Difference between revisions of "Lennox Gastaut Syndrome"
From EEGpedia
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==EEG== | ==EEG== | ||
− | * Spike | + | * [[Spike slow wave complex]] superimposed on an abnormal, slow background. |
* The [[Spike slow wave complex]] and [[Sharp wave slow wave complex]] are generalized at a frequency of 1.5 to 2.5 Hz (slow), usually with the highest amplitude frontal. | * The [[Spike slow wave complex]] and [[Sharp wave slow wave complex]] are generalized at a frequency of 1.5 to 2.5 Hz (slow), usually with the highest amplitude frontal. | ||
* Transient and shifting asymmetries are frequent | * Transient and shifting asymmetries are frequent | ||
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* Valproic acid, lamotrigine, topiramate, rufinamide, felbamate, and clobazam can be effective | * Valproic acid, lamotrigine, topiramate, rufinamide, felbamate, and clobazam can be effective | ||
* Carbamazepine can worsen the drop attacks | * Carbamazepine can worsen the drop attacks | ||
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+ | '''''Lennox gastaut in a 3 years and 10 months old boy (source) with multiple spike and slow waves and a slow basic rhythm''''' | ||
+ | [[File:Lennox_gastaut_in_a_3_years_and_10_months_old_boy_(source)_EEGpedia.png|border|none|1200px|left]] | ||
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Latest revision as of 15:16, 4 August 2017
Contents
Background
- Severe seizures in childhood
- Presentation before eight years of age, most commonly between three and five years of age
- Many cases evolve into Lennox Gastaut from other epilepsy syndromes, particularly West Syndrome (Infantile Spasm)
Clinics
- Mixture of seizure types
- Tonic
- Tonic-clonic
- Atonic (drop attacks)
- Myoclonic
- atypical absences
- Brief tonic seizures of seconds to 1 minute.
- The seizures can cause falls.
- Eyelid retraction, staring, mydriasis, and apnea are commonly associated
- The seizures are frequent
- Mental retardation before seizure onset in 20-60%
EEG
- Spike slow wave complex superimposed on an abnormal, slow background.
- The Spike slow wave complex and Sharp wave slow wave complex are generalized at a frequency of 1.5 to 2.5 Hz (slow), usually with the highest amplitude frontal.
- Transient and shifting asymmetries are frequent
- Non-REM sleep increases the discharges. In REM sleep the discharges decreases.
- Hyperventilation or photic stimulation does not trigger the discharges.
Ictal EEG
- Tonic seizure: typically fast rhythmic discarges of 10 – 20 Hz, increasing in amplitude.
- Myoclonic seizures: bursts of arrhythmic, multiple spike-wave or irregular spike-wave activity
- Atypical absence seizures: Slow (<2.5 Hz) and often asymmetric and irregular spike-and-wave activity
Treatment
- No drug is highly effective
- Valproic acid, lamotrigine, topiramate, rufinamide, felbamate, and clobazam can be effective
- Carbamazepine can worsen the drop attacks
Lennox gastaut in a 3 years and 10 months old boy (source) with multiple spike and slow waves and a slow basic rhythm