West Syndrome (Infantile Spasm) - Revision history

EEGpedia at 16:25, 29 July 2017

2017-07-29T16:25:14Z

EEGpedia at 10:18, 13 July 2017

2017-07-13T10:18:19Z

EEGpedia at 10:11, 13 July 2017

2017-07-13T10:11:29Z

EEGpedia: Created page with "West syndrome ==Background== * Severe epilepsy syndrome with the triad: Infantile spasm Hypsarrhythmia ** Mental retardation * Onset: 90% in the first 12 months after b..."

2017-07-13T10:11:00Z

Created page with "West syndrome ==Background== * Severe epilepsy syndrome with the triad: ** Infantile spasm ** Hypsarrhythmia ** Mental retardation * Onset: 90% in the first 12 months after b..."

New page

West syndrome

==Background==
* Severe epilepsy syndrome with the triad:
** Infantile spasm
** Hypsarrhythmia
** Mental retardation
* Onset: 90% in the first 12 months after birth, with a peak onset between 4 and 6 months
* Incedence: 3 to 5/10,000 live births
* 2% of childhood epilepsies
* Classified according to etiology (old International League Against Epilepsy (ILAE) classification)
** Symptomatic (almost any disorder than can cause brain damage) ie:
*** Trisomy 21 (Down syndrome)
*** Trauma
*** Infectious (meningitis, encephalitis)
*** Metabolic disorders (ie maple syrup urine disease, mitochondrial encephalopathy, pyridoxine dependency)
*** Hydrocephalus/microcephaly
*** Tuberous sclerosis
*** Ischemic encephalopathy
** Cryptogenic
*** If a symptomatic cause is most probable, however the cause is not identified.
*** 8-42% of the West syndromes
** Idiopathic
*** 9-14% of the West syndromes
*** In contrast to cryptogenic West, there is a normal development until the onset.
*** No MRI abnormalities
* New International League Against Epilepsy (ILAE) classification:
** Genetic
** Structural/metabolic
** Unknown

==Clinic==
* Psychomotor arrest or regression
* Spasms, sudden contraction of the trunk en limb lasting around 2 - 10 seconds
** Flexor spasm (Salaam spasm): Rapid bending of the head and torso forward and simultaneous raising and bending of the arms, similar to the greeting between muslims (Salaam)
** Extensor spasm: Extension of the neck and trunk and with extension and abduction of the limbs
** Mixed spasm
* 1 to 30 clusters/day of 20 to 100 spasms each cluster

==EEG==
'''Interictal: '''<br />
* Hypsarrhythmia: high voltage, chaotic, slow waves and spikes in all cortical areas. The spikes originate from multiple foci and have no rhythmical or repetitive organization.
* The typical hypsarrhythmia is usually seen in the early stage of West syndrome
* The EEG gradually evolves to a more synchronized, with increased hemispheric synchronization en symmetry.
* Hypsarrhytmia is most pronounced in sleep and the patients have reduces or absent non-REM sleep.
* Other interictal patterns: diffused (encephalopathic) or focal slowing, multifocal [[Spike slow wave complex]]
* The EEG can evolve towards a EEG seen in [[Lennox Gastaut syndrome]]

'''Ictal EEG'''<br />
* High-voltage, frontal dominant, generalized slow-wave transient followed by voltage attenuation

==Treatment==
*ACTH
*Glucocorticoids
*Vigabatrin
*Zonisamide

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'''''West syndrome with hypsarrhytmia (multifocal spike and slow waves, with little organization) in a 8 months old girl (average)'''''
[[File:West_syndrome_with_hypsarrhytmia_in_a_8_months_old_girl_(average)_EEGpedia.png|border|none|1200px|left]]

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← Older revision		Revision as of 10:11, 13 July 2017
Line 50:		Line 50:
	'''Ictal EEG'''<br />		'''Ictal EEG'''<br />
	* High-voltage, frontal dominant, generalized slow-wave transient followed by voltage attenuation		* High-voltage, frontal dominant, generalized slow-wave transient followed by voltage attenuation
−
−	~~==Treatment==~~
−	*ACTH
−	*Glucocorticoids
−	*Vigabatrin
−	*Zonisamide

@@ Line 40: / Line 40: @@
 ==EEG==
 '''Interictal: '''<br />
-* Hypsarrhythmia: high voltage, chaotic, slow waves and spikes in all cortical areas. The spikes originate from multiple foci and have no rhythmical or repetitive organization.
+* '''Hypsarrhythmia''': high voltage, chaotic, slow waves and spikes in all cortical areas. The spikes originate from multiple foci and have no rhythmical or repetitive organization.
 * The typical hypsarrhythmia is usually seen in the early stage of West syndrome
 * The EEG gradually evolves to a more synchronized, with increased hemispheric synchronization en symmetry.
 * Hypsarrhytmia is most pronounced in sleep and the patients have reduces or absent non-REM sleep.
 * Other interictal patterns: diffused (encephalopathic) or focal slowing, multifocal [[Spike slow wave complex]]
-* The EEG can evolve towards a EEG seen in [[Lennox Gastaut Syndrome]]
+* The EEG can evolve towards a EEG seen in '''[[Lennox Gastaut Syndrome]]'''

West Syndrome (Infantile Spasm) - Revision history

EEGpedia at 16:25, 29 July 2017

EEGpedia at 10:18, 13 July 2017

EEGpedia at 10:11, 13 July 2017

EEGpedia: Created page with "West syndrome ==Background== * Severe epilepsy syndrome with the triad: ** Infantile spasm ** Hypsarrhythmia ** Mental retardation * Onset: 90% in the first 12 months after b..."

EEGpedia: Created page with "West syndrome ==Background== * Severe epilepsy syndrome with the triad: Infantile spasm Hypsarrhythmia ** Mental retardation * Onset: 90% in the first 12 months after b..."