Severe Spastic Cerebral Palsy Research Articles

Muscle thickness, muscle strength, and fitness in two young adults with moderate and severe spastic cerebral palsy

Muscle thickness, muscle strength, and fitness in two young adults with moderate and severe spastic cerebral palsy

Percutaneous thermal radiofrequency rhizotomy of L2-S1 spinal nerve roots in children with cerebral palsy.

This study presents the results of an evaluation of the effectiveness of percutaneous thermal radiofrequency (RF) ablation of spinal nerve roots to reduce spasticity and improve motor function in children with cerebral palsy (CP). A retrospective analysis was conducted on the surgical treatment outcomes of 26 pediatric patients with severe CP (Gross Motor Function Classification System levels IV-V). The assessment protocol included muscle tone assessment using the modified Ashworth scale (MAS), evaluation of passive and active range of motion, gait video recording, and locomotor status evaluation using the Gross Motor Function Measure (GMFM)-88 scale. Thermal RF rhizotomy (ablation of spinal nerve roots) was performed on all patients at the L2-S1 levels at 70°C for 90 seconds. The statistical data analysis was conducted using the t-test and Mann-Whitney U-test. A p value < 0.05 was considered statistically significant. Before the operation, the average level of spasticity in the lower-limb muscles of all patients was 3.0 ± 0.2 according to the MAS. In the early postoperative period, the spasticity level in all examined muscle groups significantly decreased to a mean of 1.14 ± 0.15 (p < 0.001). In the long-term postoperative period, the spasticity level in the examined muscle groups averaged 1.49 ± 0.17 points on the MAS (p < 0.001 compared to baseline, p = 0.0416 compared to the early postoperative period). Despite the marked reduction of spasticity in the lower limbs, no significant change in locomotor status according to the GMFM-88 scale was observed in the selected category of patients. In the long-term period, during the control examination of patients, the GMFM-88 level increased on average by 3.6% ± 1.4% (from 22.2% ± 3.1% to 25.8% ± 3.6%). The findings of this study offer preliminary yet compelling evidence that RF ablation of spinal nerve roots can lead to a significant and enduring decrease in muscle tone among children with severe spastic CP. Further studies and longer-term data of the impact on functionality and quality of life of patients with CP after spinal root RF ablation are needed.

Palliative single-level selective dorsal rhizotomy for children with spastic cerebral palsy Gross Motor Function Classification System level IV and V: a case series and systematic review of the literature.

Single-level selective dorsal rhizotomy (SDR), typically indicated for ambulatory patients, is a controversial topic for severe spastic cerebral palsy (CP) with Gross Motor Function Classification System (GMFCS) level IV or V. The objective of this case series and systematic literature review was to outline the indication and outcome of palliative SDR for nonambulatory patients with CP and GMFCS level IV and V, focusing on improvement of spasticity and of patient and caregiver reported quality of life assessment. A retrospective case series of patients with CP and GMFCS level IV or V who underwent single-level SDR at the authors' institution is presented. Furthermore, two databases (PubMed and Embase) were searched and a systematic review with a search string based on the terms "selective dorsal rhizotomy," "cerebral palsy," and "outcome" was conducted. The primary outcome was the reduction of spasticity based on the modified Ashworth scale (MAS). Secondary outcomes were change on the Gross Motor Function Measure-66 (GMFM-66), evaluation of patient-reported outcome measures (PROMs), surgical morbidity, and mortality. Eleven consecutive children under the age of 25 years undergoing palliative single-level SDR were included. All patients showed a reduction in MAS score (mean 1.09 ± 0.66 points) and no surgical morbidity and mortality occurred. For the systematic review results from our case series, in addition to 4 reports, 274 total patients were included. Reduction of spasticity based on MAS score was noted in all studies (mean range 1.09-3.2 points). Furthermore, in 2 studies spasticity of the upper extremities showed a MAS score reduction as well (range 1.7-2.8 points). The GMFM-66 score improved in 72% of the patients, while bladder function improved in 78% of the patients. Based on the PROMs, 92% of the patients/caregivers were satisfied with the outcome and their quality of life after the procedure. Two wound infections (2.7%) and one CSF leak (1.3%) occurred, while no surgery-related deaths were described. This analysis showed an improvement in spasticity, daily care, and comfort for patients with CP and GMFCS levels IV and V. Larger cohorts analyzing the outcome of palliative single-level SDR, based on the MAS, GMFM-66, and PROMs, are still needed and should be the focus of future studies. Systematic review registration no.: CRD42024495762 (https://www.crd.york.ac.uk/prospero/).

