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PubMed1997

[A pilot investigation into the effects of extracorporeal shock waves on muscular dysfunction in children with spastic movement disorders].

INTRODUCTION: Extracorporeal shock waves (ESW) are being used increasingly frequently for the treatment of narrowly localized pain in the musculoskeletal system. Pain is normally associated with muscular dysfunction. Beginning with the observation that not only is the pain soothed by treatment with ESW, but that the muscular dysfunction itself is relieved, an attempt has been made to treat the impaired function in cases of infantile cerebral palsy with ESW. METHOD: In 35 children and young people with mainly spastic disturbances of movement, the bellies of those muscles producing the flexion contracture of the lower limb were each treated with 500 non-focused pulses of low-energy ESW. The focus (focal pressure 10 MPA=0.06 mJ/mm) of the shock waves itself was placed either within the coupling cushion of the therapy source (Minilith, Storz Medical, CH-Kreuzlingen) or obliquely outside the patient's body. In this way ESW could be dispersed throughout the muscles and the risk of undesirable effects was minimized. The energy used amounted to about 30% of the threshold value at which cellular damage from focused treatment with ESW had been observed in the endothelium of the human umbilical cord. RESULTS: The degree of movement at the hip joint increased after one treatment by an average of 17 degrees, at the knee joint by 12.3 degrees and at the ankle joint by 7.1 degrees, each increase being statistically significant. Muscular stiffness, spastic co-contractions, and myofascial viscoelasticity, as well as dyskinetic and ataxic symptoms, decreased. It appeared as if a systemic action had entered into the motor control. Together with an increase in general proprioception, there was an improvement in body image, in economy of movement and also in the limbs that had not been treated. A few patients showed euphoric reactions for about 2 days after treatment. No undesirable effects were observed during a follow-up of 12 months. The treatment was painless and is therefore suitable for children. The biomechanical improvement remained for several weeks and this time could be used for initiating an improved pattern of movement. CONCLUSION: ESW is suitable for use as an additional instrument for physiotherapeutic treatment of infantile cerebral palsy. The effective action of ESW on disturbed peripheral muscles has been discussed in terms of positive pressure and tensile wave forces and/or forces acting by cavitation. The results obtained in this pilot investigation showed particular a decrease in muscle stiffness. A controlled investigation with gait analysis and psychological control is to be undertaken.

English Abstract↗
PubMed2001

Treatment of functional limitations at the knee in ambulatory children with cerebral palsy.

The gait of ambulatory children with cerebral palsy frequently involves abnormal knee motion. Spasticity, muscle contracture formation, impairments of motor control, weakness, balance deficits, and extrapyramidal motions can all contribute to the functional limitations imposed at the knee. Careful clinical evaluation of the child and their gait must be performed in order to determine the best individual course of treatment. Often, three-dimensional motion analysis with assessment of muscle activity and force is necessary to completely assess the complexities of gait. Several typical gait patterns have been described involving the knee, including 'jump knee', 'crouch', 'true equinus', 'apparent equinus', 'recurvatum' and 'stiff knee' gait. Each of these gait patterns is defined here and discussed using case examples. These typical gait patterns are usually accompanied by involvement at the hip and ankle and may be combined with transverse plane rotational abnormalities. Treatment options such as rehabilitation (physiotherapy, casting, strengthening, and/or orthoses), spasticity management (intramuscular injections of phenol, alcohol, and botulinum toxin type A) and orthopaedic approaches are discussed for each entity.

Biomechanical Phenomena↗
PubMed1976

Knee flexion deformity and patella alta in spastic cerebral palsy.

A study on spastic knee flexion deformity has revealed a recurrence rate of 32 per cent following attempted surgical correction on 170 knees (103 patients). This rate appeared to be unrelated to concomitant surgery for correction of hip and ankle deformity. An additional study demonstrated patella alta in 72 per cent of 100 spastic knees (50 patients) and only 2 per cent and 50 knees of a comparable group of 25 non-spastic patients. It is suggested that restoration of a normal patello-femoral relationship may reduce both the functional disability and recurrence rate of spastic knee flexion deformity when this disease is managed surgically.

Cerebral Palsy↗
PubMed1998

Musculoskeletal modelling in determining the effect of botulinum toxin on the hamstrings of patients with crouch gait.

This study aimed to determine the effect of hamstring botulinum toxin A (Btx-A) injection in 10 children with crouch gait in terms of changes in muscle length and lower-limb kinematics. Before Btx-A injection limb kinematics were recorded. Maximum hamstring lengths and excursions were calculated by computer modelling of the lower limb. Data were compared with the averaged hamstring lengths of 10 control children. Hamstrings were defined as short if their length was shorter than the average maximum length minus one standard deviation. Gait analysis was repeated 2 weeks after isolated hamstring Btx-A injection. Pre- and postinjection kinematic data and muscle lengths were then compared. Four of 18 injected limbs in three subjects had short medial hamstring before injection, none of the subjects had short lateral hamstrings. Muscle excursion was significantly reduced in the short and adequate maximum muscle length groups. A significant increase in the semimembranosus and semitendinosus length in all of the injected limbs was noted. Only in the short muscle group was a significant increase in muscle excursion observed. Knee extension improved by 13 degrees in the adequate muscle length group and by 15.6 degrees in the short muscle length group. Pelvic tilt and hip flexion increased in both groups non-significantly. Average walking speed postinjection increased from 0.60 ms(-1) to 0.71 ms(-1). Short hamstrings are over-diagnosed in crouch gait. Hamstring Btx-A injection in patients with crouch gait produces significant, repeatable muscle lengthening and improved ambulatory function.

Botulinum Toxins, Type A↗
PubMed2004

[Knee-bending and -stretching-spastic in infant cerebral palsy. Surgery aimed at functional improvement and its results].

Any treatment of disorders of the knee in patients with cerebral palsy is a complex task in terms of indications and surgical technique. Surgical management has the potential to considerably improve function in walkers as well as tetraparetic patients. The close functional relationship between the knee joint and the adjacent hip and ankle joints requires an integrated view. An increased anterior pelvic tilt aggravates any shortening of the hamstring muscles. An equinus foot deformity increases knee extensor movement. When evaluating postoperative results, it is never sufficient to indicate the postoperative range of motion only. Instead, functional evaluation criteria complemented by instrumented gait analysis must be used to define the results more quickly and to uncover postoperative problems.

Cerebral Palsy↗
PubMed1975

Insufficiency and stress fractures of the long bones occurring in patients with rheumatoid arthritis.

The occurrence of stress and insufficiency fractures in patients with long-standing rheumatoid arthritis has not been sufficiently emphasized. Osteoporosis due to rheumatoid arthritis and corticosteroid therapy, joint stiffness, contracture, and angular deformity of the extremity, and the unaccustomed exercise after reconstructive joint surgery combine to predispose to the occurrence of these fractures in these patients. The pain and disability caused by these fracture is often attributed to rheumatoid joint involvement. The fracture may not be visible on radiographs obtained near the onset of symptoms, and serial examinations may be needed for diagnosis.

Adult↗
PubMed1993

Determinants of muscle function in the spastic lower extremity.

The upper motor neuron lesion that causes hemiplegia impairs the patient's selective control and exposes primitive modes of muscle activation. Significant inconsistency between the clinical findings and the patient's gait may result. Dynamic electromyography revealed the primitive mechanisms leading to these inconsistencies. The rate of stretch does not differentiate spasticity from contracture, since either a quick or slow stretch frequently causes a sustained muscle response. Using knee flexion to differentiate gastrocnemius and soleus spasticity is not reliable, since the change in neurologic input with flexion may inhibit the extensor muscle's response to stretch so that the soleus is also relaxed. The change in body position from lying supine to sitting can double the intensity of soleus spasticity, and standing further increases the tone. Primitive patterns of mass extension and flexion, while voluntary, inhibit normal progression during walking. Simultaneous activation of the soleus and gastrocnemius with the knee's quadriceps causes premature ankle plantar flexion as the limb is loaded in stance. The primitive flexion synergy between the hip, knee, and ankle (dorsiflexion) inhibits terminal swing knee extension while the hip remains flexed. Consequently, surgical planning for the hemiparetic limb must rely heavily on gait analysis findings (systematic observation or by instrumentation).

Adolescent↗
PubMedDate not supplied

[The effect of cryo- and cryoelectrotherapy on neuromuscular function in coxarthrosis patients].

