Lennox Gastaut Syndrome

Owen had another EEG on Wednesday afternoon.  By my count, in his two short years, he has now completed six EEGs.  The good news is he really enjoys them!  Owen loves having his head touched and rubbed, so having sensors glued on to it is like a giant massage for him.  The past month has brought increased seizure activity and irritability.  After a few months of Owen being in a fantastic mood, he is reverting back to the archy, screamy, crabby, awake little boy I know so well.  

It comes as no surprise that his seizure diagnosis has progressed from Infantile Spasms to Lennox Gastaut Syndrome.  Here is the information about this syndrome as described by the National Institute of Neurological Disorders and Stroke (NINDS):

What is Lennox-Gastaut Syndrome?

Lennox-Gastaut syndrome is a severe form of epilepsy. Seizures usually begin before 4 years of age. Seizure types, which vary among patients, include tonic (stiffening of the body, upward deviation of the eyes, dilation of the pupils, and altered respiratory patterns), atonic (brief loss of muscle tone and consciousness, causing abrupt falls), atypical absence (staring spells), and myoclonic (sudden muscle jerks). There may be periods of frequent seizures mixed with brief, relatively seizure-free periods. Most children with Lennox-Gastaut syndrome experience some degree of impaired intellectual functioning or information processing, along with developmental delays, and behavioral disturbances. Lennox-Gastaut syndrome can be caused by brain malformations, perinatal asphyxia, severe head injury, central nervous system infection and inherited degenerative or metabolic conditions. In 30-35 percent of cases, no cause can be found.

Is there any treatment?

Treatment for Lennox-Gastaut syndrome includes anti-epileptic medications such as valproate, lamotrigine, felbamate, or topiramate. There is usually no single antiepileptic medication that will control seizures. Children who improve initially may later show tolerance to a drug or have uncontrollable seizures.

What is the prognosis?

The prognosis for individuals with Lennox-Gastaut syndrome varies. There is no cure for the disorder. Complete recovery, including freedom from seizures and normal development, is very unusual.

Owen demonstrates all four seizure types many times a day.  We are sad for our guy that he continues to be on such a steep downward trajectory.  The good news is the prognosis of his cuteness remains excellent as demonstrated here: