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WEST SYNDROME
    Dr.Dhritiman Choudhury
    Tripura medical college
introduction
• West syndrome is a severe epilepsy syndrome
  composed of the triad of infantile spasms, an
  interictal electroencephalogram (EEG) pattern
  termed hypsarrhythmia, and mental
  retardation, although the diagnosis can be
  made even if 1 of the 3 elements is missing
  (according to international classification)
• The syndrome's namesake, Dr W J West, gave the
  first detailed description of infantile
  spasms, which occurred in his own child.In a
  letter to the editor of The Lancet in 1841, West
  described the events as "bobbings" that "cause a
  complete heaving of the head forward towards
  his knees, and then immediately relaxing into the
  upright position.These bowings and relaxings
  would be repeated alternately at intervals of a
  few seconds, and repeated from 10 to 20 or more
  times at each attack, which would not continue
  more than 2 or 3 minutes; he sometimes has 2, 3
  or more attacks in the day."
Classification based on etiology:
• Symptomatic:Patients are diagnosed with
  symptomatic infantile spasms if an identifiable
  factor is responsible for the syndrome.
  Virtually any disorder that can produce brain
  damage can be associated with infantile
  spasms.
  – Hydrocephalus
  – Microcephaly
  – Sturge weber syndrome
– Tuberous sclerosis
– HIE
– Congenital infections
– Meningitis
– Encephalitis
– Pyridoxine deficiency
– Maple syrup urine disease
– Phenylketonuria
– Biotinidase deficiency
– Trauma
• Cryptogenic:Patients have cryptogenic
  infantile spasms if no cause is identified but a
  cause is suspected and the epilepsy is
  presumed to be symptomatic.
• Idiopathic:Patients may be considered to have
  idiopathic infantile spasms if normal
  psychomotor development occurs prior to the
  onset of symptoms, no underlying disorders or
  presumptive causes are present, and no
  neurologic or neuroradiologic abnormalities
  exist. Some investigators use the terms
  idiopathic and cryptogenic interchangeably.
A case study:
• A 5 month old baby presented with
  generalized convulsion since 20 days.
• Frequency: 4-5/day each lasting for 2-4 min.
• h/o cough-10days
• No H/O LOC ,vomiting, fever, trauma.
• No autonomic involvement
• Perinatal history-uneventful.
• Immunization up to date.
• No similar history in the family.
• No history of drug intake.
On examination:
• Conscious,feeding well.
• No bulging of fontanel.
• No evidence of meningeal irritation.
• No pallor, cyanosis, icterus,
  lymphadenopathy,hepatosplenomegaly or
  abdominal mass.
• No evidence of any dermatologic lesion.
• Developmental milestones normal.
Ictal manifestations:
• Spasms begin with a sudden, rapid, tonic
  contraction of trunk and limb musculature
  that gradually relaxes over 0.5-2 seconds.
  Spasms last for 3-4 sec. The intensity of
  spasms may vary from a subtle head nodding
  to a powerful contraction of the body.
• The spasms are of mixed variety consisting of
  flexion of the neck and trunk with extension
  and abduction of limbs. They are associated
  with a cry. The patient then relaxes, and the
  jerk repeats. These attacks occur in clusters
  throughout the day and last for 2-4 min.
• These manifestations are also known as
  salaam or jackknife attacks: a flexor spasm
  with rapid bending of the head and torso
  forward and simultaneous raising and bending
  of the arms while partially drawing the hands
  together in front of the chest. If one imagined
  this act in slow motion, it would appear
  similar to the ceremonial greeting (Salaam).
Investigations:
•   Hb 11.0 gm%
•   TLC 9,400/cumm
•   DLC 52/45/01/02/00
•   ESR 18mm/1st hr
•   Sr. Ca 8.9 mg/dl
•   NCCT brain- within normal limit.
•   ECG- within normal limit.
•   EEG- hypsarrhythmia.
hypsarrhythmia
• Hypsarrhythmia (seen in the image below) is
  the characteristic interictal EEG pattern. It
  consists of chaotic, high- to extremely high–
  voltage, polymorphic delta and theta rhythms
  with superimposed multifocal spikes and wave
  discharges.
