Total Body Involvement Research Articles

Dyskinetic vs Spastic Cerebral Palsy: A Cross-sectional Study Comparing Functional Profiles, Comorbidities, and Brain Imaging Patterns.

The authors aimed to describe the distribution of predominant and secondary motor types and compare functional profiles, comorbidities, and brain imaging patterns between dyskinetic and spastic cerebral palsy. Children recruited from a cerebral palsy register were assessed at age 5, 10, or 15. Motor types, topography, functional classifications, and comorbidities were recorded. Univariable logistic regression was used to compare dyskinesia with spasticity, with and without adjustment for topography. Neuroimaging classifications were extracted from the register. Of 243 children with spasticity or dyskinesia, the predominant motor type was spastic in 183 and dyskinetic in 56. Dyskinesia was associated with comparatively poorer function, total body involvement, and gray matter injury. After adjustment for topography, dyskinesia was associated with similar or better function. The study suggests that practical tools routinely incorporated into clinical practice would facilitate accurate and reliable classification of predominant and secondary motor types, topography, and functional abilities.

The CXCR4 Inhibitor Plerixafor Effectively Mobilizes Primary AML in NODscid IL2Rγc−/− Xenografts and Markedly Reduces but Does Not Eradicate Leukemia in Combination with Cytarabine +/− Clofarabine Chemotherapy

Abstract 1432 Background:The origin of relapse in AML is believed to be related to persistence of resistant “leukemia stem cells.” in the bone marrow microenvironment where adhesion confers drug resistance. Engraftment of human AML in immunodeficient mice is dependent on CXCR4 (Tavor et al 2004). CXCR4 inhibitors, such as AMD3100 (plexiglass,hereafter P), overcome adhesion mediated chemotherapy resistance (Zeng et al 2006) and mobilize human leukemia engrafted in immunodeficient mice (Zeng et al 2009). P also mobilized leukemia in an APL murine model and in combination with chemotherapy reduced tumor burden (Nervi et al 2009). Methods:We studied the combination of P 5mg/kg daily sc X 3, cytarabine (araC=A) 300mg/kg IP X 3 and clofarabine (C), 20mg/kg IP X 3 in the NODscid IL2R γc−/− mouse engrafted with primary patient AML CD34+ cells after 350 cGy total body irradiation. We could first detect circulating human CD45+ or human CD34+ cells, denoting engraftment, by flow cytometry as early as 5–13 weeks. We then injected plerixafor to assess mobilization capability at 8–16 weeks, followed by the combination of plerixafor and chemotherapy. Animals were sacrificed by 14–38 days after chemotherapy, and assessed for AML in blood, marrow, and spleen. Results:A single 5mg/kg dose of P, produced a 2.26 ± 0.94 (SD) fold increase in peak mobilization (at 2 hours) compared to saline control, p=0.026. P-induced mobilization was directly related to expression of CXCR4, with a patient exhibiting 10.3% CXCR4 showing 0.86× baseline, as compared to a patient with 24.7% CXCR4 exhibiting a 2.2-fold increase, and 84.9% CXCR4, a 3-fold increase. Chemotherapy,described above, was given 2 hours after plerixafor. For animals that received P/A vs. P/A/C, there was no statistically significant difference in leukemic burden (in millions of human CD34+ AML cells ± SD) of the animals sacrificed 14 days after initiation of treatment: bone marrow six bones 116.3 ± 33.7 vs. 111.7 ± 29.2 (p=0.86), spleen 50.8 ± 10 vs. 43.7 ± 19.1 (p=0.59), blood 10.9 ± 9.6 vs. 3.1 ± 1.4 (p=0.16), or estimated total body burden 178.0 ± 45.3 vs. 158.5 ± 30 (p=0.52). A comparison of 4 groups of animals, P/A/C vs. P/A vs. A/C vs. A demonstrated a statistically significant difference between certain groups, at certain time points. For example, on day 10, P/A/C treated animals had a lower human PB CD34+ count than P/A, 0.07 vs. 0.24 × 109/L (p=0.034). On day 14, P/A/C had a lower CD34+ count than A/C, 0.08 vs. 0.16 (p=0.047). But at day 38, the leukemia had already recurred, and there was no statistically significant difference in the organ or total body involvement by the leukemia. There was a very low white blood count days 5–24 post chemotherapy, with only minimal residual disease detectable by flow cytometry (analogous to a period of remission in humans), but by day 38, the leukemia had recurred (Figure 1), and there was no statistically significant difference in the organ or total body involvement by the leukemia amongst the groups (Figure 2). Conclusion:This model demonstrates the efficacy of chemotherapy in reducing circulating leukemia, but resistant cells appear to remain sheltered and give rise to relapse. Despite the beneficial effects of CXCR4 inhibitors in vitro and in vivo in a murine APL model, the combination of plerixafor with chemotherapy did not prevent or postpone leukemic relapse. These results could be attributed to either inefficient scheduling of plerixafor (for example, continuous infusion may have worked better), or non-cycling cells may be preferentially mobilized (Bonig H et al., 2009), and thus less susceptible to cytarabine treatment. These concepts are currently being explored. Alternatively, concomitant inhibition of other adhesion receptors may be necessary to prevent leukemia from homing back to the marrow. Precise timing and degree of mobilization in combination with chemotherapy may be required to optimize this approach in the clinic. [Display omitted] [Display omitted] Disclosures:Becker:Sanofi-Oncology (Genzyme): Research Funding.