Microsurgical DREZotomy in Spastic Cerebral Palsy: Poor Man's Baclofen Pump

Using an intrathecal baclofen (ITB) pump for severe refractory spastic cerebral palsy is not a viable option in resource-constraint settings. Therefore authors assessed the role of microsurgical dorsal root entry zone-otomy (MDT) as an alternative for patients in whom the circumstances did not allow ITB pump placement. A prospective clinical study was conducted from August 2016 to July 2020. All patients of spastic cerebral palsy (with III-IV Ashworth grade) who underwent MDT were included. Preoperative and postoperative Ashworth grade and Gross Motor Function Classification System level were assessed along with any change in power, sensation, and bladder functions postoperatively. At last follow-up, the patients' main care providers were asked to rate the effect of surgery (on scale of 0-5) on ease of providing care related to 6 activities of daily living. Seven patients (5 males, 2 females) of spastic cerebral palsy with an age range of 6-21 years underwent MDT over the study period. Six patients had spastic diplegia, and 1 had spastic hemiplegia. Preoperative Ashworth grade in all patients was 3-4 in various involved muscle groups. Four patients with spastic diplegia underwent MDT at L3-S1 level bilaterally, 2 patients with spastic diplegia and spastic bladder underwent MDT at L3-S4 level bilaterally, and 1 patient with right-sided spastic hemiplegia underwent MDT at C5-T1 level and L3-S1 level on the right side. Postoperative Ashworth grade in all patients improved to 0-1. The improvement in mean Ashworth grade was from 3.14 ± 0.378 preoperatively to 0.29 ± 0.488 (P value= 0.000). Most care providers reported improvement in their ability to do physiotherapy, position, clothe, ambulate, and maintain patient's personal hygiene. Care providers of 6 patients reported that they were likely to recommend MDT to another similar patient. MDT is a cost-effective tool in deceasing limb spasticity in spastic cerebral palsy, especially for those who cannot afford ITB placement.

Impacto da utilização de recursos tecnológicos no desempenho de atividades cotidianas e na satisfação da mãe de criança com paralisia cerebral grave

O objetivo geral deste estudo foi o de identificar a percepção da mãe de uma criança com paralisia cerebral quadriplégica espástica grave, com três anos de idade, sobre os recursos tecnológicos utilizados por seu filho no desempenho de tarefas funcionais da rotina diária. Os objetivos específicos foram: listar e indicar a frequência de modificações utilizadas pela criança de acordo com o Inventário de Avaliação Pediátrica (PEDI); identificar o nível de satisfação da mãe em relação as modificações, as dificuldades para usá-las e a necessidade de orientações. Para a coleta de dados utilizou-se o PEDI e um roteiro de entrevista semiestruturado. Os resultados sugerem que a mãe sentia-se satisfeita com os recursos tecnológicos utilizados por O objetivo geral deste estudo foi o de identificar a percepção da mãe de uma criança com paralisia cerebral quadriplégica espástica grave, com três anos de idade, sobre os recursos tecnológicos utilizados por seu filho no desempenho de tarefas funcionais da rotina diária. Os objetivos específicos foram: listar e indicar a frequência de modificações utilizadas pela criança de acordo com o Inventário de Avaliação Pediátrica (PEDI); identificar o nível de satisfação da mãe em relação as modificações, as dificuldades para usá-las e a necessidade de orientações. Para a coleta de dados utilizou-se o PEDI e um roteiro de entrevista semiestruturado. Os resultados sugerem que a mãe sentia-se satisfeita com os recursos tecnológicos utilizados por seu filho e estava bem instruída em relação ao modo de usá-los. Dentre os aspectos negativos que influenciavam na usabilidade dos recursos, citam-se à falta de acessibilidade das vias públicas para o uso da cadeira de rodas e a baixa renda da família. Os resultados deste estudo reforçam a necessidade de envolver a família no processo de seleção de recursos tecnológicos que exigem assistência máxima do cuidador, e reportam para a relevância de o profissional analisar criteriosamente o contexto ambiental em que eles serão inseridos a fim de facilitar a usabilidade e promover, de fato, uma melhor qualidade de vida para a pessoa com deficiência e seus familiares. seu filho e estava bem instruída em relação ao modo de usá-los. Dentre os aspectos negativos que influenciavam na usabilidade dos recursos, citam-se à falta de acessibilidade das vias públicas para o uso da cadeira de rodas e a baixa renda da família. Os resultados deste estudo reforçam a necessidade de envolver a família no processo de seleção de recursos tecnológicos que exigem assistência máxima do cuidador, e reportam para a relevância de o profissional analisar criteriosamente o contexto ambiental em que eles serão inseridos a fim de facilitar a usabilidade e promover, de fato, uma melhor qualidade de vida para a pessoa com deficiência e seus familiares.