Treatment:
• Compared with other forms of epilepsy, West
  syndrome is difficult to treat. To raise the
  chance of successful treatment and keep
  down the risk of longer-lasting effects, it is
  very important that the condition is diagnosed
  as early as possible and that treatment begins
  straight away. However, there is no guarantee
  that therapy will work even in this case.
Treatment options
Commonly used first-line treatments for infants with
West syndrome include the following:
      ACTH
      Vigabatrin
      Prednisone
      Pyridoxine (vitamin B-6)
Second-line treatments include the following:
      Benzodiazepines
      Valproic acid
      Lamotrigine
      Topiramate
      Zonisamide
      Levetiracetam
Prednisone
• A 2004 American Academy of Neurology and Child
  Neurology Society practice parameter concluded that
  "there is insufficient evidence that oral corticosteroids
  are effective in the treatment of infantile spasms”
• One study found that after approximately 2 weeks,
  hormonal therapy provided better relief from spasm
  than did vigabatrin. The 2004 multicenter, randomized,
  controlled trial compared hormonal therapy (either
  oral prednisolone or IM tetracosactide depot) with
  vigabatrin in 107 infants with infantile spasms. More
  infants assigned hormonal treatments (73%) had no
  spasms on days 13 and 14 than did infants assigned
  vigabatrin (54%).
Pyridoxine
• Two distinct treatment situations exist in which
  pyridoxine is used in patients with West syndrome.
• First is intravenous (IV) administration during
  diagnostic EEG to assess whether the patient's seizures
  and EEG abnormalities are related to pyridoxine
  deficiency. In this approach, administer 50-100 mg IV
  during a diagnostic EEG; if dramatic improvement is
  noted in the EEG, the patient is believed to have
  pyridoxine-dependent seizures.
• Second is long-term oral administration. The
  effectiveness of long-term, oral, high-dose pyridoxine
  in West syndrome has been investigated in multiple
  open-label studies, with promising results. Most
  patients who respond to long-term, oral, high-dose
  pyridoxine do so within 1-2 weeks of initiation.
Valproic acid
• Valproic acid is considered an effective
  second-line AED therapy against spasms
  associated with West syndrome.
• Dose-10-15 mg/kg/day PO/IV divided q6-8h
• Monitor: LFTs
ACTH
• A 2004 American Academy of Neurology and
  Child Neurology Society practice parameter
  concluded that "ACTH is probably effective for
  the short-term treatment of infantile spasms
  and in resolution of hypsarrhythmia” and
  "here is insufficient evidence to recommend
  the optimum dosage and duration of
  treatment with ACTH for the treatment of
  infantile spasms."
• Corticotropin is associated with serious,
  potentially life-threatening adverse effects. It
  must be administered intramuscularly, and such
  injections are painful for the infant to receive and
  are unpleasant for the parent to perform.
• A prospective, single-blind study demonstrated
  no difference in effectiveness between high-dose,
  long-duration corticotropin (150 U/m2/day for 3
  wk, tapering over 9 wk) and low-dose, short-
  duration corticotropin (20-30 U/day for 2-6 wk,
  tapering over 1 wk with respect to spasm
  cessation and improvement in the patient's EEG.
  Hypertension was more common with larger
  doses
Vigabatrin
• Vigabatrin is indicated as monotherapy for
  children aged 1 month to 2 year with infantile
  spasms. Its precise mechanism of action is
  unknown. The drug is a selective, irreversible
  inhibitor of gamma-aminobutyric acid
  transaminase (GABA-T). GABA-T metabolizes
  GABA, an inhibitory neurotransmitter, thereby
  increasing CNS GABA levels. Vigabatrin use must
  be weighed against the risk of permanent vision
  loss.Vigabatrin was approved by the US Food and
  Drug Administration (FDA) in August 2009. It is
  available only from a restricted access program.
Topiramate
• Topiramate is a sulfamate-substituted
  monosaccharide with a broad spectrum of
  antiepileptic activity that may have state-
  dependent sodium channel blocking action, may
  potentiate the inhibitory activity of the
  neurotransmitter GABA, and may block glutamate
  activity.
• A 2004 American Academy of Neurology and
  Child Neurology Society practice parameter
  concluded that "there is insufficient evidence to
  recommend topiramate for the treatment of
  infantile spasms."
