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PMR board > Peds hypertonia > Flashcards

Peds hypertonia Flashcards

1
Q

Spasticity

A

Velocity-dependent resistance to muscle stretch

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2
Q

Spasticity pathophysiology

A

Poor control of the monosynaptic reflex arc at the spinal cord modulated by upper motor neurons, specifically the corticospinal tract. A disruption of these tracks diminishes their inhibitory influences.

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3
Q

Dystonia

A

Cocontraction of antagonist and agonist muscles. Not velocity-dependent movement disorder characterized by abnormal sustained or intermittent movement causing posturing or twisting or both.

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4
Q

Dystonia pathophysiology

A

Damaged to the basal ganglia which is responsible for automatic and integrated sequences of movement and enhanced preferred motor patterns and inhibits unwanted motor pattern. Damage to the basal ganglia decreases inhibitory signals to the thalamocortical tracts.

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5
Q

Medication that can cause acute dystonia and other extrapyramidal symptoms

A

SSRIs

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6
Q

Dopa-responsive dystonia

A

DRD is a genetic syndrome, typically AD, due a mutation in the GTP cyclohydrolase 1 gene and the motor problems are responsive to low doses of levodopa.

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7
Q

Chorea

A

RANDOM, incessant, involuntary movement with identifiable discrete movement.

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8
Q

Athetosis

A

Slow, constant, writhing movements.

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9
Q

Ballismus

A

Sudden, high-amplitude movement of a limb.

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10
Q

Why basal ganglia gets affected?

A
  • PVL extension to the basal ganglia
  • BG has a high metabolic rate and are susceptible to brief severe hypoxic events
  • BG is susceptible to metabolic injuries such as iron deposition from hyperbilirubinemia.
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11
Q

Tardieu scale

A

V1- A passive movement around a joint at a slow speed
V2- A fast speed at the threshold to rigger a catch
V3- The difference between V1 and V2 angles is the angle of spasticity V3.

It also measures the quality of muscle reaction on a scale of 0 (nor resistance) to 4 (clonus).

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12
Q

Minimum weight for ITB placement

A

34lbs

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13
Q

Diazepam MoA

A

GABA A agonist in the CNS

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14
Q

Diazepam dose

A

0.2 to 0.8 mg/kg/day divided every 6-8 hours.

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15
Q

Baclofen MoA

A

GABA B agonist at the spinal cord to inhibit to monosynaptic reflex arc.

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16
Q

Baclofen dose

A

Starting dose of 2.5 to 5 mg/day, divided every 8 hours, titrated up every few days to a maximum of 20-60 mg/day.

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17
Q

Baclofen withdrawal symptoms

A

Increased spasms, hallucination, confusion, pruritus, and seizures.

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18
Q

Baclofen overdose symptoms

A

Respiratory depression, hypothermia, hypotonia, seizures, coma.

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19
Q

Dantrolene MoA

A

Inhibits the release of calcium in the sarcoplasmic reticulum

20
Q

Dantrolene dose

A

Starting dose of 0.5mg/kg once a day and increasing to 12mg/kg or 400mg/day in divided doses

21
Q

Tizanidine MoA

A

Alpha 2 agonist

22
Q

Medication to treat acute dystonia episodes

A

Benzodiazepines

23
Q

Drug alternatives for dystonia

A

Clonidine, Gabapentin, Trihexyphenidyl (Artane), Levodopa (for DRD), Tetrabenzine, Baclofen and Benzodiazepines.

24
Q

Adverse effect from Clonidine

A

sedation, bradycardia, hypotension, and pseudoileus.

25
Q

Gabapentin dose

A

3-11 years, starting dose of 10mg/kg/day up to 40mg/kg/day (3-4 years) to 60mg/kg/day (>4 years).

26
Q

Trihexyphenidyl MoA

A

Muscarinic antagonist that has anticholinergic effect and central and peripheral receptors.

27
Q

Trihexyphenidyl dose

A

Starting dose at 2mg/day and increased by 2.5mg every other week to a max of 60mg/day or up to 0.75mg/kg.

28
Q

Trihexyphenidyl side effects

A

dry mouth, constipation, blurred vision and irritability.

29
Q

Tetrabenzine MoA

A

Depletes presynaptic dopamine by blocking vesicular monoamine transporter type 2 (VMAT2)

30
Q

BoNT MoA

A

blocks the release of acetylcholine at the neuromuscular junction

31
Q

FDA max dose of BoNT

A

onabotulinum toxin A (10U/kg/BLE or 340U)
Abobotulinum toxin A (30U/kg/BLE or 1,000 U)
Incobotulinum toxin A 12 U/kg or 400U)

32
Q

Why 12 weeks in between BoNT injection is recommended?

A

To decrease risk of developing antibodies and neuromuscular recovery time.

33
Q

BoNT with less risk of antibody formation.

A

Incobotulinum toxin A (Xeomin) is very unlikely to cause antibodies since all extraneous proteins have been removed.

34
Q

Phenol dose

A

Less than 30mg/kg, higher doses can cause dysrhythmias and bradycardia.

35
Q

Adverse effect from phenol injection

A

Dysesthesia is sensory nerve is injected. Less than 5% of children who receive phenol injections have dysesthesia.

36
Q

ITB onset of action and peak of a simple continuous

A

Onset of action is 6-8 hours, peak effect at 24 to 48 hours.

37
Q

ITB onset of action and peak of a large bolus

A

Onset of action is .5 to 1 hour, peak effect at 4 hours.

38
Q

ITB half life

A

3 to 5 hours

39
Q

Environmental considerations when having a ITB

A

Changes in barometric pressure (such as Scuba diving and hyperbaric treatment) and high water temperatures (>102F).

40
Q

If acute ITB withdrawal is suspected…

A

Oral Baclofen (5 to 20mg every 4 hours) should be started immediately.

41
Q

Troubleshooting ITB pump and catheter

A
  • Interrogate for concentration, programming errors, motor stalls and low reservoir volume.
  • Program a bolus, if no improvement in hypertonia..
  • Attempt aspiration from catheter access port, if CFS is not withdrawn easily…
  • AP and lateral X-ray to look for the radiopaque catheter tip, if normal X-ray…
  • Catheter dye study ( if CSF can be withdrawn to avoid rapid delivery of Baclofen and overdose)
  • Replace catheter
42
Q

Describe SDR

A

Disinhibition of monosynaptic reflex arc by disrupting the afferent sensory fibers. Done at L1-S1/S2. Typically 25-60% of the dorsal rootlets are transected.

43
Q

Candidates for SDR

A

Ideally 4-10 years of age. If older, there is a risk of preexisting contractors and diminished of brain plasticity.
Patient also needs to be able to follow commands to participate in the intense therapy post op.

44
Q

Benefits of SDR in the long term

A

Less botulinum toxin injection and orthopedic procedures.

45
Q

Adverse effects of SDR

A

weakness
urinary retention
numbness
infections
dysesthesia- risk increased with increased number of rootlets dissected. Usually last several weeks and responds to Gabapentin.
nocturnal leg spasms- transient and respond to diazepam.

46
Q

Ventral dorsal rhizotomy

A

sensory/dorsal and ventral/motor rootlets are cut from L1-S2, 50-80%. resulting in permanent reduction in spasticity and dystonia, although some dystonia can return after 1-2 years. Might be an option for GMCS IV-V.

PMR board (21 decks)

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