Satisfaction of individuals treated long-term with continuous infusion of intrathecal baclofen by implanted programmable pump.

PURPOSE: To investigate the perspective of the individual receiving intrathecal baclofen (ITB) or his/her caregiver concerning its effects and to describe characteristics of those that were satisfied or not satisfied. METHODS: After IRB approval, potential subjects were identified who had undergone ITB pump implantation at least 1 year prior to the study. One hundred subjects/caregivers were interviewed by phone. Interview consisted of a scripted questionnaire to obtain subject/caregiver opinions about changes in function and caregiver assistance, as well as satisfaction with ITB. Medical records were reviewed to collect information including diagnosis, ITB related surgeries and medications. RESULTS: Subjects ranged in age from 5-42 years and 88 had a diagnosis of cerebral palsy. Improvement was noted in the following areas: positioning 69%, transfers 58%, dressing 69% and toileting/hygiene 51%. Fifty-four per cent reported reduction in startle movements. Sleep was improved in 43% and comfort in 53%. Twenty-two subjects experienced 32 events related to the ITB hardware or surgery. Only 12% indicated they would not choose to undergo the procedure again. DISCUSSION: Generally, subjects and their caregivers were satisfied with the results after ITB pump implantation. A majority reported improvements in positioning, transfers, dressing, toileting/hygiene and comfort.

Pharmacotherapy of spasticity: oral medications and intrathecal baclofen.

Spasticity is a common problem in children with neurologic impairment, particularly in those with cerebral palsy. Clinicians commonly make use of oral medications to attempt to reduce spasticity and increase function. Little has been published in the literature concerning the use of these medications in children and their effects on both muscle tone and function. This article is a review of the sites of action, side effects, and efficacy of benzodiazepines, baclofen, dantrolene sodium, alpha2-adrenergic agonists, and gabapentin. A brief review of intrathecal baclofen is included as well.

Spasticity management in the child with spastic quadriplegia.

In children with spastic quadriplegia, also described as 'whole body involvement', spasticity can interfere with motor function, contributes to the development of deformities and adversely impacts on care, positioning, and comfort. In this population, spasticity interventions address goals such as improving comfort, reducing pain, easing the burden of carers, slowing the progression of musculoskeletal deformities and perhaps improving function. Children with severe diplegia are distinguished from those with quadriplegia by their ability to ambulate, as well as by a greater emphasis being placed on functional motor goals even though similar treatment modalities are often employed to manage spasticity. The many treatment options currently available include, but are not limited to, botulinum toxin type A, phenol neurolysis, oral medications, intrathecal baclofen, selective dorsal rhizotomy, and orthopaedic surgery. The integration of these treatment modalities can help to optimize the overall care and function for a child with spastic quadriplegia or severe diplegia. However, the development of a management programme is complex and needs to take into account many factors, including age, weight and nutritional status, rate of progression of musculoskeletal deformities, developmental potential, comorbid conditions, current functional status and prognosis, and family and patient treatment goals. Children with marked spasticity are likely to benefit from a combination of interventions, rather than a single treatment modality. Because of these complexities, management should be planned and coordinated by a multidisciplinary team of medical and allied health professionals which recognizes the central role of the family in all decisions. Once the special characteristics of the child with spastic quadriplegia and the various treatment options are understood, outcomes can be maximized.

Late improvements in mobility after acquired brain injuries in children.

The purpose of this investigation was to describe the late improvements in functional mobility in children who have sustained severe acquired anoxic or traumatic brain injuries. Ninety-eight children from a consecutive series of 199 with acquired brain injuries met inclusion criteria. As expected, children with traumatic injuries had better mobility at time of discharge from rehabilitation than did children with anoxic injury. In addition, children with traumatic injuries improved more in mobility status during the first 2 years after injury than did children with anoxic injury. The children who continued to improve in mobility after discharge were unconscious for a shorter time. Children who became community ambulators during the first year after discharge had higher mobility ratings at discharge. Although children who eventually achieved more functional mobility were admitted to rehabilitation service sooner after injury, discriminant analysis revealed that the duration of unconsciousness, and therefore severity of injury, was more closely correlated with the final mobility status.

Failure of absorption of baclofen after rectal administration.

We wished to determine the degree to which baclofen was absorbed from an aqueous vehicle after rectal administration. A comparison was made to absorption after oral administration. After oral administration, the baclofen half-life was 2.3 to 3.4 hours and peak serum concentrations were achieved from 1 to 2.1 hours after administration. No measurable absorption was observed after rectal administration of the drug in any subject. Rectal administration of baclofen is not a clinically sound treatment option when oral administration of the drug is not possible. For patients receiving chronic baclofen therapy, other medications such as a benzodiazepine should be considered at times when oral administration of medication is not possible.

Automatic garage door openers: hazard for children.

OBJECTIVES: Despite significant advances in automatic garage door opener design, automatic garage door openers continue to severely injure or kill children. In this investigation, we sought to determine the frequency and circumstances of accidents that have caused severe injury or death to children. We also tried to develop a means by which homeowners can evaluate their door openers. METHODS: We present the histories of three children severely injured or killed by automatic garage door openers. We reviewed national data of similar accidents primarily published by the US Product Safety Commission and Underwriters Laboratories. Also, we evaluated 50 automatic door openers for safety of operation. The reversing mechanisms of door openers were tested using a cardiopulmonary resuscitation mannequin, a roll of paper towels, and a block of wood. RESULTS: In the United States, at least 85 children have had permanent brain injury or have died since 1974 as a result of accidents involving automatic door openers. A review of circumstances of the accidents illustrates that accidents are caused both by use of the openers by children and by faults in design. Most accidents have occurred when children have found access to the activation devices and have been entrapped under closing doors that failed to reverse. However, in one case, an adult activated the opener and left the premises before the door completely closed. Our evaluation of 50 garage door openers showed that although 88% percent reversed when encountering a block of wood, 40% failed to reverse when coming down on a supine, child-sized cardiopulmonary resuscitation mannequin. CONCLUSIONS: Automatic garage door openers pose a serious risk of severe injury or death to children. It is probable that many doors would not reverse if they came down on a young child. Therefore, we have devised a way for homeowners to test their door openers that closely mimics our evaluations using the mannequin by using a large roll of paper towels. If the door fails to reverse using this test, we suggest that homeowners disconnect their openers and operate the doors manually until the openers are serviced or replace their automatic openers with one that meets the latest Underwriters Laboratory standards. We also have other recommendations regarding the safe operation of the doors, including improving the safety standards for openers in apartment complexes. Compliance with these recommendations should reduce the number of injuries to children caused by garage door openers.

Kluver-Bucy syndrome following heat stroke in a 12-year-old girl.

A 12-year-old girl developed Kluver-Bucy syndrome (KBS) following heat stroke. She demonstrated the features typical of human KBS, including visual agnosia, hypermetamorphosis, hypersexuality, language disorder with aphasia, hyperorality, placidity, flat affect, and memory dysfunction. Magnetic resonance imaging 11 1/2 months after onset disclosed mild, diffuse atrophy. Fourteen months after onset, she did not consistently respond to language or communicate verbally, and was dependent on others. A comparison is made with previously reported KBS cases in children.

Outcome of severe anoxic/ischemic brain injury in children.

The outcome of 25 children who had anoxic or ischemic brain injuries at 2 months to 14 years of age is reported. Follow-up was from 1 to 14 years after injury; causes were near-drowning, 11; suffocation, 7; cardiac arrest, 3; electrocution with cardiac arrest, 2; strangulation, 1; aborted sudden infant death syndrome, 1. All patients were unconscious for at least 24 hours. Of 11 remaining in vegetative states, 5 died. Seven children regained some language skills and are in special education or self-contained classrooms. Seven are profoundly impaired and show only a social smile. Cognitive and motor outcomes were correlated with the severity of injury as indicated by the duration of unconsciousness. All children who regained language skills or the ability to walk were unconscious less than 60 days. Dystonic rigidity was observed in all children who were nonambulatory. Outcome was also correlated with the cause of injury; mortality, cognitive outcome, feeding outcome, and duration of unconsciousness were all worse in children with near-drowning.

Spinal cord injury rehabilitation in a pediatric achondroplastic patient: case report.

Achondroplasia is an autosomal dominant condition that occurs in approximately 1 of 25,000 births. It has long been associated with neurologic morbidity and mortality in adults, but more recently it has been increasingly identified in children. Neurological sequelae of achondroplasia includes spinal stenosis, spinal cord compression at the foramen magnum (which can result in fatal acute craniocervical junction compression), hydrocephalus, radiculopathy, paresis, and abnormal spinal curvature. We report the case of a 12-year-old achondroplastic patient who incurred an apparently nontraumatic cervical spinal cord infarction, with resultant quadriplegia, with no apparent cause, which was complicated by impaired tolerance of temperature changes and hypercalcemia of immobilization. Whereas persons with achondroplasia have many of the same physical and functional impairments from spinal cord injury as other SCI patients, they are more likely to experience certain types of neurologic deficits and are more subject to other problems because of their altered body habitus.

Outcome of children with prolonged unconsciousness and vegetative states.

The outcomes of 60 children unconscious for 90 days or longer following acquired brain injury are reported. Eight children who died had remained in persistent vegetative states. As expected, most neurologic improvement occurred within the first year after injury, although some delayed improvements were observed. Outcomes were strongly correlated with causes of brain injury. Better cognitive and motor function was observed with nonanoxic injuries. No child in this report with anoxic brain injury regained functional cognitive or motor skills, although 3 became socially responsive. The remarkable contrast with adults following acquired brain injury is the significantly longer survival of children. The only children who died had remained in persistent vegetative states.

Movement disorders after status epilepticus and other brain injuries.

A retrospective medical record review was conducted of 173 consecutive children hospitalized for acquired brain injuries on a specialized pediatric rehabilitation service. The chart review identified children who developed movement disorders with acquired brain injuries: 8 with status epilepticus, 2 with trauma, and 1 with anoxia. Movement disorders were observed more frequently following status epilepticus (8 of 12) than following other causes of acquired brain injury (3 of 161; P = .0001). Four additional children had severe neurologic deficits following status epilepticus but did not develop movement disorders. The 11 patients who developed movement disorders had choreiform movements predominantly. Even though status epilepticus is a clinical phenomenon resulting from a variety of etiologies, the features of movement disorders in these children were strikingly similar. The pathophysiology of this complication is unknown.

Closed head injury: comparison of children younger and older than 6 years of age.

The outcomes of 97 children with severe closed head injuries referred to a regional rehabilitation center were studied. Patients were divided according to referral source and age (less than 6 and greater than or equal to 6 years). Patients referred from more distant sources had worse outcomes in terms of cognition, motor ability, and brain atrophy for both age groups. Children 6 years of age and older had better cognitive, motor, and brain atrophy outcomes than younger patients for each referral origin. These results do not support the hypothesis that the youngest children have the best recovery after profound closed head injury. All abused children were younger than 6 years of age; compared to other age-matched, closed head injury patients, these children had significantly worse cognitive and motor abilities.

Severe adolescent head injury: implications for transition into adult life.

This report describes the outcomes of 28 children who had severe head injuries between 13-18 years of age. All were unconscious at least 24 hours and have been followed at least 2 years after injury. At present, their ages range from 18 to 27 years. Their educational achievements, social activities, marital status, functional independence, and employment at follow-up are described.

Pediatric closed head injury: outcome following prolonged unconsciousness.

This report describes outcomes for a group of 26 children who remained unconscious longer than 90 days after traumatic brain injury. Twenty children regained consciousness; 11 are able to communicate. Five children can ambulate independently. In this series, children with the best recovery (IQ greater than or equal to 70) were predicted by minimal cerebral atrophy, demonstrated by computerized brain scan (CT scan) performed two months after injury (p = 0.001). In subjects over 12 years old, minimal CT atrophy predicted a good outcome with 89% accuracy.

Precocious puberty after traumatic brain injury.

After traumatic brain injuries in 33 prepubertal children, precocious puberty was observed in seven. Precocious puberty developed significantly more frequently in girls than in boys (54.5 versus 4.5%, P less than 0.01). Six children with precocious puberty were in coma for greater than or equal to 2 weeks. Follow-up computed tomography revealed cerebral atrophy or focal encephalomalacia in all children with and 69% of children without precocious puberty. There were no striking differences in incidence of motor or cognitive deficits or posttraumatic epilepsy in children with and without precocious puberty. In four of five children, basal sex steroid levels were elevated, and the response to luteinizing hormone releasing hormone stimulation revealed a pubertal pattern after the appearance of secondary sex characteristics.

Pediatric head injury resulting from all-terrain vehicle accidents.

In our experience, accidents involving all-terrain vehicles are an increasingly frequent cause of brain injury in children. The risk associated with operation of these vehicles is not fully appreciated and should be better publicized. We believe that it is possible to delineate several steps that could significantly reduce the risk to the pediatric population without curtailing the recreation altogether. Although construction design has promised to produce safer vehicles, our experience has shown that extreme injury is still possible with the newer four-wheel machine.