Levetiracetam
• Levetiracetam's mechanism of action is the
  inhibition of N-type calcium channels, the
  modulation of GABA and glycine receptors,
  and binding to SVA2 protein.
• An open-label trial of 5 infants with new-
  onset, cryptogenic infantile spasms showed
  levetiracetam to be clinically effective. Two
  children became seizure free, while 2 others
  showed a minimum of 50% reduction in
  seizures. The dose ranged from 30-60
  mg/kg/day.
clonazepam
• Clonazepam is considered a second-line AED
  therapy against spasms associated with West
  syndrome. However, adverse effects and the
  development of tolerance limit the drug's
  usefulness over time. Nitrazepam and
  clobazam are not approved by the FDA but are
  available in many countries worldwide.
Treatment given
• Methyl prednisone 1mg/kg/day
• Pyridoxine hydrochloride 10mg/day
• Sodium valproate 10-15mg/kg/day
There was only a single episode of seizure after
the commencement of the steroid therapy.
Prognosis
• It is not possible to make a generalised
  prognosis for development due to the
  variability of causes, the differing types of
  symptoms and etiology. Each case must be
  considered individually.
• The prognosis for children with idiopathic
  West syndrome are mostly more positive than
  for those with the cryptogenic or symptomatic
  forms.
• A large proportion (up to 90%) of children suffer
  severe physical and cognitive impairments, even
  when treatment for the attacks is successful. This is
  not usually because of the epileptic fits, but rather
  because of the causes behind them (cerebral
  anomalies or their location or degree of severity).
• Permanent damage often associated with West
  syndrome in the literature include cognitive
  disabilities, learning difficulties and behavioural
  problems, cerebral palsy (up to 5 out of 10 children),
  psychological disorders and often autism (in around 3
  out of 10 children). Once more, the etiology of each
  individual case of West syndrome must be
  considered when debating cause and effect.
• Statistically, 5 out of every 100 children with
  West syndrome do not survive beyond five years
  of age, in some cases due to the cause of the
  syndrome, in others for reasons related to their
  medication. Only less than half of all children can
  become entirely free from attacks with the help
  of medication. Statistics show that treatment
  produces a satisfactory result in around three out
  of ten cases, with only one in every 25 children's
  cognitive and motoric development developing
  more or less normally.
• Sometimes West syndrome turns into a focal or
  other generalised epilepsy. Around half of all
  children develop Lennox-Gastaut syndrome.
Thank you

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West syndrome

  • 1. WEST SYNDROME Dr.Dhritiman Choudhury Tripura medical college
  • 2. introduction • West syndrome is a severe epilepsy syndrome composed of the triad of infantile spasms, an interictal electroencephalogram (EEG) pattern termed hypsarrhythmia, and mental retardation, although the diagnosis can be made even if 1 of the 3 elements is missing (according to international classification)
  • 3. • The syndrome's namesake, Dr W J West, gave the first detailed description of infantile spasms, which occurred in his own child.In a letter to the editor of The Lancet in 1841, West described the events as "bobbings" that "cause a complete heaving of the head forward towards his knees, and then immediately relaxing into the upright position.These bowings and relaxings would be repeated alternately at intervals of a few seconds, and repeated from 10 to 20 or more times at each attack, which would not continue more than 2 or 3 minutes; he sometimes has 2, 3 or more attacks in the day."
  • 4. Classification based on etiology: • Symptomatic:Patients are diagnosed with symptomatic infantile spasms if an identifiable factor is responsible for the syndrome. Virtually any disorder that can produce brain damage can be associated with infantile spasms. – Hydrocephalus – Microcephaly – Sturge weber syndrome
  • 5. – Tuberous sclerosis – HIE – Congenital infections – Meningitis – Encephalitis – Pyridoxine deficiency – Maple syrup urine disease – Phenylketonuria – Biotinidase deficiency – Trauma
  • 6. • Cryptogenic:Patients have cryptogenic infantile spasms if no cause is identified but a cause is suspected and the epilepsy is presumed to be symptomatic. • Idiopathic:Patients may be considered to have idiopathic infantile spasms if normal psychomotor development occurs prior to the onset of symptoms, no underlying disorders or presumptive causes are present, and no neurologic or neuroradiologic abnormalities exist. Some investigators use the terms idiopathic and cryptogenic interchangeably.