The Effect of a Rehabilitation Specific Gaming Software Platform to Achieve Individual Physiotherapy Goals in Children with Severe Spastic Cerebral Palsy: A Randomized Crossover Trial.

Cerebral palsy (CP) is the most common cause of permanent neurological disabilities in children. Many children require long-term daily physiotherapy (PT), and videogaming is a promising tool to increase motivation in rehabilitation. The short- and medium-term effects of an intervention with rehabilitation specific videogames were evaluated on individually defined therapy goals, gross motor function, and motivation. Thirty-two children with bilateral spastic CP, Gross Motor Function Classification level III-IV, and 6-15 years were randomized into an intervention group (regular PT and gaming) or a control group (regular PT), followed by a crossover. The effects of both training periods (each 12 weeks) were compared using the Goal Attainment Scale (GAS), Trunk Control Measurement Scale (TCMS), Pediatric Balance Scale (PBS), Gross Motor Function Measure-88 (GMFM-88), and Dimensions of Mastery Motivation Questionnaire (DMQ). After 3 months follow-up, children were retested using the GMFM, TCMS, and PBS. The GAS change scores were significantly higher after the intervention compared to the control period (8.5 and 2.4, P < 0.001). The change scores for standing exercises (3.85 and 0.22, P = 0.04) and dynamic sitting balance (5.9 and -1.7, P < 0.001) were also significantly higher. After 3 months follow-up the results did not persist. A combined approach of regular PT and rehabilitation specific gaming showed significant effects on individually defined therapy goals, dynamic sitting balance, and standing exercises. However, the lack of persistent effect indicates that continuous individual goal-oriented PT with the addition of gaming is needed.

The Relationship between Swallowing Dynamics and Post-Swallow Residue in Persons with Severe Spastic Cerebral Palsy and Profound Intellectual Disability

The Relationship between Swallowing Dynamics and Post-Swallow Residue in Persons with Severe Spastic Cerebral Palsy and Profound Intellectual Disability

Decline in severe spastic cerebral palsy at term in Denmark 1999-2007.

To analyse trends in prevalence and severity of cerebral palsy (CP) in Denmark in birth years 1999-2007 and compare with previous periods. Data has been collected uniformly in the Danish cerebral palsy national register nationwide since 1995. Rates in the time periods 1999-2001, 2002-2004 and 2005-2007 covering 585,393 births were analysed by gestational age and subtypes. Total number of CP cases in the period was 1165. The overall prevalence of CP decreased significantly from 2.1 in 1999-2001 to 1.8 in 2005-2007 per 1000 livebirths (p=0.022). The decline was only significant for children born at term (p=0.007) but not for the preterm (p=0.44). The decline in children born at term was based on a decrease in bilateral spastic CP (n=117 in years 1999-2001 and n=59 in 2005-2007). Multidisciplinary obstetric skills training with neonatal resuscitation in Denmark was initiated in 2003 and timely associated with the decrease. The prevalence of unilateral spastic CP the prevalence did not change, but in the two last time periods more children had a right-sided than left-sided unilateral spastic CP. The decline in rate of CP seen in 2005-2007 as compared to 1999-2001 was mainly based on fewer cases of severe spastic CP in term infants. We hypothesize that improved neonatal resuscitation in the delivery room may be partly responsible for the decrease. In premature children the decline was not significant in this time period, but has been dramatically decreasing in the years before the time period here analysed.

