lennox gastaut syndrome enrique feoli md north east regional epilepsy group
TRANSCRIPT
![Page 1: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/1.jpg)
Lennox Gastaut Syndrome
Enrique Feoli MD
North East Regional Epilepsy Group
![Page 2: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/2.jpg)
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
![Page 3: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/3.jpg)
Syndrome types in different age groups
Infancy;
-West Syndrome
-Ohtahara Synd.
-Dravet Syndrome
Late Childhood (5-10y)
-Absence Epilepsy
-Landau-K syndrome
-Benign Rolandic E.
Early Childhood (1-5y)
-LGS
-Febrile Seizures
Adolescence
-JME
-Juvenile Absence epilepsy
![Page 4: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/4.jpg)
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 %
![Page 5: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/5.jpg)
Etiology
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.
![Page 6: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/6.jpg)
LGS Diagnosis; Pediatrics
-Onset 3 to 8 years of age
-Seizures type; -Tonic-atonic drop attacks -Tonic clonic - Atypical absences; Gradual onset and termination Behavioral pause/stare (30 + seconds) Clonic activity
![Page 7: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/7.jpg)
LGS Diagnosis; Pediatrics Cont
May present as non-convulsive status epilepticus in 50 to 75 % of patients
-Cognitive impairment and developmental delay.
-EEG; irregular, gneneralized 2 to 2 ½ Hz spikes and wave pattern
![Page 8: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/8.jpg)
![Page 9: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/9.jpg)
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 seizuresSeizures; arms asymetrically elevated Vocalization 5 to 20 seconds duration
![Page 10: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/10.jpg)
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.
![Page 11: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/11.jpg)
![Page 12: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/12.jpg)
![Page 13: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/13.jpg)
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
Diet; Ketogenic, Modified Atkins diet.a-Lancman et al. AES 2011 Abstract 3.313
![Page 14: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/14.jpg)
![Page 15: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/15.jpg)
LGS treatment with AEDs
Effective in randomized, double blind placebo controlled trial, approved by FDA;
Clobazam, Felbamate,Lamotrigine, Rufinamide, Topiramate.
Effective in open level trials Vigabatrin, Zonisamide
![Page 16: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/16.jpg)
LGS treatment with AEDs
Often used
Valproic acid, Clonazepam
Limited utility (may exacerbate seizures)
CBZ, Phenobarbital, Primidone
![Page 17: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/17.jpg)
![Page 18: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/18.jpg)
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
![Page 19: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/19.jpg)
What Medication to use first
Based on Syndrome Based on gender Based on side effects
Choices; Depakote, Topiramate, Lamotrigine, Felbamate.
![Page 20: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/20.jpg)
What medication to use Next
Based on the first medicine Based on Additive effect Based on side effects
Options; Rufinamide, Zonisamide, Vigabatrin, same as the previous list, Benzodiazepines, Levetiracetam
![Page 21: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/21.jpg)
Diet for LGS
Ketogenic Diet Modified Atkins diet for Epilepsy Low glycemic index diet
![Page 22: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/22.jpg)
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.
![Page 23: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/23.jpg)
History
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
![Page 24: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/24.jpg)
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.
![Page 25: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/25.jpg)
Efficacy
20-30% – seizures were completely controlled
50-70% - seizure frequency decreased by 50%
20-30% - not effective
![Page 26: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/26.jpg)
Modified Atkins
Absence of protein, fluid, or calorie restriction
64% fat 30% protein 6% carbohydrate (10 to 20% on regular
Atkins diet)
![Page 27: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/27.jpg)
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
![Page 28: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/28.jpg)
Modified Atkins
Is it Effective? 60 to 70 % >50% improvement, 20 to 30 % >90% improvement 5 to 10 % seizure free
![Page 29: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/29.jpg)
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
![Page 30: Lennox Gastaut Syndrome Enrique Feoli MD North East Regional Epilepsy Group](https://reader035.vdocuments.us/reader035/viewer/2022062423/5697c0251a28abf838cd4fef/html5/thumbnails/30.jpg)
Summary 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