Intrathecal baclofen withdrawal simulating neuroleptic malignant syndrome in a child with cerebral palsy

Abstract

Intrathecal baclofen infusion (IBI) is being used with increasing frequency in children to treat spasticity and dystonia. In this report, we summarize the clinical course of a 9–year‐old boy with quadriplegic cerebral palsy with mixed tonal abnormalities (spasticity and dystonia) experiencing withdrawal from intrathecal baclofen. His clinical course is compared to that of adults experiencing withdrawal from IBI and to neuroleptic malignant syndrome. If unrecognized, this disorder may have significant potential for morbidity and mortality. Clues to diagnosis, appropriate evaluation, and potential treatments are discussed. When a child treated with IBI presents with unexplained multiorgan system dysfunction, particularly if accompanied by evidence of rhabdomyolysis, the integrity of the IBI system must be evaluated. In some cases, evaluation might necessitate surgical exploration. Caregivers most commonly seek urgent evaluation and treatment from their primary care provider when their child experiences fever or acute illness. Primary care providers of children treated with IBI should be made aware of this clinical scenario to prevent delays in diagnosis.

Neuroleptic Malignant Syndrome Induced by Lamotrigine

“…10 Of course we cannot exclude the possibility that this patient's NMS was induced by ARP. Although the exact mechanism for how NMS is associated with the GABAergic system is not yet completely understood, it is known that the GABAergic medication bacrofen may cause symptoms of NMS.…”

Baclofen Withdrawal: A Cause of Prolonged Fever in the Intensive Care Unit

“…GABA B receptors in the spinal cord are down-regulated after a week of baclofen therapy in an animal model 5 . Thus, abrupt withdrawal of baclofen may be associated with rebound excitation at all levels of the neuro-axis that may not be overcome by the administration of lower dosages of baclofen or other GABA agonists [6][7][8][9] .…”

“…Baclofen withdrawal following abrupt discontinuation of intrathecal or oral baclofen therapy is a lifethreatening and even fatal syndrome [6][7][8][9][10] . Symptoms including fever, autonomic dysregulation, sepsis-like syndrome, muscle spasticity, dyskinesia, psychosis and seizure have been reported 6,7,11,12 . Baclofen underdosing during attempts to taper oral baclofen or replace intrathecal with oral baclofen may cause severe baclofen withdrawal syndrome 8,9 .…”

In Support of Initial Parenteral Medical Management of Intrathecal Baclofen Withdrawal in Spasticity Patients

“…It has been mistaken for toxic syndromes, sepsis, neuroleptic malignant syndrome, and malignant hyperthermia, to name a few. 1,6-8 The difficulty in making the correct diagnosis from a protean constellation of signs and symptoms may contribute to delay of definitive treatment and progressive worsening of the patient’s condition, especially so if the physician is unfamiliar with it. 3,4,6,9-11 Symptoms of withdrawal may manifest with surprising rapidity.…”