Joan Gold

Nonoperative management of spasticity in children

Central to the longitudinal management of childhood hypertonia are nonsurgical treatments. These include physical and occupational therapy, electrical stimulation, orthotics, botulium toxin, and drugs. This manuscripts reviews these treatment modalities as well as evaluation tools available to assess their impact on a child’s hypertonia.

Selective functional rhizotomy for the treatment of spasticity in children

During the past three years interest in the use of selective functional rhizotomy to treat spasticity has increased, particularly in the United States. This is due largely to the positive experiences of surgeons employing various techniques of sensory rhizotomies during the 1960’s, 1970’s and early 1980’s in Europe and South Africa (Fasano et al. 1980, Fraioli and Guidetti 1977, Gros et al. 1973, Laitinen et al. 1983, Peacock et al. 1987, Privat et al. 1976, Sindou et al. 1974, 1987). To understand the rationale of this procedure, a summary of the current theories of the pathophysiology is helpful.

Rehabilitation of Strokes in Children

Recent literature has devoted a great deal of attention to the etiologies of cerebrovascular accidents in children, especially in light of newly identified causes such as Protein S and C deficiencies (Van Kuijck, Rotteveel, Osstrom & Novakova, 1994). However, literature which addresses the rehabilitation of these patients is infrequent and anecdotal. Most of the information cited is abstracted either from experience with adults or with the hemiplegic cerebral palsy population. Minimal information is available to guide clinicians or parents as to potential outcomes and/or the specific interventions that enhance progress. In view of these omissions from the literature, it is the aim of this article to: (1) describe the deficits associated with childhood strokes; (2) address the unique medical conditions that make their rehabilitation challenging; and (3) discuss the treatment options available as well as how these need to be modified according to the child’s ongoing physical, cognitive and emotional development.

Atypical Variant of Guillain-Barre Syndrome in a Pediatric Patient: A Case Report

Oxygen consumption in walking test pre and post hamstring lengthening. Results or Clinical Course: 11.5 percent of our population presented with knee hyperextension after hamstring lengthening. Regression analysis showed that the main predictor of hyperextension after hamstring lengthening is knee flexion at midstance (mean of 23.9 degrees). Analysis of oxygen consumption data did not reveal any significant changes in children with hyperextended knees vs. nonhyperextended knees. Conclusions: Incidence of knee hyperextension after hamstring lengthening in our population is 11.5 percent. Pre operative knee flexion at midstance was correlated with the presence of knee hyperextension post surgery. Interestingly, there was no significant change in gait efficiency as measured by oxygen consumption in hyperextended knees vs non-hyper extended knees.

Adolescent Spinal Stenosis as a Sign of Diabetes Mellitus: A Case Report

Conclusions: Although multiple studies have evaluated longterm outcomes after SDR, this is the first study to our knowledge using controls matched for diagnosis, age, and gait analysis criteria. In long-term follow up, validated self-report measures and objective data demonstrated clinically significant and superior gains in patients who underwent SDR. Although self-reported quality of life did not differ between the groups, the non-SDR group had higher self-reported pain interfering with their daily life activities.

Poster 102 Lumbosacral Plexopathy After Gunshot Wounds: A Case Report

In addition, a nonsystemic vasculitis in childhood is even rarer. Conclusions: Asymmetric sensory or motor symptoms that develop in previously healthy children may suggest an acute vasculitis. Electrodiagnostic evaluation can determine the extent and nature of the possible neuropathic process. Sural nerve biopsy can identify vasculitic disorders. Prompt initiation of immunosuppressive therapy with a rehabilitation program offers the best opportunity of functional recovery.

Poster 399 The Effects of Extracorporeal Membrane Oxygenation in Pediatric Rehabilitation: A Case Series

Disclosures: A. Myers, none. Program Description: A 16-year-old girl presented with generalized weakness of all extremities and superimposed plegia of the right lower extremity. She developed an acute febrile illness, which prompted admission to a local hospital. She had an abrupt onset of cardiovascular collapse, was diagnosed with Coxsackie myocarditis with subsequent heart failure and was placed on extracorporeal membrane oxygenation (ECMO) for 16 days. Her course was remarkable for infections, secondary renal failure that required hemodialysis, and retroperitoneal bleed with right lumbar plexopathy. A 7-year-old girl presented with abdominal pain, emesis, and fever. She was admitted to the pediatric intensive care unit, tested positive for H1N1 influenza, was started on oseltamivir and received IVIG therapy. She became hypotensive and tachycardic. ECHO revealed poor biventricular function. She was diagnosed with myocarditis, which required pericardiocentesis and was placed on ECMO for 6 days, with subsequent left leg compartment syndrome and underwent 2 fasciotomies. An 11-year-old boy presented with gastroenteritis, fever, hypotension, after 2 cardiac arrests and resuscitations. He was diagnosed with myocarditis, which required ECMO for 4 days. While intubated, the patient had increased tone, spasticity, and weakness. Magnetic resonance imaging showed multiple bilateral infarctions. Setting: A tertiary care hospital. Results: ECMO was initially used in the pediatric patient in neonates with severe respiratory distress syndrome but expanded to the older population with cardiac dysfunction. Access and bleeding issues, however, can result in these medically frail children with obvious and more occult medical problems for which they need to be screened, especially upon admission to a rehabilitation unit to prevent additional morbidity. This strategy should be associated with fever complications and reduced length of stay in the rehabilitation with improved quality of care. Discussion: The children in this case series had ECMO-associated events with functional deficits that were addressed and improved with therapy. It is important for physicians to be aware of these events to better help these patients improve their function.

