New Diagnosis: Lennox-Gastaut Syndrome (LGS)

Last week Friday, Stephanie was diagnosed with Lennox-Gastaut Syndrome (LGS).  We saw the neurosurgeon and neurologist on March 8th. Her new LP Shunt is working exceptionally well and she seems to be more "aware" of her environment and is talking more and learning a few new words.  She will be weaned off her increased pressure meds and we'll see how she does.

Unfortunately, Steph also started having a new kind of seizure last week. The neurologist now had enough information to diagnose Stephanie with Lennox-Gastaut syndrome, among everything else. We finally know that even after our best efforts, we may never be able to rid her of these many different kinds of seizures and the best we can do is reduce the impact as much as possible with medications, the brain surgery she already had and next probably a vagus nerve stimulator implanted in her chest. In addition to the seizures, LGS can also cause cognitive/mental regression, even mobility regression.  I am planning to work with her therapists to be a little more aggressive with speech, occupational, and physio therapy.  I found video of her from just a few years ago that is of a completely different little girl than who we see today.  It really shows how much she has slowed downed and even regressed.  It's not always easy to notice these changes in the day to day of raising a child with special needs, especially when medical care has been the number one priority. 

I am a little more heart broken again.  One step forward and two steps back, this is beginning to be a theme in our lives.  My saving grace is that Stephanie still lives in the moment and enjoys all the small things life has to offer - Miss Piggy, toys, her close friends, snacks, and music. I know she doesn't need much more than this but I so wish I could give her the world! I guess I am going to have to try my darndest to do just that!

Here's a little excerpt from the LGS Foundation:

"Lennox-Gastaut syndrome (LGS) is a rare and often debilitating form of childhood-onset epilepsy. The syndrome is characterized by a triad of signs including multiple seizure types, moderate to severe cognitive impairment, and an abnormal EEG with slow spike- wave complexes. LGS constitutes between 1-4% of pediatric epilepsies and typically appears between the second and sixth year of life.

Some of the known causes of Lennox-Gastaut Syndrome include brain injury associated with pregnancy or birth (including asphyxia, low birth weight, and prematurity), severe brain infections (including encephalitis, meningitis and rubella), developmental malformations of the brain, or a history of infantile spasms. In 30-35 percent of cases, no cause can be found.

Most children are typically developing normal when first diagnosed, but then begin to lose skills, sometimes dramatically, in association with uncontrolled seizures. Young children with LGS may exhibit behavioral issues, personality disturbances, mood instability, and slowing of psychomotor development. Behavioral disturbances can include poor social skills and attention seeking behavior, which can be caused by the effects of the medication, difficulty interpreting information, or the electric disturbances in the brain. Some children with LGS are prone to develop non-convulsive status epilepticus (a continuous seizure state that is associated with a change in the child's level of awareness). This requires medical intervention to bring it to an end." ~ LGS Foundation