Syncope in Complex Regional Pain Syndrome

Complex regional pain syndrome (CRPS) is a debilitating pain syndrome characterized clinically by severe pain along with signs and symptoms of autonomic dysfunction. Presyncope and syncope are common in these patients. The purpose of this study was to investigate the cause of these symptoms in these patients. Patients with CRPS are more prone to presyncope and syncope as evidenced by head-up tilt table testing (HUTT). Patients with CRPS underwent a complete cardiac evaluation that included a 12-lead electrocardiogram, 2-dimensional echocardiography, 24-hour Holter monitoring, and HUTT. Seventy-four patients with CRPS were evaluated. Eight CRPS patients (10%) could not complete a HUTT due to pain. Of the 66 CRPS patients who completed a HUTT, 15 (37.9%) reported pretest symptoms of presyncope or syncope. Twenty-eight CRPS patients (42.4%) had a positive HUTT. CRPS patients with lower limb involvement were more likely to have vasovagal syncope or orthostasis on HUTT than those with upper extremity or total body involvement (12 of 18 [67%] vs 16 of 48 [33%]; P = 0.015). Syncope is common in patients with CRPS, especially with lower limb involvement. Autonomic dysregulation of the lower extremities leads to impaired sympathetic vasoconstriction and venous pooling, which can predispose these patients to syncope. Physician awareness of this syndrome will lead to improved recognition and treatment of their symptoms of presyncope or syncope.

Health and fitness management through yoga

IntroductionModern technology is trying hard to make our life easier. Now throughout the world man appears to be living a more and physically inactive life, he rides instead of walks,...

Development and treatment of spinal deformity in patients with cerebral palsy

Scoliosis is a common deformity in children and adolescents with cerebral palsy. This is usually associated with pelvic obliquity due to extension of the curve to the sacrum. Sagittal plane deformity is less common and often develops along with scoliosis. Spinal deformity in patients with severe neurological handicaps can affect their ability to sit and cause significant back pain or pain due to rib impingement against the elevated side of the pelvis on the concavity of the curvature. Surgical correction followed by spinal arthrodesis is indicated in patients with progressive deformities which interfere with their level of function and quality of life. Spinal deformity correction is a major task in children with multiple medical co-morbidities and can be associated with a high risk of complications including death. A well-coordinated multidisciplinary approach is required in the assessment and treatment of this group of patients with the aim to minimize the complication rate and secure a satisfactory surgical outcome. Good knowledge of the surgical and instrumentation techniques, as well as the principles of management is needed to achieve optimum correction of the deformity and balancing of the spine and pelvis. Spinal fusion has a well-documented positive impact even in children with quadriplegia or total body involvement and is the only surgical procedure which has such a high satisfaction rate among parents and caregivers.

