- Email: [email protected]
Use of Intrathecal Baclofen in a Child With Spastic Paraparesis Related to Human Immunodeficiency Virus Infection: A Case Report
1001
CLINICAL NOTE
Use of Intrathecal Baclofen in a Child With Spastic Paraparesis Related to Human Immunodeficiency Virus Infection: A Case Report Kat Kolaski, MD ABSTRACT. Kolaski K. Use of intrathecal baclofen in a child with spastic paraparesis related to human immunodeficiency virus infection: a case report. Arch Phys Med Rehabil 2006;87:1001-3. This case report describes a 2-year treatment course of a 6-year-old with spastic paraparesis due to human immunodeficiency virus (HIV) encephalopathy. She continued to develop worsening musculoskeletal problems and a decline in mobility despite aggressive antispasticity interventions. In addition to meeting the usual clinical criteria for treatment with continuous intrathecal baclofen (ITB), she was believed to be an appropriate candidate for ITB because of adequate treatment of her HIV infection and associated overall low risk of mortality. For over 12 months, she has been successfully treated with ITB and has not developed any complications related to her HIV infection. To my knowledge, this is the first reported successful treatment of spasticity with ITB in a patient with stable HIV infection. Key Words: Baclofen; Case report; HIV; Muscle spasticity; Pediatrics; Rehabilitation. © 2006 by the American Congress of Rehabilitation Medicine and the American Academy of Physical Medicine and Rehabilitation INCE THE BEGINNING OF the acquired immunodeficiency syndrome (AIDS) epidemic in the mid 1980s, the S number of people living with human immunodeficiency virus
(HIV) infection has been steadily increasing.1 Fortunately, the rate of AIDS infection in children in the United States has decreased over the last decade and is currently less than 1%.1 This is related to public health initiatives for the identification and early treatment of HIV-infected mothers and their infants.2 However, when these preventative measures fail, HIV encephalopathy occurs as the fifth most common AIDS-defining condition in children. It may be the initial or an early presenting symptom, a clinical characteristic that is not seen in adults.3 In infants with perinatally acquired HIV infection, risk factors for encephalopathy include early age of infection, high viral loads, advanced maternal disease, and maternal encephalopathy. The neurologic presentation of HIV encephalopathy in children is variable, with characteristic features of microcephaly or
From the Department of Orthopaedic Surgery, Wake Forest University School of Medicine, Winston-Salem, NC. No commercial party having a direct financial interest in the results of the research supporting this article has or will confer a benefit upon the authors or upon any organization with which the authors are associated. The Department of Orthopaedic Surgery of Wake Forest University School of Medicine receives research funding from Medtronic, but the author is not supported by these research funds. Reprint requests to Kat Kolaski, MD, Dept of Orthopaedic Surgery, Wake Forest University School of Medicine, Medical Center Blvd, Winston-Salem, NC 27157, e-mail: [email protected]. 0003-9993/06/8707-10581$32.00/0 doi:10.1016/j.apmr.2006.03.015
brain atrophy, motor abnormalities primarily affecting lowerextremity function, spasticity, and cognitive delays or mental retardation.3 Spastic paraparesis, quadriparesis, and hemiparesis have been described in case series of children with perinatally acquired HIV infection.4,5 Aggressive antiretroviral treatment may prevent progression of the encephalopathic process.3 There is no available literature reporting specifically on the treatment of the motor abnormalities related to HIV encephalopathy in children. However, the etiology and clinical presentation of HIV encephalopathy are consistent with the classic definition of cerebral palsy (CP)6; thus, it is likely that children with these diagnoses are evaluated and treated similarly. This is illustrated in the following case report of a child with spastic paraparesis related to HIV encephalopathy. CASE DESCRIPTION The patient was born at 42 weeks gestation via cesarean section because of breech presentation with a birth weight of 2900g (6lb 7oz) to an HIV-infected mother who received zidovudine (AZT) during pregnancy. The immediate postnatal period was uncomplicated, and the infant was given AZT. HIV infection, with a high viral load and low CD4 count, was diagnosed in the patient 10 weeks after birth. A combination antiretroviral regimen was initiated. Around 1 year of age, developmental delay