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