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document
Delineation of cryptogenic Lennox-Gastaut syndrome and myoclonic astatic epilepsy using multiple correspondence analysis . PURPOSE To distinguish various types of childhood severe cryptogenic/idiopathic generalised epilepsy on the basis of reproducible diagnostic criteria , using multiple correspondence analysis ( MCA ) . METHODS We applied MCA to a series of 72 children with no evidence of brain damage , starting epilepsy between 1 and 10 years , with two or more types of generalised seizures . We excluded patients with infantile spasms or typical absences . MCA was performed on all clinical and EEG parameters , first throughout follow-up , then restricted to the first year of the disease . RESULTS When including all follow-up variables , there were three groups : ( 1 ) Thirty-seven children with male predominance , familial history of epilepsy , simple febrile convulsions , massive myoclonus , tonic-clonic fits . Outcome was favourable , with no seizures and mildly affected cognitive functions . Interictal EEG showed short sequences of irregular 3-Hz spike-waves . ( 2 ) In 18 children , clinical characteristics were similar to those of the first group at the early stage , but 95 % exhibited myoclonic status and vibratory tonic seizures , with persisting seizures on follow-up . EEG showed long sequences of generalised irregular spike and slow waves . Those two groups meet the characteristics of childhood onset myoclonic-astatic epilepsy ( MAE ) with respectively , favourable and unfavourable outcome . ( 3 ) Eleven children had later onset , atypical absences , tonic and partial seizures , and no myoclonus , or vibratory tonic seizures . All had mental retardation and persisting seizures . EEG showed long sequences of slow spike-wave activity and half the patients had spike and slow wave foci . These patients met the major characteristics of Lennox-Gastaut syndrome . Initial parameters failed to distinguish the first two groups , but Lennox-Gastaut syndrome ( the third group ) was distinct from both groups of myoclonic astatic epilepsy from the onset . Within MAE groups combined , clinical and EEG risk factors for mental retardation could be identified . CONCLUSION It is possible to validate statistically the distinction between discrete epileptic syndromes . Myoclonic astatic epilepsy is therefore distinct from Lennox-Gastaut syndrome , and the distinction appears from the first year of the disorder .
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Intervention_Educational
multiple correspondence analysis
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Intervention_Educational
multiple correspondence analysis ( MCA )
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Intervention_Educational
MCA
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Intervention_Other
EEG
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Outcome_Physical
no seizures and mildly affected cognitive functions
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Outcome_Other
short sequences of irregular 3-Hz spike-waves
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Outcome_Physical
clinical characteristics
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Outcome_Physical
myoclonic status
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Outcome_Physical
vibratory tonic seizures
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Outcome_Physical
persisting seizures
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Outcome_Other
long sequences of generalised irregular spike and slow waves
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Outcome_Mental
atypical absences
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Outcome_Mental
tonic and partial seizures
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Outcome_Mental
mental retardation and persisting seizures
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Outcome_Other
long sequences of slow spike-wave activity and half the patients had spike and slow wave foci
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Participant_Condition
childhood severe cryptogenic/idiopathic generalised epilepsy