Pediatric Movement Disorders and Spasticity Flashcards
- Spasticity is best described as:
a. Velocity-dependent resistance to passive muscle stretch usually due to upper motor neuron lesion
b. Velocity-dependent resistance to active muscle stretch usually due to upper motor neuron lesion
c. Velocity-dependent resistance to passive muscle stretch usually due to lower motor neuron lesion
d. Force-dependent resistance to passive
muscle stretch usually due to upper motor neuron lesion
e. Force-dependent resistance to active
muscle stretch usually due to lower motor neuron lesion
f. Force-dependent resistance to passive
muscle stretch usually due to lower motor neuron lesion
a. Velocity-dependent resistance to passive muscle stretch usually due to upper motor neuron lesion
Velocity-dependent resistance to passive
muscle stretch usually due to upper motor
neuron lesion. Symptoms include muscle
tightness, cramping/pain, and fatigue. It is
due to a loss of descending GABA inhibition
of muscle groups resulting in hypertonia (coactivation of agonist and antagonist muscles
during volitional movement)
The most common cause of spasticity in children is likely to be:
a. Cerebral palsy
b. Multiple sclerosis
c. Stroke
d. Traumatic spinal injury
e. Wilson’s disease
a. Cerebral palsy
Cerebral palsy. Occurs in 1.5-3 per 1000
and accounts for 75% of spasticity in children. This is defined as a range of nonprogressive syndromes of posture and motor
impairment due to an insult to the developing
nervous system. It is often accompanied by
disturbances in sensation (visual and tactile),
cognition/behavior, communication, and
epilepsy (30%). CP is classified into spastic,
dyskinetic, or mixed. Spastic CP is the most
common and divided into spastic quadraplegia, diplegia, hemiplegia, or monoplegia.
Severity of spasticity is graded according to
Ashworth or Modified Ashworth scales. Dyskinetic cerebral palsy can be divided into dystonic and choreoathetoid forms.
You see a 4-year-old child in clinic with a right spastic hemiparesis from a previously ruptured left basal ganglia cavernoma. She is currently taking oral baclofen and gabapentin. On examination, the right upper limb has increased tone throughout the range of movement but is easy to move, while the right leg has a considerable
increased in muscle tone with passive movement being difficult. What are the Modified Ashworth scale grades for the right arm and leg?
a. Right arm¼=1, Right leg¼=2
b. Right arm¼=1 +, Right leg¼=2
c. Right arm¼=1 +, Right leg¼=3
d. Right arm¼=2, Right leg¼=3
e. Right arm¼=3, Right leg¼=4
d. Right arm¼=2, Right leg¼=3
Right arm¼2, Right leg¼3. Generally
patients with an Ashworth score of 3 are
candidates for intrathecal baclofen, although
those with less severe spasticity may also
benefit depending on the clinical context.
Additionally, during ITB test dose (25-
50 μg in children) for spasticity an improvement of one point or greater is considered
positive.
Which one of the following is LEAST likely to be a therapeutic end goal for intrathecal baclofen?
a. Absence of limb spasticity
b. Facilitating care
c. Increasing range of motion
d. Reducing painful muscle spasms
e. Slowing the development of muscle
contractures
a. Absence of limb spasticity
—Absence of limb spasticity. This is
because spasticity can help people with standing, walking or transferring and complete
removal of spasticity can result in loss of
function in some patients and thus intrathecal
baclofen pump insertion is inappropriate.
Intrathecal baclofen treatment in children with cerebral palsy is most commonly associated with?
a. Increased generalized itching
b. Increased headache
c. Increased progression of scoliosis
d. Increased seizure frequency
e. Reduced physostigmine requirements
e. Reduced physostigmine requirements
Headache. CSF leaks occur in 5-15% of
children with cerebral palsy, compared to
3% in adults. This is thought to be due to
smaller size, thinner tissues, malnutrition of
chronically disabled children and occult
hydrocephalus in cerebral palsy.
A 27-year-old patient with spastic diplegia presents 2 years after baclofen pump insertion with increasing baclofen requirements.
Until 6 months ago he had been stable for 1 year with a requirement of 300 μg/day. On the X-ray image of his baclofen pump below, which port would allow you to determine catheter patency?
a. Blue arrow
b. Purple arrow
c. Red circle
d. Yellow arrow
e. Access port is usually in-line with catheter in lumbar region
a. Blue arrow
Blue arrow. The general parts in the most
commonly used pump (Medtronic Synchromed) consist of pump roller (red ring),
pump reservoir port for filling (yellow arrow),
catheter access port (blue arrow) and pumpcatheter connector (purple arrow). In general, catheter malfunction can be assessed with plain radiographs (disconnection, kinking,
migration) and either fluoroscopic or CT
imaging after injection of contrast material
via the catheter access port (catheter leak or
extrathecal catheter-see image below).
