Recommended
More Related Content
What's hot
What's hot (20)
Similar to West Syndrome Diagnosis and Treatment
Similar to West Syndrome Diagnosis and Treatment (20)
Recently uploaded
Recently uploaded (20)
West Syndrome Diagnosis and Treatment
- 1. Sunrise to “WEST” “Sunrise to WEST” Jeewana Leelaratne Senior Registrar in Paediatric Neurology “SUNRISE TO WEST”
- 2. Outline 1. Case scenarios 2. Clinical features 3. Etiology 4. Diagnosis 5. Treatment 6. Prognosis and Prevention
- 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 minutes • 3 -5 cluster/day; on awakening or falling asleep • Development was mildly delayed but regression also noted with the onset of head drops
- 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 Tuberous Sclerosis • 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 encephalopathy • Epileptic spasms are mostly seen in West Syndrome. • 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 syndrome • Infantile spasms/ Epileptic spasms • Electroencephalograph shows Hypsarrhythmia • Development arrest or retardation • WS comprises 2% of childhood epilepsies and 13%‐45% of infantile‐onset epilepsies
- 11. History • Dr William James West, surgeon in England, gave the first detailed description of infantile spasms, which occurred in his own child, James Edwin West, • 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 “hypsarrhythmia” • The eponym West syndrome was created in the early 1960s by Drs. Gastaut, Poirier, and Pampiglione.
- 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.
- 15. Etiological classification • Etiology is classified according to the ILAE 2017 epilepsy classification
- 17. Differential Diagnosis • Non Epileptic • Epileptic
- 18. Non-epileptic • Benign Sleep Myoclonus • Benign Myoclonus of Infancy • GORD • Infantile Colic • Benign Infantile Head Drops • Exaggerated startles caused by Colic or to other external stimuli
- 19. Epileptic • Early infantile developmental and epileptic encephalopathy : – < 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 epileptic spasms. – There should be a normal background with generalized spike wave discharges.
- 20. Interictal EEG • Hypsarrhythmia – High amplitude – Excessive slowing – Multifocal epileptiform discharges • NREM sleep record needed
- 23. The ictal EEG • The ictal recording of a spasm is characterized by – 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.
- 24. Treatment • 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
- 25. First-Line Treatments • ACTH • Prednisolone • Vigabatrin • Pyridoxine (vitamin B-6) • Well studied drugs of first choice for the spasms
- 26. West syndrome Unknown etiology /structural Prednisolone /ACTH Tuberous sclerosis Vigabatrin
- 28. • According to the United Kingdom Infantile Spasms Study- UKISS an advantage of using steroids (73%) over vigabatrin (56%) is clearly shown. • 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 with vigabatrin.
- 30. Side Effects Steroids Vigabatrin • Irritability • Increased appetite • Weight gain • Gastro- intestinal upset. • Systemic hypertension • Hyperglycemia • Sleep disturbance • Immunosuppression • Allergic rash or anaphylaxis • Drowsiness • Increased appetite • Weight gain • Visual field constriction • Susceptibility to infection • Gastro-intestinal upset • Sleep disorder
- 31. Second-Line Treatments • Benzodiazepines • Valproic acid • Lamotrigine • Topiramate • Zonisamide • Levetiracetam
- 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
- 33. Prognosis
- 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 epilepseis • Some genetic etiologies have better prognosis
- 35. Prognosis • The long-term overall prognosis is poor • Directly related to the etiology • Normal or near normal development in only 15-25% . • Seventeen percent had an IQ >85 • In 20-35 years of follow up, nearly all patients with a normal or near normal outcome held jobs .
- 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 spasms
- 37. Prevention • Around 70 % of case of WEST syndrome is related to acquired brain injuries • Identify subtle spasms in children who has birth insults. • Other 10 % of case of unknown etiology, prompt identification of clinical symptoms diagnosis and treatment is the mainstay of achieving better prognosis.
- 38. Prevention • 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.
- 39. Summary • 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
- 40. Summary • 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 cognitive achievement. • Parental education on symptom identification is utmost important.
- 41. References • 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 and Definitions • 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.
- 42. Thank you