Poster 319: Functional Outcomes in 4 Children With Transverse Myelitis: A Case Series

Disclosures: K. Rothman, None. Patients or Programs: Patient 1, a 35-month-old girl, former 30.5-week-old preemie, with spastic diplegic cerebral palsy. Patient 2, a 25-month-old girl with congenital brain malformation and left spastic hemiparesis. Program Description: Both children underwent OnabotulinumtoxinA (obtx-A) motor point blocks of gastrocnemii muscles for management of spasticity. Patient 1 had bilateral gastrocnemii injected, whereas patient 2 just the left. Both subjects received a total of 50 units of obtx-A. Patient 1 weighed 10.8 kg, and patient 2 weighed 12.2 kg. Within 24 hours, patient 1 developed new onset status epilepticus. Patient 2 had a seizure 4 days after the injection. Setting: University tertiary care pediatric hospital. Results: Both patients were placed on long-term antiepileptic drugs. Discussion: Obtx-A is commonly used to treat spasticity in children with cerebral palsy. It is generally thought to have a good side-effect profile, though the FDA has recently required a Black Box warning. There is evidence that obtx-A may have central side effects. Research suggests that Obtx-A may affect spinal cord circuitry, the brainstem, and the motor cortex. There are several proposed mechanisms by which these central circuits are affected, including blocking gamma motor endings and reducing spindle afferent input from the treated muscle, plastic changes at both the level of the motor neuron and muscle, and retrograde transport and transcytosis. Central nervous system disruptions may potentially lower seizure threshold, especially in children with underlying risk factors for epilepsy. Seizures have been reported after obtx-A injections, however, this has been poorly documented. The above 2 cases developed seizures shortly after their injections. This raises concern that obtx-A may increase risk of seizures in children receiving obtx-A injections. Conclusions: Obtx-A motor point blocks may increase risk of seizure in children at risk for seizures. Poster 318 Functional Improvement in Swallowing and Social Cognition After Intrathecal Baclofen Pump Placement in a 4-Year-Old Boy With Quadriplegic Cerebral Palsy Secondary to Shaken Baby Syndrome: A Case Report. Sathya Vadivelu, DO (University of Missouri-Columbia, Columbia, MO); Jane A. Emerson, MD.

Abstract 10: Standing induced via segmental reflex pathways of persons with spinal paralysis: a case series.

Abstract Setting: Urban hospital. Patients: 12 persons with paralysis of spinal origin. Case Description: Reflex standing is induced by means of whole body vibration in persons with paralysis of spinal origin. A case series of persons who had paralysis due to spinal infection, paraplegia after traumatic injury at the thoracic and lumbar levels, or quadriplegia after cervical trauma. Subjects were 1 year postonset, had stable skeletal systems, and tone in the thighs. Subjects reflexively stood on a vibratory device as muscles contracted and relaxed in response to segmental reflex loops triggered by vibration delivered at amplitudes ranging from 0 to 5.1mm, and frequencies ranging from 0 to 28Hz. Assessment/Results: Standing time with and without vibratory stimulaton; degree of volitional movement in trunk and limbs; and carry-over to voluntary standing and walking. Subjects were able to stand for periods (up to 30min) with vibration and for brief periods (1–3min) after vibration ceased. Some patients recovered the ability to stand and to ambulate. Discussion: Long periods of immobility and loss of sensation can produce skin breakdown with fatal consequences if left untreated. A sedentary life style resulting from a loss of voluntary movement and sensation may aggravate existing dysfunction of the bowel and bladder and lead to constipation and urinary infection, as well as increased vulnerability to pneumonia and respiratory infection. Spinal cord injury increases the vulnerability to chronic conditions such as cardiovascular disease, diabetes, and osteoporosis. Fitness has been improved through treadmill-induced reflex walking in humans for 2 decades. Likewise, the act of standing, be it for brief periods, benefits patients physically and provides a source of emotional, psychologic, and social satisfaction. Conclusion: Vibratory-induced standing represents a promising modality for use in the rehabilitation of persons with motor dysfunction of spinal origin.

Spinal cord injuryAbstract 10: Standing induced via segmental reflex pathways of persons with spinal paralysis: a case series.1

Abstract Setting: Urban hospital. Patients: 12 persons with paralysis of spinal origin. Case Description: Reflex standing is induced by means of whole body vibration in persons with paralysis of spinal origin. A case series of persons who had paralysis due to spinal infection, paraplegia after traumatic injury at the thoracic and lumbar levels, or quadriplegia after cervical trauma. Subjects were 1 year postonset, had stable skeletal systems, and tone in the thighs. Subjects reflexively stood on a vibratory device as muscles contracted and relaxed in response to segmental reflex loops triggered by vibration delivered at amplitudes ranging from 0 to 5.1mm, and frequencies ranging from 0 to 28Hz. Assessment/Results: Standing time with and without vibratory stimulaton; degree of volitional movement in trunk and limbs; and carry-over to voluntary standing and walking. Subjects were able to stand for periods (up to 30min) with vibration and for brief periods (1–3min) after vibration ceased. Some patients recovered the ability to stand and to ambulate. Discussion: Long periods of immobility and loss of sensation can produce skin breakdown with fatal consequences if left untreated. A sedentary life style resulting from a loss of voluntary movement and sensation may aggravate existing dysfunction of the bowel and bladder and lead to constipation and urinary infection, as well as increased vulnerability to pneumonia and respiratory infection. Spinal cord injury increases the vulnerability to chronic conditions such as cardiovascular disease, diabetes, and osteoporosis. Fitness has been improved through treadmill-induced reflex walking in humans for 2 decades. Likewise, the act of standing, be it for brief periods, benefits patients physically and provides a source of emotional, psychologic, and social satisfaction. Conclusion: Vibratory-induced standing represents a promising modality for use in the rehabilitation of persons with motor dysfunction of spinal origin.