Professional Documents
Culture Documents
Research from the last 5 years on the pathophysiology and treatment of upper extremity
sequelae of cerebral palsy (CP) is presented. The development of new treatments of CP-
affected limbs, utilizing the brain’s inherent neuroplasticity, remains an area of promising
and active research. Functional magnetic resonance imaging scans have evaluated the role of
neuroplasticity in adapting to the initial central nervous system insult. Children with CP
appear to have greater recruitment of the ipsilateral brain for motor and sensory functions of
the affected upper limb. Studies have also shown that constraint-induced movement therapy
Current Concepts
From the Department of Orthopaedic Surgery and the Medical School, University of Minnesota, Corresponding author: Ann E. Van Heest, MD, Department of Orthopaedic Surgery,
Minneapolis, MN. University of Minnesota, 2450 Riverside Ave., Ste. R200, Minneapolis, MN 55454; e-mail:
Received for publication November 10, 2014; accepted in revised form November 20, 2014. vanhe003@umn.edu.
No benefits in any form have been received or will be received related directly or indirectly 0363-5023/15/4005-0032$36.00/0
http://dx.doi.org/10.1016/j.jhsa.2014.11.025
to the subject of this article.
results in localized increase in gray matter volume of the sensorimotor cortex contralateral to
the affected arm targeted during rehabilitation. Recent therapy interventions have emphasized
the role of home therapy programs, the transient effects of splinting, and the promise of
constraint-induced movement therapy and bimanual hand training. The use of motion labo-
ratory analysis to characterize the movement pattern disturbances in children with CP con-
tinues to expand. Classification systems for CP upper limb continue to expand and improve
their reliability, including use of the House Classification, the Manual Ability Classification
System, and the Shriner’s Hospital Upper Extremity Evaluation. Surgical outcomes have
greater patients’ satisfaction when they address functional limitations, also in addition to
aesthetics, which may improve patients’ self-esteem. Surgical techniques for elbow, wrist,
fingers, and thumb continue to be refined. Research into each of these areas continues to
expand our understanding of the nervous system insults that cause CP, how they may be
modified, and how hand surgeons can continue to serve patients by improving their upper
limb function and aesthetics. (J Hand Surg Am. 2015;40(5):1035e1040. Copyright 2015
by the American Society for Surgery of the Hand. All rights reserved.)
Key words Cerebral palsy, stereognosis.
T
HE PURPOSE OF THIS ARTICLE IS TO review litera- timing of the insult during development. Structural
ture from the last 5 years to update hand neuroplasticity has already been demonstrated in
surgeons regarding the pathophysiology and adult stroke patients undergoing neurorehabilitation.
treatment of upper extremity sequelae secondary to Several interventions are now being employed to
cerebral palsy (CP). By definition, CP is “a group of increase upper limb function in children with uni-
disorders of development of movement and posture lateral CP. Inguaggiato et al3 found that successful
causing activity limitations that are attributed to non- noninvasive rehabilitative strategies, including
progressive disturbances that occurred in the devel- constraint-induced movement therapy (CIMT) and
oping fetal or infant brain.”1 virtual reality therapy, result in enlargement of the
Etiologies can include fetal stroke, brain anoxia, primary hand motor area contralateral to the paretic
central nervous system (CNS) infections, maternal in- hand. Increased activation was also found in the
fections, or CNS congenital malformations. Population- contralateral sensory cortex, supplementary motor
based studies report prevalence estimates of CP ranging area, premotor cortex, and cerebellum. These plastic
from 1.5 to 4 per 1,000 live births, which makes it the changes correlate with the enhancement of motor
most common motor disability in childhood.2 Tradi- skills of the paretic upper limb.