and lower-extremity spasticity were documented. The patient underwent magnetic resonance imaging of the brain that showed diffuse atrophy for age, confirming a diagnosis of HIV encephalopathy. During the first few years of life, the patient’s HIV infection was not optimally controlled, suspected as a result of poor compliance with medications. Social services became involved with the family, and after age 3 years, the patient had good HIV-infection control and no progression of her neurologic symptoms. The patient’s motor delays were treated with physical therapy. At 4 to 5 years of age, the patient’s spasticity was treated with oral baclofen and lower-extremity botulinum toxin injections with uncertain results. A dorsal rhizotomy was planned, but the patient was placed in foster care and relocated, necessitating a change in her health care providers. The patient presented for an initial evaluation by the author in a pediatric orthopedic clinic at the age of 6 years. Her medications included a combination antiretroviral regimen and 5mg of oral baclofen twice daily. In terms of gross motor function, the patient could sit independently and pull to stand, but she crawled as her primary means of mobility. Her foster parent reported difficulty with dressing and hygiene because of lower-extremity muscle tightness. On examination, she was cognitively intact for her age and had no abnormal findings in the upper extremities. In the lower extremities, weakness and spasticity were significant, with Ashworth Scale scores7 of 4 in the hip adductors, hip flexors and extensors, knee extensors, and ankle plantarflexors. In addition, the patient had contractures of the hip adductors, hamstrings, and ankle plantarflexors. Initial treatment included an increase of the oral baclofen dose to 10mg thrice daily. Although this dose was well tolerArch Phys Med Rehabil Vol 87, July 2006
1002
BACLOFEN FOR HIV-RELATED SPASTICITY, Kolaski
ated, the medication had an equivocal effect on spasticity. In an attempt to facilitate the fitting of lower-extremity orthotics to allow standing, the patient underwent botulinum toxin type A (BTX-A) injections to the bilateral gastrocnemius-soleus muscles (14.3U/kg of body weight) followed by serial casting of the ankles. She was fitted with knee-ankle-foot orthotics but had difficulty tolerating them because of her hip and knee spasticity and contractures. Because of the failure of these interventions and the clinical severity of her spasticity, she was considered to be an appropriate candidate for intrathecal baclofen (ITB).8 Rhizotomy was not recommended because of significant underlying lower-extremity weakness.9 The patient underwent a trial of ITB with a dose of 50g, resulting in an average change in lower-extremity Ashworth Scale scores of 2.4 points. Although the patient’s response to the ITB trial bolus dose was positive, pump implantation was postponed because of 2 main issues: (1) the patient was being treated by an infectious disease specialist at another institution and (2) she was in the legal custody of the local department of social services. Although her foster family could provide excellent psychosocial support, long-term placement issues were unclear. In addition, social service representatives raised concerns about the lack of experience with ITB in patients with HIV infection. Subsequently, the patient was treated with ongoing physical therapy and the following: (1) multiple sessions of chemical denervation procedures including phenol neurolysis of the bilateral obturator nerves, ethanol motor point blocks of the bilateral hamstrings and gastrocnemius muscles, and BTX-A injection (20U/kg of body weight) of the iliopsoas and hamstring muscles; (2) serial casting of the ankles and knees; and (3) a gradual increase of the oral baclofen dose to 30mg thrice daily. With these treatments, the patient progressed to ambulating in the house and classroom using custom-molded polypropylene ankle-foot orthoses (AFOs) and a posterior rolling walker. However, the patient’s improved mobility was short-lived. She developed progression of equinovalgus deformities necessitating the discontinuation of her AFOs because of heel and midfoot skin breakdown. She began to experience poor endurance for ambulation, likely due to the combined effects of ambulation without orthotics, high energy costs of her preferred swing-through gait pattern, and a growth spurt. ITB was reconsidered based on the following: (1) her medical care had been transferred to a local pediatric infectious disease specialist; and (2) the patient was in the process of being adopted by her foster parents. The patient underwent