3. Case 01
• 4 month old baby boy
• Presented with sudden flexion of upper and lower
limbs, just few seconds
• Complicated birth History
• Development delay
4. • EEG - Hypsarrhythmia
• Treatment -High dose steroids
• Spasms resolved and EEG cleared partially in
2 week, completely at 4 weeks.
• On intense habilitation program
5. Case 02
• 8 month old baby girl
• Subtle head drops in clusters lasting about 10
• 3 -5 cluster/day; on awakening or falling
• Development was mildly delayed but
regression also noted with the onset of head
6. • On clinical examination he had hypomelanotic
macules, shagreen patches
• MRI brain showed cortical tubers in left
frontal lobe and right parietal lobe
• 2D ECO showed Rhabdomyoma
8. • EEG shows typical hypsarrhythmia
• Clinical and Investigation findings confirms
• At 8 month of age started Vigabatrin with
reasonable spasm control at 2 weeks of
treatment and further continued for 6 months
with very good spasm control.
9. West syndrome
• 2017 ILAE classification categorize epileptic
spasms under category of developmental and
• Epileptic spasms are mostly seen in West
• Epileptic spasms/ Infantile spasms/ West
Syndrome are synonymously used
• It is rare, affecting 3 to 5 per 10,000 live births
10. West syndrome
• Severe childhood epileptic encephalopathy
• Infantile spasms/ Epileptic spasms
• Electroencephalograph shows Hypsarrhythmia
• Development arrest or retardation
• WS comprises 2% of childhood epilepsies and
13%‐45% of infantile‐onset epilepsies
• Dr William James
West, surgeon in
England, gave the first
detailed description of
infantile spasms, which
occurred in his own
child, James Edwin
• In a letter to the editor
of The Lancet in 1841,
West described the
events as "bobbings"
12. • This detailed clinical description was followed
approximately 100 years later by the report of
the typical interictal EEG pattern termed
• The eponym West syndrome was created in the
early 1960s by Drs. Gastaut, Poirier, and
13. Epileptic Spasms
• Manifest as involuntary contractions of the
head, neck, and trunk and/or uncontrolled
flexion or extension of the legs and/or arms.
• The duration, intensity, and muscle groups
affected by seizures vary from infant to infant.
• These spasms may occur upon drowsiness,
awakening or after feeding but all along the
day these can be there.
• Benign Sleep Myoclonus
• Benign Myoclonus of Infancy
• Infantile Colic
• Benign Infantile Head Drops
• Exaggerated startles caused by Colic or to
other external stimuli
• Early infantile developmental and epileptic
– < 3 months of age.
– Spasms may be present,
– Other seizure types may tonic, myoclonic and sequential
seizures typically co-exist.
• Myoclonic Epilepsy of Infancy :
– Presents with myoclonic seizures, not epileptic spasms.
– The EEG and EMG can distinguish myoclonus from
– There should be a normal background with generalized
spike wave discharges.
23. The ictal EEG
• The ictal recording of a spasm is characterized
– A high amplitude,
– Generalized sharp slow waves
– Followed by low amplitude, fast activity which
may appear as a brief electrodecrement.
– Hypsarrhythmia typically attenuates or stops
during a series of epileptic spasms.
– EMG helps to distinguish epileptic spasms from
myoclonic seizures and tonic seizures.
• Infantile spasms are difficult to treat.
• Conventional ASMs are not effective
• Hormonal therapies used since 1958
• Vigabatrin approved in the 1990s as an
effective treatment for infantile spasms,
especially in tuberous sclerosis
28. • According to the United Kingdom Infantile
Spasms Study- UKISS an advantage of using
steroids (73%) over vigabatrin (56%) is clearly
• They described that short term spasm control is
better with steroid treatment than with vigabatrin.
• It further showed that better cognitive outcome
(assessed at 18 and 42 months) occurs in those
with cryptogenic IS if treated with steroids than
30. Side Effects
• Increased appetite
• Weight gain
• Gastro- intestinal upset.
• Systemic hypertension
• Sleep disturbance
• Allergic rash or
• Increased appetite
• Weight gain
• Visual field constriction
• Susceptibility to infection
• Gastro-intestinal upset
• Sleep disorder
32. Other Treatment Modalities
• Epilepsy surgery
– Consider in atypical, asymmetrical spasms, or
other suggestion of focality in seizure semiology,
supported by lesional identification on
neuroimaging and localisation on EEG.
• Ketogenic Diet
– In Medical refractory cases
34. Long term outcome
• Rarely spasms may persist in adulthood
• 50-70% of patients evolve to other epilepsies
• 18-50% Lenox Gastaut Syndrome or focal
• Some genetic etiologies have better prognosis
• The long-term overall prognosis is poor
• Directly related to the etiology
• Normal or near normal development in only
• Seventeen percent had an IQ >85
• In 20-35 years of follow up, nearly all patients
with a normal or near normal outcome held
36. Good Prognostic factors
• Children with unknown etiology
• Short time lag from onset to treatment
• Early and sustained response to treatment
• Normal development prior to onset of epileptic
• Around 70 % of case of WEST syndrome is
related to acquired brain injuries
• Identify subtle spasms in children who has
• Other 10 % of case of unknown etiology,
prompt identification of clinical symptoms
diagnosis and treatment is the mainstay of
achieving better prognosis.
• Parental education is utmost important during
antenatal clinic visits regarding these
catastrophic epilepsy type.
• Unknown etiology group make 10% of overall
disease burden but it has the best prognosis.
• Who gets the disease is unknown for this
group, no specialized risk factors found.
• Early identification and treatment is the only
hope to raise the sun to the WEST Syndrome.
• WEST syndrome is a developmental and
epileptic encephalopathy and neurological
emergency in infants.
• It has unique presentation irrespective of its
large number of etiologies.
• EEG is Pathognomonic and Diagnostic
• WEST syndrome is treated with steroids.
• Early identification and early treatment is the
only hope for a better outcome as each
additional day without treatment costs in final
• Parental education on symptom identification
is utmost important.
• Andrew L Lux, et al The United Kingdom Infantile Spasms Study comparing
vigabatrin with prednisolone or tetracosactide at 14 days: a multicentre,
randomised controlled trial
• Sameer M Zuberi1 et al ILAE Classification & Definition of Epilepsy Syndromes in
the Neonate and Infant: Position Statement by the ILAE Task Force on Nosology
• Surana et al Infantile Spasms: Etiology, lead time and treatment response in a
resource limited setting.
• Matti Sillanaa et al Long‐term mortality of patients with West syndrome
• Priyanka Madaan et al Management practices for West syndrome in South Asia: A
survey study and
• Riikonen R. et al Long-term outcome of patients with West syndrome. Brain Dev 2001;23:683-7
• Darke et al Developmental and epilepsy outcomes at age 4 years in the UKISS trial
comparing hormonal treatments to vigabatrin for infantile spasms: a multi-centre
randomised trial. Arch Dis Child 2010;95:382-6. 73.