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Cerebral palsy (CP) is a motor impairment due to a brain malformation or a brain lesion before the age of two. Spasticity, hypertonus in flexor muscles, dyscoordination and an impaired sensorimotor control are cardinal symptoms. The brain lesion is non-progressive, but the flexor muscles of the limbs will during adolescence become relatively shorter and shorter (contracted), forcing the joints into a progressively flexed position. This will worsen the positions of already paretic and malfunctioning arms and legs. Due to bending forces across the joints, bony malformations will occur, worsening the function even further. Currently, the initial treatment of choice is the use of braces, which diminishes the shortening somewhat, but eventually lengthenings of tendons and release of aponeuroses around the muscles often is needed, and transfers of wrist flexors to wrist extensors may improve wrist position. But the long-term results are unpredictable- how much does the muscle need to be lengthened? What muscles should be transferred for a better position of the wrist, and at what tension? A method to measure sarcomere length in vivo has been developed. The sarcomere, the distance between two striations, is the smallest contractile unit in the striated muscle. When, during surgery, a muscle fiber bundle is transilluminated with a low energy laser light, a diffraction pattern is formed. This diffraction pattern reflects the sarcomere length, and thereby an instant measure of how the stretch of the muscle is obtained. When performing tendon transfers of e.g. wrist flexors to wrist extensors, the setting of the tension of the transfer is arbitrary, and the long-term result is unpredictable. Laser diffraction measurements will give a guide to a precise setting of tension. It is known that there may be pathological changes in muscle in cerebral palsy that also will affect the long-term results of tendon lengthenings and transfers. In order to also take these changes into account, small muscle biopsies will be taken during the same surgeries. These will be examined with immuno-histochemical and biochemical techniques, gel-electrophoresis as well as electron microscopy.
Cerebral palsy (CP) is a motor impairment due to a brain malformation or a brain lesion before the age of two. Spasticity, hypertonus in flexor muscles, dyscoordination and an impaired sensorimotor control are cardinal symptoms. The brain lesion is non-progressive, but the flexor muscles of the limbs will during adolescence become relatively shorter and shorter (contracted), forcing the joints into a progressively flexed position. This will worsen the positions of already paretic and malfunctioning arms and legs. Due to bending forces across the joints, bony malformations will occur, worsening the function even further. Currently, the initial treatment of choice is the use of braces, which diminishes the shortening somewhat, but eventually lengthenings of tendons and release of aponeuroses around the muscles often is needed, and transfers of wrist flexors to wrist extensors may improve wrist position. But the long-term results are unpredictable- how much does the muscle need to be lengthened? What muscles should be transferred for a better position of the wrist, and at what tension? A method to measure sarcomere length in vivo has been developed. The sarcomere, the distance between two striations, is the smallest contractile unit in the striated muscle. When, during surgery, a muscle fiber bundle is transilluminated with a low energy laser light, a diffraction pattern is formed. This diffraction pattern reflects the sarcomere length, and thereby an instant measure of how the stretch of the muscle is obtained. When performing tendon transfers of e.g. wrist flexors to wrist extensors, the setting of the tension of the transfer is arbitrary, and the long-term result is unpredictable. Laser diffraction measurements will give a guide to a precise setting of tension. It is known that there may be pathological changes in muscle in cerebral palsy that also will affect the long-term results of tendon lengthenings and transfers. In order to also take these changes into account, small muscle biopsies will be taken during the same surgeries. These will be examined with immuno-histochemical and biochemical techniques, gel-electrophoresis as well as electron microscopy.
Research questions:
Significance: Children with cerebral palsy have a motor impairment and progressive contractures that we often treat late; when tendon and bony surgery are the only options to realign the joints. More information on the muscle architecture, composition and growth potential will give more knowledge the reasons for increasing flexion contractures in children with cerebral palsy. With this knowledge, hopefully new treatment regimens and improved surgical techniques for this patient group can be developed.
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| No intervention | Other | No intervention |
| Measure | Description | Time Frame |
|---|---|---|
| Sarcomere lenght measurements intraooperatively in vivo with laser diffraction | micrometer | One hour |
| Muscle biopsy structure, fiber area | square mm | one hour |
| Muscle biopsy gene expression and protein content | percentage % | one hour |
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Inclusion Criteria:
Exclusion Criteria:
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Children who due to clinical indications need surgery of the arm or the lower leg. Patients are consecutively recruited.
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Eva M Pontén, MD PhD | Contact | +46706303052 | eva.ponten@ki.se | |
| Ferdinand von Walden, MD PhD | Contact | Ferdinand.vonWalden@ki.se |
| Name | Affiliation | Role |
|---|---|---|
| Eva M Pontén, MD PhD | Karolinska Institutet | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Karolinska University Hospital | Recruiting | Stockholm | 17176 | Sweden |
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| ID | Term |
|---|---|
| D002547 | Cerebral Palsy |
| D003286 | Contracture |
| D009128 | Muscle Spasticity |
| ID | Term |
|---|---|
| D001925 | Brain Damage, Chronic |
| D001927 | Brain Diseases |
| D002493 | Central Nervous System Diseases |
| D009422 | Nervous System Diseases |
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Muscle biopsies taken during surgery
| Karolinska University Hospitla | Recruiting | Stockholm | 17176 | Sweden |
|
| Karolinska | Recruiting | Stockholm | 17176 | Sweden |
|
| D007592 | Joint Diseases |
| D009140 | Musculoskeletal Diseases |
| D009135 | Muscular Diseases |
| D009122 | Muscle Hypertonia |
| D020879 | Neuromuscular Manifestations |
| D009461 | Neurologic Manifestations |
| D012816 | Signs and Symptoms |
| D013568 | Pathological Conditions, Signs and Symptoms |