uncomplicated ITB pumpa placement with catheter placement at the T9 level. Over the first 90 days after pump implantation, her dose was titrated to 400g/d on a simple, continuous program and resulted in significantly decreased lower-extremity spasticity. The patient also experienced some decreased ability to stand, likely due to both her underlying weakness and decreased ability to recruit her spasticity. Four months postimplant, the patient presented with acute ITB withdrawal symptoms including anxiety, lower-extremity pruritis, and a dramatic increase in lower-extremity spasticity. Plain spinal radiographs showed a distal catheter disconnection that was found at surgery to be at the lumbar fascia; she underwent prompt catheter revision, thus restoring ITB infusion and spasticity control. Over the next several months, her ITB dose was further adjusted and at 250g/d, an optimal balance between spasticity reduction and functional tone was achieved. Arch Phys Med Rehabil Vol 87, July 2006
Because of persistent equinovalgus deformities, the patient was fitted for double upright metal AFOs with valgus-preventing T straps. These orthotics were chosen to provide stability of the ankle without skin breakdown. Using these orthotics, the patient progressed to ambulating short community distances with a posterior rolling walker. She primarily used a reciprocating gait pattern but tended to revert to a swing-through gait pattern at higher cadences. Over 1 year postimplant, the patient’s spasticity continues to be controlled with a maintenance ITB dose of 250g/d. She has not experienced any other ITB-related complications, and her HIV infection remains in good control on antiretroviral therapy. There has been no radiographic evidence of progression of hip subluxation or development of neuromuscular scoliosis. To address her lower-extremity contractures, the patient underwent mid-third gastrocnemius lengthenings and bilateral adductor tenotomies. This surgery enabled her to tolerate polypropylene AFOs. She is currently ambulating community distances with forearm crutches. The need for further ITB dose adjustments—as well as possible further orthopedic surgical intervention—is anticipated. DISCUSSION This case report illustrates the similarity in the presentation and treatment of a child with neurologic sequelae of HIV infection compared with children with CP. It also illustrates the fact that for potentially ambulatory pediatric patients, adequate control of spasticity and achievement of optimal functional outcomes often involves multiple treatment interventions. In addition, the selection and timing of these interventions must be based on both individual patient characteristics and the psychosocial situation. Growth is a patient characteristic unique to the pediatric population. Several aspects of this case show how growth affected spasticity management. First, the patient had developed some degree of contracture before her initial presentation at age 6 years, thus making the need for orthopedic surgical intervention highly likely. Second, changes in the dosages of medications may be necessary during growth to maintain maximal results. Third, changes in the need for and types of orthotics and/or assistive devices may also change over time because of changes in spasticity, biomechanic alterations related to increases in the child’s height and weight, and/or changes in the child’s environment. Comorbidities are another important patient characteristic that must be considered in the management of spasticity. There is no specific information in the literature on the treatment of spasticity in patients with HIV infection. A case report describes the use of intrathecal morphine via an implantable device for successful treatment of bladder pain in an adult patient with AIDS.10 In the case presented here, the patient’s diagnosis of HIV encephalopathy did not exclude her from consideration of the full range of treatment options for spasticity management in pediatric patients, including ITB. The patient met the basic ITB clinical inclusion criteria for age and size, her spasticity was severe and clinically significant, treatment goals were appropriate, and she had an excellent psychosocial support system.11 In terms of her general health status, the patient did not meet any exclusion criteria for ITB; with normal CD4 counts and a nondetectable viral load, she is not considered to be immunocompromised and should react similarly to a non–HIV-infected child to any pump-related procedures. The estimated probability of death within 12 months in pediatric patients over 5 years of age with normal CD4 counts who are not receiving antiretroviral therapy is less than 0.5%.12 Thus, the patient discussed