Risk Factors for Developing Scoliosis in Cerebral Palsy: A Cross-Sectional Descriptive Study.

This study aims to identify the risk factors leading to the development of severe scoliosis among children with cerebral palsy. A cross-sectional descriptive study of 70 children (aged 12-18 years) with severe spastic and/or dystonic cerebral palsy treated in a single specialist unit is described. Statistical analysis included Fisher exact test and logistic regression analysis to identify risk factors. Severe scoliosis is more likely to occur in patients with intractable epilepsy ( P = .008), poor gross motor functional assessment scores ( P = .018), limb spasticity ( P = .045), a history of previous hip surgery ( P = .048), and nonambulatory patients ( P = .013). Logistic regression model confirms the major risk factors are previous hip surgery ( P = .001), moderate to severe epilepsy ( P = .007), and female gender ( P = .03). History of previous hip surgery, intractable epilepsy, and female gender are predictors of developing severe scoliosis in children with cerebral palsy. This knowledge should aid in the early diagnosis of scoliosis and timely referral to specialist services.

Effect of Therapeutic Horseback Riding on Posture in Children with Cerebral Palsy

The purpose of this study was to measure postural changes in children with spastic cerebral palsy after participation in a therapeutic horseback riding program. Eleven children with moderate to severe spastic cerebral palsy, aged 2 years 4 months to 9 years 6 months, were selected for this study and underwent postural assessments according to a repeated-measures design. Assessment of posture was performed by a panel of three pediatric physical therapists, using a postural assessment scale designed by the author. A composite score for each test interval was calculated for each child, and a median score was calculated for the entire group at each test interval. Data were analyzed using a Friedman test, assuming an alpha level of .05. A statistically significant difference was found between the three test intervals with significant improvement occurring during the period of therapeutic riding. Clinical improvements were also noted in muscle tone and balance as evidenced by improved functional skills. These results constitute the first objective measure supporting the efficacy of therapeutic horseback riding on posture in children with cerebral palsy.

Autonomic responses to correct outcomes and interaction errors during single-switch scanning among children with severe spastic quadriplegic cerebral palsy

BackgroundThe combination of single-switch access technology and scanning is the most promising means of augmentative and alternative communication for many children with severe physical disabilities. However, the physical impairment of the child and the technology’s limited ability to interpret the child’s intentions often lead to false positives and negatives (corresponding to accidental and missed selections, respectively) occurring at rates that frustrate the user and preclude functional communication. Multiple psychophysiological studies have associated cardiac deceleration and increased phasic electrodermal activity with self-realization of errors among able-bodied individuals. Thus, physiological measurements have potential utility at enhancing single-switch access, provided that such prototypical autonomic responses exist in persons with profound disabilities.MethodsThe present case series investigated the autonomic responses of three pediatric single-switch users with severe spastic quadriplegic cerebral palsy, in the context of a single-switch letter matching activity. Each participant exhibited distinct autonomic responses to activity engagement.ResultsOur analysis confirmed the presence of the autonomic response pattern of cardiac deceleration and increased phasic electrodermal activity following true positives, false positives and false negatives errors, but not subsequent to true negative outcomes.ConclusionsThese findings suggest that there may be merit in complementing single-switch input with autonomic measurements to improve augmentative and alternative communications for pediatric access technology users.