tionally, the hand surgeon has treated the secondary Eyre et al4 examined brain plasticity utilizing
peripheral manifestations of the primary CNS insult, transcranial magnetic stimulation to characterize
because few CNS treatments exist. However, future corticospinal tract development in healthy children,
treatments of upper extremity pathology due to CP children with perinatal stroke with hemiplegia, and
may evolve by gaining a greater understanding of the bilateral lesions. Magnetic resonance imaging (MRI)
primary CNS insults and CNS plasticity from such in- and anatomical studies showed that ipsilateral corti-
sults. Considerable work has been done in the last cospinal axons from the noninfarcted hemisphere to
5 years in attempt to better understand these processes the paretic side undergo compensatory hypertrophy.
regarding upper extremity use and function. Such hypertrophy predicts severe impairment in the
Current Concepts
Holmström et al6 have reported that, of the various constraining the less-affected upper limb with CIMT
lesions responsible for CP, the lesion associated with may lessen primary motor cortex activity controlling
the most favorable hand function is white matter both upper limbs—a suboptimal therapy. Specula-
damage of immaturity, in which there is mild white tively, bimanual training may be a more appropriate
matter loss and the presence of contralateral motor therapy for these children, but its efficacy may depend
projections to the paretic hand. The most impaired on interhemispherical connections.
hand function was observed in children with ipsilat-
eral motor projections, regardless of the type of
damage. In addition, van de Winckel et al7 examined PATIENT EVALUATION
brain activation patterns in children with unilateral Several studies in the last 5 years describe advances in
CP compared to normally developing children dur- motion laboratory assessment for evaluation of chil-
ing active and passive movement as well as tactile dren with upper extremity CP. By definition, CP is a
stimulation. They concluded that, in children with movement disorder, but the type of movement im-
CP, there is an increased reliance on the ipsilateral pairment may clinically manifest as spastic, flaccid,
brain for motor and sensory function to the paretic dystonic, or a combination thereof. In addition, the
limb. manifestations of CP may vary in the different seg-
Using MRI and transcranial magnetic stimulation, ments of the limb: shoulder, elbow, forearm, wrist,
Mackey et al8 found decreased intracortical and in- fingers, and thumb.
terhemispherical inhibitory mechanisms in children Fitoussi et al11 report using a specific kinematic
with CP. More intact inhibition correlates with protocol to measure the ability of hemiplegic patients
higher limb function. Hopefully, interventions can be with CP to carry out simple daily tasks before and
developed that would enhance intracortical and after therapy with either botulinum toxin injection or
interhemispherical inhibition in order to restore the surgery. The study found that, as problems in the
excitatory balance in the brain in patients with CP. forearm, wrist, and thumb were addressed and im-
Such interventions are already being explored in the proved with therapy, so did the proximal kinematic
adult stroke population. problems. This suggests that proximal deficits could
be related to compensatory movement strategies and/
Role of therapy based on neuroplasticity or cocontractions and should be addressed second-
CIMT, a rehabilitative modality rooted in neuro- arily after treatment of distal issues.
plasticity, has been used for years in the adult pop- Klotz et al12 report that motion analysis is a useful
ulation and has more recently been shown to improve clinical tool for evaluating both coordination and
upper extremity functionality in children with CP. range of motion limitations. They describe use of a
Sterling et al9 found that CIMT resulted in localized fingertip reach task, in which the child touches the
increase in contralateral gray matter volume of the top of a bottle with the index fingertip followed
sensorimotor cortex during rehabilitation. In addition, immediately by touching the nose tip. This analysis
increased gray matter volumes were observed in the shows movement deviations due to lack of coordi-
ipsilateral sensorimotor cortex and contralateral hip- nation very clearly for children with CP.
pocampus. There was a significant improvement in Jaspers et al’s study13 of spatiotemporal and ki-
spontaneous use of the upper extremities in daily nematic parameters in 3-dimensional motion anal-
activities following CIMT. Although not yet proven, ysis revealed that children with hemiplegic CP have
there seems to be a causal relationship between in- longer movement durations and less straight tra-
creases in gray matter volume and the magnitude of jectories in upper extremity movements. Compa-
motor improvement. red with normally developing children, children
Gordon et al10 have suggested that there may be with CP also have more wrist flexion and less
Current Concepts
limitations to the utility of CIMT, particularly when elbow extension and forearm supination during
there is early-onset damage. When ipsilateral pro- reach, reach to grasp, and gross motor tasks. In
jections from the unaffected hemisphere reorganize addition, children with CP have increased trunk
and are strengthened to the paretic hand, and con- movements and reduced shoulder elevation during
tralateral projections are weakened, the patients reach activities.