BACLOFEN FOR HIV-RELATED SPASTICITY, Kolaski
in this report will likely survive into adulthood and beyond because she is receiving antiretroviral therapy and close disease monitoring. Finally, this case highlights the critical role of a child’s psychosocial situation in the management of spasticity. The treatment course of the child described here—not unlike many children with CP—was time and effort intensive, involving frequent appointments, therapies, and procedures. The patient’s caregivers showed a high level of commitment to—and compliance with—the treatment course. Their compliance was facilitated by their ability to readily access facilities offering pediatric multidisciplinary, subspecialty care for both infectious disease and spasticity management. Legal adoption eliminated placement uncertainties and also simplified care by eliminating the need to coordinate the patient’s complex care through the department of social services, an often challenging process. Clinical decision-making during the course of treatment of the patient described here was based on careful and detailed evaluation of these individual patient characteristics and psychosocial issues. Continued aggressive management of the patient’s spasticity and its related potential for negative impact on health and function is advocated by the author (and other health care providers involved in this patient’s care) as both clinically indicated and appropriate. CONCLUSIONS HIV encephalopathy in children may result in a clinical picture similar to CP, with motor impairments and spasticity as major features. With medical treatment, long-term survival is likely, and therefore, these patients should be considered potential candidates for the full spectrum of antispasticity treatment options available. This case report shows the safety and benefits of ITB therapy—as part of a multimodality approach to spasticity management—in the treatment of a child with spastic paraparesis related to HIV infection. Clinical decisionmaking will continue to be guided by additional information on longer-term outcomes in this patient in particular and in general for pediatric patients with other diagnoses treated with ITB. Acknowledgements: I thank Beth Smith, PhD, Department of Orthopedics, Wake Forest University School of Medicine; Charles Woods, MD, Department of Pediatrics, Wake Forest University School of Medicine; and Thomas Belhorn, MD, PhD, Department of
1003
Pediatric Infectious Diseases, University of North Carolina School of Medicine, Chapel Hill, for their advice and comments. References 1. Centers for Disease Control and Prevention. Cases of HIV infection and AIDS in the United States, 2002. HIV/AIDS Surveill Rep 2002;14:1-48. 2. Centers for Disease Control and Prevention. Pediatric HIV/AIDS surveillance L262 slide series. 2003. Available at: http:// www.cdc.gov/hiv/graphics/pediatri.htm. Accessed March 24, 2006. 3. Mitchell W. Neurological and developmental effects of HIV and AIDS in children and adolescents. Ment Retard Dev Disabil Res Rev 2001;7:211-6. 4. Angelini L, Zibordi F, Triulzi F, et al. Age-dependent neurologic manifestations of HIV infection in childhood. Neurol Sci 2000; 21:135-42. 5. Udgirkar VS, Tullu MS, Bavdekar SB, Shaharao VB, Kamat JR, Hira PR. Neurological manifestations of HIV infection. Indian Pediatr 2003;40:230-4. 6. Bax MC. Terminology and classification of cerebral palsy. Dev Med Child Neurol 1964;6:295-307. 7. Ashworth B. Preliminary trial of carisoprodol in multiple sclerosis. Practitioner 1964;192:540-2. 8. Albright AL, Gilmartin R, Swift D, Krach LE, Ivanhoe CB, McLaughlin JF. Long-term intrathecal baclofen therapy for severe spasticity of cerebral origin. J Neurosurg 2003;98:291-5. 9. von Koch CS, Park TS, Steinbok P, Smyth M, Peacock WJ. Selective posterior rhizotomy and intrathecal baclofen for the treatment of spasticity. Pediatr Neurosurg 2001;35:57-65. 10. Johnsson E, Coombs DW, Hunstad D, et al. Continuous infusion of intrathecal morphine to control acquired immunodeficiency syndrome-associated bladder pain. J Urol 1992;147:687-9. 11. Albright AL. Intrathecal baclofen in cerebral palsy movement disorders. J Child Neurol 1996;11(Suppl 1):S29-35. 12. Working Group on Antiretroviral Therapy and Medical Management of HIV-infected Children. Guidelines for the use of antiretroviral agents in pediatric HIV infection. Nov 3, 2005. Available at: http://www.aidsinfo.nih.gov/ContentFiles/PediatricGL_SupIIIPDA. pdf. Accessed March 24, 2006. Supplier a. Model 8637-40, SynchroMed II; Medtronic Inc, 710 Medtronic Pkwy, Minneapolis, MN 55432.
