The Pathophysiological Basis of Weakness in Children With Cerebral Palsy

Purpose: To examine the evidence concerning the neurologic and muscular pathophysiology that contributes to clinically observed weakness in children and young people with cerebral palsy (CP). Method: Literature concerning the neural or muscular changes in subjects with CP was found by searching 6 databases plus supplementary searching. Results: A final set of 51 articles was identified by 2 independent reviewers. Summary of Key Points: Muscle weakness is due to reduced central drive, possible abnormal neural maturation, insufficient and disorganized motor recruitment, impaired voluntary control, impaired reciprocal inhibition, altered setting of muscle spindles, and reinforcement of abnormal neural circuits. Muscle tissue is altered, with selective atrophy of fast fibers and altered myosin expression, changes in fiber length and cross-sectional area, changes in the length-tension curve, reduced elasticity, and impoverished muscle tissue development. Conclusion: Children with CP are weak because of both neurologic and muscular changes.

[1]  C Tardieu,et al.  Muscle hypoextensibility in children with cerebral palsy: I. Clinical and experimental observations. , 1982, Archives of physical medicine and rehabilitation.

[2]  Richard L Lieber,et al.  Spastic muscle cells are shorter and stiffer than normal cells , 2003, Muscle & nerve.

[3]  Overstretching of sarcomeres may not cause cerebral palsy muscle contracture , 2004, Journal of orthopaedic research : official publication of the Orthopaedic Research Society.

[4]  Jack R Engsberg,et al.  Relation between spasticity and strength in individuals with spastic diplegic cerebral palsy , 2002, Developmental medicine and child neurology.

[5]  Richard L Lieber,et al.  Structural and mechanical alterations in spastic skeletal muscle. , 2005, Developmental medicine and child neurology.

[6]  Robyn Gardiner The pathophysiological and clinical implications of neuro-muscular changes following cerebrovascular accident. , 1996, The Australian journal of physiotherapy.

[7]  J. Rose,et al.  Muscle pathology and clinical measures of disability in children with cerebral palsy , 1994, Journal of orthopaedic research : official publication of the Orthopaedic Research Society.

[8]  Relation between muscle thickness, spasticity, and activity limitations in children and adolescents with cerebral palsy , 2008, Developmental medicine and child neurology.

[9]  C L Vaughan,et al.  MUSCLE RESPONSE TO HEAVY RESISTANCE EXERCISE IN CHILDREN WITH SPASTIC CEREBRAL PALSY , 1995, Developmental medicine and child neurology.

[10]  Kazuhiko Cho,et al.  Muscle histopathology in spastic cerebral palsy , 1996, Brain and Development.

[11]  R. Lieber,et al.  Inferior mechanical properties of spastic muscle bundles due to hypertrophic but compromised extracellular matrix material , 2003, Muscle & nerve.

[12]  B. Bobath Motor development, its effect on general development, and application to the treatment of cerebral palsy. , 1971, Physiotherapy.

[13]  S. Amor,et al.  Disorders of the neuromuscular junction , 2011, Journal of Neurology.

[14]  J. Engsberg,et al.  Maximum Active Resultant Knee Joint Torques in Children With Cerebral Palsy , 1998 .

[15]  F. Haddad,et al.  highlighted topics Plasticity in Skeletal, Cardiac, and Smooth Muscle Invited Review: Effects of different activity and inactivity paradigms on myosin heavy chain gene expression in striated muscle , 2000 .

[16]  A. Bakheit Management of Muscle Spasticity , 1996 .

[17]  T Sinkjaer,et al.  Passive, intrinsic and reflex-mediated stiffness in the ankle extensors of hemiparetic patients. , 1994, Brain : a journal of neurology.

[18]  G. Cioni,et al.  Immunohistochemical study of muscle biopsy in children with cerebral palsy , 2002, Brain and Development.

[19]  G C Elder,et al.  Improved ankle function in children with cerebral palsy after computer‐assisted motor learning , 1998, Developmental medicine and child neurology.

[20]  S. Õunpuu,et al.  Kinematic and Kinetic Evaluation of the Ankle After Lengthening of the Gastrocnemius Fascia in Children with Cerebral Palsy , 1993, Journal of pediatric orthopedics.

[21]  B. Dan,et al.  A report: the definition and classification of cerebral palsy April 2006 , 2007, Developmental medicine and child neurology. Supplement.

[22]  Mati Pääsuke,et al.  Quadriceps femoris muscle voluntary force and relaxation capacity in children with spastic diplegic cerebral palsy. , 2008, Pediatric exercise science.

[23]  Richard W. Bohannon Is the measurement of muscle strength appropriate in patients with brain lesions? A special communication. , 1989, Physical therapy.

[24]  F. Stanley,et al.  Intrauterine growth and spastic cerebral palsy II. The association with morphology at birth. , 1992, Early human development.

[25]  C Andersson,et al.  Adults with cerebral palsy: a survey describing problems, needs, and resources, with special emphasis on locomotion , 2001, Developmental medicine and child neurology.

[26]  S. Farmer Key factors in the development of lower limb co-ordination: implications for the acquisition of walking in children with cerebral palsy , 2003, Disability and rehabilitation.

[27]  Jing-Xia Liu,et al.  Myosin Heavy Chain Composition of Muscle Spindles in Human Biceps Brachii , 2002, The journal of histochemistry and cytochemistry : official journal of the Histochemistry Society.