typically have more adversely affected hand function. Motion laboratory analysis coupled with dynamic
The earlier the onset of the damage, the greater the electromyography testing was used in tendon transfer
reorganization that occurs. In children with sub- surgery.14 Van Heest et al14 compared the preoper-
stantial ipsilateral corticospinal tract reorganization, ative and postoperative firing pattern of the flexor
account the mental health of patients with CP when elbow flexion contractures,29,30 swan neck de-
making treatment decisions. Although movement de- formities,31 and thumb-in-palm deformity.32 An al-
viations in upper and lower extremities often occur gorithm for treatment of elbow flexion contractures
simultaneously in patients with unilateral CP, the au- has been outlined by Carlson and Carlson.31 The
thors report that upper extremity deviations correlate amount of preoperative contracture must be accoun-
most with lower self-esteem. Even in high-functioning ted for when determining the extent of elbow muscle
patients with mild CP, self-esteem may be adversely lengthening. In this study, patients with fixed elbow
affected by such deviations. Elbow flexion deformity is deformities less than 45 underwent partial length-
the main contributor to decreased self-esteem, when ening, whereas those with fixed deformities greater than
compared with shoulder flexion, shoulder abduction, 45 underwent full release. A direct correlation exists
and wrist flexion deformity. between increasing age and the degree of preoperative
Similarly, Makki et al25 have reported that co- contracture; patients 12 years of age or older are 5.5
smetic appearance after surgical correction may have times as likely to have fixed contracture (passive
a greater influence on patient’s satisfaction than extension 45 ) than patients younger than 12 years
functional outcome. Older children (> 12 y) are more of age. The appropriate surgical treatment, based
self-conscious about the appearance of their hand. on the degree of preoperative contracture, can
These authors suggest that it is important not to un- greatly improve elbow flexion posture angle at
derestimate the psychosocial implications of hand ambulation, active and passive extension, and total
deformity. Libberecht et al26 reported that patient range of motion. Van Heest32 described use of thumb
satisfaction is significantly higher in patients with adductor release, extensor pollicis longus rerouting
considerable cosmetic improvement after surgery. It and metacarpophalangeal stabilization procedures
is not unreasonable to suspect that patients may be depending on the preoperative thumb-in-palm
seeking treatment primarily for cosmetic reasons. As deformity.
such, even if surgical intervention is not expected to Research from the last 5 years on the pathophysiology
deliver considerable functional improvement, cor- and treatment of CP upper extremity sequelae is pre-
recting the deformity for cosmetic reasons can be a sented in this update article. Functional MRI scans have
worthwhile endeavor for the patient. evaluated the role of neuroplasticity in adapting to the
Gong et al27 have reported on the use of the initial CNS insult. Children with CP appear to have
MACS to categorize outcomes after surgery. They greater recruitment of the ipsilateral brain for motor and
reported that, by dichotomizing the MACS into high sensory function of the affected upper limb. Studies have
and low levels for baseline hand function prior to also shown that CIMT results in localized increases in
surgery, one can effectively predict surgical outcome, gray matter volume of the sensorimotor cortex contra-
regardless of the specific type of surgery performed. lateral to the affected arm targeted during rehabilitation.
