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Poster 231: Botulinum Toxin Type A for Tongue Protrusion and Extrusion Disorder: A Case Report
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ACADEMY ANNUAL ASSEMBLY ABSTRACTS
Poster 230 Prolonged, Severe Intrathecal Baclofen Withdrawal Syndrome: A Case Report. Colby R. Hansen, MD (University of Utah Health Sciences Ctr, Salt Lake City, UT); Judith L. Gooch, MD; Teresa Such-Neibar, DO. Disclosure: C.R. Hansen, None; J.L. Gooch, None; T. Such-Neibar, None. Setting: Tertiary care children’s hospital. Patient: An 11-year-old girl with spastic quadriplegic cerebral palsy managed with a baclofen pump. Case Description: The patient developed an infected pump and subsequent meningitis, prompting the removal of her pump and catheter. She subsequently developed a severe, prolonged baclofen withdrawal syndrome marked by increased spasticity, agitation, hypertension, and tachycardia that lasted nearly 2 months, requiring intensive care unit care and continuous intravenous sedation with benzodiazepines and opiates. Assessment/Results: Her pump was eventually replaced on hospital day 56 and within 24 hours her symptoms dramatically improved. She was eventually weaned off sedating medications and returned to baseline functional status. Discussion: Intrathecal baclofen (ITB) withdrawal is a well-recognized complication when drug delivery is disrupted for any reason. ITB withdrawal varies widely in its severity and poses the very real possibility of death if not promptly managed. Cases of withdrawal lasting greater than 1 or 2 weeks, however, are sparse. This case represents the first pediatric case, to our knowledge, of a prolonged, severe withdrawal from ITB. In addition, the literature to date has not discussed the potential role for opiates in managing baclofen withdrawal, yet a growing body of literature is examining the interplay between opiates and GABA B pathways. Conclusions: ITB withdrawal can persist well beyond the usual expected course of 1 to 2 weeks. Opiates may have a potential role in managing severe baclofen withdrawal. Reinstitution of ITB should be considered the definitive therapy for ITB withdrawal. Key Words: Baclofen; Cerebral palsy; Rehabilitation; Substance withdrawal syndrome. Poster 231 Botulinum Toxin Type A for Tongue Protrusion and Extrusion Disorder: A Case Report. Satish Mahajan, MD; Eugenio Monasterio, MD; Jenny Andrus, MD (Virginia Commonwealth University, Richmond, VA); Colleen Wunderlich, MD, MSc. Disclosure: S. Mahajan, None; E. Monasterio, None; J. Andrus, None; C. Wunderlich, None. Setting: Pediatric long-term care facility. Patient: A 17-year-old woman with spastic quadriplegic cerebral palsy (CP). Case Description: Patient had severe oral ulceration at the tongue base with secondary dysplastic overgrowth consistent with Riga-Fede disease. The lesion was secondary to frequent tongue protrusion and extrusion. Treatment required both dental and otolaryngology surgical interventions and prolonged course of medication. Botulinum toxin injection was performed to decrease aberrant tongue movements. 200U of botulinum toxin type A (BTX-A) was injected into the genioglossus muscle in 2 sites (100U each), using a bilateral paramedian submandibular approach. Assessment/Results: Nursing and medical staff noted a 50% decrease in the aberrant tongue movements, with less protrusion and extrusion of the tongue. Effect lasted approximately 3 months. The ulcer has not returned to date. The patient has been more comfortable and tolerant of oral care. Discussion: BTX-A has been used successfully in a number of head and neck conditions, including cervical, spasmodic, oromandibular, and lingual dystonias, as well as bruxism, blepharospasm, hemifacial spasm, and sialorrhea. However, review of the literature shows only several case reports and a small series of patients with tongue protrusion dystonia secondary to Meige’s syndrome, oromandibuArch Phys Med Rehabil Vol 88, September 2007
lar, or tardive dyskinesia that have been treated successfully with botulinum toxin injection into the genioglossus. No one to date has reportedly used BTX-A to decrease tongue protrusion and extrusion in children with CP. Conclusions: The use of BTX-A to control tongue protrusion and extrusion disorders in CP may be warranted to prevent secondary complications such as Riga-Fede disease. Key Words: Botulinum toxins; Cerebral palsy; Oral ulcer; Rehabilitation. Poster 232 Improvement of Pediatric Nonalcoholic Fatty Liver Disease by Diet and Physical Exercise: A Case Report. Osamu Ito, MD, PhD (Tohoku University School of Medicine, Sendai, Japan); Yoshikazu Muroya, MD; Nobuyoshi Mori, MD; Makoto Nagasaka, MD, PhD; Masayuki Kanazawa, MD, PhD; Masahiro Kohzuki, MD, PhD. Disclosure: O. Ito, None; Y. Muroya, None; N. Mori, None; M. Nagasaka, None; M. Kanazawa, None; M. Kohzuki, None. Setting: University hospital. Patient: A 9-year-old boy with obesity and hypertransaminasemia. Case Description: On admission, his height, body weight, and body mass index (BMI) were 149cm, 93kg, and 41.9kg/m2, respectively. Blood pressure was normal. Serum alanine and aspartate transaminases and lactate dehydrogenase were 144, 244, and 370IU/L, respectively. Levels of serum triglyceride and low- and high-density lipoprotein cholesterols were in the normative range. Oral glucose tolerance test showed normal glucose tolerance. The value of the homeostatic model assessment was 3.8, which indicated insulin resistance. Plain computer tomography (CT) showed thick subcutaneous fat, low CT densities of the liver, and normal sizes of visceral fat and pancreas. Assessment/Results: He was admitted for 40 days to undergo a diet of 1900kcal and physical exercise with bicycle ergometer (at 15W for 20min/d, 4d/wk) and walking (10,000 steps/d, 7d/wk), and he followed the diet and physical exercise at home for 12 months. After 12 months, BMI decreased to 35kg/m2, and levels of serum liver enzymes normalized, as did CT densities of the liver. Discussion: Nonalcoholic fatty liver disease (NAFLD) is a series of diseases from asymptomatic steatosis with or without elevated transaminase to cirrhosis with complications of liver failure and hepatocellular carcinoma. Paralleling the increasing prevalence of obesity, NAFLD has become common and potentially serious even in children. There are few reports of the effect of therapeutic intervention on pediatric NAFLD. Conclusions: Therapeutic intervention with diet and physical exercise has beneficial effects in pediatric NAFLD. Key Words: Exercise, physical; Fatty liver; Obesity; Rehabilitation. Poster 233 Orthoses and Conditioning in a Patient With Pompe’s Disease: A Case Report. Lainie Holman, MD (Cincinnati Children’s Hospital Medical Ctr, Yellow Springs, OH); Linda Michaud, MD, PT; Diane E. Von Stein, MD; Nancy Leslie, MD; Deborah Guebard, PT. Disclosure: L. Holman, None; L. Michaud, None; D.E. Von Stein, None; N. Leslie, None; D. Guebard, None. Setting: Pediatric tertiary care hospital. Patient: A 5-year-old girl with late infant. Pompe’s disease (glycogen storage disease type II). Case Description: This child presented at age 4 months with hypotonia and cardiomegaly. Deficiency of alpha-glucosidase in muscle confirmed a diagnosis of Pompe’s disease. At age 3, her cardiomyopathy and ventilation were stable and she could scoot on her bottom but could not ambulate. Enzyme replacement therapy
ACADEMY ANNUAL ASSEMBLY ABSTRACTS
Poster 230 Prolonged, Severe Intrathecal Baclofen Withdrawal Syndrome: A Case Report. Colby R. Hansen, MD (University of Utah Health Sciences Ctr, Salt Lake City, UT); Judith L. Gooch, MD; Teresa Such-Neibar, DO. Disclosure: C.R. Hansen, None; J.L. Gooch, None; T. Such-Neibar, None. Setting: Tertiary care children’s hospital. Patient: An 11-year-old girl with spastic quadriplegic cerebral palsy managed with a baclofen pump. Case Description: The patient developed an infected pump and subsequent meningitis, prompting the removal of her pump and catheter. She subsequently developed a severe, prolonged baclofen withdrawal syndrome marked by increased spasticity, agitation, hypertension, and tachycardia that lasted nearly 2 months, requiring intensive care unit care and continuous intravenous sedation with benzodiazepines and opiates. Assessment/Results: Her pump was eventually replaced on hospital day 56 and within 24 hours her symptoms dramatically improved. She was eventually weaned off sedating medications and returned to baseline functional status. Discussion: Intrathecal baclofen (ITB) withdrawal is a well-recognized complication when drug delivery is disrupted for any reason. ITB withdrawal varies widely in its severity and poses the very real possibility of death if not promptly managed. Cases of withdrawal lasting greater than 1 or 2 weeks, however, are sparse. This case represents the first pediatric case, to our knowledge, of a prolonged, severe withdrawal from ITB. In addition, the literature to date has not discussed the potential role for opiates in managing baclofen withdrawal, yet a growing body of literature is examining the interplay between opiates and GABA B pathways. Conclusions: ITB withdrawal can persist well beyond the usual expected course of 1 to 2 weeks. Opiates may have a potential role in managing severe baclofen withdrawal. Reinstitution of ITB should be considered the definitive therapy for ITB withdrawal. Key Words: Baclofen; Cerebral palsy; Rehabilitation; Substance withdrawal syndrome. Poster 231 Botulinum Toxin Type A for Tongue Protrusion and Extrusion Disorder: A Case Report. Satish Mahajan, MD; Eugenio Monasterio, MD; Jenny Andrus, MD (Virginia Commonwealth University, Richmond, VA); Colleen Wunderlich, MD, MSc. Disclosure: S. Mahajan, None; E. Monasterio, None; J. Andrus, None; C. Wunderlich, None. Setting: Pediatric long-term care facility. Patient: A 17-year-old woman with spastic quadriplegic cerebral palsy (CP). Case Description: Patient had severe oral ulceration at the tongue base with secondary dysplastic overgrowth consistent with Riga-Fede disease. The lesion was secondary to frequent tongue protrusion and extrusion. Treatment required both dental and otolaryngology surgical interventions and prolonged course of medication. Botulinum toxin injection was performed to decrease aberrant tongue movements. 