[28]  C. Buckon,et al.  Objective measurement of muscle strength in children with spastic diplegia after selective dorsal rhizotomy. , 2002, Archives of physical medicine and rehabilitation.

[29]  Kevin W. Hollander,et al.  Hip Spasticity and Strength in Children with Spastic Diplegia Cerebral Palsy , 2000 .

[30]  L. Leahey,et al.  Contributing factors to muscle weakness in children with cerebral palsy , 2003, Developmental medicine and child neurology.

[31]  L. Brain The Nervous System , 1963, Nature.

[32]  Neil Armstrong,et al.  Assessment and Interpretation of Isokinetic Muscle Strength During Growth and Maturation , 2003, Sports medicine.

[33]  J. Engsberg,et al.  Spasticity and strength changes as a function of selective dorsal rhizotomy. , 1998, Neurosurgical focus.

[34]  J. Marques Lower-extremity strength profiles in spastic cerebral palsy. , 2002, Pediatric physical therapy : the official publication of the Section on Pediatrics of the American Physical Therapy Association.

[35]  M. Abel,et al.  Spasticity versus strength in cerebral palsy: relationships among involuntary resistance, voluntary torque, and motor function , 2001, European journal of neurology.

[36]  L. Lefaucheur,et al.  Low birth weight is associated with enlarged muscle fiber area and impaired meat tenderness of the longissimus muscle in pigs. , 2006, Journal of animal science.

[37]  P. Raina,et al.  Prognosis for gross motor function in cerebral palsy: creation of motor development curves. , 2002, JAMA.

[38]  H. Sarnat Cerebral dysgeneses and their influence on fetal muscle development , 1986, Brain and Development.

[39]  H. Goh,et al.  Relationships among Measures of Knee Musculoskeletal Impairments, Gross Motor Function, and Walking Efficiency in Children with Cerebral Palsy , 2006, Pediatric physical therapy : the official publication of the Section on Pediatrics of the American Physical Therapy Association.

[40]  J. Eng Strength Training in Individuals with Stroke. , 2004, Physiotherapy Canada. Physiotherapie Canada.

[41]  T N Theologis,et al.  Collagen accumulation in muscles of children with cerebral palsy and correlation with severity of spasticity. , 2001, Developmental medicine and child neurology.

[42]  R. Kearney,et al.  Intrinsic and reflex stiffness in normal and spastic, spinal cord injured subjects , 2001, Experimental Brain Research.

[43]  L. Ada,et al.  Spasticity: Research Findings and Implications for Intervention , 1995 .

[44]  V. Calvisi,et al.  Histological and morphological aspects of muscle in infantile cerebral palsy. , 1989, Italian journal of orthopaedics and traumatology.

[45]  Adam P Shortland,et al.  The morphology of the medial gastrocnemius in typically developing children and children with spastic hemiplegic cerebral palsy. , 2007, Journal of electromyography and kinesiology : official journal of the International Society of Electrophysiological Kinesiology.

[46]  J R Engsberg,et al.  Ankle spasticity and strength in children with spastic diplegic cerebral palsy , 2000, Developmental medicine and child neurology.

[47]  Richard L Lieber,et al.  Structural and functional changes in spastic skeletal muscle , 2004, Muscle & nerve.

[48]  J. Rose,et al.  The motor unit in cerebral palsy , 1998, Developmental medicine and child neurology.

[49]  C. Reggiani,et al.  Molecular diversity of myofibrillar proteins: gene regulation and functional significance. , 1996, Physiological reviews.

[50]  D. Damiano,et al.  Muscle thickness reflects activity in CP but how well does it represent strength? , 2008, Developmental medicine and child neurology.

[51]  Richard L Lieber,et al.  Spasticity causes a fundamental rearrangement of muscle–joint interaction , 2002, Muscle & nerve.

[52]  Richard L. Lieber,et al.  Skeletal Muscle Structure, Function, and Plasticity , 2009 .

[53]  P. Huijing,et al.  Spastic muscle properties are affected by length changes of adjacent structures , 2005, Muscle & nerve.

[54]  K. Stokes Skeletal muscle: from molecules to movement , 2006, British Journal of Sports Medicine.

[55]  G. Margaritondo,et al.  Reactivity of Au with ultrathin Si layers: A photoemission study , 2001 .

[56]  J. Rose,et al.  Promotion of Physical Fitness and Prevention of Secondary Conditions for Children With Cerebral Palsy: Section on Pediatrics Research Summit Proceedings , 2007, Physical Therapy.

[57]  D. Damiano,et al.  Should we be testing and training muscle strength in cerebral palsy? , 2002, Developmental medicine and child neurology.

[58]  Stuart A Binder-Macleod,et al.  Voluntary muscle activation, contractile properties, and fatigability in children with and without cerebral palsy , 2005, Muscle & nerve.

[59]  J. Carey,et al.  Movement dysfunction following central nervous system lesions: a problem of neurologic or muscular impairment? , 1993, Physical therapy.

[60]  P D Neilson,et al.  Mechanisms of Muscle Growth Related to Muscle Contracture in Cerebral Palsy , 1989, Developmental medicine and child neurology.

[61]  M. Abel,et al.  Deficits in eccentric versus concentric torque in children with spastic cerebral palsy. , 2001, Medicine and science in sports and exercise.

[62]  R. Herbert The passive mechanical properties of muscle and their adaptations to altered patterns of use. , 1988, The Australian journal of physiotherapy.