A classification for hip disease in cerebral palsy

hip disorders in patients with CP was equally poor, with some research stating that up to 50% of patients with ‘dislocating’ hips developed hip pain when the patient aged. Who were these patients and what were their hip disorders? Reimers, in his classic work on the stability of the hip in children, added the migration percentage of hip subluxation to the lexicon of classification systems. 2 While this has become the standard of hip measurement in CP, it is still difficult to determine where in the spectrum of disease a particular patient lies. Reimers’ classification also does not address the degree of acetabular involvement. Why do classification systems matter? CP is a diverse spectrum of disorders ranging from mild motor disorder (GMFCS level I) to severe total body involvement (GMFCS level V). Similarly, mild hip disorders with minimal structural changes need not have any treatment while severe hip dislocation with femoral head destruction and acetabular involvement may adversely affect the quality of life of a patient who has such a hip. Comparing large groups of patients who have similar, measureable conditions (in this case hip dysplasia) enables the researcher and the clinician to understand the natural history of the problem and, perhaps, make conclusions about the best way to treat their patients. Treatments such as physical therapy, chemical denervation, bracing, orthopedic surgery, etc. purport to ‘improve’ the hip, yet it is often difficult to determine the degree of involvement that any particular patient had prior to or after the treatment. A classification system that has discrete parameters enables one to ‘put that disorder into a box’ for communication with other clinicians and other researchers. While I applaud this attempt to categorize hip problems in CP, there are a few limitations of the Robin et al. study. First, although the authors performed validity testing, there are other studies demonstrating the inter- and intrarater error in using the Reimers’ index. 3 Second, the quality of the radiographs, i.e. standardization of positioning of the hip was not discussed. The measurements become more difficult and less reliable with pelvic obliquity and rotation. Third, this is a classification on skeletally mature patients and it may be too late to intervene to improve hip function by the time that the patient is skeletally mature and may lessen the impact of a classification system. Last, hip disorders in CP are a three-dimensional problem. Often the plain radiograph appears fairly normal, yet the hip may be subluxated posteriorly. Clearly three-dimensional imaging (computed tomography or magnetic resonance imaging) would add to the classification of hip disorders in children with CP. 4 However, with these caveats, the authors should be congratulated for taking a complex problem and attempting to break it down to discrete categories. This, I believe, is one of the challenges of science: taking continuous data that is overwhelming and attempting to organize it for our human brains (especially orthopedic surgeon’s brains). Combining this hip classification schema with the GMFCS will enable us to evaluate our patients in the clinic and in our clinical research to determine if any of our therapies change the natural history of the hip in children with CP. Why another classification system? It’s all about communication.

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.

Retentissement de la scoliose chez les patients polyhandicapés : étude descriptive de 61 enfants et adultes polyhandicapés avec ou sans arthrodèse vertébrale

Introduction The literature is controversial about how to manage scoliosis in cerebral palsy patients with total body involvement. Spinal fusion is admitted as the only means to stop the evolution of the curve but remains associated with many complications. Objectives The aim of this descriptive study was to formulate hypotheses concerning the interest of spinal fusion in this population. Methods This descriptive cross-sectional study was conducted in 61 patients aged 4 to 48 years (mean 24 years, median 20 years) with severe scoliosis (Cobb score > 40°). Each patient had undergone recent X-ray of the spine and hips and a clinical exam. Data were collected on orthopaedic and functional status, health level (pulmonary, digestive, nutrition, skin parameters), pain, and subjective quality of life (sQoL). Results Data were collected for all 61 patients (35 females, 26 males), 16 underwent spinal fusion at a mean age of 16.5 years (prevalence of 32.0%). No significant difference was found between patients with a spinal instrumentation and others in variables tested. A difference might be present with functional level, but only results from a longitudinal study would be conclusive. Pain was prevalent (19.7%), not treated sufficiently (only 50% receive treatment), and contributes to poor sQoL ( p < 0.042). Among the other data collected, nutritional level has the greatest influence on general health status ( p < 0.05). Conclusions Systematic spinal fusion cannot be generalized for this population of patients with cerebral palsy and total body involvement. However, health status and quality of life could be improved with better care of nutritional problems and pain.

