Judith L. Gooch

Prolonged paralysis after treatment with neuromuscular junction blocking agents

ObjectivesPrevious reports have described prolonged paralysis after treatment with neuromuscular junction blocking agents in critically ill patients. The purpose of this study was to further describe a group of patients who developed prolonged weakness after treatment with these agents. DesignClinical information, electrodiagnostic and muscle pathology results are described in this group of patients. Clinical information includes diagnoses, dosage of neuromuscular junction blocker, other medications affecting the neuromuscular system, and neuromuscular examination and clinical course. SettingAll patients were seen in the ICUs of three local hospitals. PatientsIncluded were critically ill patients with a variety of diagnoses, all of whom developed severe weakness after discontinuation of neuromuscular junction blocking agents. InterventionsElectrodiagnostic studies and muscle biopsies were performed on several of the patients. Measurements and Main ResultsAll patients had pronounced weakness without sensory loss. Electrodiagnostic and muscle pathology findings were consistent with failed neuromuscular transmission. Although many patients had disorders or were taking medications that can injure the neuromuscular system, no disorder or medication was common to all. Recovery of strength often took several months and most patients were slow to wean from mechanical ventilator support. ConclusionsAlthough alternative explanations cannot be excluded with certainty, the use of neuromuscular junction blocking agents may lead to neurogenic atrophy and care must be taken when using them.

Complications of Intrathecal Baclofen Pumps in Children

Intrathecal baclofen is increasingly being used to manage severe spasticity in children. Although substantial tone reduction with this treatment has been documented, complications also occur. In this study, we describe the device- and major non-device-related complications in a group of 100 consecutive children and young adults who received 117 intrathecal baclofen pumps for the management of severe spasticity. Twenty-four patients (24%) experienced a total of 48 complications. The most common complication was disconnection of the catheter at its connection to the pump, occurring in 9% of pumps implanted. This complication occurred more frequently in pumps with catheter access ports (16%) than in those without ports (2%). Catheter dislodgement from the intrathecal space was the next most common complication, occurring in 8% of pumps implanted (13% of pumps with ports, 4% of pumps without ports). To decrease the occurrence of the most common complications of intrathecal pumps, we now typically implant pumps without catheter access ports, and we use 2-piece catheters. Although the lack of an access port may be a disadvantage for troubleshooting, most complications can be detected in pumps without a port. Patient and family education is critical in preventing serious consequences of baclofen withdrawal resulting from catheter-related complications.

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

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.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.

Stereotactic endoscopic placement of third ventricle catheter for long-term infusion of baclofen in patients with secondary generalized dystonia.

Continuous infusion of baclofen is a treatment option for severe generalized dystonia. Catheter insertion within the third ventricle has been described as an alternative to standard intrathecal placement to maximize intracranial concentrations of baclofen. The authors describe their experience with a novel technique for stereotactic endoscopic insertion of baclofen infusion catheters in the third ventricle in 3 patients with severe secondary generalized dystonia. Insertion was successful in all 3 patients, and all of them experienced significant improvement in dystonia scores on the Barry-Albright Dystonia Scale. Follow-up ranged from 5.5 to 7 months (mean 6 months), and no mechanical complications or CSF leaks were observed. The stereotactic endoscopic insertion of a baclofen infusion catheter into the third ventricle appears to be a safe method for continuous intraventricular baclofen infusion in patients with generalized secondary dystonia.

Spinal Stenosis after Total Lumbar Laminectomy for Selective Dorsal Rhizotomy

Knowledge of long-term outcome and complications of selective dorsal rhizotomy is limited due to the relatively recent introduction of the procedure. We describe 2 patients with cerebral palsy who developed lumbar spinal stenosis several years after selective dorsal rhizotomy. These patients also had substantial lateral trunk sway during gait and walked for several years with limited assistive devices. This abnormal gait pattern in combination with the changes from the selective dorsal rhizotomy may lead to the development of spinal stenosis.

Force perception before and after maximal voluntary contraction.

Capacity to match a low level of elbow flexion force maintained in the control arm was measured in the experimental arm in 16 subjects before and after maximal voluntary contraction (MVC). Prior to a 1-min. MVC, the mean force exerted by the experimental arm was 3.4 ± 1.0 kg when attempting to match the tension of a 2.3-kg weight in the control arm. After the MVC, the mean force exerted in the experimental arm was 4.4 ± 2.6 kg. The change in perception of force after a prolonged MVC as demonstrated in this study may be due to postcontraction potentiation of contraction, which has been demonstrated by others after a brief MVC.

Orthopedic surgery in children with intrathecal baclofen pumps

AIMS Children with cerebral palsy often have severe spasticity leading to deformity that requires multiple orthopedic surgeries. Intrathecal baclofen pump implantation effectively decreases severe spasticity. The objective of this study was to determine whether children who have a baclofen pump implanted at a young age have fewer orthopedic surgeries than those who have a baclofen pump implanted at later ages. METHODS In this retrospective study of 310 children, we compared occurrence of surgery in relation to having or not having the baclofen pump, by using survival analysis with surgery as the outcome, presence of baclofen pump as the exposure of interest, modeled as a time-dependent variable, and age as the time scale. RESULTS There was no significant effect of pump placement on overall surgery frequency. Analyses by type of surgery showed that those without a pump in place had a 64% lower hazard of scoliosis surgery. CONCLUSIONS No evidence was found to indicate that children who have a baclofen pump implanted at a young age have fewer orthopedic surgeries than those who have a baclofen pump implanted at later ages.