| Introduction |
 Nonprogressive upper motor neuron disease (static encephalopathy) due to injury to immature brain
- by definition onset must be before first two years of life
- leads to muscle imbalance with a mixture of weakness and spasticity
- the encephalopathy is static while the affected portion of the musculoskeletal system changes with growth
- Cause is usually not identifiable. Some identifiable causes include
- prematurity (most common)
- anoxic injuries
- prenatal intrauterine factors
- perinatal infections (toxoplasmosis, rubella, cytomegalovirus infection, herpes simplex, ToRCH)
- meningitis
- brain malformations
- Characterized by the following orthopaedic manifestations
- primary
- abnormal tone
- loss of motor control
- impaired balance
- secondary (growth and spasticity related)
- muscles contractures
- bony deformities
- joint subluxation and dislocation
- scoliosis
- foot deformities
- Prognosis
- most reliable predictor for ability to walk is independent sitting by age 2
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| Classification |
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Physiologic Classification
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| Spastic (most common) |
Velocity-dependent increased muscle tone and hyperreflexia with slow restricted movement due to simultaneous contraction of agonist and antagonist muscles. Most amenable to operative treatments. |
| Athetoid |
Characterized by constant succession of slow, writhing, involuntary movements |
| Ataxic |
Characterized by inability to coordinate muscle movements. Results in unbalanced wide based gait. |
| Mixed |
Usually mixed spastic and athetoid features and involves the entire body |
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Anatomic Classification
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| Quadriplegic |
Total body involvement and nonambulatory with a low IQ and a high mortality |
| Diplegic |
Legs more than arms but usually still ambulatory. IQ may be normal (injury in brain is midline)  |
| Hemiplegic |
Arms and legs on one side of the body, usually with spasticity; all will eventually be able to walk, regardless of treatment
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| Gross Motor Function Classification Scale (GMFCS) |
| Level I |
Near normal gross motor function, independent ambulator |
| Level II |
Walks independently, but with limitations |
| Level III |
Dependent ambulator |
| Level IV |
Minimal walking ability, uses adult assisted or powered devices for community ambulation |
| Level V |
Nonambulator with global involvment, dependent in all aspects of care |
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| Evaluation |
- History
- clinical history
- perinatal history
- growth & development
- prior medical treatments
- functional status
- nutritional status
- respiratory function
- sitting/standing posture
- upper and lower extremities function
- communication skills
- acuity of hearing and vision
- Physical Exam
- musculoskeletal exam
- motion, tone, and strength
- hamstring contractures (lead to decreased lumbar lordosis)
- hip contractures (lead to excessive lumbar lordosis)
- spine exam
- look at flexibility of curve
- spinal balance and shoulder height
- pelvic obliquity
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| Imaging |
- MRI
- MRI of brain shows periventricular leukomalacia
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| General Treatment |
- Nonoperative
- physical therapy, bracing/orthotics, and medications for spasticity
- spasticity control
- Botox (botulinum - A toxin)
- competitive inhibitor of presynaptic cholinergic receptors with a finite lifetime (usually lasts 2-3 months)
- used to maintain joint motion during rapid growth when a child is too young for surgery
- often injected into gastrocnemius
- baclofen
- reduces tone via unknown mechanism
- thought to act as GABA agonist
- intra-thecal administration is preferred route to avoid cognitive impairment seen with oral administration
- Operative
- general
- surgery to improve function should be considered in a child >3 years old with spasticity and voluntary muscle control
- multi-level soft tissue procedures
- indications
- perform early (< 5 years of age)
- techniques
- tenotomies for continuously active muscles (e.g. hip adductor)
- tendon lengthening for continuously active muscles (e.g. achilles tendon or hamstring)
- tendon transfers for muscles firing out of phase (e.g. rectus tendon or tibialis posterior)
- tendon transfers in the upper extremity show the best improvement in function in patients with voluntary motor control
- bony procedures (pelvic osteotomies, scoliosis surgery)
- indications
- done later in life ( > 5 years of age)
- rhizotomy
- neurosurgical resection of dorsal rootlets that do not show a myographic or clinical response to stimulation
- indications
- ages 4 to 8, ambulatory spastic diplegia, and a stable gait pattern that is limited by lower extremity spasticity
- contraindications
- athetoid CP
- nonambulatory patients with spastic quadriplegia (associated with significant spinal deformities)
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| Fractures |
- Introduction
- associated with non-ambulators secondary to low bone mineral density
- Treatment
- bisphosphonates
- IV pamidronate can be considered in patients with >3 fractures and a DEXA z-score <2 SD
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| Upper Extremity Conditions |
- See Cerebral Palsy Upper Extremity Disorders
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| Spine Disorders |
- See Cerebral Palsy Spine Disorders
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| Hip Conditions |
- See Cerebral Palsy Hip Conditions
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| Gait disorders |
- See Cerebral Palsy Gait disorders
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| Foot Deformities |
- Introduction
- treatment goal - obtain a plantigrade, painless, braceable foot
- foot deformities are common in cerebral palsy and may take several forms including
- Equinovalgus
- Equinovarus
- Cavus foot & Hallux clawing
- Hallux Valgus
- treatment
- first metatarsophalangeal joint arthrodesis
- has the highest overall success rate compared to other surgeries in ambulatory and nonambulatory children with cerebral palsy.
