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Poster 319: Functional Outcomes in 4 Children With Transverse Myelitis: A Case Series
S140
PRESENTATIONS
progressive stenosis in those with spastic and dystonic CP, at no common cervical level, and at GMFCS levels I -III. Conclusions: New neurologic findings or changes in function in people with CP must be fully investigated and cannot be explained as progression of their CP. Even minor changes noted on scans can result in functional changes in those with already existing impairments in motor control.
Poster 318
Poster 316: Cancelled
Disclosures: S. Vadivelu, None. Patients or Programs: A 13-month-old boy with spastic quadriplegic cerebral palsy (CP) secondary to shaken baby syndrome (SBS) seen in the Physical Medicine and Rehabilitation (PM&R) clinic for developmental delays and spasticity management. Program Description: The patient had developmental delays, vision impairments, spastic quadriplegia, and dysphagia due to shaken baby syndrome. Botulinum toxin A and clonazepam were initiated to control spasticity and oral baclofen was added later. He received physical and occupational therapies. Multiple modified barium swallow studies (MBS) were performed revealing dysphagia requiring no oral intake. To further control spasticity, at age 2, he underwent an intrathecal baclofen (ITB) trial. His sitting balance improved significantly, and spasticity was reduced during the trial, but pump placement was delayed secondary to child custody issues. Two years later, the patient underwent ITB pump placement. He transferred to inpatient rehabilitation 1 day after pump implantation for dose adjustments and therapy. Setting: Outpatient/inpatient rehabilitation center. Results: Three days after pump placement, improvements in spasticity and dysphagia were evident. The patient was able to purposefully activate switches, make choices, and “give me 5.” He was also vocalizing, prompting a request for an MBS. The MBS showed improvements in swallowing; therefore, his diet was advanced from nothing by mouth to pureed solids and thin liquids. Functional Independence Measure (FIM) scores also reflected a change in social interaction, memory, and problem solving. FIM scores advanced from total assistance (1) to supervision (5)/modified independent (6) in these areas. Discussion: This case illustrates that ITB not only reduced spasticity, but also resulted in functional improvements in swallowing and in communication as measured by MBS and FIM scores. Conclusions: ITB can result in functional improvements. Further research is warranted to determine if ITB can help other CP patients with dysphagia.
Poster 317 Do OnabotulinumtoxinA Injections Affect Seizure Threshold in Children? A Report on 2 Cases. Kori Rothman, DO (University of Michigan, Ann Arbor, MI); Joseph E. Hornyak, MD, PhD; Edward A. Hurvitz, MD; Sucheta Joshi, MD. 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.
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.
Poster 319 Functional Outcomes in 4 Children With Transverse Myelitis: A Case Series. Zahava T. Traeger, MD (NYU Medical Center, New York, NY); Joan Gold; Eric T. Lee, MD, MA.
PM&R
Disclosures: Z. T. Traeger, None. Patients or Programs: 4 cases of pediatric transverse myelitis (TM). Program Description: Patient (Pt) #1: 12-year-old girl with TM at T10 to conus medullaris with sudden onset of bilateral lower extremity (BLE) weakness. She was moderate to maximal assist (max A) in activities of daily living (ADL) and unable to ambulate. Her course was complicated by urinary incontinence, deep vein thrombosis and acute worsening of TM. She improved to modified independence level (mod I) for ambulation and most ADL. Pt #2: 9-year-old boy (M) with progressive BLE weakness diagnosed with TM 4 days after a viral infection. His course was uncomplicated and he was bowel and bladder continent. He required minimal to maxA with ADL on admission and ambulated with circumduction. He improved to supervision for his ADL and ambulated with minimal assist. Pt #3: 13-year-old M with numbness and weakness in all 4 extremities diagnosed with TM at C1-C4 on magnetic resonance imaging (MRI) 18 days after his varicella and hepatitis A vaccines. He required max A for bed mobility (BM), was dependent in his ADL and unable to ambulate on admission. His course was complicated by restrictive pulmonary disease and neurogenic bowel and bladder retention. He is currently continuing inpatient rehabilitation. Pt #4: 13-year-old M diagnosed with TM at C3-T4 after 1 day of BLE weakness and urinary incontinence. He required max A in BM, transfers and ambulation. At discharge he was independent in BM, transfers, bowel and bladder management and mod I with ambulation. Setting: Pediatric acute rehabilitation unit. Results: TM pts vary greatly in their functional abilities pre- and post-rehabilitation. Discussion: TM is caused by spinal cord inflammation and has various etiologies and outcomes, with the most prevalent deficits in bowel and bladder functions, and ambulation difficulties. Improvements in MRI to localize lesions has led to advances in understanding the disease, the ability to provide anticipatory guidance, and the ability to differentiate it from multiple sclerosis and other postinflammatory conditions. Conclusions: Understanding TM, its causes, course, and complications allows the rehabilitation team to maximize functional gains by better targeting medical and rehabilitation management to overcome both current and future obstacles.