Arch Phys Med Rehabil Vol 87, July 2006
CLINICAL NOTE
Use of Intrathecal Baclofen in a Child With Spastic Paraparesis Related to Human Immunodeficiency Virus Infection: A Case Report Kat Kolaski, MD ABSTRACT. Kolaski K. Use of intrathecal baclofen in a child with spastic paraparesis related to human immunodeficiency virus infection: a case report. Arch Phys Med Rehabil 2006;87:1001-3. This case report describes a 2-year treatment course of a 6-year-old with spastic paraparesis due to human immunodeficiency virus (HIV) encephalopathy. She continued to develop worsening musculoskeletal problems and a decline in mobility despite aggressive antispasticity interventions. In addition to meeting the usual clinical criteria for treatment with continuous intrathecal baclofen (ITB), she was believed to be an appropriate candidate for ITB because of adequate treatment of her HIV infection and associated overall low risk of mortality. For over 12 months, she has been successfully treated with ITB and has not developed any complications related to her HIV infection. To my knowledge, this is the first reported successful treatment of spasticity with ITB in a patient with stable HIV infection. Key Words: Baclofen; Case report; HIV; Muscle spasticity; Pediatrics; Rehabilitation. © 2006 by the American Congress of Rehabilitation Medicine and the American Academy of Physical Medicine and Rehabilitation INCE THE BEGINNING OF the acquired immunodeficiency syndrome (AIDS) epidemic in the mid 1980s, the S number of people living with human immunodeficiency virus
(HIV) infection has been steadily increasing.1 Fortunately, the rate of AIDS infection in children in the United States has decreased over the last decade and is currently less than 1%.1 This is related to public health initiatives for the identification and early treatment of HIV-infected mothers and their infants.2 However, when these preventative measures fail, HIV encephalopathy occurs as the fifth most common AIDS-defining condition in children. It may be the initial or an early presenting symptom, a clinical characteristic that is not seen in adults.3 In infants with perinatally acquired HIV infection, risk factors for encephalopathy include early age of infection, high viral loads, advanced maternal disease, and maternal encephalopathy. The neurologic presentation of HIV encephalopathy in children is variable, with characteristic features of microcephaly or
From the Department of Orthopaedic Surgery, Wake Forest University School of Medicine, Winston-Salem, NC. No commercial party having a direct financial interest in the results of the research supporting this article has or will confer a benefit upon the authors or upon any organization with which the authors are associated. The Department of Orthopaedic Surgery of Wake Forest University School of Medicine receives research funding from Medtronic, but the author is not supported by these research funds. Reprint requests to Kat Kolaski, MD, Dept of Orthopaedic Surgery, Wake Forest University School of Medicine, Medical Center Blvd, Winston-Salem, NC 27157, e-mail: [email protected]. 0003-9993/06/8707-10581$32.00/0 doi:10.1016/j.apmr.2006.03.015
brain atrophy, motor abnormalities primarily affecting lowerextremity function, spasticity, and cognitive delays or mental retardation.3 Spastic paraparesis, quadriparesis, and hemiparesis have been described in case series of children with perinatally acquired HIV infection.4,5 Aggressive antiretroviral treatment may prevent progression of the encephalopathic process.3 There is no available literature reporting specifically on the treatment of the motor abnormalities related to HIV encephalopathy in children. However, the etiology and clinical presentation of HIV encephalopathy are consistent with the classic definition of cerebral palsy (CP)6; thus, it is likely that children with these diagnoses are evaluated and treated similarly. This is illustrated in the following case report of a child with spastic paraparesis related to HIV encephalopathy. CASE DESCRIPTION The patient was born at 42 weeks gestation via cesarean section because of breech presentation with a birth weight of 2900g (6lb 7oz) to an HIV-infected mother who received zidovudine (AZT) during pregnancy. The immediate postnatal period was uncomplicated, and the infant was given AZT. HIV infection, with a high viral load and low CD4 count, was diagnosed in the patient 10 weeks after birth. A combination antiretroviral regimen was initiated. Around 1 year of age, developmental delay and lower-extremity spasticity were documented. The patient underwent magnetic resonance imaging of