  • 7. A case study: • A 5 month old baby presented with generalized convulsion since 20 days. • Frequency: 4-5/day each lasting for 2-4 min. • h/o cough-10days • No H/O LOC ,vomiting, fever, trauma. • No autonomic involvement • Perinatal history-uneventful. • Immunization up to date. • No similar history in the family. • No history of drug intake.
  • 8. On examination: • Conscious,feeding well. • No bulging of fontanel. • No evidence of meningeal irritation. • No pallor, cyanosis, icterus, lymphadenopathy,hepatosplenomegaly or abdominal mass. • No evidence of any dermatologic lesion. • Developmental milestones normal.
  • 9. Ictal manifestations: • Spasms begin with a sudden, rapid, tonic contraction of trunk and limb musculature that gradually relaxes over 0.5-2 seconds. Spasms last for 3-4 sec. The intensity of spasms may vary from a subtle head nodding to a powerful contraction of the body. • The spasms are of mixed variety consisting of flexion of the neck and trunk with extension and abduction of limbs. They are associated with a cry. The patient then relaxes, and the jerk repeats. These attacks occur in clusters throughout the day and last for 2-4 min.
  • 10. • These manifestations are also known as salaam or jackknife attacks: a flexor spasm with rapid bending of the head and torso forward and simultaneous raising and bending of the arms while partially drawing the hands together in front of the chest. If one imagined this act in slow motion, it would appear similar to the ceremonial greeting (Salaam).
  • 11. Investigations: • Hb 11.0 gm% • TLC 9,400/cumm • DLC 52/45/01/02/00 • ESR 18mm/1st hr • Sr. Ca 8.9 mg/dl • NCCT brain- within normal limit. • ECG- within normal limit. • EEG- hypsarrhythmia.
  • 12. hypsarrhythmia • Hypsarrhythmia (seen in the image below) is the characteristic interictal EEG pattern. It consists of chaotic, high- to extremely high– voltage, polymorphic delta and theta rhythms with superimposed multifocal spikes and wave discharges.
  • 13. Treatment: • Compared with other forms of epilepsy, West syndrome is difficult to treat. To raise the chance of successful treatment and keep down the risk of longer-lasting effects, it is very important that the condition is diagnosed as early as possible and that treatment begins straight away. However, there is no guarantee that therapy will work even in this case.
  • 14. Treatment options Commonly used first-line treatments for infants with West syndrome include the following: ACTH Vigabatrin Prednisone Pyridoxine (vitamin B-6) Second-line treatments include the following: Benzodiazepines Valproic acid Lamotrigine Topiramate Zonisamide Levetiracetam
  • 15. Prednisone • A 2004 American Academy of Neurology and Child Neurology Society practice parameter concluded that "there is insufficient evidence that oral corticosteroids are effective in the treatment of infantile spasms” • One study found that after approximately 2 weeks, hormonal therapy provided better relief from spasm than did vigabatrin. The 2004 multicenter, randomized, controlled trial compared hormonal therapy (either oral prednisolone or IM tetracosactide depot) with vigabatrin in 107 infants with infantile spasms. More infants assigned hormonal treatments (73%) had no spasms on days 13 and 14 than did infants assigned vigabatrin (54%).
  • 16. Pyridoxine • Two distinct treatment situations exist in which pyridoxine is used in patients with West syndrome. • First is intravenous (IV) administration during diagnostic EEG to assess whether the patient's seizures and EEG abnormalities are related to pyridoxine deficiency. In this approach, administer 50-100 mg IV during a diagnostic EEG; if dramatic improvement is noted in the EEG, the patient is believed to have pyridoxine-dependent seizures. • Second is long-term oral administration. The effectiveness of long-term, oral, high-dose pyridoxine in West syndrome has been investigated in multiple open-label studies, with promising results. Most patients who respond to long-term, oral, high-dose pyridoxine do so within 1-2 weeks of initiation.
