West Syndrome

Synonyms:

a.k.a. Infantile spasms

Diagnosis:

Clinical features plus EEG

Clinical features:

Onset 3 months – 3 years, Jack-knifing, myoclonus
+/-mental retardation

Findings on Investigations:

EEG: hypsarrhythmia i.e. chaotic slow-wave high voltage background activity & sharp components
High amplitude (voltage >200 microV)
Slow waves, Sharps waves & spikes in all areas
Sharps & spikes appear randomly from many foci (different areas); also they lack rhythmicity or periodicity
Most pronounced during non-REM sleep & reduced/abolished during REM sleep
Ictally:

  • Electrodecremental peroid i.e. sudden flattening
  • Or Lower amplitude slow waves

Treatment:

Corticosteroids (prednisolone, ACTH)
Vigabatrin

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