›Start at the lower end and titrate to effect
›Verify against current pediatric guideline before use
›Observe for iatrogenic overdose (hypotonia, hyporeflexia, hypoventilation, obtundation) for at least several hours after any bolus, with airway and ventilatory support at the bedside because there is no reversal agent
›Escalating rigidity, hyperthermia, or autonomic instability despite enteral baclofen at maximum dosing and escalating benzodiazepines is the trigger to give intrathecal baclofen and move to intensive care
›Do not keep climbing the enteral baclofen dose alone while the patient deteriorates
›Reassess for an untreated cause: catheter disconnection, granuloma, pocket fill, unrecognized infection, or coexisting sepsis
›Restore the patient's own device as the definitive endpoint
›Emergent reservoir refill, reprogramming, or surgical catheter or pump revision as dictated by interrogation and imaging
Adjuncts for hyperthermia, rigidity, and autonomic instability
›Active external cooling for core temperature at or above 39°C, with cooling blankets, cold packs to the groin and axillae, and cooled intravenous fluid
›Antipyretics such as acetaminophen are largely ineffective because the heat is muscular in origin
›Dantrolene for life-threatening rigidity and hyperthermia not controlled by benzodiazepines
›Dantrolene 1 to 2.5 mg/kg IV, repeated every 6 hours as needed, maximum 10 mg/kg per day
›Monitor liver enzymes with repeated dosing
›Cyproheptadine as a reported adjunct for withdrawal with prominent serotonergic overlap
›Cyproheptadine 4 to 8 mg enteral every 6 to 8 hours, maximum 32 mg per day
›Sedation and anticholinergic effects are the main downsides
›Dexmedetomidine infusion for autonomic arousal and agitation without respiratory depression
›Dexmedetomidine 0.2 to 1.5 mcg/kg per hour IV with no loading bolus
›Titrate by 0.1 to 0.2 mcg/kg per hour every 30 minutes
›Bradycardia and hypotension are dose-limiting
›Short-acting agents for hypertensive surges, chosen so therapy can be stopped quickly when pressure swings to hypotension
›Esmolol 25 to 50 mcg/kg per minute IV infusion when heart rate and blood pressure both need control, titrated to effect
›Nicardipine 5 mg per hour IV, titrated by 2.5 mg per hour every 5 to 15 minutes to a maximum of 15 mg per hour, when blood pressure alone is the target
›Nicardipine is a dihydropyridine calcium channel blocker and an arterial vasodilator; it gives no rate control and can cause reflex tachycardia, so pair it with a beta blocker when tachycardia is also problematic
›Treating autonomic signs is secondary to baclofen and benzodiazepine replacement
›For any titrated infusion (dexmedetomidine, benzodiazepine, propofol, esmolol, nicardipine, norepinephrine): continuous ECG, pulse oximetry, and capnography, an arterial line for the labile pressures, vital signs and muscle tone reassessed every 15 minutes during titration, and creatine kinase every 6 to 12 hours
Supportive and organ-protective care
›Isotonic crystalloid resuscitation targeting urine output of 1 to 2 ml/kg per hour in rhabdomyolysis
›In dialysis-dependent or advanced chronic kidney disease patients, involve nephrology early and avoid volume overload
›Serial potassium and ECG monitoring for hyperkalemia from muscle breakdown
›ECG shows peaked T waves, PR prolongation, and QRS widening with hyperkalemia, and QT prolongation or bradyarrhythmia with baclofen overdose
›Calcium gluconate 1 to 3 g IV, then regular insulin 10 units with 25 g dextrose IV, then potassium removal, repeating the ECG after each step
›Venous thromboembolism prophylaxis for immobility unless disseminated intravascular coagulation or active bleeding is present
›Empiric antimicrobial coverage when device infection or meningitis cannot be excluded
›Vancomycin 15 to 20 mg/kg IV every 8 to 12 hours plus cefepime 2 g IV every 8 hours in adults, adjusted to cultures and renal function
›Intrathecal baclofen recipients are generally older than 3 to 4 years, so neonatal dosing does not apply; for a younger child, verify the regimen, including whether to add gentamicin, against a current pediatric guideline