Effect of a hyperbolide mastication apparatus for the treatment of severe sleep bruxism in a child with cerebral palsy: Long-term follow-up

Sleep bruxism is common among the various oromotor alterations found in individuals with cerebral palsy (CP). Few studies have investigated the use of the mastication device denominated "hyperbola" (HB) and none was found describing the use of such a device for the treatment of bruxism in children with CP. The aim of the present study was to evaluate the effect of the HB on electromyographic (EMG) activity in the jaw-closing muscles and the reduction in sleep bruxism in a child with CP using surface EMG analysis before and after nine months of treatment. A seven-year-old boy with severe spastic CP and sleep bruxism was enrolled in this study. The HB was chosen as the treatment option for sleep bruxism in this case because the child did not accept an occlusal splint. The HB has a hyperbolic shape and is made of soft, non-toxic, odorless, tasteless silicone. There are five different sizes of HB manufactured based on the diversity of tooth sizes. This device produces proprioceptive excitation in the dentoalveolar nerve, spindles and Golgi tendon organs. HB has been employed for the treatment of temporomandibular disorder, abnormal oro-dental development, abnormal occlusion, xerostomy, halitosis and bruxism. HB therapy was performed for 5min six times a day over a nine-week period. Surface EMG of the mandible at rest and during maximum contraction was performed on the masseter and temporalis muscles bilaterally to evaluate electromyographic activity before and after nine months of HB usage. HB usage led to a visible tendency toward the reorganization of mastication dynamics, achieving a marked balance in electromyographic activity of the jaw-closing muscles and improving the child's quality of life. Based on the findings of the present study, this noninvasive therapy may be useful for individuals with cerebral palsy due to its positive effects and low cost, which allows its use in the public health realm. Further clinical studies with a larger sample size are needed to validate these results and allow the development of a new treatment protocol for patients with spastic cerebral palsy.

Controlled study of the effects of continuous intrathecal baclofen infusion in non-ambulant children with cerebral palsy

To measure changes in children with severe spastic cerebral palsy (CP) after continuous intrathecal baclofen (ITB) infusion over 18 months and to compare the results with those of a comparison group awaiting treatment. Thirty-eight children with severe spastic CP considered suitable for ITB were assessed when first seen, just before insertion of an intrathecal pump, and 9 months and 18 months later. Eighteen children waited around 9 months for a pump (group 1: nine males, nine females; mean age 9y 11mo [SD 3y 7mo], nine in Gross Motor Function Classification System [GMFCS] level IV, nine in level V). This baseline period was used as a control for comparison with the first and second 9-month periods after the pump for the remaining 20 children (group 2: 11 males, nine females; mean age 10y 2mo [SD 3y 1mo], nine in GMFCS level IV, 11 in level V). The main outcome measure was the Pediatric Evaluation of Disability Inventory (PEDI); other assessments were of function, ease of care, quality of life, and costs of new equipment. No significant change was found in the PEDI between group 1 while awaiting treatment and group 2 in the two periods afterwards, nor in the Lifestyle Assessment Questionnaire or the cost of new equipment. Significant changes were found in group 2 in the first 9 months according to the modified Ashworth score (difference between mean values for groups -1.7, standard error 0.58; p=0.008), Penn Spasm score (-1.3, 0.37; p=0.001), mean joint range of movement (8.3°, 2.8; p=0.005), and Caregiver Questionnaire (-19.7, 5.1; p=0.01), and in the second 9 months for the Modified Ashworth Scale score (-0.62, 0.12; p=0.001). ITB in children with severe spastic CP over the first 18 months improves their quality of life in terms of comfort and ease of care. It has less effect on function, participation in society, or the overall cost of new equipment.

