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    Movement Disorders Babcock

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    Baiq Trisna Satriana
    This document provides scenarios and information about pediatric movement disorders. It discusses two scenarios involving an 8 year old boy with neck popping movements diagnosed with Tourette's syndrome, and a 6 year old girl presenting with abnormal arm and neck twisting who is diagnosed with dystonia likely caused by recent headache medication use. It outlines steps for observing, classifying, diagnosing and treating movement disorders. It provides information on specific disorders like tics, tremor, chorea, dystonia, Tourette's syndrome, Sydenham's chorea, opsoclonus-myoclonus syndrome, Rett syndrome, and provides a sample PREP question involving chorea.

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    Movement Disorders Babcock

    Uploaded by

    Baiq Trisna Satriana
    111 views19 pages
    This document provides scenarios and information about pediatric movement disorders. It discusses two scenarios involving an 8 year old boy with neck popping movements diagnosed with Tourette's syndrome, and a 6 year old girl presenting with abnormal arm and neck twisting who is diagnosed with dystonia likely caused by recent headache medication use. It outlines steps for observing, classifying, diagnosing and treating movement disorders. It provides information on specific disorders like tics, tremor, chorea, dystonia, Tourette's syndrome, Sydenham's chorea, opsoclonus-myoclonus syndrome, Rett syndrome, and provides a sample PREP question involving chorea.

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    This document provides scenarios and information about pediatric movement disorders. It discusses two scenarios involving an 8 year old boy with neck popping movements diagnosed with Tourette's syndrome, and a 6 year old girl presenting with abnormal arm and neck twisting who is diagnosed with dystonia likely caused by recent headache medication use. It outlines steps for observing, classifying, diagnosing and treating movement disorders. It provides information on specific disorders like tics, tremor, chorea, dystonia, Tourette's syndrome, Sydenham's chorea, opsoclonus-myoclonus syndrome, Rett syndrome, and provides a sample PREP question involving chorea.

    Copyright:

    © All Rights Reserved
    Download as PPT, PDF, TXT or read online from Scribd
    Download as ppt, pdf, or txt
    111 views19 pages

    Movement Disorders Babcock

    Uploaded by

    Baiq Trisna Satriana
    This document provides scenarios and information about pediatric movement disorders. It discusses two scenarios involving an 8 year old boy with neck popping movements diagnosed with Tourette's syndrome, and a 6 year old girl presenting with abnormal arm and neck twisting who is diagnosed with dystonia likely caused by recent headache medication use. It outlines steps for observing, classifying, diagnosing and treating movement disorders. It provides information on specific disorders like tics, tremor, chorea, dystonia, Tourette's syndrome, Sydenham's chorea, opsoclonus-myoclonus syndrome, Rett syndrome, and provides a sample PREP question involving chorea.

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    © All Rights Reserved
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    The document discusses various pediatric movement disorders including tics, dystonia, chorea, and myoclonus. It provides scenarios to classify movements and make diagnoses.

    The main types of movement disorders discussed are tics, dystonia, chorea, myoclonus, and opsoclonus-myoclonus syndrome. Tics, dystonia and chorea are described in detail with examples given.

    Tics are repetitive, brief movements that are usually suppressible and associated with an urge. They disappear during sleep and are often worsened by anxiety. Transient tics last less than 1 year while chronic tics last more than 1 year.

    Pediatric Neurology Quick Talks

    Movement Disorders
    Michael Babcock
    Summer 2013
    Scenarios

    • Scenario 1 • Scenario 2
    • 8 yo boy -6 yo girl
    • PMHx – dx'd with allergic -in ED with abnormal sustained
    rhinitis unresponsive to nasal twisting posture of L arm and
    steroids neck
    • 1 year of recurrent neck popping, -4 recent ER visits for headache with
    multiple times daily vomiting
    • No LOC and is aware of -has taken headache medicine for the
    movements last 5 days
    • Pt says he feels “relaxed” after -no other medications
    movements -no other significant history
    • What is dx? What comorbidities -what is diagnosis?
    should you ask about? Does he
    -what is treatment?
    really have rhinitis?
    Step 1:Observe and Step 2: Describe

    • 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

    • Hyperkinetic (most common) • Hypokenitic (uncommon for


    – Tic peds – mainly Parkinsons)
    – Tremor – Bradykinesia
    – Myoclonus – Freezing
    – Chorea – Rigidity
    • Ballismus
    – Athetosis
    – Dystonia
    – Dyskinesia
    – Akathesia
    – Myokymia
    – Asterixis
    Step 4: Diagnose and Step 5: Treatment

    • Scenario 1 • Scenario 2
    • Tics – Neck popping movements • Dystonia – abnormal twisting
    are repetitive and stereotypic, movement sustained for a long
    relieve an inner feeling of time.
    tension. • Acute onset is likely medication
    • Diagnosis of rhinitis unrelieved related – she was recently given
    with steroids is probably headache medication, probably
    sniffing/throat clearing that is dopamine antagonist like
    also a tic. phenergan.
    • Diagnosis is Tourette's • Tx – benedryl
    • Treatment can be observation
    unless significantly bothersome
    or comorbidities.
    Basal Ganglia Circuit – Just to discuss treatment
    Tics

    • 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

    • 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

    • 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

    • 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

    • Athetosis • Sterotypies
    – Slow, continuous, writhing – Taskforce, “stereotypies are
    movements of distal body repetitive, simple
    parts, especially fingers and movements that can be
    hands voluntarily suppressed”
    – Can see in cerebral palsy and – Patterned, episodic,
    Rett syndrome. repetative, purposeless
    • Myoclonus – These are different from tics
    – Sudden, brief, shock-like – See often in autism, Rett
    movements
    – May be repetitive or
    rhythmic
    Benign movements (may need to rule out other
    causes)
    • 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

    • 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

    • 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

    • 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
    • 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

     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
    References

    -Uptodate articles
    -on call neurology
    -http://www.unifr.ch/biochem/index.php?id=120

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