approach. An initial TPM dose of 0.5-1 mg/kg/day followed by weekly increments of 0.5-1 mg/kg is usually well tolerated. Based on these studies, topiramate appears to be an important addition to our pediatric AED armamentarium.
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PMID:Topiramate use in pediatric patients. 970 34

Children with childhood epileptic encephalopathy (Lennox-Gastaut syndrome) frequently have both multiple seizure types and nonepileptic stereotyped events that are difficult to differentiate. We hypothesize that electroencephalogram (EEG) video monitoring is essential for correct identification of atypical absence seizures in this population. All video/EEG monitoring records on patients with confirmed Lennox-Gastaut syndrome between September 1992 and December 1996 were reviewed for clinical events and EEG changes. A subset of patients with suspected atypical absence seizures during the video/EEG formed the cohort for analysis. Thirty-eight patients had 48 monitoring periods ranging from 1 to 4 days (mean, 2.2 days). Twenty-six monitoring periods captured suspected atypical absence seizures and formed the study cohort. Suspected atypical absence seizures were epileptic seizures in only 27% (7 of 26) of the study cohort. By contrast, parents reliably and correctly identified tonic, atonic, and tonic-clonic seizures in the study cohort. Reliable diagnosis and subsequent counting of atypical absence seizures in patients with Lennox-Gastaut syndrome cannot be made on the basis of observation and/or history alone. Future outpatient studies of investigational anticonvulsant medications for patients with Lennox-Gastaut syndrome should consider parental counts of atypical absence seizures unreliable. We recommend that video/EEG monitoring be done on all Lennox-Gastaut syndrome patients with suspected atypical absence seizures not controlled by medication.
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PMID:Need for electroencephalogram video confirmation of atypical absence seizures in children with Lennox-Gastaut syndrome. 979 56

The author first reviews the definition of myoclonia as an epileptic crisis differentiated from tonic crises and infantile spasms. He reviews the prevalence and incidence found in bibliographic data, under the following headings 1. Early myoclonic encephalopathy or neonatal myoclonic encephalopathy 2. Early epileptic syndrome with bursts of suppression or Otahara's syndrome. 3. West's syndrome. 4. The benign myoclonic epilepsy syndrome of children. 6. Syndrome of myoclonic epilepsy in non-progressive encephalopathy. 7. Early myoclonic epilepsy of children or Dose's syndrome. 8. Lennox-Gastaut syndrome. 9. Syndrome of epilepsy with absences in children. 10. Myoclonic absence epilepsy syndrome. 11. Landau-Kleffner syndrome and the syndrome of continuous slow spike-and-wave epilepsy during slow sleep. 12. Photosensitive epilepsy. 13. Absence epilepsy in young patients. 14. Juvenile myoclonic epilepsy. 15. Syndrome of gran mal epilepsy on waking. 16. Progressive myoclonic epilepsies. The author reviews 6,450 cases, 408 patients who had myoclonic crises, that is 6.3%. The differences seen in this total group of patients were: the myoclonic crises which presented alone, myoclonic crises accompanied by simple typical absences, those initially accompanied by generalized tonic-clonic crises and those presenting typical absences, tonic-clonic generalized crises and myoclonus simultaneously. The course of the different groups is analyzed.
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PMID:[Severity and epidemiology of myoclonic epilepsy]. 1071 95

Myoclonic jerks occur in a number of different syndromes. There is many classifications of myoclonus. It is preferred the Fejerman classification, slightly modified that present the following five groups: 1. Myoclonus without encephalopathy and without epilepsy, which includes physiological myoclonus; 2. Encephalopathies with non epileptic myoclonus, which includes Kinsbourne syndrome and certain types of hyperekplexia which pose differential diagnosis problems with reflex myoclonic epilepsy; 3. Progressive encephalopathies with myoclonic seizures which includes typical and atypical progressive myoclonus epilepsies; 4. Epilepsies and epileptic encephalopathies with myoclonic seizures, which includes severe epilepsies which leads to mental retardation, as Otahara syndrome, West syndrome and Lennox-Gastaut syndrome, and other epilepsies which present sometimes myoclonic seizures, as Landau-Kleffner syndrome, 5. Comprises true myoclonic epilepsies, differentiating syndromes recognized as idiopathic, -benign myoclonic epilepsy of infancy, reflex form of benign myoclonic epilepsy in infancy, eyelid myoclonic with absences, perioral myoclonic with absences and juvenile myoclonic epilepsy-, cryptogenic-severe myoclonic epilepsy of infancy, myoclonic-astatic epilepsy and epilepsy with myoclonic absences-, and symptomatic as the generalized myoclonus in children with static encephalopathies. The epileptic syndromes of the last group are described. Despite this classification, apparently clear, there is still a great deal of confusion and in clinical practice, many cases are difficult to classify.
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PMID:[Myoclonus and myoclonic epilepsies in childhood]. 1071 97

