What are stroke-like episodes in SWS and how are they treated?

Answer:

Symptoms of stroke-like episodes in SWS include weakness on one (or both) sides of the body, usually after a seizure or with a migraine, which lasts longer than is typical.  There can also be tingling or numbness in the arm or leg on the affected side, and .   that side of the face can droop, causing difficulty talking, drooling, or problems with mouth movements. This can be very scary for the patient experiencing it firsthand and their family members who are witnessing it.

After a seizure, some patients may experience weakness anywhere from a few hours to an entire day (this is called a Todd’s paralysis). As a result of this weakness, some patients may experience migraines and other neurological symptoms. Though patients may look like they are having a stroke, an MRI may reveal that they’re actually not experiencing a stroke. It is common for most patients to fully recover from these symptoms.

However, in infants and young children, repeated stroke-like episodes, or failure to meet developmental milestones (such as walking and talking on time),are often associated with progressive neurologic impairment. Infants with SWS are more prone to brain injury from seizures and venous stasis (blood flow problems, which develops over the first year of life).  Infants and very young children, and occasionally older children and adults, do not always fully recover - in this case, they are said to have had a stroke. SWS patients who have experienced a stroke are usually affected by increased brain atrophy (shrinkage) and calcification (abnormal calcium deposits).Stroke-like episodes are initially treated by getting the seizures and/or migraine under control. Often this requires a hospitalization or ER visit, particularly if the episode has been triggered by an illness with vomiting.  IV fluids, seizure medications and continuous EEG to rule out subtle seizure activity are all very important. Migraine treatment may include any of the following, depending on the age of the patient and their response to medications: IV fluids, IV Toradol, Compazine or reglan with Benadryl, Depakote, Magnesium or steroids. Sleep is also very important and may even resolve the symptoms if they can be caught early.

It is important to not overdo physical therapy early on, as with resolution of the seizures and/or migraines the neurologic symptoms will most likely go away, and thus, the priority needs to be fluids, sleep, and medication.  If neurologic deficit persists after the illness, migraine or seizure episode fully resolves, then rehabilitation needs can be addressed with therapy.