Lennox-Gastaut Syndrome

    
      Re: Lennox-Gastaut Syndrome
    


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Posted by ccf neuro M.D. on May 26, 1997 at 11:49:45:

In Reply to: Lennox-Gastaut Syndrome posted by Fred Roedl on May 18, 1997 at 11:27:34:

: My 23 year old son has a mixed seizure disorder that we
  have been unable to control all of his life.  He began
  having petit mal seizures when he was 9 months old after
  a normal pregnancy and infant development. His first
  grand mal seizure was on his first birthday, 12 hours
  after his MMR immunization.  This seizure lasted approx.
  15 minutes. Since that time (22 years) he has frequent,
  uncontolled grand mal and myoclonic seizures. Currently,
  he has some form of seizure activity every day.  
  He is developmentally disabled, with a functioning
  mental capacity of age 3-4.
  He has been on numerous combinations of all FDA
  approved seizure medications since his seizures began.
  We have had no success in controlling these seizures.
  The myoclnic episodes are becoming more frequent(daily)
  and are severely limiting his quality of life. We have
  recently been told by doctors that our son suffers from
  Lennox-Gestault Syndrome. This is a new diagnosis for
  us, after having our son evaluated by many different
  neurologists. I realize you will not be able to comment
  with absolute certainly, but does this diagnosis sound
  accurate. If so, what are possible treatments for
  Lennox-Gastault that will improve my son's seizure
  control.
==========================================================
Lenox-Gastaut syndrome is a term applied to a severe, intractable, mixed seizure disorder that can include generalized convulsions, atonic seizures, tonic seizures, and so called atypical absence seizures (prolonged staring spells, plus or minus twitches or eye fluttering etc.) It is somewhat unusual for it to present so early in infancy and does not commonly follow a precipitating event like a vaccine. There is a characteristic EEG pattern for the disorder called generalized slow spike and wave activity, which assists in confirming the syndrome. As it is a syndrome, multiple different pathways of brain injury may produce it. It may be that your son had an immunologic reaction to one of the componenets of the MMR vaccine that damaged his brain. Irrespective of whether or not your son meets the specific criteria for the syndrome, he clearly has intractable epilepsy like those who suffer from the syndrome have. Mental retardation and global developmental delay are the rule with such epilepsies, since the brain has little chance to recover long enough to continue developing in between seizures. Recent newer seizure drugs that might be worth trying in such intractable cases include felbamate (which has been proven clinically effective in achieving better seizure control in some persons with Lenox-Gastaut syndrome specifically)--- but note, reports of rare fatal anemia and liver damage have greatly diminished the drug's use, vigabitrin (very close to FDA approval and very safe overall), lamotrigine, topiramate (not sure if yet approved), and tiagabine (not yet approved). Neurontin is another drug that may be worth adding--- not likely to be effective, but minimal side effects and, uniquely, NO DRUG INTERACTIONS. You could try to obtain some of the not yet approved drugs from the manufacturer on a compassionate use basis. Vigabitrine is available in Canada currently. If your son has Lenox-Gastuat syndrome, he will probably never have what would be considered good control of his seizures, but may, with continued        experimentation with combinations of drugs be able to achieve a reasonable quality of life. Myoclonic seizures are mosty often initially treated with valproate (Depakote/Depakene) and clonazepam (Klonapin) and its cousins. There is a cousin of clonazepam called nitrazepam that is available on a compassionate use basis also that may be worth looking into in your son's case. The Cleveland Clinic is world renowned in the treatment of epilepsy. If you would like a second opinion for your son (don't expect miracles), I would suggest seeing Dr. Hans Luders, Dr. Harold Morris, or Dr. Elaine Wyllie (pediatric epilepsy but you might be able to talk her into seeing your son since this is a pediatric syndrome). Any of these or our other epileptologists could review your son's seizures history and EEGs and give you a definite YES or NO regarding your specific question of Lenox-Gastaut syndrome, and may be able to work with you and your local neurologist to obtain some of the not yet officially approved drugs on a
compassionate use basis to try. We wish you well in dealing with this very challenging and exasperating problem. You may wish to contact the Epilepsy Foundation of America (EFA) for advice on 1) a recommended board certified epileptologist nearer to you if Cleveland is far and 2)support groups. Their address is 4351 Garden City Drive, Landover MD 20785-4951; phone 1-800-EFA-1000/(301)-459-3700; FAX [phone]; e-mail: ***@****; web page http://www.efa.org. I hope you find this information useful
  Thank you and I look forward to your reply, as we are
  desperate for help.