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EEG IN WEST
SYNDROME:
Dr.Roopchand.PS
Senior Resident Academic
Department of Neurology
TDMC, Alappuzha
Overview
• Severe epilepsy syndrome composed of the triad of
  infantile spasms, an interictal EEG pattern termed
  hypsarrhythmia, and mental retardation.
• Dr W J West, gave the first detailed description of
  infantile spasms.
• Published in The Lancet in 1841.
• Generalized Flexion Epilepsy, Infantile Epileptic
  Encephalopathy, Infantile Myoclonic Encephalopathy,
  jackknife convulsions, Massive Myoclonia , Salaam
  spasms.
• Reflect abnormal interactions between the cortex and
  brainstem structures.
• Insult to the immature CNS.
• Brain-adrenal axis.
  • stressors in the immature brain produces an abnormal, excessive
   secretion of corticotropin-releasing hormone (CRH), causing
   spasms.
• Based on etiology classified in to
  • Symptomatic
  • Cryptogenic
  • idiopathic.
• Two specific genetic defects have a phenotypic
 presentation similar to that of the early onset of infantile
 spasms.
  • gene ARX mutation
  • cyclin-dependent kinase-like protein 5 (CDKL5) mutation
• 2% of childhood epilepsies, 25% of epilepsy with onset in
  the first year of life.
• Males are affected slightly more then females.
• Onset is before 12 mo of age.
  • Peak onset between 4 to 6 mo.
• Only 14% of infants with symptomatic West syndrome
  have normal or borderline-normal cognitive development.
• 50-70% of patients develop other seizure types.
• 18-50% of patients will develop Lennox-Gastaut
  syndrome or some other form of symptomatic generalized
  epilepsy.
• 70% dies before 20 yrs of age.
Hypsarrhythmia:
• Hypsarrhythmia is the characteristic interictal EEG
 pattern.
• Chaotic, high- to extremely high–voltage, polymorphic
    delta and theta rhythms with superimposed multifocal
    spikes and wave discharges.
•   Gibbs and Gibbs described hypsarrhythmia in 1952.
•   Unilateral hypsarrhythmia and asymmetrical ictal EEG
    changes during spasms are correlated with focal or
    asymmetrical cerebral lesions on imaging studies.
•   Hypsarrhythmia either disappears or improves during a
    cluster of spasms and/or REM sleep.
•   Hypsarrhythmia rarely persists beyond the age of 24
    months.
Defining characteristics(Gibs and Gibs):


• Essentially continuous
• Present in both awake and sleep
• Consist of random high voltage slow waves and spikes.
• Spikes vary in location and duration – focal or multifocal
• Occasionally generalized discharges but never in a
 rhythmic or highly organized pattern.
• Five variants of the "classical" hypsarrhythmic pattern
    have been identified.
•   Hypsarrhythmia with increased interhemispheric
    synchronization (35%).
•   Asymmetric hypsarrhythmia (12%).
•   Hypsarrhythmia with a consistent focus of abnormal
    discharge (26%).
•   Hypsarrhythmia with episodes of voltage attenuation
    (11%).
•   Hypsarrhythmia without spike or sharp activity (7%).
Hypsarrhythmia with increased
interhemispheric synchronization:
Asymmetric hypsarrhythmia:
Focal Hypsarrhythmia:
Hypsarrhythmia with episodes of voltage
attenuation:
Factors influencing Hypsarrhythmia
pattern:
• It is a highly dynamic pattern:
• Sleep:
  • NREM – increase in amplitude of waves, grouping of spikes,
    sharps and slow waves, sometimes attenuation.
  • REM: complete to near complete disappearance of hypsarrhythmia
    pattern.
  • Normalization can also be seen upon waking up.
• Ictal events:
   • After a seizure episode there can be transient periods of decreased
     abnormal activity and normalization of background.
• Evolution with time:
  • Hypsarrhythmia pattern tends to decrease with time and
    disappears by 5 to 7 years.
Precursors of hypsarrhythmia:




• Focal or multifocal spikes
• B/L parieto temporal dominant spikes
• Burst suppression pattern.
Significance of interictal pattern:
• Diagnostic value: very strong indicator of infantile spasms.
• Correlation with etiology:
  • Asymmetric and focal findings correlate with a symptomatic
    etiology.
  • Hemi hypsarrhythmia pattern seen in cerebral dysgenesis
  • HIE- absence of sleep pattern.
• Correlation with outcome:
ICTAL PATTERNS:



• Kellaway et al.
• Described 11 different patterns.
• High amplitude fast activity is the most commonest.
• Asymmetry indicates possible symptomatic etiology.
• Infantile Spasms: Diagnosis, Management and
 Prognosis
 • James D. Frost Jr., Richard A. Hrachovy - 2003
We st syndrome eeg

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We st syndrome eeg

  • 1. EEG IN WEST SYNDROME: Dr.Roopchand.PS Senior Resident Academic Department of Neurology TDMC, Alappuzha
  • 2. Overview • Severe epilepsy syndrome composed of the triad of infantile spasms, an interictal EEG pattern termed hypsarrhythmia, and mental retardation. • Dr W J West, gave the first detailed description of infantile spasms. • Published in The Lancet in 1841. • Generalized Flexion Epilepsy, Infantile Epileptic Encephalopathy, Infantile Myoclonic Encephalopathy, jackknife convulsions, Massive Myoclonia , Salaam spasms.
  • 3. • Reflect abnormal interactions between the cortex and brainstem structures. • Insult to the immature CNS. • Brain-adrenal axis. • stressors in the immature brain produces an abnormal, excessive secretion of corticotropin-releasing hormone (CRH), causing spasms. • Based on etiology classified in to • Symptomatic • Cryptogenic • idiopathic.
  • 4. • Two specific genetic defects have a phenotypic presentation similar to that of the early onset of infantile spasms. • gene ARX mutation • cyclin-dependent kinase-like protein 5 (CDKL5) mutation • 2% of childhood epilepsies, 25% of epilepsy with onset in the first year of life. • Males are affected slightly more then females. • Onset is before 12 mo of age. • Peak onset between 4 to 6 mo.
  • 5. • Only 14% of infants with symptomatic West syndrome have normal or borderline-normal cognitive development. • 50-70% of patients develop other seizure types. • 18-50% of patients will develop Lennox-Gastaut syndrome or some other form of symptomatic generalized epilepsy. • 70% dies before 20 yrs of age.
  • 6. Hypsarrhythmia: • Hypsarrhythmia is the characteristic interictal EEG pattern.
  • 7. • Chaotic, high- to extremely high–voltage, polymorphic delta and theta rhythms with superimposed multifocal spikes and wave discharges. • Gibbs and Gibbs described hypsarrhythmia in 1952. • Unilateral hypsarrhythmia and asymmetrical ictal EEG changes during spasms are correlated with focal or asymmetrical cerebral lesions on imaging studies. • Hypsarrhythmia either disappears or improves during a cluster of spasms and/or REM sleep. • Hypsarrhythmia rarely persists beyond the age of 24 months.
  • 8. Defining characteristics(Gibs and Gibs): • Essentially continuous • Present in both awake and sleep • Consist of random high voltage slow waves and spikes. • Spikes vary in location and duration – focal or multifocal • Occasionally generalized discharges but never in a rhythmic or highly organized pattern.
  • 9. • Five variants of the "classical" hypsarrhythmic pattern have been identified. • Hypsarrhythmia with increased interhemispheric synchronization (35%). • Asymmetric hypsarrhythmia (12%). • Hypsarrhythmia with a consistent focus of abnormal discharge (26%). • Hypsarrhythmia with episodes of voltage attenuation (11%). • Hypsarrhythmia without spike or sharp activity (7%).
  • 13. Hypsarrhythmia with episodes of voltage attenuation:
  • 14. Factors influencing Hypsarrhythmia pattern: • It is a highly dynamic pattern: • Sleep: • NREM – increase in amplitude of waves, grouping of spikes, sharps and slow waves, sometimes attenuation. • REM: complete to near complete disappearance of hypsarrhythmia pattern. • Normalization can also be seen upon waking up. • Ictal events: • After a seizure episode there can be transient periods of decreased abnormal activity and normalization of background. • Evolution with time: • Hypsarrhythmia pattern tends to decrease with time and disappears by 5 to 7 years.
  • 15. Precursors of hypsarrhythmia: • Focal or multifocal spikes • B/L parieto temporal dominant spikes • Burst suppression pattern.
  • 16. Significance of interictal pattern: • Diagnostic value: very strong indicator of infantile spasms. • Correlation with etiology: • Asymmetric and focal findings correlate with a symptomatic etiology. • Hemi hypsarrhythmia pattern seen in cerebral dysgenesis • HIE- absence of sleep pattern. • Correlation with outcome:
  • 17. ICTAL PATTERNS: • Kellaway et al. • Described 11 different patterns. • High amplitude fast activity is the most commonest. • Asymmetry indicates possible symptomatic etiology.
  • 18. • Infantile Spasms: Diagnosis, Management and Prognosis • James D. Frost Jr., Richard A. Hrachovy - 2003