200U of botulinum toxin type A (BTX-A) was injected into the genioglossus muscle in 2 sites (100U each), using a bilateral paramedian submandibular approach. Assessment/Results: Nursing and medical staff noted a 50% decrease in the aberrant tongue movements, with less protrusion and extrusion of the tongue. Effect lasted approximately 3 months. The ulcer has not returned to date. The patient has been more comfortable and tolerant of oral care. Discussion: BTX-A has been used successfully in a number of head and neck conditions, including cervical, spasmodic, oromandibular, and lingual dystonias, as well as bruxism, blepharospasm, hemifacial spasm, and sialorrhea. However, review of the literature shows only several case reports and a small series of patients with tongue protrusion dystonia secondary to Meige’s syndrome, oromandibuArch Phys Med Rehabil Vol 88, September 2007
lar, or tardive dyskinesia that have been treated successfully with botulinum toxin injection into the genioglossus. No one to date has reportedly used BTX-A to decrease tongue protrusion and extrusion in children with CP. Conclusions: The use of BTX-A to control tongue protrusion and extrusion disorders in CP may be warranted to prevent secondary complications such as Riga-Fede disease. Key Words: Botulinum toxins; Cerebral palsy; Oral ulcer; Rehabilitation. Poster 232 Improvement of Pediatric Nonalcoholic Fatty Liver Disease by Diet and Physical Exercise: A Case Report. Osamu Ito, MD, PhD (Tohoku University School of Medicine, Sendai, Japan); Yoshikazu Muroya, MD; Nobuyoshi Mori, MD; Makoto Nagasaka, MD, PhD; Masayuki Kanazawa, MD, PhD; Masahiro Kohzuki, MD, PhD. Disclosure: O. Ito, None; Y. Muroya, None; N. Mori, None; M. Nagasaka, None; M. Kanazawa, None; M. Kohzuki, None. Setting: University hospital. Patient: A 9-year-old boy with obesity and hypertransaminasemia. Case Description: On admission, his height, body weight, and body mass index (BMI) were 149cm, 93kg, and 41.9kg/m2, respectively. Blood pressure was normal. Serum alanine and aspartate transaminases and lactate dehydrogenase were 144, 244, and 370IU/L, respectively. Levels of serum triglyceride and low- and high-density lipoprotein cholesterols were in the normative range. Oral glucose tolerance test showed normal glucose tolerance. The value of the homeostatic model assessment was 3.8, which indicated insulin resistance. Plain computer tomography (CT) showed thick subcutaneous fat, low CT densities of the liver, and normal sizes of visceral fat and pancreas. Assessment/Results: He was admitted for 40 days to undergo a diet of 1900kcal and physical exercise with bicycle ergometer (at 15W for 20min/d, 4d/wk) and walking (10,000 steps/d, 7d/wk), and he followed the diet and physical exercise at home for 12 months. After 12 months, BMI decreased to 35kg/m2, and levels of serum liver enzymes normalized, as did CT densities of the liver. Discussion: Nonalcoholic fatty liver disease (NAFLD) is a series of diseases from asymptomatic steatosis with or without elevated transaminase to cirrhosis with complications of liver failure and hepatocellular carcinoma. Paralleling the increasing prevalence of obesity, NAFLD has become common and potentially serious even in children. There are few reports of the effect of therapeutic intervention on pediatric NAFLD. Conclusions: Therapeutic intervention with diet and physical exercise has beneficial effects in pediatric NAFLD. Key Words: Exercise, physical; Fatty liver; Obesity; Rehabilitation. Poster 233 Orthoses and Conditioning in a Patient With Pompe’s Disease: A Case Report. Lainie Holman, MD (Cincinnati Children’s Hospital Medical Ctr, Yellow Springs, OH); Linda Michaud, MD, PT; Diane E. Von Stein, MD; Nancy Leslie, MD; Deborah Guebard, PT. Disclosure: L. Holman, None; L. Michaud, None; D.E. Von Stein, None; N. Leslie, None; D. Guebard, None. Setting: Pediatric tertiary care hospital. Patient: A 5-year-old girl with late infant. Pompe’s disease (glycogen storage disease type II). Case Description: This child presented at age 4 months with hypotonia and cardiomegaly. Deficiency of alpha-glucosidase in muscle confirmed a diagnosis of Pompe’s disease. At age 3, her cardiomyopathy and ventilation were stable and she could scoot on her bottom but could not ambulate. Enzyme replacement therapy