Transcript Movement Disorders

Pediatric Neurology Quick Talks
Movement Disorders
Michael Babcock
Summer 2013
Scenarios
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Scenario 1
8 yo boy
PMHx – dx'd with allergic
rhinitis unresponsive to nasal
steroids
1 year of recurrent neck popping,
multiple times daily
No LOC and is aware of
movements
Pt says he feels “relaxed” after
movements
What is dx? What comorbidities
should you ask about? Does he
really have rhinitis?
• Scenario 2
-6 yo girl
-in ED with abnormal sustained
twisting posture of L arm and
neck
-4 recent ER visits for headache with
vomiting
-has taken headache medicine for the
last 5 days
-no other medications
-no other significant history
-what is diagnosis?
-what is treatment?
Step 1:Observe and Step 2: Describe
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Are the movements normal or abnormal?
Are they paroxysmal (sudden on/off), continual (repeated again and
again), or continuous (non-stop)?
How does voluntary movement affect abnormal movements?
Are movements present at rest, posture, action, intention, or combination?
Do environmental stimuli/emotion precipitate/exacerbate/alleviate?
Is patient aware of movements?
Do movements stop with sleep?
Step 3: Classify
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Hyperkinetic (most common)
– Tic
– Tremor
– Myoclonus
– Chorea
• Ballismus
– Athetosis
– Dystonia
– Dyskinesia
– Akathesia
– Myokymia
– Asterixis
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Hypokenitic (uncommon for
peds – mainly Parkinsons)
– Bradykinesia
– Freezing
– Rigidity
Step 4: Diagnose and Step 5: Treatment
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Scenario 1
Tics – Neck popping movements
are repetitive and stereotypic,
relieve an inner feeling of
tension.
Diagnosis of rhinitis unrelieved
with steroids is probably
sniffing/throat clearing that is
also a tic.
Diagnosis is Tourette's
Treatment can be observation
unless significantly bothersome
or comorbidities.
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Scenario 2
Dystonia – abnormal twisting
movement sustained for a long
time.
Acute onset is likely medication
related – she was recently given
headache medication, probably
dopamine antagonist like
phenergan.
Tx – benedryl
Basal Ganglia Circuit – Just to discuss treatment
Tics
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Taskforce on Childhood Movement Disorders, “tics are repeated,
individually recognizable, intermittent movements or movement fragments
that are almost always briefly suppressible and are usually associated with
awareness of an urge to perform the movement”
Sometimes difficult for young children to describe urge
Disappear with sleep
Often worsened by anxiety
Transient Tic disorder – 25% of children – tics last less than 1 year
Chronic Tic disorder – tics last more than 1 year
Tourette's – Chronic tics with both motor and vocal tics
Tremor
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Rhythmic oscillation around a central point involving one or more body
parts.
Different tremors occur at different times – rest, postural, action, intention
Rest tremor – Parkinsons, Wilson's
Postural -physiologic tremor, essential tremor
Intention – cerebellar disease
Chorea
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Taskforce - “ongoing random-appearing sequence of one or more discrete
involuntary movements or movement fragments.”
Chaotic, purposeless
Not as rapid as myoclonus
Can see in CP, Sydenham, Post-pump, kernicterus, hereditary
Ballism – Large amplitude chorea of proximal muscles
Dystonia
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Task force – “a movement disorder in which involuntary sustained or
intermittent muscle contractions cause twisting and repetitive movements,
abnormal postures, or both.”
Dystonic postures are repeated, particular patterns or postures are
characteristic of any one patient at a given time.
Postures maybe be sustained or occur during brief intervals
Often triggered by certain voluntary movements – writer's cramp.
Others
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Athetosis
– Slow, continuous, writhing
movements of distal body
parts, especially fingers and
hands
– Can see in cerebral palsy and
Rett syndrome.
Myoclonus
– Sudden, brief, shock-like
movements
– May be repetitive or
rhythmic
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Sterotypies
– Taskforce, “stereotypies are
repetitive, simple
movements that can be
voluntarily suppressed”
– Patterned, episodic,
repetative, purposeless
– These are different from tics
– See often in autism, Rett
Benign movements (may need to rule out other
causes)
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benign neonatal sleep myoclonus
benign myoclonus of infancy
jitteriness
shuddering
paroxysmal tonic upgaze of infancy
spasmus nutans
benign paroxysmal torticollis
benign idiopathic dystonia of infancy
Tourette's Syndrome
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Chronic vocal and motor tics
Comorbidites: ADHD, OCD, anxiety – often these guide treatment
Tx: only needed if there is physical discomfort, functional problems, or psychosocial
problems (class disturbance, self-esteem, social anxiety)
Tx: alpha-adrengergic meds (clonidine, guanfacine), SSRI, AEDs (topamax, keppra). Can
consider antipsychotics but higher side-effect profile – pimozide and haldol.
