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West syndrome in tuberous sclerosis complex.

Publication ,  Journal Article
Husain, AM; Foley, CM; Legido, A; Chandler, DA; Miles, DK; Grover, WD
Published in: Pediatr Neurol
September 2000

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.

Duke Scholars

Published In

Pediatr Neurol

DOI

ISSN

0887-8994

Publication Date

September 2000

Volume

23

Issue

3

Start / End Page

233 / 235

Location

United States

Related Subject Headings

  • Tuberous Sclerosis
  • Treatment Outcome
  • Survival Rate
  • Spasms, Infantile
  • Neurology & Neurosurgery
  • Male
  • Magnetic Resonance Imaging
  • Infant
  • Humans
  • Follow-Up Studies
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Husain, A. M., Foley, C. M., Legido, A., Chandler, D. A., Miles, D. K., & Grover, W. D. (2000). West syndrome in tuberous sclerosis complex. Pediatr Neurol, 23(3), 233–235. https://doi.org/10.1016/s0887-8994(00)00186-7
Husain, A. M., C. M. Foley, A. Legido, D. A. Chandler, D. K. Miles, and W. D. Grover. “West syndrome in tuberous sclerosis complex.Pediatr Neurol 23, no. 3 (September 2000): 233–35. https://doi.org/10.1016/s0887-8994(00)00186-7.
Husain AM, Foley CM, Legido A, Chandler DA, Miles DK, Grover WD. West syndrome in tuberous sclerosis complex. Pediatr Neurol. 2000 Sep;23(3):233–5.
Husain, A. M., et al. “West syndrome in tuberous sclerosis complex.Pediatr Neurol, vol. 23, no. 3, Sept. 2000, pp. 233–35. Pubmed, doi:10.1016/s0887-8994(00)00186-7.
Husain AM, Foley CM, Legido A, Chandler DA, Miles DK, Grover WD. West syndrome in tuberous sclerosis complex. Pediatr Neurol. 2000 Sep;23(3):233–235.
Journal cover image

Published In

Pediatr Neurol

DOI

ISSN

0887-8994

Publication Date

September 2000

Volume

23

Issue

3

Start / End Page

233 / 235

Location

United States

Related Subject Headings

  • Tuberous Sclerosis
  • Treatment Outcome
  • Survival Rate
  • Spasms, Infantile
  • Neurology & Neurosurgery
  • Male
  • Magnetic Resonance Imaging
  • Infant
  • Humans
  • Follow-Up Studies