Poster 320 Hypothermic-Induced Axonal Peripheral Polyneuropathy After Drowning Episode: A Case Report. Talia R. Collier, MD (Children's Mercy Hospital, Kansas City, MO); Robert Rinaldi, MD. Disclosures: T. R. Collier, None. Patients or Programs: A 6-year-old girl with axonal peripheral polyneuropathy secondary to hypothermic drowning event.
Vol. 2, Iss. 9S, 2010
S141
Program Description: The patient was transferred to inpatient rehabilitation after a drowning episode of an estimated 30 minutes of submersion in an icy pond. Core temperature was noted to be 84°F at the scene. Resuscitation efforts were performed for 2 hours. Brain imaging at the outside facility was negative. Before transfer to the rehabilitation service, she made an excellent cognitive recovery and she was reported to be at her baseline. Her motor recovery was limited. Central cord syndrome was ruled out with negative spine imaging at outside facility. Upon admission to the rehabilitation service, she had weak proximal upper extremity strength with no wrist or finger movements. Her proximal lower extremities were stronger than her upper extremities and she had trace plantar flexion. She did not have active dorsiflexion or toe movement. Her extremities were areflexic and hypotonic bilaterally. She had allodynia to palpation of hands and feet, but otherwise gross tactile sensation was intact. She did not make significant strength gains after receiving intensive rehabilitation services. EMG/ NCS was performed and demonstrated axonal peripheral polyneuropathy. Setting: Inpatient rehabilitation service at pediatric hospital. Results: An EMG/NCS was performed on hospital day 7 of the rehabilitation service due to concerns for peripheral neuropathy due to her hypotonia, distal greater than proximal weakness and areflexia. EMG/NCS demonstrated no responses for nerve conduction studies. Needle examination demonstrated denervation potentials. The findings were consistent with axonal peripheral polyneuropathy. The patient will have repeated EMG/NCS in 3 months in which results will be discussed. Discussion: The patient’s axonal polyneuropathy is thought to be secondary to hypothermic-induced ischemia that occurred during the submersion episode. Hypothermicinduced polyneuropathy is a rare occurrence and there have been very few documented clinical cases to our knowledge. Conclusions: Prolonged hypothermic exposure causing hypothermic induced ischemia is the likely etiology of axonal peripheral polyneuropathy in this patient.
Poster 321 “I Forget To Use My Arm When I Dance”: TMS Evidence of Learned Non-Use in Birth Brachial Plexus Palsy: A Case Report. Tobias J. Tsai, MD (Cincinnati Children's Hosp Med Ctr, Cincinnati, OH); Donald L. Gilbert, MD, MS; Charles T. Mehlman, DO, MOH; Linda J. Michaud, MD. Disclosures: T. J. Tsai, None. Setting: Tertiary care pediatric hospital. Results: An 11-year-old girl with history of left birth brachial plexus palsy (BBPP) who had recently undergone partial subscapularis release and latissimus dorsi tendon transfer presented to brachial plexus clinic. Although more indepen-
PRESENTATIONS
progressive stenosis in those with spastic and dystonic CP, at no common cervical level, and at GMFCS levels I -III. Conclusions: New neurologic findings or changes in function in people with CP must be fully investigated and cannot be explained as progression of their CP. Even minor changes noted on scans can result in functional changes in those with already existing impairments in motor control.