Cryotherapy (CT) and cryoelectrotherapy (CET) in combination with therapeutic exercise were assessed for neuromuscular effects in patients with coxarthritis and necrosis of caput femoris. The above complex is found effective in reducing the rigidity of the muscles and joints, in attenuation of functional contractures. It increases muscle strength nearby the affected joints. The best results were achieved with CET. Single CT and CET procedures increase the amplitude of muscular bioelectrical activity at maximal muscular tension, the highest effect being observed at the sites of the direct exposure. Marked analgetic activity of the physical factors was confirmed by electroneuromyographic findings.

Combined Modality Therapy↗
PubMedDate not supplied

Relationship between dynamic deformities and joint moments in children with cerebral palsy.

A relationship between dynamic flexion-extension motion ranges during walking and the magnitude of flexion and extension moments was analyzed for 38 ambulatory children with spastic cerebral palsy. Moments tending to flex the hip and the knee were more than a factor of two times greater than those occurring during normal gait. At the knee, the magnitude of the moments was related to the amount of flexion deformity, whereas at the hip there was no correlation between moment magnitude and extension motion range. The maximum dorsiflexion and plantarflexion ankle moments were of less than normal value in all deformity groupings. An understanding of the longterm influence of these abnormal moments on the terms of the progression or stabilization of the dynamic deformities during walking is an important step toward improving our understanding of and, potentially, our ability to treat patients with cerebral palsy.

Adolescent↗
PubMed2005

[Analysis of the expression of collagen VI in congenital muscular dystrophy].

Congenital muscular dystrophy (CMD) composes a group of disorders characterized by hypotonia and muscular weakness noticed in the first year of life. The Ullrich's form is characterized by proximal joint contractures and distal hiperextensibility. About 40% of these patients present mutations in one of the genes that codify the sub-units of the collagen VI protein (COL6), producing total or partial deficiency of the protein expression. We analyzed, through immunohistochemistry, the expression of COL6 in muscle fragments of 50 patients with CMD; 20 of them presented merosin expression deficiency. We identified 4 cases with total COL6 deficiency (8% of the total), representing 13% of the cases with normal merosin expression. The histological findings of patients with deficiency of COL6 were indistinguishable from other forms of CMD, but milder than that abnormalities observed in merosin deficient patients. In three COL6 deficient patients were observed hypotonia and weakness in the neonatal period, delayed of motor milestones, muscular retractions of knees and elbows, distal joint hiperextensibility and congenital hip dislocation (two patients). One patient lost the ability to walk; and one died due to respiratory problems. The analysis of COL6 expression, as well as merosin expression, in the muscle tissue from CMD patients, can be important for identification and phenotypic characterization of different CMD subtypes.

Biomarkers↗
PubMed2007

Multisystem Proteinopathy

CLINICAL CHARACTERISTICS: Multisystem proteinopathy (MSP) is a genetically heterogeneous, multisystem degenerative disorder characterized by adult-onset proximal and distal muscle weakness (clinically resembling a limb-girdle muscular dystrophy syndrome), early-onset Paget disease of bone (PDB), and premature frontotemporal dementia (FTD). Muscle weakness progresses to involve other limb and respiratory muscles. PDB involves focal areas of increased bone turnover that typically lead to spine and/or hip pain and localized enlargement and deformity of the long bones; pathologic fractures occur on occasion. Early stages of FTD are characterized by dysnomia, dyscalculia, comprehension deficits, and paraphasic errors, with minimal impairment of episodic memory; later stages are characterized by inability to speak, auditory comprehension deficits for even one-step commands, alexia, and agraphia. Mean age at diagnosis for muscle disease is 43 years, PDB is 41 years, and FTD is 56 years. Dilated cardiomyopathy, amyotrophic lateral sclerosis, and Parkinson disease are now known to be part of the spectrum of findings associated with MSP. DIAGNOSIS/TESTING: The diagnosis of MSP is established in a proband with typical clinical findings and a heterozygous pathogenic variant in VCP, HNRNPA1, HNRNPA2B1, or SQSTM1 identified by molecular genetic testing. MANAGEMENT: Treatment of manifestations: Weight control to avoid obesity; physical therapy and stretching exercises to promote mobility and prevent contractures; occupational therapy and mechanical aids (canes, walkers, orthotics, wheelchairs) as needed for ambulation/mobility; surgical intervention for foot deformity and scoliosis as needed; respiratory aids when indicated; assisted living arrangements for muscle weakness and/or dementia; bisphosphonates to relieve pain and disability from PDB; social and emotional support; education regarding safety precautions. Surveillance: Echocardiogram and electrocardiogram with repeat cardiac evaluation every two to three years or earlier if symptomatic; annual pulmonary function studies; sleep studies as clinically indicated; annual alkaline phosphatase measurement; skeletal imaging as indicated for evaluation of PDB; neurologic and neuropsychological assessment every two to three years or more frequently as needed; multidisciplinary monitoring for respiratory, cardiac, musculoskeletal, and cognitive decline. GENETIC COUNSELING: MSP is inherited in an autosomal dominant manner. Most individuals diagnosed with MSP have an affected parent. Estimates based largely on VCP-MSP suggest that approximately 5% of individuals have a de novo pathogenic variant. Each child of an individual with MSP has a 50% chance of inheriting the MSP-related pathogenic variant. Marked intrafamilial variability may be observed among heterozygous family members, including differences in age at onset, severity, rate of progression, and the specific combination of manifestations. Once the MSP-related pathogenic variant has been identified in an affected family member, predictive testing for at-risk family members and prenatal/preimplantation genetic testing are possible.

