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>> The Selective Dorsal Rhizotomy is a procedure
that we use to help control the muscle tone
in young children who have spastic diplegia.
That means that they have more difficulty walking,
but their arms and their cognition
and social development are usually on track.
And what we do is make a small incision
in the back under the microscope
and cut some of the nerves that have abnormal signals
and contribute to that spasticity.
And by doing that, we sort of reset the meter
for the tone in the muscles in the legs.
After that procedure, the kids need intensive rehab
to, sort of, relearn to use
the remaining circuits really well.
And that intense rehab usually is several weeks.
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The best candidates are kids who
are approximately three to 12 years old,
although we sometimes consider kids outside of that range
and they have difficulty with
spasticity in their lower extremities,
usually not so much in their upper extremities.
They're typically kids that have
the potential to walk, even without devices,
so they may currently be walking
with a walker or using canes or even just orthotics.
And the kids also need to have
the, sort of, the maturation and motivation
to learn to improve their gait on their own.
So, initially kids cooperate,
you know, with physical therapy
because you bribe 'em or other negotiating techniques.
But we really want these kids to have, sort of,
the inner awareness that they're working really hard
on their therapy to help themselves walk better.
And so that usually takes till somebody's
at least three or four years old
to be able to have that maturity.
We do like to intervene early,
before they hit, sort of, the middle of grade school
and the peer pressure really kicks in
on how differently they're walking.
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So, if the child undergoes the Selective Dorsal Rhizotomy
and they're able to complete the intensive rehab afterwards,
the most common improvement is
to improve by one device level.
So, if they're walking with a walker,
we can typically expect that they walk with canes.
They may improve so much that they
are just with orthotics alone
and don't need an extra cane or crutches or anything.
But we, you know, on average, they improve one level.
There's a few children who are extremely motivated
and have the underlying potential
that they can do really well
and get close to a typical gait.
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So we typically do not perform Selective Dorsal Rhizotomy
for kids that don't have the potential to ambulate
on their own, at least with the help of a device.
And so, for more severely involved kids,
they respond much better to intrathecal baclofen.
Intrathecal baclofen is a thousand times more potent
than baclofen they may take by mouth
or by their feeding tube.
And it works much more effectively.
It does not go through the bloodstream
and give 'em the side effects,
and it can be precisely regulated.
So it's a much better option for the duration.
We're looking at interventions
to help the kids over many years to decades
and the pump can provide that kind of relief.
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So, for dystonia, we have two main categories.
There's the kids that have primary dystonia,
which is due to a genetic cause.
Those kids typically are fairly healthy
until they reach seven or eight,
although there are exceptions,
and some kids are sicker earlier.
The kids with primary dystonia respond
much better to deep brain stimulation.
So that's a device with electrodes placed in the brain
and then a generator that's very similar
to, like, the generator used for a pacemaker.
So we can implant that in kids who are seven years or older
and it works very well for those kids.
The outcomes with secondary dystonia,
or dystonia from other causes, is not nearly as good,
and it's, some kids respond
to the intrathecal baclofen pump better
whereas other kids respond better
to the deep brain stimulation.
And we're actively working on trying to discern
which populations would respond better to which option.
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So, primary dystonia is due, usually, to a genetic mutation
and the kids have difficulty.
They usually have a normal brain MRI.
They usually have a normal perinatal history.
By contrast, the kids with secondary dystonia
have acquired dystonia from some other abnormality
that may be a metabolic abnormality
like glutaric acidosis or kernicterus,
other causes of problems with the basal ganglia.
They also may have severe dystonia
or mixed spasticity from perinatal brain injury,
such as hypoxic ischemic encephalopathy or preterm birth.
The kids that have secondary dystonia
tend to respond better to an intrathecal baclofen pump,
although some can respond to deep brain stimulation.
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So at Johns Hopkins, we are very lucky
because we're able to work with our colleagues
at Kennedy Krieger Institute
and Mt. Washington Pediatric Hospital for rehabilitation.
At both places we have great, dedicated teams
for children with cerebral palsy
and dystonia and other related disorders.
We work very closely with our colleagues
in rehab medicine, developmental pediatrics,
neurology, orthopedics and all our therapists
to really design the most unique,
individualized treatment plan for each child.
We work very closely with the families
to work out a plan that works for everyone
and our goal is to have the kids be as independent
and comfortable and functional
as they can be as they head into adulthood.
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(children laughing)