- the recurrence rate is unacceptably high with the other procedures listed above
- proximal phalanx (Akin) osteotomy
- indicated when there is valgus interphalangeus associated with hallux valgus
- should be treated at the same time as the hallux valgus
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Please Rate Educational Value!
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3.0
t-4084
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Average 3.0 of 12 Ratings
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Qbank (3 Questions)
TAG
(OBQ09.171)
In patients with cerebral palsy, voluntary control of motion best predicts improvement in function after which of the following?
Review Topic
DISCUSSION:
Tendon transfer surgery is used to maximize function in patients with cerebral palsy.
Van Heest et al. (1999) evaluated 134 patients where 718 procedures were performed in the upper extremities. Treatment was tailored to each child's needs and included soft tissue releases of deforming spastic muscles, tendon transfers to augment antagonistic activity, and joint stabilization. Using a 9-level functional use score, the authors showed an average improvement of 2.6 functional levels for all patients. Patients with good voluntary control had the greatest improvement in functional use scores.
The Van Heest (2003) review discusses how appropriate tendon release/transfer can improve motor function in children with cerebral palsy.
REFERENCES:
1.
Van Heest AE, House JH, Cariello C. Upper extremity surgical treatment of cerebral palsy. J Hand Surg Am. 1999 Mar;24(2):323-30.
PMID:10194018 (Link to Abstract)
2.
Van Heest AE. Surgical management of wrist and finger deformity. Hand Clin.2003 Nov;19(4):657-65. Review.
PMID:14596556 (Link to Abstract)
|
Please Rate Educational Value!
|
3.0
q-2984
|
Average 3.0 of 19 Ratings
|
TAG
(OBQ09.266)
Which of the following decreases acetylcholine levels in the synaptic cleft by blocking the presynaptic release of acetylcholine?
Review Topic
DISCUSSION:
Decreased acetylecholine levels in the synaptic cleft weaken the strength of muscle contraction and are used in the treatment of spastic muslce disorders like cerebral palsy. While botulinum toxin A, acetylcholinesterase, and baclofen all decrease muscular contraction, only botulinum toxin A does so by blocking the presynaptic release of acetylcholine. Baclofen acts as a Gamma-aminobutyric acid (GABA) agonist. Gamma-aminbutyric acid blocks both the presynaptic and postsynaptic release of acetylcholine. Acetylcholinesterase is an enzyme that degrades acetylcholine. Pyridostigmine blocks the function of acetylcholinesterase, thus increasing acetylcholine levels. Finally lidocaine blocks the voltage gated sodium channels, unrelated to acetylcholine metabolism. Abbruzzese and Berardelli review the mechanism of action, treatment options, and side effects of botulinum toxin type A. Das and Park treated 6 patients with spasticity due to stroke-related hemiplegia with botulinum toxin type A injection into skeletal muscle. The patients showed subjective and objective improvement without any significant side-effects.
REFERENCES:
1.
Abbruzzese G, Berardelli A. Neurophysiological effects of botulinum toxin type A. Neurotox Res. 2006 Apr;9(2-3):109-14. Review.
PMID:16785106 (Link to Abstract)
2.
Das TK, Park DM. Effect of treatment with botulinum toxin on spasticity. Postgrad Med J. 1989 Apr;65(762):208-10.
PMID:2594595 (Link to Abstract)
|
Please Rate Educational Value!
|
4.0
q-3079
|
Average 4.0 of 12 Ratings
|
Cases
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HPI -
Fixed Flexion deformity bilateral knee joints since birth
What should be the treatment plan
|
3/14/2013
115
responses
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See More Cases
Groups
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Van Heest AE, House JH, Cariello C. Upper extremity surgical treatment of cerebral palsy. J Hand Surg Am. 1999 Mar;24(2):323-30.
PMID:10194018 (Link to Abstract)
-
Das TK, Park DM. Effect of treatment with botulinum toxin on spasticity. Postgrad Med J. 1989 Apr;65(762):208-10.
PMID:2594595 (Link to Abstract)
Level of Evidence 5 and Other Journal Articles (includes Case Reports, Expert Opinions,
Personal Observations, and Biomechanic Studies)
-
Abbruzzese G, Berardelli A. Neurophysiological effects of botulinum toxin type A. Neurotox Res. 2006 Apr;9(2-3):109-14. Review.
PMID:16785106 (Link to Abstract)
-
Van Heest AE. Surgical management of wrist and finger deformity. Hand Clin.2003 Nov;19(4):657-65. Review.
PMID:14596556 (Link to Abstract)
Textbooks
- Review of Orthopaedics, 6th Edition, Mark D. Miller MD, Stephen R. Thompson MBBS MEd FRCSC, Jennifer Hart MPAS PA-C ATC, an imprint of Elsevier, Philadelphia, Copyright 2012
- AAOS Comprehensive Orthopaedic Review, Jay R. Leiberman. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2009
- Orthopaedic Knowledge Update 10, John M Flyn. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2011
- Hoppenfeld SP. Surgical Exposures in Orthopaedics: The Anatomic Approach. Lipponcott, Williams, and Wilkins, Philadelphia, PA, Copyright 2009
- Orthopaedic In-training Examination (OITE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012
- Self-Assessment Examination (SAE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012
Undefined
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OKU 8 p164
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Renshaw, TS: Cerebral Palsy, in Morrissy RT, Weinstein SL (eds): Lovell and Winter’s Pediatric Orthopaedics, ed. 5. Philadelphia, PA, Lippencott Williams & Wilkins, 2001, pp563-99.
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