Inclusion Body Myopathy with Early-Onset Paget Dis↗
PubMed1993

Simultaneous multiple operations for spastic diplegia. Outcome and functional assessment of walking in 18 patients.

We assessed the outcome after simultaneous multiple operations performed on 18 children with spastic diplegia, with emphasis on the changes in the physiological cost index (PCI) of walking. Fourteen patients had a measurable reduction at one year, but the more severely affected patients took up to two years to reach a new functional plateau. The level of the preoperative PCI allows prediction of the outcome of surgery in terms of reducing the effort of walking, or improving its appearance only. Intrapelvic intramuscular psoas tenotomy produced an improvement of hip flexion deformity in 15 of 17 patients without the loss of muscle power to initiate the swing phase. Fractional lengthening corrected hamstring tightness in 17 cases, and the mean popliteal angle was reduced from 63 degrees preoperatively to 30.2 degrees, with almost complete resolution of the fixed knee flexion deformity present in ten patients. Distal transfer of the rectus femoris, when it was shown to be contracting inappropriately, improved the knee flexion arc during walking from a mean of 28.3 degrees to 45.2 degrees.

Adolescent↗
PubMed1986

[Determination of rotational defects of the femur by computer tomographic determination of the antetorsion angle of the femoral neck].

The first of all unexplained but persisting static complaints after femoral fractures are due to rotational deformities. The clinical examination can only sustain the suspicion of a rotational deformity but can not determine its exact extent. The usual X-ray determination of the anteversion angle in the technique of Dunn and Rippstein as a measure for torsional deformities is often very difficult, because of the exact positioning conditions of patients with posttraumatic axial deformities, soft tissue contractures and painful motility restrictions. With the total body computertomography we have a safe and quick technique, independent of the patient's positioning, to determine the anteversion angle without problems. In comparing the two sides, the rotational deformity can be recognized and its extent can exactly be measured. Because of the simple technique and the exact determination of rotatory deformity a computertomographic measuring of the anteversion of the femoral head always should be made before planning a corrective osteotomy to fix the extent of correction, whenever a total body scan is available.

Adult↗
PubMed1993

Mucolipidosis III presenting as a rheumatological disorder.

Genetic disorders of the osseous skeleton constitute a challenging area of differential diagnosis. Three patients, 2 girls and 1 boy, were referred to our pediatric rheumatology clinic because of progressive stiffness of their hands and flexion contractures of fingers, accompanied by additional musculoskeletal changes. One of the girls had been diagnosed in early childhood as having juvenile rheumatoid arthritis, the other was suspected to have scleroderma, and the boy had been labelled with "Hurler's syndrome." On evaluation, all 3 patients had the clinical and roentgenographic features of mucolipidosis III; the diagnosis of mucolipidosis III was confirmed by enzymatic assays. We call for an awareness of pediatricians, rheumatologists and orthopedic surgeons to the "rheumatological" presentation of mucolipidosis III. The establishment of a precise diagnosis will lead to adequate management and will allow appropriate genetic counseling.

