PMID- 11071139
OWN - NLM
STAT- MEDLINE
DCOM- 20010531
LR  - 20111117
IS  - 0174-304X (Print)
IS  - 0174-304X (Linking)
VI  - 31
IP  - 4
DP  - 2000 Aug
TI  - Nonconvulsive status epilepticus--a possible cause of mental retardation in 
      patients with Lennox-Gastaut syndrome.
PG  - 169-74
AB  - Lennox-Gastaut syndrome (LGS) is one of the most severe types of childhood 
      epilepsy. It is usually resistant to treatment and associated with mental 
      retardation. To delineate the risk factors associated with the outcome of LGS, we 
      evaluated, in a retrospective and multicentre study, the course of the disease, 
      EEG tracings, and intellectual function in 101 patients. Inclusion criteria were 
      the presence of tonic seizures as well as slow spike and wave complexes in the 
      EEG. The average documented observation period was 16 years (range 4-31 years). 
      Overall, the intellectual and neurological outcome was poor. At the last 
      follow-up, 38% of the patients could not speak, 21% were unable to walk and only 
      4% were free of seizures. Four independent risk factors for severe mental 
      retardation were identified by multivariate analysis. These were in a decreasing 
      order of importance: nonconvulsive status epilepticus (NCSE), odds ratio (OR) 
      25.2, a previous diagnosis of West syndrome (OR 11.6), a symptomatic etiology of 
      epilepsy (OR 9.5), and an early age at onset of epilepsy (OR 4.7). The results 
      highlight the association between NCSE and the severity of mental retardation in 
      patients with LGS; this association appears to be independent of symptomatic 
      etiology. Our data provide an indirect evidence that, at least in some of the 
      patients, NCSE is not only a concomitant feature, but also a cause of severe 
      mental retardation.
FAU - Hoffmann-Riem, M
AU  - Hoffmann-Riem M
AD  - Department of Neuropediatrics and Muscular Disorders, University of Freiburg, 
      Germany.
FAU - Diener, W
AU  - Diener W
FAU - Benninger, C
AU  - Benninger C
FAU - Rating, D
AU  - Rating D
FAU - Unnebrink, K
AU  - Unnebrink K
FAU - Stephani, U
AU  - Stephani U
FAU - Ernst, H P
AU  - Ernst HP
FAU - Korinthenberg, R
AU  - Korinthenberg R
LA  - eng
PT  - Journal Article
PL  - Germany
TA  - Neuropediatrics
JT  - Neuropediatrics
JID - 8101187
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Atrophy/pathology
MH  - Cerebral Cortex/pathology
MH  - Child
MH  - Child, Preschool
MH  - Epilepsy/*complications
MH  - Female
MH  - Humans
MH  - Intellectual Disability/*etiology
MH  - Male
MH  - Retrospective Studies
MH  - Risk Factors
MH  - Status Epilepticus/*complications
MH  - Syndrome
EDAT- 2000/11/09 11:00
MHDA- 2001/06/02 10:01
CRDT- 2000/11/09 11:00
PHST- 2000/11/09 11:00 [pubmed]
PHST- 2001/06/02 10:01 [medline]
PHST- 2000/11/09 11:00 [entrez]
AID - 10.1055/s-2000-7456 [doi]
PST - ppublish
SO  - Neuropediatrics. 2000 Aug;31(4):169-74. doi: 10.1055/s-2000-7456.