Poster 318
Poster 316: Cancelled
Disclosures: S. Vadivelu, None. Patients or Programs: A 13-month-old boy with spastic quadriplegic cerebral palsy (CP) secondary to shaken baby syndrome (SBS) seen in the Physical Medicine and Rehabilitation (PM&R) clinic for developmental delays and spasticity management. Program Description: The patient had developmental delays, vision impairments, spastic quadriplegia, and dysphagia due to shaken baby syndrome. Botulinum toxin A and clonazepam were initiated to control spasticity and oral baclofen was added later. He received physical and occupational therapies. Multiple modified barium swallow studies (MBS) were performed revealing dysphagia requiring no oral intake. To further control spasticity, at age 2, he underwent an intrathecal baclofen (ITB) trial. His sitting balance improved significantly, and spasticity was reduced during the trial, but pump placement was delayed secondary to child custody issues. Two years later, the patient underwent ITB pump placement. He transferred to inpatient rehabilitation 1 day after pump implantation for dose adjustments and therapy. Setting: Outpatient/inpatient rehabilitation center. Results: Three days after pump placement, improvements in spasticity and dysphagia were evident. The patient was able to purposefully activate switches, make choices, and “give me 5.” He was also vocalizing, prompting a request for an MBS. The MBS showed improvements in swallowing; therefore, his diet was advanced from nothing by mouth to pureed solids and thin liquids. Functional Independence Measure (FIM) scores also reflected a change in social interaction, memory, and problem solving. FIM scores advanced from total assistance (1) to supervision (5)/modified independent (6) in these areas. Discussion: This case illustrates that ITB not only reduced spasticity, but also resulted in functional improvements in swallowing and in communication as measured by MBS and FIM scores. Conclusions: ITB can result in functional improvements. Further research is warranted to determine if ITB can help other CP patients with dysphagia.
Poster 317 Do OnabotulinumtoxinA Injections Affect Seizure Threshold in Children? A Report on 2 Cases. Kori Rothman, DO (University of Michigan, Ann Arbor, MI); Joseph E. Hornyak, MD, PhD; Edward A. Hurvitz, MD; Sucheta Joshi, MD. 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.
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.
Poster 319 Functional Outcomes in 4 Children With Transverse Myelitis: A Case Series. Zahava T. Traeger, MD (NYU Medical Center, New York, NY); Joan Gold; Eric T. Lee, MD, MA.
PM&R
Disclosures: Z. T. Traeger, None. Patients or Programs: 4 cases of pediatric transverse myelitis (TM). Program Description: Patient (Pt) #1: 12-year-old girl with TM at T10 to conus medullaris with sudden onset of bilateral lower extremity (BLE) weakness. She was moderate to maximal assist (max A) in activities of daily living (ADL) and unable to ambulate. Her course was complicated by urinary incontinence, deep vein thrombosis and acute worsening of TM. She improved to modified independence level (mod I) for ambulation and most ADL. Pt #2: 9-year-old boy (M) with progressive BLE weakness diagnosed with TM 4 days after a viral infection. His course was uncomplicated and he was bowel and bladder continent. He required minimal to maxA with ADL on admission and ambulated with circumduction. He improved to supervision for his ADL and ambulated with minimal assist. Pt #3: 13-year-old M with numbness and weakness in all 4 extremities diagnosed with TM at C1-C4 on magnetic resonance imaging (MRI) 18 days after his varicella and hepatitis A vaccines. He required max A for bed mobility (BM), was dependent in his ADL and unable to ambulate on admission. His course was complicated by restrictive pulmonary disease and neurogenic bowel and bladder retention. He is currently continuing inpatient rehabilitation. Pt #4: 13-year-old M diagnosed with TM at C3-T4 after 1 day of BLE weakness and urinary incontinence. He required max A in BM, transfers and ambulation. At discharge he was independent in BM, transfers, bowel and bladder management and mod I with ambulation. Setting: Pediatric acute rehabilitation unit. Results: TM pts vary greatly in their functional abilities pre- and post-rehabilitation. Discussion: TM is caused by spinal cord inflammation and has various etiologies and outcomes, with the most prevalent deficits in bowel and bladder functions, and ambulation difficulties. Improvements in MRI to localize lesions has led to advances in understanding the disease, the ability to provide anticipatory guidance, and the ability to differentiate it from multiple sclerosis and other postinflammatory conditions. Conclusions: Understanding TM, its causes, course, and complications allows the rehabilitation team to maximize functional gains by better targeting medical and rehabilitation management to overcome both current and future obstacles.