the brain that showed diffuse atrophy for age, confirming a diagnosis of HIV encephalopathy. During the first few years of life, the patient’s HIV infection was not optimally controlled, suspected as a result of poor compliance with medications. Social services became involved with the family, and after age 3 years, the patient had good HIV-infection control and no progression of her neurologic symptoms. The patient’s motor delays were treated with physical therapy. At 4 to 5 years of age, the patient’s spasticity was treated with oral baclofen and lower-extremity botulinum toxin injections with uncertain results. A dorsal rhizotomy was planned, but the patient was placed in foster care and relocated, necessitating a change in her health care providers. The patient presented for an initial evaluation by the author in a pediatric orthopedic clinic at the age of 6 years. Her medications included a combination antiretroviral regimen and 5mg of oral baclofen twice daily. In terms of gross motor function, the patient could sit independently and pull to stand, but she crawled as her primary means of mobility. Her foster parent reported difficulty with dressing and hygiene because of lower-extremity muscle tightness. On examination, she was cognitively intact for her age and had no abnormal findings in the upper extremities. In the lower extremities, weakness and spasticity were significant, with Ashworth Scale scores7 of 4 in the hip adductors, hip flexors and extensors, knee extensors, and ankle plantarflexors. In addition, the patient had contractures of the hip adductors, hamstrings, and ankle plantarflexors. Initial treatment included an increase of the oral baclofen dose to 10mg thrice daily. Although this dose was well tolerArch Phys Med Rehabil Vol 87, July 2006
1002
BACLOFEN FOR HIV-RELATED SPASTICITY, Kolaski
ated, the medication had an equivocal effect on spasticity. In an attempt to facilitate the fitting of lower-extremity orthotics to allow standing, the patient underwent botulinum toxin type A (BTX-A) injections to the bilateral gastrocnemius-soleus muscles (14.3U/kg of body weight) followed by serial casting of the ankles. She was fitted with knee-ankle-foot orthotics but had difficulty tolerating them because of her hip and knee spasticity and contractures. Because of the failure of these interventions and the clinical severity of her spasticity, she was considered to be an appropriate candidate for intrathecal baclofen (ITB).8 Rhizotomy was not recommended because of significant underlying lower-extremity weakness.9 The patient underwent a trial of ITB with a dose of 50g, resulting in an average change in lower-extremity Ashworth Scale scores of 2.4 points. Although the patient’s response to the ITB trial bolus dose was positive, pump implantation was postponed because of 2 main issues: (1) the patient was being treated by an infectious disease specialist at another institution and (2) she was in the legal custody of the local department of social services. Although her foster family could provide excellent psychosocial support, long-term placement issues were unclear. In addition, social service representatives raised concerns about the lack of experience with ITB in patients with HIV infection. Subsequently, the patient was treated with ongoing physical therapy and the following: (1) multiple sessions of chemical denervation procedures including phenol neurolysis of the bilateral obturator nerves, ethanol motor point blocks of the bilateral hamstrings and gastrocnemius muscles, and BTX-A injection (20U/kg of body weight) of the iliopsoas and hamstring muscles; (2) serial casting of the ankles and knees; and (3) a gradual increase of the oral baclofen dose to 30mg thrice daily. With these treatments, the patient progressed to ambulating in the house and classroom using custom-molded polypropylene ankle-foot orthoses (AFOs) and a posterior rolling walker. However, the patient’s improved mobility was short-lived. She developed progression of equinovalgus deformities necessitating the discontinuation of her AFOs because of heel and midfoot skin breakdown. She began to experience poor endurance for ambulation, likely due to the combined effects of ambulation without orthotics, high energy costs of her preferred swing-through gait pattern, and a growth spurt. ITB was reconsidered based on the following: (1) her medical care had been transferred to a local pediatric infectious disease specialist; and (2) the patient was in the process of being adopted by her foster parents. The patient underwent uncomplicated ITB pumpa placement with catheter placement at the T9 level. Over