Although patients in both groups experience sub- The development of new treatments of CP-affected
stantial improvement, patients with high MACS limbs, utilizing the brain’s inherent neuroplasticity, re-
levels have greater improvements in function (utiliz- mains an area of promising and active research.
ing the House functional scale) and overall satisfac- Recent reviews of therapy interventions have
tion, but less improvement in hygiene status emphasized the role of therapy extending into home
compared with those with low MACS levels. programs, the transient effects of fabricating an
Using the SHUEE to assess therapeutic outcomes, orthosis, and the promise of CIMT and bimanual
Smitherman et al28 found that single-event multilevel hand training. The use of motion laboratory analysis
surgery for children with hemiplegic CP can signifi- to characterize the movement pattern disturbances
cantly improve thumb, finger, wrist, and forearm present in children with CP continues to expand.
segmental positioning as well as spontaneous func- Classification systems for upper limb involvement
Current Concepts
tion, but does not significantly change grasp-release due to CP continue to expand and improve their
ability. In addition, the single-event multilevel sur- reliability, including use of the House classification,15
gical approach provides the patients and families with the MACS, and the SHUEE. Outcomes after surgery
the benefits of avoiding staged surgical interventions. have been reported to have greater patient satisfaction
The SHUEE has been demonstrated to be reliable in when they address not only functional limitations but
clinical decision making and in assessment of func- also aesthetics, which may improve patients’ self-
tional outcomes after surgery. esteem. Lastly, surgical techniques continue to be
Other specific surgical technique articles and their refined for treatment of the elbow, wrist, fingers, and
results have been published regarding treatment of thumb. Continued research into each of these areas
continues to expand our understanding of the CNS 16. Arner M, Eliasson AC, Nicklasson S, Sommerstein K, Hagglund G.
Hand function in cerebral palsy. Report of 367 children in a
insults that cause CP, how they may be modified, and population-based longitudinal health care program. J Hand Surg Am.
how we, as hand surgeons, can continue to serve 2008;33(8):1337e1347.
our patients to improve their upper limb function and 17. Gajewska E, Sobieska M, Samborski W. Associations between
esthetics. manual abilities, gross motor function, epilepsy, and mental capacity
in children with cerebral palsy. Iran J Child Neurol. 2014;8(2):
45e52.
REFERENCES 18. Compagnone E, Maniglio J, Camposeo S, et al. Functional classifi-
cations for cerebral palsy: correlations between the Gross Motor
1. Bax M, Goldstein M, Rosenbaum P, et al. Proposed definition and Function Classification System (GMFCS), the Manual Ability Clas-
classification of cerebral palsy, April 2005. Dev Med Child Neurol. sification System (MACS) and the Communication Function
2005;47(8):571e576. Classification System (CFCS). Res Dev Disabil. 2014;35(11):
2. Winter S, Autry A, Boyle C, Yeargin-Allsopp M. Trends in the 2651e2657.
prevalence of cerebral palsy in a population-based study. Pediatrics. 19. Davids JR, Peace LC, Wagner LV, Gidewall MA, Blackhurst DW,
2002;110(6):1220e1225. Roberson WM. Validation of the Shriners Hospital for Children
3. Inguaggiato E, Sgandurra G, Perazza S, Guzzetta A, Cioni G. Brain Upper Extremity Evaluation (SHUEE) for children with hemiplegic
reorganization following intervention in children with congenital cerebral palsy. J Bone Joint Surg Am. 2006;88(2):326e333.
hemiplegia: a systematic review. Neural Plast. 2013;2013:356275. 20. Sakzewski L, Ziviani J, Boyd RN. Efficacy of upper limb therapies
4. Eyre JA, Smith M, Dabydeen L, et al. Is hemiplegic cerebral palsy for unilateral cerebral palsy: a meta-analysis. Pediatrics. 2014;133(1):
equivalent to amblyopia of the corticospinal system? Ann Neurol. e175ee204.
2007;62(5):493e503. 21. Taub E, Uswatte G. Importance for CP rehabilitation of transfer
5. Krageloh-Mann I, Cans C. Cerebral palsy update. Brain Dev. of motor improvement to everyday life. Pediatrics. 2014;133(1):
2009;31(7):537e544. e215ee217.