Incidence and risk factors of hip joint pain in children with severe cerebral palsy

Purpose. Pain is a serious complication associated with hip dislocation in cerebral palsy (CP), limiting patient independence and quality of life. This study aimed to determine the frequency of pain in severe CP patients with hip dislocation and to reveal factors associated with the hip pain.Methods. Seventy-three consecutive new-intervention CP patients admitted to authors' institution with spastic quadriplegia, mean age 10.8 years (range 4.0–18.0 years) were enrolled: 31 females and 42 males, totally 99 dislocated hips. All patients were assessed level IV or V according to the Gross Motor Function Classification Scale (GMFCS) and had poor communication skills. Pain severity was evaluated according to the Numeric Rating Scale (NRS-11). Data concerning previously applied physiotherapy was collected to divide the patients into subgroups: A – no abduction therapy (n == 24), B – abduction therapy (n == 35) and C – abduction therapy and horse-back riding (n == 13). On the pelvic antero-posterior radiographs head migration percentage was measured to reveal hip dislocation. Femoral head cartilage degenerative lesions were evaluated for size and location in 45 hips undergoing surgical treatment.Results. Overall pain prevalence was 56%%. The appearance of pain was associated with the patient age (p == 0.048), previous abduction physiotherapy (p < 0.00001), previous horse-back riding therapy (p < 0.00001) and anterior location of degenerative changes of the femoral head (p == 0.03). Pain intensity was related to the size of the degenerative cartilage lesions (p == 0.004) and to the degree of femoral anteversion (p < 0.0001).Conclusions. Extensive abduction exercises, hippotherapy and presence of degenerative cartilage lesions on the anterior part of femoral head may be considered risk factors for hip pain appearance in the dislocated hip of a child with severe spastic CP. Other associated factors are abduction exercise intensity, age, excessive femoral anteversion and size of degenerative cartilage lesions.

Continous intrathecal baclofen (cITB) therapy terminates spinal spasms mimicking tonic seizures in a teenager with severe spastic cerebral palsy with total body involvement and cervical dystonia

Introduction: Cerebral palsy (CP) after brain injury in children is associated with velocity dependent muscle stiffness and motor function impairment. Continuous intrathecal baclofen (cITB) is established for children with CP but the implantation of a baclofen pump is reserved for patients with severe symptoms refractory to oral drug management or with adverse side effects (ASEs) under high oral baclofen doses. The advantages of cITB are a low dose, a low rate of ASEs and improved muscle relaxation.

Divided We Stand, United We Fall: Personal Budgets Versus Universal Coverage

Divided We Stand, United We Fall: Personal Budgets Versus Universal Coverage

Dental Treatment of a Patient with Cerebral Palsy under General Anesthesia

Dental Treatment of a Patient with Cerebral Palsy under General Anesthesia Jun-Min Chung, D.D.S., Kwang-Suk Seo, M.D., Young-Eun Yi, D.D.S., Hee-Jung Han*, R.N., Jin-Hee Han*, R.N., Hye-Jung Kim*, R.N., Teo-Jeon Shin, M.D., Hyun-Jeong Kim, M.D., Kwang-Won Yum, M.D., and Juhe Chang, D.D.S. Department of Dental Anesthesiology, Seoul National University, School of Dentistry, *Department of Dental Anesthesiology, † Clinic for Persons with Disabilities, Seoul National University Dental Hospital, Seoul, Korea Background: Cerebral palsy (CP) is non-progressive disorder of motion and posture. In CP patient, there are difficulties in dental treatment because of uncontrolled movement of limb and head, and conjoined disabilities such as cognitive impairment, sensory loss, seizures, communication and behavioral disturbances. It is reported that CP patients have high incidence in caries and a higher prevalence of periodontal disease. But, despite the need for oro-dental care, these patients often are unlikely to receive adequate treatment without sedation or general anesthesia because of uncontrolled movements of the trunk or head. Methods: We reviewed the 58 cases of 56 patients with CP who underwent outpatient general anesthesia for dental treatment at the clinic for the disabled in Seoul National University Dental Hospital. Results: The mean age was 19 (2-54) years. The number of male patient was 40 and that of female was 18. They all had severe spastic cerebral palsy and 22 had sever mental retardation, 15 epilepsy, 8 organic brain disorder, 1 blindness, 2 deafness and cleft palate. For anesthesia induction, 14 cases was needed physical restriction who had sever mental retardation and cooperation difficulty, but 44 cases showed good or moderate cooperation. Drugs used for anesthesia induction were thiopental (37 cases), sevoflurane (14 cases), ketamine (3 cases ) and propofol (4 cases). All patients except one were done nasotracheal intubation for airway management and 4 cases were needed difficult airway management and 1 patient already had tracheostomy tube. Mean total anesthetic time was 174 ± 56 min and staying time at PACU was 88 ± 39 min. There was no death or long term hospitalization because of severe complications. Conclusion: If general anesthesia is needed, pertinent diagnostic tests and workup about anomaly, and appropriate anesthetic planning are essential for safety. (JKDSA 2008; 8: 22~28) 󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏

Epiphyseal separations in spastic cerebral palsy

Metaphyseal and diaphyseal fractures have been reported in children with severe spastic cerebral palsy, but there is no reference to epiphyseal separations in these patients. We report nine epiphyseal separations involving the distal femur and proximal humerus in four severely affected children with spastic quadriplegic cerebral palsy. The clinico-radiological features confirmed the cause to be scurvy. These epiphyseal separations have a good prognosis unlike the physeal injuries reported in children with myelodysplasia. Treatment with vitamin C and splintage resulted in rapid healing with excellent remodeling. Scurvy should be considered a potential cause for an epiphyseal slip in a child with severe spastic cerebral palsy. Routine dietary supplementation of vitamin C is recommended for these malnourished and nonambulant children.

Agreement of aspiration tests using barium videofluoroscopy, salivagram, and milk scan in children with cerebral palsy

To study the agreement between three tests for aspiration, barium videofluoroscopy, salivagram, and milk scan we studied 63 children with severe non-ambulant spastic quadriplegic cerebral palsy (CP) aged 14 months to 16 years (32 males, 31 females). The salivagram was most frequently positive (56%, 95% confidence interval 43 to 68%); the next most frequently positive was barium videofluoroscopy when aspiration was defined as the presence of either laryngeal penetration of material or frank aspiration (39%, 95% confidence interval 26 to 53%). The milk scan was rarely positive (6%, 95% confidence interval 2 to 16%). Agreement between the tests of aspiration was poor. The maximum agreement (kappa=0.20) was between aspiration as diagnosed with the salivagram and by barium videofluoroscopy. Positive tests for aspiration are frequent in children with severe CP. Frequency varies widely depending on the investigation used. There is poor agreement between tests used for the diagnosis of aspiration. This information is of importance in assessing the significance of test results.

Mental Retardation: Management and Prognosis

1. Chris Plauche Johnson, MEd, MD* 2. William Otis Walker Jr, MD† 1. *Professor, Department of Pediatrics, University of Texas Health Science Center at San Antonio, San Antonio, Tex 2. †Director, Neurodevelopmental/Birth Defects Clinics, Children’s Hospital & Regional Medical Center. The University of Washington, Seattle, Wash After completing this article, readers should be able to: 1. List key components of the special education system that benefit children who have mental retardation (MR). 2. Describe associated conditions that commonly occur in children who have MR. 3. Recognize the importance of family supports, including support of siblings, in the management of children who have MR. 4. Discuss the most likely adult outcomes for persons who have various levels of MR. 5. Delineate the factors, in addition to cognitive skills, that help determine long-term outcomes of persons who have MR. 6. Know when it is appropriate to pursue guardianship proceedings for an adult who is mentally retarded. Management of mental retardation (MR) begins with breaking the news to parents of affected children sensitively, compassionately, and culturally appropriately. It is important to emphasize the child’s strengths in addition to describing the delays or deficits. It also is important to be realistic without taking away hope. If the child is younger than 6 years of age at the time of diagnosis, it may be more appropriate to use the term “global developmental delay” unless the delays are due to a recognizable syndrome known to be associated with MR. When the child enters elementary school and standardized testing provides more reliable and predictive results of adult cognitive impairment, the diagnosis may be revised to “mental retardation.” Parents should be informed that the child will continue to progress, albeit more slowly, than his or her peers. Families need additional patience and persistence when raising a child who has MR. Unlike typically developing children, who seem to learn skills simply by modeling their parents, siblings, and peers, children who have MR may need specific instruction to master a skill. If the global developmental delay or MR is due to a known syndrome, appropriate genetic counseling and up-to-date literature should be …