Although for most children epilepsy is a relatively benign disorder, for some, epilepsy can be designated as "catastrophic" because the seizures are so difficult to control and because they are strongly associated with mental retardation. The catastrophic childhood epilepsies include uncommon disorders such as early infantile epileptic encephalopathy with suppression burst, severe myoclonic epilepsy of infancy, and epilepsy with myoclonic-astatic seizures. There are other syndromes that are relatively common such as infantile spasms, Lennox-Gastaut syndrome, and Sturge-Weber syndrome. Many children with catastrophic epilepsy have the seizures as a result of underlying brain abnormalities that will inevitably lead to mental retardation whether or not they have seizures. In some patients, however, the mental retardation appears to be caused by the seizures. Developmental plasticity provides children with an opportunity to recover from significant brain injuries. However, the plasticity may also be the cause of the mental retardation. In such patients, control of the seizures may lead to more normal intellectual development. Thus, every effort should be made to control seizures in children with catastrophic epilepsy.
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PMID:Catastrophic epilepsy in childhood. 1088 34

Several epileptic syndromes that occur during childhood are characterized by severe treatment-resistant seizures, progressive loss of higher intellectual functions, and characteristic electroencephalographic abnormalities. These catastrophic epileptic syndromes include epileptic encephalopathy with diffuse slow spike waves (Lennox-Gastaut syndrome), West syndrome, progressive myoclonic epilepsies, and electrical status epilepticus during sleep. This article summarizes each syndrome and reviews the most recent information concerning the effectiveness of topiramate with respect to each condition. Suggestions are offered to help clinicians maximize topiramate's efficacy and tolerability in patients suffering with these syndromes. Overall, topiramate is a valuable antiepileptic medication in the treatment of catastrophic pediatric epileptic syndromes.
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PMID:Topiramate in the catastrophic epilepsies of childhood. 1121 52

The diagnosis of Angelman syndrome (AS) can be confirmed by genetic laboratory in about 80% of cases. In 20%, the diagnosis remains clinical, but often there is uncertainty about the correctness of the clinical diagnosis and alternative diagnoses may be investigated. In evaluating individuals for AS in our center since 1989, we have encountered several mimicking conditions, and additional ones have been reported in the literature. Mimicking conditions can be grouped into the areas of chromosome, single gene, and symptom complex anomalies. Microdeletions or microduplications include chromosome regions 2,4,17, 22, and 15. Single gene conditions include methylene tetrahydrofolate reductase deficiency (MTHFR), Rett syndrome, alpha-thalassemia retardation syndrome (ATR-X), and Gurrieri syndrome. Symptom complexes include cerebral palsy, static encephalopathy, Lennox-Gastaut syndrome, autism spectrum disorder, pervasive developmental delay (PDD), and mitochondrial disorders. We present a review of these mimicking disorders to increase the awareness about conditions that can lead to an incorrect clinical diagnosis of AS.
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PMID:Angelman syndrome: mimicking conditions and phenotypes. 1175 74

The subjects were 25 children, including West syndrome, Lennox-Gastaut syndrome, childhood absence epilepsy (CAE) and localization-related epilepsies. Clinical seizures occurred only during waking state in 14 patients (including the cases of West syndrome, CAE and epilepsy with myoclonic absences (EMA)), only during sleeping state (especially during non-REM sleep in five patients, including the case of benign epilepsy with centrotemporal spike), and diffusely during waking and sleeping states in six patients (including the case of early infantile epileptic encephalopathy with suppression burst). These three types were observed in the cases of Lennox-Gastaut syndrome and localization-related epilepsies. Subclinical ictal discharges occurred during REM sleep in West syndrome, and during REM and non-REM sleep especially during non-REM sleep in CAE and EMA.
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PMID:Epileptic seizures and sleep-wake rhythm. 1142 28

A long-term follow-up study of Lennox-Gastaut syndrome (LGS) ( > 10 years) was conducted with 38 patients with severe motor and intellectual disabilities (SMID) to clarify the relationship between the rapid development of dysphagia and epileptic seizures, and to elucidate the long-term evolution of LGS in patients with SMID. Those who showed a relatively favourable seizure outcome were compared to those with a poor seizure outcome. Poor seizure outcome correlated strongly with: (a) an early appearance of dysphagia and additional deterioration of the already retarded mental function; (b) a predominance of atypical absence seizures; and (c) persistent frequent epileptiform discharges during electroencephalographic evaluations. Neither age at seizure onset nor intelligence level at the time of the last examination was correlated with seizure prognosis. Further, seizure prognosis was not related to the aetiology of LGS. Repeated seizures apparently caused development of progressive epileptic encephalopathy, in addition to the underlying severe brain damage. Since development of dysphagia burdens an already severely handicapped patient with intensive medical care, efforts to reduce the seizures and design a long-term care plan are of great importance.
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PMID:Long-term follow-up study of Lennox-Gastaut syndrome in patients with severe motor and intellectual disabilities: with special reference to the problem of dysphagia. 1143 19


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