Transcript Lennox Gastaut Syndrome

Lennox Gastaut
Syndrome
Enrique Feoli MD
North East Regional Epilepsy Group
Goals
Understand the definition of LGS
 Outline the clinical presentation of LGS
 Compare the different medications
available to treat LGS
 Develop a rational treatment approach for
LGS
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Syndrome types in different age groups
Infancy;
-West Syndrome
-Ohtahara Synd.
-Dravet Syndrome
Early Childhood (1-5y)
-LGS
-Febrile Seizures
Late Childhood (5-10y)
-Absence Epilepsy
-Landau-K syndrome
-Benign Rolandic E.
Adolescence
-JME
-Juvenile Absence epilepsy
LGS Prevalence and Incidence
Prevalence is 1 to 10 % of all childhood
epilepsy
 Incidence rate for LGS of all new onset
epilepsies is 0.6 %
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Etiology
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Brain malformations
Hypoxic-ischemic brain injury
Meningitis &Encephalitis/Congenital infections
Neurocutaneous syndromes-Tuberous sclerosis
Trauma
Brain tumors
Cortical dysplasia/Bilateral perisylvian syndrome
17-30% with LGS will have history of infantile spasms.
Few have metabolic issues such as Leigh’s
encephalomyelopathy or Channelopathies.
LGS Diagnosis; Pediatrics
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-Onset 3 to 8 years of age
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-Seizures type;
-Tonic-atonic drop attacks
-Tonic clonic
- Atypical absences;
Gradual onset and termination
Behavioral pause/stare (30 + seconds)
Clonic activity
LGS Diagnosis; Pediatrics Cont
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May present as non-convulsive status
epilepticus in 50 to 75 % of patients
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-Cognitive impairment and developmental delay.
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-EEG; irregular, gneneralized 2 to 2 ½ Hz spikes
and wave pattern
LGS Diagnosis; adult
Onset 4 to 8 years of age
 Cognitive impairment and developmental delay
 Seizures Type;
Childhood onset tonic-atonic drop attacks
Complex partial/frontal lobe seizures
Seizures; arms asymetrically elevated
Vocalization
5 to 20 seconds duration
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LGS Diagnosis; adult
EEG (variable)
- Background; normal (rare) to diffuse
slowing
- Ictal; diffuse attenuation
generalized irregular 2 to 2 ½ Hz
spikes/wave generalized might lateralized
without clear focal findings.
LGS treatment
Antiepileptic drugs
 Surgery;
-Vagus Nerve stimulator VNS shows a 24%–
42% global seizure reduction. Comparable
outcome to corpus
callosotomy without
invasive surgery (a)
-Corpus callosotomy
most beneficial for atonic seizures
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Diet; Ketogenic, Modified Atkins diet.
a-Lancman et al. AES 2011 Abstract 3.313
LGS treatment with AEDs
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Effective in randomized, double blind
placebo controlled trial, approved by FDA;
Clobazam, Felbamate,Lamotrigine,
Rufinamide, Topiramate.
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Effective in open level trials
Vigabatrin, Zonisamide
LGS treatment with AEDs
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Often used
Valproic acid, Clonazepam
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Limited utility (may exacerbate seizures)
CBZ, Phenobarbital, Primidone
Aeds Additive effects
Post-hoc sub analysis of the CLB trial;
Evaluated drop seizures rate from baseline
to 12 week in patient receiving CLB with
LTG or VPA.
Average reduction in drop seizures was
grater for either combination vs placebo.
Efficacy was dose dependent
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What Medication to use first
Based on Syndrome
 Based on gender
 Based on side effects
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Choices; Depakote, Topiramate,
Lamotrigine, Felbamate.
What medication to use Next
Based on the first medicine
 Based on Additive effect
 Based on side effects
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Options; Rufinamide, Zonisamide,
Vigabatrin, same as the previous list,
Benzodiazepines, Levetiracetam
Diet for LGS
Ketogenic Diet
 Modified Atkins diet for Epilepsy
 Low glycemic index diet
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Ketogenic diet
A treatment option for epilepsy (LGS)
 A very strict diet that involves fluid
restriction, high fat and low carbohydrate +
protein intake.
 The goal: alter the body’s fuel source from
glucose to fat.
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History
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The basis of the diet – fasting
Devised in the 1920’s at Johns Hopkins
University School of Medicine.
Popularity faded in the 40’s and 50’s when new
anticonvulsant drugs were discovered
Resurgence recently because, among others, of
the TV movie based on Charlie Abrahams
Who is a candidate
Children – usually 2-10 years of age
 Most effective in kids with “drop” type
seizures
 The children considered have at least 3
seizures/week
 The antiepileptic medication is not
working.
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Efficacy
20-30% – seizures were completely
controlled
 50-70% - seizure frequency decreased by
50%
 20-30% - not effective
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Modified Atkins
Absence of protein, fluid, or calorie
restriction
 64% fat
 30% protein
 6% carbohydrate (10 to 20% on regular
Atkins diet)
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Modified Atkins
The modified Atkins diet is a modification
of the traditional ketogenic
 Foods are not weighed and measured, but
carbohydrate counts are monitored by
patients and parents.
 It is started outside of the hospital
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Modified Atkins
Is it Effective?
 60 to 70 % >50% improvement,
 20 to 30 % >90% improvement
 5 to 10 % seizure free
Surgical Options
 Vagus
Nerve stimulator VNS shows a
24%–42% global seizure reduction.
Comparable outcome to corpus
callosotomy without invasive surgery.
 Corpus
callosotomy
most beneficial for atonic seizures
Summary
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Lennox Gastaut Syndrome
Difficult epileptic encephalopathy
Onset in childhood
Persist into adult life
Optimal Treatment
Requires identification of the syndrome
Selection of effective therapy
Ovoid treatments that exacerbate seizures