Pediatric Movement (Payne) Flashcards
What questions should we ask pediatric patients when trying to diagnose movement disorders? (8)
- Paroxysmal or continuous
- Acute or chronic
- Triggered by external stimuli
- Suppressible
- Abnormal neurologic exam
- Hereditary
- Degenerative
- Type of movements
Chorea, Athetosis (snake like), Dystonia, Dyskinesia (disordered), Tics
are all classified as?
- Where in the brain does this come from?
Movement disorders
- Globus pallidus
Diagnosis?
- Compare this to “weakness”!
- Can be ocular, truncal, appendicular, gait
- Causes: infectious/post-infectious, lesional (vascular, demyelinating, traumatic, mass), toxins, metabolic, hereditary, neurochemical imbalance, seizures
Ataxia
Diagnosis?
- Physical exam:
- Frequent, brief, unpredictable, purposeless movements
- Flows from one body part to another
- Chaotic
- “piano playing” “milkmaid’s grip” “chameleon tongue”
- Mental processing can bring out the tremor
- Seen in:
- juvenile onset Huntington disease
- Sydenham’s ___
- Cerebral Palsy - perinatal HIE
- Kernicterus
- Toxin - stimulant meds (*Most common)
- Post pump ___ (after cardiac surgery
- ___ gravidarum
- Infection
- Degenerative disorders
- Metabolic disorders
- Acute disseminating encephalomyelitis
- SLE, hyperthyroidism
Chorea, Athetosis
Diagnosis?
- Major JONES criteria for Acute Rhematic Fever
- 10-40% of children with ARF have this
- 5-15 years of age
- 2:1, females to males
- Weeks to months after GABHS infection
- Gradual progressive emotional lability followed by choreiform movements
- Asymmetric or unilateral
Sydenham’s Chorea
How is Sydenham’s chorea diagnosed?
- ______ and + ______
- Most children are + for _____ or ______
- Throat culture may be negative
- 40-75% of children with this have carditis
Tx: Usually COMPLETELY resolves - waxes and wanes for months
- Meds to treat?
- Clinical history and + serology
- Anti-streptolysin O or antiDNAase B antibodies
- Pimozide, Carbamazepine, Valproate, Benzodiazepine, Corticosteroids, Penicillin
Which cause/help treat chorea?
A. Benzodiazepines, valproate, carbamazepine, pimozide, haloperidol
B. Stimulant meds, cocaine, lithium
A. Help treat chorea
B. Cause chorea
Diagnosis?
- Contraction of agonist and antagonist muscle groups
- Primary: hereditary, metabolic
- Secondary: cerebral palsy, post-traumatic, post-stroke, toxin
- Types
- Focal
- Segmental
- Multifocal
Dystonia
Dystonia:
- May worsen during _______
- May ____ over time
- May be triggered by certain body ______
- Not present during ____ – be careful for arousals
- May be suppressed by _______, “geste antagoniste”
- Stress/excitement/pain
- Fluctuate
- Movements
- Sleep
- Touch of a certain body part
Primary Dystonia in Childhood:
- A: Most common, excellent response to low-dose levodopa
- Begins with foot dystonia
- B: AD DYT1 mutation; Begins in a limb (usually lower extremity)
- Generalizes within 5 years
- A: Dopa-responsive Dystonia
- B: Idiopathic generalized Torsion Dystonia
Diagnosis?
- Chromosome 13q14-q21, AR inheritance
- Defect in the copper transporter that carries copper out of the hepatic cells
- Many neuropsychiatric manifestations -
- dysarthria
- dystonia (fixed pseudosmile = risus sardonicus)
- rigidity
- gait disturbance
- tremor
Wilson Disease
What are usually associated with each other in the same affected limb?
- ____ = VELOCITY DEPENDENT TONE – felt when the patient is at rest
- ____ = Action, patient may not be able to be completely at rest
***Both exacerbated by stress, excitement, pain
- Weak muscles
- Spasticity
- Dystonia
What can be used to treat dystonia?
- 3 classes?
- Goals: Ease of care, ADLs, More control of voluntary movement, Ambulation - but be careful too much can impair function, Remove the tone, lose the strength
- Muscle relaxants
- Baclofen
- Tizanidine
- Anticholinergic
- Trihexyphenidyl
- Benzodiazepines
- Diazepam
What are the two surgical treatments of dystonia?
A. Inserted at any level in the spinal cord - helps symptomatically, decreased systemic side effects
B. Probably doesn’t work for spasticity because the circuit is interrupted
A. Intrathecal Baclofen pump
B. Deep brain stimulation
Common culprits of drug induced movemment disorders:
- Dopamine antagonists - can cause?
- Amphetamines - can cause?
- Cocaine - can cause?
- Lithium - can cause?
- Acute dystonic reaction, tardive dyskinesia, parkinsonism, neuroleptic malignant syndrome
- Chorea, tremor
- Chorea
- Chorea, tremor
Treatment for drug induced movement disorders? e.g. Acute dystonic reaction (oculogenic crisis, torticollis, axial dystonia)
IV Benadryl or cogentin
Tics usually:
- Begin by age __
- _____ tics precede _____ tics
- Worsen around age ____ and lessen around age ____
- CHANGE OVER TIME!
- get checked out - blinking (ophtho), nose/throat (ENT), etc.
- 6
- Motor precede vocal
- 10, 18
FYI:
- Tics may be triggered by an external stimulus - contacts
- May be suppressed - then more “ticcing” after!
- May NOT be present during sleep - be careful of arousals
- Begin with a sense of urge - with anxiety, urge is a type of obsession
- Feeling of relief after performing
In comparison with TICs - what are:
- repetitive purposeless movements
- Involuntary +/-
- Do not change over time
- Occur when excited, stressed, fatigued
- Autism- hand flapping, Rett- hand flapping, etc.
Treatment?
Stereotypies
Tx: SSRIs, especially for anxiety
How are tics usually treated?
- What is treated first?
- If none - kids? adults?
- Comorbidities are treated first
- eg underlying anxiety
- If none, alpha 2 adrenergic agonist first for kids (try nothing, then guanfacine/clonidine, then topiramate, depakote)
For adults - CBT
_______ causes tics, OCD, or both
- Prepubertal onset
- episodic course
- temporal relationship with infection and tic/ocd exacerbations
**Do NOT use abx to treat exacerbations, use prophylaxis for patients after rheumatic fever
- Use standard meds for tics
Group A beta-hemolytic streptococcal infection
Some common causes of: ______
A. Cerebellitis, neuroblastoma, brain tumor, ingestion, miller fisher syndrome, MS, postconcussive syndrome, vertebrobasilar occlusion, cerebellar hemorrhage, Kawasaki
B. Metabolic, hereditary, migraine, BPPV
C. Congenital, brain tumor, MS< Freidrich’s ataxia, hereditary, ataxia-telangiectasia
A. Acute ataxia
B. Paroxysmal ataxia
C. Chronic ataxia
Localizes to..?
- Truncal ataxia - _______
- Limb ataxia - ______
- Eye movements - _____
- Vermis
- hemispheres
- brainstem
FYI:
- Obtaining CSF in ataxia - smart! INfection (varicella, HSV, EBV, mumps)
- Elevated protein (MS, Miller fisher)
- Brain imaging! tumor
- Blood EtOH - little kids, mouthwash!
- Check toxicities/levels: phenytoin, carbamazepine, sedatives, ethylene glycol, lead, mercury, thallium