Arthrography↗
PubMed2006

Correction of severe crouch gait in patients with spastic diplegia with use of multilevel orthopaedic surgery.

BACKGROUND: Severe crouch gait in patients with spastic diplegia causes excessive loading of the patellofemoral joint and may result in anterior knee pain, gait deterioration, and progressive loss of function. Multilevel orthopaedic surgery has been used to correct severe crouch gait, but no cohort studies or long-term results have been reported, to our knowledge. METHODS: In order to be eligible for the present retrospective cohort study, a patient had to have a severe crouch gait, as defined by sagittal plane kinematic data, that had been treated with multilevel orthopaedic surgery as well as a complete clinical, radiographic, and instrumented gait analysis assessment. The surgical intervention consisted of lengthening of contracted muscle-tendon units and correction of osseous deformities, followed by the use of ground-reaction ankle-foot orthoses until stable biomechanical realignment of the lower limbs during gait was achieved. Outcome at one and five years after surgery was determined with use of selected sagittal plane kinematic and kinetic parameters and valid and reliable scales of functional mobility. Knee pain was recorded with use of a Likert scale, and all patients had radiographic examination of the knees. RESULTS: Ten subjects with severe crouch gait and a mean age of 12.0 years at the time of surgery were studied. After surgery, the patients walked in a more extended posture, with increased extension at the hip and knee and reduced dorsiflexion at the ankle. Pelvic tilt increased, and normalized walking speed was unaltered. Knee pain was diminished, and patellar fractures and avulsion injuries healed. Improvements in functional mobility were found, and, at the time of the five-year follow-up, fewer patients required the use of wheelchairs or crutches in the community than had been the case prior to intervention. CONCLUSIONS: Multilevel orthopaedic surgery for older children and adolescents with severe crouch gait is effective for relieving stress on the knee extensor mechanism, reducing knee pain, and improving function and independence.

Adolescent↗
PubMed1975

Ectopic calcification around joints of paralysed limbs in hemiplegia, diffuse brain damage, and other neurological diseases.

Mechanical deformity associated, with neurological disease is commonly encountered in the form of contractures, or pre-existing arthritis. Less common is the occurrence of ectopic calcification around the joints of a paralysed limb. Two forms of this are presented in a series of twenty patients. Para-articular calcification with the appearance of myositis ossificans around large joints of the affected limbs occurred most often. The interior of the joints was not affected, nor was there evidence of bony injury. In some cases, however, the excessive outgrowth of bone around the joint eventually resulted in a functional ankylosis by a uniting bar of bone outside the joint. The second form of calcification was periosteal, which unlike the first type was resorbed and left an area of cortical bony thickening.

Aged↗
PubMed2005

Anterolateral thigh flap reconstruction of large external facial skin defects: a follow-up study on functional and aesthetic recipient- and donor-site outcome.

BACKGROUND: The purpose of this study was to investigate the subjective and the objective functional and aesthetic follow-up results of the recipient and donor sites after reconstruction of extensive facial defects with the anterolateral thigh flap. METHODS: Between December of 2001 and April of 2003, the anterolateral thigh flap was used to reconstruct large facial skin defects after malignant tumor resection in 23 white patients. All patients had a standardized interview, physical examination, and clinical photographs. RESULTS: The mean flap size was 108 cm2. Fasciocutaneous anterolateral thigh flaps were used in 15 patients and musculocutaneous flaps were used in eight patients with exposed dura, open sinuses, or orbital defects. An extra free osteocutaneous fibula flap was necessary to reconstruct the affected mandible in 10 patients. The donor site was skin grafted in 18 patients. The flap survival rate was 96 percent. At follow-up, color mismatch (71 percent) and flap bulkiness (50 percent) were encountered most often. Four of five patients with speech problems had received an additional free osteocutaneous fibula flap. Three flap contractures were seen in the neck region. A contour defect of the upper leg was encountered in five patients. Sensory disturbances of the upper leg were observed in 12 patients. Cold intolerance occurred three times after skin grafting. No significant impairment was found in range of motion and muscle strength of the donor leg. CONCLUSIONS: Careful patient selection may further improve aesthetic outcome of the anterolateral thigh flap. The versatility in design and composition of the anterolateral thigh flap and the low donor-site morbidity and satisfactory recipient-site outcome make it a valuable option in reconstruction of external skin defects in the head and neck region.

Aged↗