›Analgesia for painful spasms
›Acetaminophen 15 mg/kg up to 1 g enteral or IV every 6 hours, maximum 4 g per day, and 3 g per day with hepatic impairment or chronic use
›Morphine 0.05 to 0.1 mg/kg IV every 2 to 4 hours titrated, with continuous capnography given concurrent benzodiazepines
›Avoid tramadol 50 to 100 mg and meperidine 1 to 1.5 mg/kg because they lower the seizure threshold
›Oxygen titrated to a patient-specific target, since over-oxygenation in chronic hypercapnia can worsen ventilatory failure
›Treat autonomic dysreflexia in spinal cord injury by removing the trigger first: sit the patient up, drain the bladder, and disimpact the rectum, then use the short-acting antihypertensives above if pressure remains dangerous
Management of baclofen overdose
›Overdose is managed with airway and ventilatory support and time, because there is no antidote
›Intubate for airway protection or hypoventilation and expect recovery over 24 to 72 hours
›Atropine 0.5 mg IV for symptomatic bradycardia; isotonic fluid and norepinephrine 0.05 to 0.5 mcg/kg per minute for hypotension
›Verify against current pediatric guideline before use
›Address the device to stop ongoing delivery
›The implanting service programs the pump to minimum or stops it and aspirates drug from the catheter access port
›Correct a pocket fill by aspirating the subcutaneous depot
›Enteral activated charcoal 1 g/kg to a maximum of 50 g only for a witnessed recent oral baclofen ingestion with a protected airway
›Hemodialysis for severe oral baclofen overdose with renal failure, because baclofen is dialyzable
›Dialysis does not help pump-delivered intrathecal overdose, where the dose is small and cleared from cerebrospinal fluid over hours
›Do not give physostigmine 0.5 to 2 mg IV; reported arousal is transient and it risks bradyarrhythmia and seizures
›Watch for withdrawal to emerge as the overdose resolves, because the underlying dependence persists
Iatrogenic harms to avoid
›Airway and paralytic errors
›Succinylcholine 1 to 1.5 mg/kg IV for rapid sequence intubation can cause hyperkalemic cardiac arrest in these chronically immobilized, often spinal cord injured patients and with rhabdomyolysis; use rocuronium 1.2 mg/kg IV
›Extubating before intrathecal delivery is restored, when rigidity and hypoventilation can recur
›Wrong-mechanism drugs
›Dopamine antagonists (haloperidol 2 to 5 mg, metoclopramide 10 mg IV, prochlorperazine 10 mg IV) given for agitation, nausea, or presumed neuroleptic malignant syndrome worsen rigidity, lower the seizure threshold, and delay effective treatment
›Under-dosing benzodiazepines by treating them as sedation rather than the primary therapy, or substituting an antipsychotic, which treats nothing
›Relying on antipyretics for hyperthermia that is muscular in origin, delaying active cooling and benzodiazepines
›Hemodynamic and hematologic errors
›Treating tachycardia and hypertension as the primary problem with long-acting antihypertensives or beta blockers, which are unopposed when the autonomic picture swings to hypotension
›Vasopressors are appropriate for hypotension after sedation, but use short-acting titratable agents such as norepinephrine 0.05 to 0.5 mcg/kg per minute because pressure is labile
›Fluid loading without monitoring in a patient with possible stress cardiomyopathy or advanced kidney disease
›Anticoagulation or fibrinolysis for the tachycardia and troponin rise of stress cardiomyopathy, which is not thrombotic and is dangerous if disseminated intravascular coagulation is developing
›Mechanical circulatory support such as an intra-aortic balloon pump is rarely indicated and only for refractory cardiogenic shock from stress cardiomyopathy, not for the hyperdynamic state of withdrawal
›Diagnostic and analgesic errors
›Reading improved spasticity plus new drowsiness as recovery, then discharging a patient who later arrests from respiratory depression
›Withholding cultures and empiric antibiotics because withdrawal explains the fever, missing catheter-related central nervous system infection
›Opioids or tramadol as the main strategy for spasm pain, adding respiratory depression and, for tramadol, seizures