Poster 320 Hypothermic-Induced Axonal Peripheral Polyneuropathy After Drowning Episode: A Case Report. Talia R. Collier, MD (Children's Mercy Hospital, Kansas City, MO); Robert Rinaldi, MD. Disclosures: T. R. Collier, None. Patients or Programs: A 6-year-old girl with axonal peripheral polyneuropathy secondary to hypothermic drowning event.
Vol. 2, Iss. 9S, 2010
S141
Program Description: The patient was transferred to inpatient rehabilitation after a drowning episode of an estimated 30 minutes of submersion in an icy pond. Core temperature was noted to be 84°F at the scene. Resuscitation efforts were performed for 2 hours. Brain imaging at the outside facility was negative. Before transfer to the rehabilitation service, she made an excellent cognitive recovery and she was reported to be at her baseline. Her motor recovery was limited. Central cord syndrome was ruled out with negative spine imaging at outside facility. Upon admission to the rehabilitation service, she had weak proximal upper extremity strength with no wrist or finger movements. Her proximal lower extremities were stronger than her upper extremities and she had trace plantar flexion. She did not have active dorsiflexion or toe movement. Her extremities were areflexic and hypotonic bilaterally. She had allodynia to palpation of hands and feet, but otherwise gross tactile sensation was intact. She did not make significant strength gains after receiving intensive rehabilitation services. EMG/ NCS was performed and demonstrated axonal peripheral polyneuropathy. Setting: Inpatient rehabilitation service at pediatric hospital. Results: An EMG/NCS was performed on hospital day 7 of the rehabilitation service due to concerns for peripheral neuropathy due to her hypotonia, distal greater than proximal weakness and areflexia. EMG/NCS demonstrated no responses for nerve conduction studies. Needle examination demonstrated denervation potentials. The findings were consistent with axonal peripheral polyneuropathy. The patient will have repeated EMG/NCS in 3 months in which results will be discussed. Discussion: The patient’s axonal polyneuropathy is thought to be secondary to hypothermic-induced ischemia that occurred during the submersion episode. Hypothermicinduced polyneuropathy is a rare occurrence and there have been very few documented clinical cases to our knowledge. Conclusions: Prolonged hypothermic exposure causing hypothermic induced ischemia is the likely etiology of axonal peripheral polyneuropathy in this patient.
Poster 321 “I Forget To Use My Arm When I Dance”: TMS Evidence of Learned Non-Use in Birth Brachial Plexus Palsy: A Case Report. Tobias J. Tsai, MD (Cincinnati Children's Hosp Med Ctr, Cincinnati, OH); Donald L. Gilbert, MD, MS; Charles T. Mehlman, DO, MOH; Linda J. Michaud, MD. Disclosures: T. J. Tsai, None. Setting: Tertiary care pediatric hospital. Results: An 11-year-old girl with history of left birth brachial plexus palsy (BBPP) who had recently undergone partial subscapularis release and latissimus dorsi tendon transfer presented to brachial plexus clinic. Although more indepen-