(v) The hip in cerebral palsy

Summary Deformity and displacement of the hip is the second most common orthopaedic problem to affect children with cerebral palsy. The severity and incidence of hip pathology increases with the severity of cerebral palsy and up to 70% of patients with total body involvement cerebral palsy suffer from hip displacement. Hip deformity can also be problematic in patients who walk as it results in rotational malalignment of the lower limb in gait. Awareness of the problems and appropriate screening permits timely intervention. This may involve management of muscle tone or soft tissue procedures. In more advanced cases of hip displacement and deformity, femoral and pelvic osteotomies are usually required. The goals of treatment range from improving the efficiency of gait to prevention of severe postural problems and pain in the most severely affected.

Natural history of scoliosis in spastic cerebral palsy

Although the frequent occurrence of scoliosis in patients who have spastic cerebral palsy is well known and surgical treatment has often been recommended for these patients, little is known about the natural history of scoliosis in this population. We aimed to clarify the natural history of scoliosis from childhood through to adulthood and provide objective data on proper surgical indications for such patients. The participants were 37 institutionalised patients with severe spastic cerebral palsy and scoliosis. All the participants had a series of radiographs taken, starting at a mean age of 7.8 years; they were followed up for an average of 17.3 years. We retrospectively reviewed radiographs and assessed the effect of five factors on progression of scoliosis: sex, degree of spasticity, initial physical capability, pattern of spinal curve, and location of curve. Scoliosis usually started before the age of 10 years and progressed rapidly during the growth period. In many cases, even after growth had ended, continuous progression was seen. The mean magnitude of the curves at final examination was 55 degrees (Cobb angle). In 11 (85%) of 13 patients who had a spinal curve of more than 40 degrees before age 15 years, the scoliosis progressed to more than 60 degrees by the time of the final examination. Meanwhile, in only three (13%) of 24 patients who had a curve of less than 40 degrees at age 15 years, did the scoliosis progress to more than 60 degrees. Severe scoliosis (> or = 60 degrees) developed predominantly in those who had total body involvement (67%), were bedridden (100%), or had throacolumbar curves (57%). The risk factors for progression of scoliosis in spastic cerebral palsy are: having a spinal curve of 40 degrees before age 15 years; having total body involvement; being bedridden; and having a thoracolumbar curve. Patients with these risk factors might benefit from early surgical intervention to prevent progression to severe scoliosis.

A combined bony and soft-tissue tarsal stabilization procedure (Grice-Schede) for hindfoot valgus in children with cerebral palsy.

This study deals with a combined bony and soft-tissue procedure (Grice-Schede) for the treatment of pes (equino) planovalgus in children suffering from cerebral palsy. Results of 43 treated feet in 28 children were assessed clinically and radiologically at a mean follow-up time of 6.7 years (range from 0.6 years to 13.8 years). In all, 58.1% excellent or good results, 14.0% satisfactory and 27.9% poor results were found. The procedure can be recommended for patients with hemiplegic and diplegic conditions. It is not suited for patients with total body involvement.

Cicatricial Ectropion in Ichthyosis

Four kinds of ichthyosiform dermatoses have been described. Only lamellar ichthyosis or ichthyosis congenita is associated with the development of ectropion and subsequent eye symptoms. Conservative treatments have been tried but surgical correction of the ectropion was ultimately required for symptomatic relief. Autografts have been used successfully, provided an available donor site can be found. Grafts taken from the arm, eyelids, postauricular skin, and groin have been used with success. An uncircumcised youth with total body involvement from ichthyosis developed bilateral upper and lower ectropion. The penile foreskin was the only possible suitable donor site because it seemed unaffected by the disease. A circumcision was performed and the foreskin divided into four separate full-thickness skin graft triangles to treat the four-lid ectropion. There was successful resolution of the eye symptoms and a watertight closure. To our knowledge, this is a unique case in which penile foreskin has been used to correct cicatricial ectropion.

Iliopsoas transfer in cerebral palsy: the long-term outcome.

Thirty-three patients with cerebral palsy had iliopsoas transfers as part of their surgery for hip instability. All had total-body involvement, spastic cerebral palsy and none could walk. At the time of surgery, eight hips were subluxated and 39 were dislocated or severely subluxated. Mean follow-up was 8 years in patients between 8-25 years old. Forty-five of the 47 hips were located. Thirty patients had an accompanying scoliosis, and, in 10 patients, the rib cage impinged on the pelvis. Hip flexion had decreased in most patients. Sitting ability had not improved in any patient, and had in fact deteriorated in 50%.