the first 90 days after pump implantation, her dose was titrated to 400g/d on a simple, continuous program and resulted in significantly decreased lower-extremity spasticity. The patient also experienced some decreased ability to stand, likely due to both her underlying weakness and decreased ability to recruit her spasticity. Four months postimplant, the patient presented with acute ITB withdrawal symptoms including anxiety, lower-extremity pruritis, and a dramatic increase in lower-extremity spasticity. Plain spinal radiographs showed a distal catheter disconnection that was found at surgery to be at the lumbar fascia; she underwent prompt catheter revision, thus restoring ITB infusion and spasticity control. Over the next several months, her ITB dose was further adjusted and at 250g/d, an optimal balance between spasticity reduction and functional tone was achieved. Arch Phys Med Rehabil Vol 87, July 2006
Because of persistent equinovalgus deformities, the patient was fitted for double upright metal AFOs with valgus-preventing T straps. These orthotics were chosen to provide stability of the ankle without skin breakdown. Using these orthotics, the patient progressed to ambulating short community distances with a posterior rolling walker. She primarily used a reciprocating gait pattern but tended to revert to a swing-through gait pattern at higher cadences. Over 1 year postimplant, the patient’s spasticity continues to be controlled with a maintenance ITB dose of 250g/d. She has not experienced any other ITB-related complications, and her HIV infection remains in good control on antiretroviral therapy. There has been no radiographic evidence of progression of hip subluxation or development of neuromuscular scoliosis. To address her lower-extremity contractures, the patient underwent mid-third gastrocnemius lengthenings and bilateral adductor tenotomies. This surgery enabled her to tolerate polypropylene AFOs. She is currently ambulating community distances with forearm crutches. The need for further ITB dose adjustments—as well as possible further orthopedic surgical intervention—is anticipated. DISCUSSION This case report illustrates the similarity in the presentation and treatment of a child with neurologic sequelae of HIV infection compared with children with CP. It also illustrates the fact that for potentially ambulatory pediatric patients, adequate control of spasticity and achievement of optimal functional outcomes often involves multiple treatment interventions. In addition, the selection and timing of these interventions must be based on both individual patient characteristics and the psychosocial situation. Growth is a patient characteristic unique to the pediatric population. Several aspects of this case show how growth affected spasticity management. First, the patient had developed some degree of contracture before her initial presentation at age 6 years, thus making the need for orthopedic surgical intervention highly likely. Second, changes in the dosages of medications may be necessary during growth to maintain maximal results. Third, changes in the need for and types of orthotics and/or assistive devices may also change over time because of changes in spasticity, biomechanic alterations related to increases in the child’s height and weight, and/or changes in the child’s environment. Comorbidities are another important patient characteristic that must be considered in the management of spasticity. There is no specific information in the literature on the treatment of spasticity in patients with HIV infection. A case report describes the use of intrathecal morphine via an implantable device for successful treatment of bladder pain in an adult patient with AIDS.10 In the case presented here, the patient’s diagnosis of HIV encephalopathy did not exclude her from consideration of the full range of treatment options for spasticity management in pediatric patients, including ITB. The patient met the basic ITB clinical inclusion criteria for age and size, her spasticity was severe and clinically significant, treatment goals were appropriate, and she had an excellent psychosocial support system.11 In terms of her general health status, the patient did not meet any exclusion criteria for ITB; with normal CD4 counts and a nondetectable viral load, she is not considered to be immunocompromised and should react similarly to a non–HIV-infected child to any pump-related procedures. The estimated probability of death within 12 months in pediatric patients over 5 years of age with normal CD4 counts who are not receiving antiretroviral therapy is less than 0.5%.12 Thus, the patient discussed