6. Holmström L, Vollmer B, Tedroff K, et al. Hand function in relation 22. Jackman M, Novak I, Lannin N. Effectiveness of hand splints in
to brain lesions and corticomotor-projection pattern in children children with cerebral palsy: a systematic review with meta-analysis.
with unilateral cerebral palsy. Dev Med Child Neurol. 2010;52(2): Dev Med Child Neurol. 2014;56(2):138e147.
145e152. 23. Koman LA, Smith BP, Williams R, et al. Upper extremity spasticity
7. Van de Winckel A, Klingels K, Bruyninckx F, et al. How does brain in children with cerebral palsy: a randomized, double-blind, placebo-
activation differ in children with unilateral cerebral palsy compared controlled study of the short-term outcomes of treatment with botu-
to typically developing children, during active and passive move- linum A toxin. J Hand Surg Am. 2013;38(3):435e446.e1.
ments, and tactile stimulation? An fMRI study. Res Dev Disabil. 24. Riad J, Brostrom E, Langius-Eklof A. Do movement deviations in-
2013;34(1):183e197. fluence self-esteem and sense of coherence in mild unilateral cerebral
8. Mackey A, Stinear C, Stott S, Byblow WD. Upper limb function and palsy? J Pediatr Orthop. 2013;33(3):298e302.
cortical organization in youth with unilateral cerebral palsy. Front 25. Makki D, Duodu J, Nixon M. Prevalence and pattern of upper
Neurol. 2014;5:117. limb involvement in cerebral palsy. J Child Orthop. 2014;8(3):
9. Sterling C, Taub E, Davis D, et al. Structural neuroplastic change 215e219.
after constraint-induced movement therapy in children with cerebral 26. Libberecht K, Sabapathy SR, Bhardwaj P. The relation of patient
palsy. Pediatrics. 2013;131(5):e1664ee1669. satisfaction and functional and cosmetic outcome after correction of
10. Gordon AM, Bleyenheuft Y, Steenbergen B. Pathophysiology of the wrist flexion deformity in cerebral palsy. J Hand Surg Eur Vol.
impaired hand function in children with unilateral cerebral palsy. Dev 2011;36(2):141e146.
Med Child Neurol. 2013;55(Suppl):432e437. 27. Gong HS, Chung CY, Park MS, Shin HI, Chung MS, Baek GH.
11. Fitoussi F, Diop A, Maurel N, Laasel el M, Ilharreborde B, Functional outcomes after upper extremity surgery for cerebral palsy:
Pennecot GF. Upper limb motion analysis in children with hemiplegic comparison of high and low manual ability classification system
cerebral palsy: proximal kinematic changes after distal botulinum toxin levels. J Hand Surg Am. 2010;35(2):277e283.e1e3.
or surgical treatments. J Child Orthop. 2011;5(5):363e370. 28. Smitherman JA, Davids JR, Tanner S, et al. Functional outcomes
12. Klotz MC, van Drongelen S, Rettig O, et al. Motion analysis of the following single-event multilevel surgery of the upper extremity for
upper extremity in children with unilateral cerebral palsy—an assess- children with hemiplegic cerebral palsy. J Bone Joint Surg Am.
ment of six daily tasks. Res Dev Disabil. 2014;35(11):2950e2957. 2011;93(7):655e661.
13. Jaspers E, Desloovere K, Bruyninckx H, et al. Three-dimensional 29. Gong HS, Cho HE, Chung CY, Park MS, Lee HJ, Baek GH. Early
upper limb movement characteristics in children with hemiplegic results of anterior elbow release with and without biceps lengthening
cerebral palsy and typically developing children. Res Dev Disabil. in patients with cerebral palsy. J Hand Surg Am. 2014;39(5):
2011;32(6):2283e2294. 902e909.
14. Van Heest A, Stout J, Wervey R, Garcia L. Follow-up motion 30. Carlson MG, Hearns KA, Inkellis E, Leach ME. Early results
laboratory analysis for patients with spastic hemiplegia due to ce- of surgical intervention for elbow deformity in cerebral palsy
rebral palsy: analysis of the flexor carpi ulnaris firing pattern before
Current Concepts