Work-up – consider secondary causes if tics begin abruptly (parents remember date), are
persistent (don't wax/wane), or particularly problematic.
Secondary causes:
– Infections (encephalitis, Sydenham's chorea, ?-PANDAS)
– Drugs (stimulants, levodopa, some AEDs, antipsyhcotics
– Toxins (carbon monoxide)
– Other (head trauma, stroke, static encephalopathy, chromosomal abnormalities,
neurocutaneous/neuro-degenerative syndromes, schizophrenia)
Sydenham's Chorea
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Most common form of acquired chorea in childhood
1-18 months after GAS infection (contrast to carditis and arthritis that appear within
1 month)
ARF – Chorea alone can lead to presumptive diagnosis.
Can also have emotional change (deteriorating school performance),psychiatric
symptoms-OCD, irritability, hypotonia. grip-strength testing has continuous
increase/decrease – Milking sign.
Work-up: EKG/Echo to look for carditis, ASO, DnaseB
– other causes of chorea – SLE, Huntington's, encephalitis, Wilson's
Typically improves gradually, mean duration 3-4 months
Tx – treatment dose penicillin for 10 days, followed by antibiotic ppx against GAS.
Specific treatment may be needed – depakote, phenobarbital, haldol, pimozide,
valium, carbamazepine. Steroids may shorten duration.
Opsoclonus-Myoclonus Syndrome
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Dancing eyes – dancing feet
Mean age – 2 yo.
Opsoclonus – uncontrolled, frequent, conjugate, saccidic movement of eyes in all
directions.
can also have ataxia (OMA); irritability, sleep problems
Auto-immune: Paraneoplastic – neuroblastoma (unknown Ab); parainfectious
Differential: other causes of myoclonus, ataxia; toxic/metabolic dz, structural
Work-up – Look for neuroblastoma – CAP MRI, VMA/HVA, MIBG scan. If no
neuroblastoma, then MRI brain to look for structural, toxic/metabolic, med review,
infections – viral (hep C, Lyme, EBV, HIV, coxsackie, rota, mycoplasma, GAS).
Tx - Treat movements with immunosuppression- steroids, IVIG, rituximab.
Rett Syndrome
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X-linked dominant – mostly sporadic – Mutations in MECP2 gene on Xq28
1:10,000 females
Typically presentation – female at 6-18 months with previously normal growth and
development with regression in verbal and motor skills
– Postnatal microcephaly
Lose purposeful movements of hands
Sterotypies of hands resembling hand washing and kneading
Differential – autism, CP, Fragile X, Angelman, leukdoystrophies, neurocutaneous,
metabolic disorders, NCL.
Work-up – MRI, EEG, Chromosome analysis, FISH/methylation for Angelman, metabolic
studies
Can have seizures – need to differentiate from sterotypies
80% are ambulatory, though some will lose this during regression period
Long-term issues – EKG – prolonged QTC. GI – poor growth, GERD, gallbladder,
constipation. Respiratory – breath-holding and abnormal breathing. Orthopedic –
osteopenia and scolisos.
PREP Question
A 10 year old boy presents to the emergency department with a 2 day history of progressive
difficulty with speech and coordination. On PE, the restless but otherwise quiet child has a
normal mental status and eye movements. His speech is slurred, and he cannot maintain
tongue protrusion without an in-and-out darting movement. Continuous flowing and jerky
movements occur when he holds his hands outstretched or overhead. Although his grip is
strong, he cannot maintain it well because of irregular hand and arm movements. He had a
sore throat and fever 2 months ago. You diagnose chorea.
Of the following, the MOST effective treatment for suppressing the chorea for this boy is:
A. Carbamazepine
B. Clonazepam
C. Haloperidol
D. Penicillin
E. Trihexyphenidyl
C. Haloperidol
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Chorea
“milk maids grip,” “darting tongue,” continuous flowing and jerky movements
Syndeham: poststrep, autoimmune; ASO, anti-DNAse B
Other conditions associated: SLE, APA, hyperthyroid
Haloperidol: Dopamine receptor blocker; can use low dose; short term therapy
typically
Other therapeutic options: Depakote, riperidone
Regarding other choices:
A. Carbamazepine: Anticonvulsant; may induce chorea
B. Clonazepam: Sedating to hit therapeutic dosing
D. Penicillin: Secondary prevention for patients with RF, but doesn't treat
chorea.
E. trihexyphenidyl: reduces dystonia, worsens chorea
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References
-Uptodate articles
-on call neurology
-http://www.unifr.ch/biochem/index.php?id=120