BACLOFEN FOR HIV-RELATED SPASTICITY, Kolaski
in this report will likely survive into adulthood and beyond because she is receiving antiretroviral therapy and close disease monitoring. Finally, this case highlights the critical role of a child’s psychosocial situation in the management of spasticity. The treatment course of the child described here—not unlike many children with CP—was time and effort intensive, involving frequent appointments, therapies, and procedures. The patient’s caregivers showed a high level of commitment to—and compliance with—the treatment course. Their compliance was facilitated by their ability to readily access facilities offering pediatric multidisciplinary, subspecialty care for both infectious disease and spasticity management. Legal adoption eliminated placement uncertainties and also simplified care by eliminating the need to coordinate the patient’s complex care through the department of social services, an often challenging process. Clinical decision-making during the course of treatment of the patient described here was based on careful and detailed evaluation of these individual patient characteristics and psychosocial issues. Continued aggressive management of the patient’s spasticity and its related potential for negative impact on health and function is advocated by the author (and other health care providers involved in this patient’s care) as both clinically indicated and appropriate. CONCLUSIONS HIV encephalopathy in children may result in a clinical picture similar to CP, with motor impairments and spasticity as major features. With medical treatment, long-term survival is likely, and therefore, these patients should be considered potential candidates for the full spectrum of antispasticity treatment options available. This case report shows the safety and benefits of ITB therapy—as part of a multimodality approach to spasticity management—in the treatment of a child with spastic paraparesis related to HIV infection. Clinical decisionmaking will continue to be guided by additional information on longer-term outcomes in this patient in particular and in general for pediatric patients with other diagnoses treated with ITB. Acknowledgements: I thank Beth Smith, PhD, Department of Orthopedics, Wake Forest University School of Medicine; Charles Woods, MD, Department of Pediatrics, Wake Forest University School of Medicine; and Thomas Belhorn, MD, PhD, Department of
1003
Pediatric Infectious Diseases, University of North Carolina School of Medicine, Chapel Hill, for their advice and comments. References 1. Centers for Disease Control and Prevention. Cases of HIV infection and AIDS in the United States, 2002. HIV/AIDS Surveill Rep 2002;14:1-48. 2. Centers for Disease Control and Prevention. Pediatric HIV/AIDS surveillance L262 slide series. 2003. Available at: http:// www.cdc.gov/hiv/graphics/pediatri.htm. Accessed March 24, 2006. 3. Mitchell W. Neurological and developmental effects of HIV and AIDS in children and adolescents. Ment Retard Dev Disabil Res Rev 2001;7:211-6. 4. Angelini L, Zibordi F, Triulzi F, et al. Age-dependent neurologic manifestations of HIV infection in childhood. Neurol Sci 2000; 21:135-42. 5. Udgirkar VS, Tullu MS, Bavdekar SB, Shaharao VB, Kamat JR, Hira PR. Neurological manifestations of HIV infection. Indian Pediatr 2003;40:230-4. 6. Bax MC. Terminology and classification of cerebral palsy. Dev Med Child Neurol 1964;6:295-307. 7. Ashworth B. Preliminary trial of carisoprodol in multiple sclerosis. Practitioner 1964;192:540-2. 8. Albright AL, Gilmartin R, Swift D, Krach LE, Ivanhoe CB, McLaughlin JF. Long-term intrathecal baclofen therapy for severe spasticity of cerebral origin. J Neurosurg 2003;98:291-5. 9. von Koch CS, Park TS, Steinbok P, Smyth M, Peacock WJ. Selective posterior rhizotomy and intrathecal baclofen for the treatment of spasticity. Pediatr Neurosurg 2001;35:57-65. 10. Johnsson E, Coombs DW, Hunstad D, et al. Continuous infusion of intrathecal morphine to control acquired immunodeficiency syndrome-associated bladder pain. J Urol 1992;147:687-9. 11. Albright AL. Intrathecal baclofen in cerebral palsy movement disorders. J Child Neurol 1996;11(Suppl 1):S29-35. 12. Working Group on Antiretroviral Therapy and Medical Management of HIV-infected Children. Guidelines for the use of antiretroviral agents in pediatric HIV infection. Nov 3, 2005. Available at: http://www.aidsinfo.nih.gov/ContentFiles/PediatricGL_SupIIIPDA. pdf. Accessed March 24, 2006. Supplier a. Model 8637-40, SynchroMed II; Medtronic Inc, 710 Medtronic Pkwy, Minneapolis, MN 55432.
Arch Phys Med Rehabil Vol 87, July 2006