In our continuing series of EEG in Epilepsy let’s move on to another rare neurological condition that we might also see in our labs.
Sturge Weber syndrome is present at birth and is accompanied by a large port-wine stain birthmark on the patient’s face.
This condition consists of abnormal blood vessels and calcifications in the cortex on the same side as the birthmark.
Neurological symptoms may include:
Seizures that begin in infancy and may worsen with age. Convulsions usually happen on the side of the body opposite the birthmark.
Muscle weakness
Developmental delays
Glaucoma (increased pressure within the eye) at birth or later. The increased pressure within the eye can cause the eyeball to enlarge and bulge out of its socket (buphthalmos)
Migraines
What might the EEG findings look like?
Abnormal Spike and Sharp Wave discharges on the same side as the patient’s birthmark and progressive worsening of the EEG over time.
Also, abnormalities can be more pronounced in sleep.
Repeat studies can also be useful in seizure management.
The following tracings demonstrate the progression:
In many cases, seizures can be controlled with anti-seizure medications. In very severe cases, seizures may need to be treated with vagus nerve stimulation implants or surgery.
For those where surgery is the best option for seizure control, it often involves removing the whole affected hemisphere. With this procedure being done at such a young age the remaining hemisphere is often able to take over many of the functions from the missing hemisphere.
Most patients will develop seizures before their first birthday so for those of us working in a pediatric lab knowing this disorder and the EEG findings is essential to being a good resource for our patients and their families.
For a look back at other posts in our EEG in Epilepsy Series - just click here: (Jeavon’s Syndrome) and (Rassmussen Syndrome).
Roya Tompkins, MS, REEG/EP T, RPSGT
Resources:
Boston Childrens
National Institute of Health
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