RT Book, Section
A1 Lerner, Jason T.
A1 Matsumoto, Joyce H.
A1 Wu, Joyce Y.
A2 Sirven, Joseph I.
A2 Stern, John M.
SR Print(0)
ID 1103049656
T1 Infantile Spasms
T2 Atlas of Video-EEG Monitoring
YR 2011
FD 2011
PB McGraw-Hill Education
PP New York, NY
SN 9780071597425
LK neurology.mhmedical.com/content.aspx?aid=1103049656
RD 2024/04/19
AB Infantile  spasms,  also  known  as  infantile  epileptic  encephalopathy  (IEE),  is  a  severe  form  of  childhood  epilepsy  that  typically  manifests  within  the  first  year  of  life  and  appears  to  be  tied  to  a  particular  neurodevelopmental  period.  A  high-amplitude,  chaotic  interictal  electroencephalographic  (EEG)  background,  known  as  hypsarrhythmia,  is  often  seen  in  association  with  infantile  spasms.  In  the  wake  of  infantile  spasms,  the  majority  of  children  are  left  with  significant  cognitive  delay.  The  triad  of  infantile  spasms,  developmental  arrest  or  regression,  and  the  characteristic  EEG  background  of  hypsarrhythmia  has  been  dubbed  West  syndrome,  after  the  English  physician  William  West,  who  first  provided  a  detailed  description  of  spasms  in  1841,  as  seen  in  his  4-month-old  son.1  The  broader  term  epileptic  spasms  is  sometimes  used  to  denote  events  of  a  similar  semiology  that  tend  to  present  in  a  slightly  older  age  range  and  are  not  associated  with  hypsarrhythmia  on  EEG.