  • 17. Valproic acid • Valproic acid is considered an effective second-line AED therapy against spasms associated with West syndrome. • Dose-10-15 mg/kg/day PO/IV divided q6-8h • Monitor: LFTs
  • 18. ACTH • A 2004 American Academy of Neurology and Child Neurology Society practice parameter concluded that "ACTH is probably effective for the short-term treatment of infantile spasms and in resolution of hypsarrhythmia” and "here is insufficient evidence to recommend the optimum dosage and duration of treatment with ACTH for the treatment of infantile spasms."
  • 19. • Corticotropin is associated with serious, potentially life-threatening adverse effects. It must be administered intramuscularly, and such injections are painful for the infant to receive and are unpleasant for the parent to perform. • A prospective, single-blind study demonstrated no difference in effectiveness between high-dose, long-duration corticotropin (150 U/m2/day for 3 wk, tapering over 9 wk) and low-dose, short- duration corticotropin (20-30 U/day for 2-6 wk, tapering over 1 wk with respect to spasm cessation and improvement in the patient's EEG. Hypertension was more common with larger doses
  • 20. Vigabatrin • Vigabatrin is indicated as monotherapy for children aged 1 month to 2 year with infantile spasms. Its precise mechanism of action is unknown. The drug is a selective, irreversible inhibitor of gamma-aminobutyric acid transaminase (GABA-T). GABA-T metabolizes GABA, an inhibitory neurotransmitter, thereby increasing CNS GABA levels. Vigabatrin use must be weighed against the risk of permanent vision loss.Vigabatrin was approved by the US Food and Drug Administration (FDA) in August 2009. It is available only from a restricted access program.
  • 21. Topiramate • Topiramate is a sulfamate-substituted monosaccharide with a broad spectrum of antiepileptic activity that may have state- dependent sodium channel blocking action, may potentiate the inhibitory activity of the neurotransmitter GABA, and may block glutamate activity. • A 2004 American Academy of Neurology and Child Neurology Society practice parameter concluded that "there is insufficient evidence to recommend topiramate for the treatment of infantile spasms."
  • 22. Levetiracetam • Levetiracetam's mechanism of action is the inhibition of N-type calcium channels, the modulation of GABA and glycine receptors, and binding to SVA2 protein. • An open-label trial of 5 infants with new- onset, cryptogenic infantile spasms showed levetiracetam to be clinically effective. Two children became seizure free, while 2 others showed a minimum of 50% reduction in seizures. The dose ranged from 30-60 mg/kg/day.
  • 23. clonazepam • Clonazepam is considered a second-line AED therapy against spasms associated with West syndrome. However, adverse effects and the development of tolerance limit the drug's usefulness over time. Nitrazepam and clobazam are not approved by the FDA but are available in many countries worldwide.
  • 24. Treatment given • Methyl prednisone 1mg/kg/day • Pyridoxine hydrochloride 10mg/day • Sodium valproate 10-15mg/kg/day There was only a single episode of seizure after the commencement of the steroid therapy.
  • 25. Prognosis • It is not possible to make a generalised prognosis for development due to the variability of causes, the differing types of symptoms and etiology. Each case must be considered individually. • The prognosis for children with idiopathic West syndrome are mostly more positive than for those with the cryptogenic or symptomatic forms.
  • 26. • A large proportion (up to 90%) of children suffer severe physical and cognitive impairments, even when treatment for the attacks is successful. This is not usually because of the epileptic fits, but rather because of the causes behind them (cerebral anomalies or their location or degree of severity). • Permanent damage often associated with West syndrome in the literature include cognitive disabilities, learning difficulties and behavioural problems, cerebral palsy (up to 5 out of 10 children), psychological disorders and often autism (in around 3 out of 10 children). Once more, the etiology of each individual case of West syndrome must be considered when debating cause and effect.
  • 27. • Statistically, 5 out of every 100 children with West syndrome do not survive beyond five years of age, in some cases due to the cause of the syndrome, in others for reasons related to their medication. Only less than half of all children can become entirely free from attacks with the help of medication. Statistics show that treatment produces a satisfactory result in around three out of ten cases, with only one in every 25 children's cognitive and motoric development developing more or less normally. • Sometimes West syndrome turns into a focal or other generalised epilepsy. Around half of all children develop Lennox-Gastaut syndrome.