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Review Question - QID 215570

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QID 215570 (Type "215570" in App Search)
A 7-year-old boy with a history of GMFCS IV spastic cerebral palsy is being treated with intrathecal baclofen to decrease spasticity. During his treatment course, you receive a phone call from an emergency department provider who is concerned that the patient's baclofen pump has malfunctioned. If this were the case, which of the following would be a sign of baclofen withdrawal?

Decreased serum potassium

14%

211/1557

Decreased serum creatine phosphokinase (CPK) levels

6%

98/1557

Elevated serum creatinine

53%

830/1557

Hypothermia

9%

146/1557

Muscle atony

17%

257/1557

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Baclofen withdrawal in the setting of pump failure can result in rhabdomyolysis and multi-system organ failure, which could be reflected by elevated serum creatinine.

Intrathecal baclofen is used primarily in nonambulatory patients with cerebral palsy patients in order to decrease muscle tone. Intrathecal baclofen acts as a GABA-b agonist and is generally better tolerated than oral baclofen with decreased risk of sedation and respiratory depression. Long-term baclofen usage down-regulates GABA-b receptors over time. Abrupt baclofen discontinuation (such as from a pump malfunction) results in a predominance of excitatory CNS signals with increased muscle rigidity, myoclonus, hyperthermia, and possibly rhabdomyolysis with multi-system organ failure. Laboratory signs include an increase in serum creatinine, potassium, and CPK levels.

Ertzgaard et al. compared the use of oral versus intrathecal baclofen in patients with cerebral palsy. They found that while oral baclofen can be effective in most patients, between 25-75% have significant side effects such as somnolence, muscle weakness, and nausea. They state that intrathecal baclofen can be a reasonable alternative in patients who do not tolerate oral medication, but caution against overdose or abrupt withdrawal if intrathecal baclofen is chosen.

Albright et al. reviewed the long-term use of intrathecal baclofen when used for severe cerebral-related spasticity. They note that improvements in spasticity were significant and were maintained throughout the > 10-year follow-up. Of note, adverse events occurred in half of the studied patients, with the most common (31%) being catheter-related issues.

Incorrect Answers:
Answer 1: Increased potassium would be seen in cases of rhabdomyolysis associated with renal failure
Answer 2: Increased CPK levels would be seen in cases of rhabdomyolysis due to muscle breakdown
Answer 4: Hyperthermia is often seen in cases of rhabdomyolysis and multisystem organ failure.
Answer 5: Increased rigidity would be seen if the baclofen pump were to fail, as there would be less GABA agonism.

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