The fluoroscopic image below most likely shows:
a. Catheter leak
b. Curling of catheter in subcutaneous tissues
c. Disconnection at pump catheter
d. Extradural position of catheter
e. Kinking of catheter
a. Catheter leak*
Selective dorsal rhizotomy for spastic cerebral palsy works by:
a. Electrical stimulation of dorsal root entry zone
b. Interruption of a subset of alpha-motor neurons
c. Interruption of gamma-motor neurons
d. Interruption of the spinal reflex arc
e. Restoration of GABAergic inhibition of
alpha-motor neurons
d. Interruption of the spinal reflex arc
Interruption of the spinal reflex arc. Deficient descending inhibition of spinal reflexes
leads to hyperreflexia, hypertonia hence
selective interruption of afferent fibers from
spastic muscles will stop this. Briefly, in
SDR (see diagram below) the conus is
exposed and L2-S2 dorsal roots separated
from motor roots using a silastic sheet, Each
dorsal root is divided into 3-5 fascicles using a
Scheer needle, and each of these is examined
with EMG. Only rootlets which display a significant EMG response after stimulation are
sectioned, while others are spared. L1 rhizotomy is required to reduce spasticity in hip
flexors.
Which one of the following treatments for pediatric dystonia works via vesicular monoamine transporter inhibition?
a. Baclofen
b. Carbamazepine
c. Carbidopa-levodopa
d. Clonazepam
e. Tetrabenazine
f. Trihexiphenidyl
e. Tetrabenazine
The surgical treatment of choice for primary dystonia is likely to be:
a. Bilateral GPi DBS
b. Epidural motor cortex stimulation
c. Intrathecal baclofen pump
d. Pallidotomy
e. Selective dorsal rhizotomy
a. Bilateral GPi DBS
A child with cerebral palsy and a long-term intrathecal infusion pump presents with fever, seizures, and rebound spasticity. This is most likely to be:
a. Acute dystonic reaction
b. Baclofen withdrawal
c. Malignant hyperthermia
d. Neuroleptic malignant syndrome
e. Status dystonicus
b. Baclofen withdrawal
Half-life is 4-5 h, therefore 24 h is required to achieve steady-state concentration after a change in dosage
Drug treatment of spasticity:
a. Carbidopa-levodopa
b. Cyproheptadine
c. Dantrolene
d. Diazepam
e. Gabapentin
f. Intrathecal baclofen
g. Oral baclofen
h. Physostigmine
i. Pregabalin
j. Tetrabenazine
k. Tizanidine
l. Trihexyphenidyl
Intrathecal baclofen
Reduces intracellular calcium by binding ryanodine receptor in skeletal muscle
Drug treatment of spasticity:
a. Carbidopa-levodopa
b. Cyproheptadine
c. Dantrolene
d. Diazepam
e. Gabapentin
f. Intrathecal baclofen
g. Oral baclofen
h. Physostigmine
i. Pregabalin
j. Tetrabenazine
k. Tizanidine
l. Trihexyphenidyl
Dantrolene
Alpha2 adrenergic antagonist
Drug treatment of spasticity:
a. Carbidopa-levodopa
b. Cyproheptadine
c. Dantrolene
d. Diazepam
e. Gabapentin
f. Intrathecal baclofen
g. Oral baclofen
h. Physostigmine
i. Pregabalin
j. Tetrabenazine
k. Tizanidine
l. Trihexyphenidyl
Tizanidine
Dystonia worse in late afternoon, parkin sonism, and spastic gait but no history con sistent with cerebral palsy, low biopterin and homovanillic acid levels
Dystonia:
a. Dopa-responsive dystonia (Segawa’s
disease)
b. Dystonic cerebral palsy
c. DYT-1 dystonia
d. Gangliosidoses
e. Glutaric aciduria
f. Leigh’s disease
g. Mitochondrial disorders
h. Pantothenate kinase-associated
neurodegeneration
i. Post-head injury dystonia
j. Post-stroke dystonia
k. Rett’s syndrome
l. Wilson’s disease
Dopa-responsive dystonia
