Serum and CSF neuron-specific enolase in patients with West syndrome.
OBJECTIVE
To determine whether frequent seizures and/or hypsarrhythmia may cause neuronal injury in West syndrome.


BACKGROUND
West syndrome is an age-related epileptic syndrome of infancy characterized by clusters of epileptic spasms, a peculiar interictal EEG pattern of hypsarrhythmia, and mental deterioration. Recent clinical studies demonstrated that serum and CSF neuron-specific enolase (NSE)-a marker of neuronal injury-were increased after status epilepticus.


METHODS
The authors examined serum and CSF NSE levels in 18 newly diagnosed infants (8.4 +/- 2.2 months) with West syndrome (3 cryptogenic, 15 symptomatic). In patients who showed complete resolution of spasms and disappearance of hypsarrhythmia (responders), additional serum NSE levels were determined several weeks after cessation of seizures. Serum NSE levels were obtained from 28 age-matched infants with normal neurologic development (control group), and 10 infants with an acute neurologic insult.


RESULTS
There were no significant differences (p > 0.05) in serum NSE levels between the group with West syndrome (12.9 +/- 3.4 ng/mL) and the control group (13.2 +/- 3.1 ng/mL). The serum NSE value in the group with an acute insult (100.3 +/- 67.4 ng/mL) was significantly higher (p < 0.0001) than that for the West syndrome and the control groups. The mean +/- SD CSF NSE level was 7.3 +/- 3.6 ng/mL, which is similar to the reported CSF NSE levels of Japanese infants without neurologic disease. Thirteen responders showed no significant (p > 0.05) change in serum NSE after cessation of epileptic spasms.


CONCLUSIONS
Normal serum and CSF neuron-specific enolase levels provided no evidence that seizures and/or hypsarrhythmia induced neuronal injury in West syndrome.
Suzuki
Y
Y
Division of Pediatric Neurology, Osaka Medical Center and Research Institute for Maternal and Child Health, Izumi, Japan.
Toribe
Y
Y
Goto
M
M
Kato
T
T
Futagi
Y
Y
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D005260:Female; D006801:Humans; D008297:Male; D009422:Nervous System Diseases / Q000097:blood / Q000134:cerebrospinal fluid / Q000503:physiopathology; D010751:Phosphopyruvate Hydratase / Q000097:blood* / Q000134:cerebrospinal fluid*; D012016:Reference Values; D013036:Spasms, Infantile / Q000097:blood* / Q000134:cerebrospinal fluid* / Q000503:physiopathology
D010751:Phosphopyruvate Hydratase