Management of hip posture in cerebral palsy.

Seating arrangements for cerebral palsy children with total body involvement are often unsatisfactory and can pose considerable problems for the multi-disciplinary team. Hip joints at risk of dislocation must be kept in an abducted position in order to minimize pain. A new wheelchair with a barrel-shaped cylindrical seat has been developed which improves the femoral head location and alleviates pain.

Surgery of Spinal Deformity in Cerebral Palsy Twelve Years in the Evolution of Scoliosis Management

In order to assess the lessons learned from 12 years of surgery on patients with cerebral palsy and spinal deformity, the cases may be divided into three groups classified according to type of posterior spinal fusion, instrumentation, and time period. Group I (1976-1980) included patients who had Harrington rods, usually with Dwyer instrumentation. Group II (1980-1985) consisted of patients with unlinked Luque or wired-in Harrington rods. Group III (1985-1988) comprised patients with a unit Luque rod extending to the pelvis. Most patients were retarded nonwalkers who had total body involvement, pelvic obliquity, and severe thoracolumbar curves (Group I average, 97 degrees; Group II average, 72 degrees; Group III average, 89 degrees). The frontal plane correction at follow-up study averaged 51% in Group I, 47% in Group II, and 76% in Group III. The correction of the pelvic obliquity averaged 71% in Group I, 58% in Group II, and 86% in Group III. The general trend was toward longer fusion, use of the unit 0.625-cm Luque rod, and first-stage anterior discectomy and fusion without anterior instrumentation. The second-stage posterior arthrodesis and fusion is now performed only one week after the first-stage anterior procedure. Skeletal traction has been abandoned. The Luque rod instrumentation without fusion has also been abandoned.

Treated and Untreated Unstable Hips in Severe Cerebral Palsy

One hundred patients with severe cerebral palsy (total body involvement) and dislocated hips were examined to determine their level of pain, sitting ability, pelvic obliquity, scoliosis, nursing care difficulties and complications of decubitus ulcers and fractures. 50 of the patients had undergone surgical procedures to treat the hip; 50 had received no treatment. No significant differences were found in the frequency of pain or other complications between the two groups. Nursing care difficulties and the ability to sit did not depend on the status of the hip. Pelvic obliquity and scoliosis were related to the severity of neurological damage rather than to hip stability. These findings suggest that surgical treatment of already dislocated hips of patients with severe cerebral palsy is not helpful.

Seat insert for cerebral-palsied children with total body involvement.

The sitting problems of cerebral-palsied children with total body involvement are discussed. A moulded seat-insert has been devised which resolves most of these problems. Custom-made for each child, the insert incorporates a head-support and the seat components are connected by hinges to allow some range of movement.

Orthopaedic Management of Cerebral Palsy

Orthopaedic surgery appears to have established a permanent and important place in the of cerebral palsy. So writes Eugene E. Bleck, who, drawing from his understanding and experience, has written what may well prove the definitive review of this subject. The book's format follows the clinical classification of cerebral palsy, with chapters devoted to its three main forms—hemiplegia, diplegia, and total body involvement. A thorough outline of Orthopaedic Assessment (including methods for documenting findings such as photographic recordings and pedographs), a provocative presentation of Neurobiology, Prognosis and Structural Changes, and a sensible outline of Goals and Methods of Treatment, which stresses management tempered by social consideration (The goal of treatment is a healthy functionally independent person, not a permanent patient), round out the text. The anatomy, physiology, and biomechanics of all major spastic deformities are discussed. Clinical examination, indications for surgery, alternative techniques (eg, myoneural block and biofeedback), operative

A phenomenon of rebirth: coming alive in analytic and medical patients.

Instances of phenomena of rebirth in physical and psychological illness have been presented. The phenomenon of rebirth has been discussed from the point of view of total body involvement, including some physiological components of the process. Some aspects of the symbolizing process in rebirth crises have been indicated. The main focus of the paper has been toward understanding the phenomenon of rebirth as a peak emotional experience and as an identity crisis through application of the concepts of the cyclical nature of integrating and disintegrating and of the alienating and dealienating processes. It is hoped that this presentation will stimulate greater awareness of the phenomenon in psychiatric liaison work and in the analytic situation, and will further application of our theory.