Cerebral Palsy Research August 13, 2012

1. Arch Dis Child Educ Pract Ed. 2012 Aug;97(4):122-31.

Cerebral palsy: the whys and hows.

Fairhurst C.

Correspondence to Dr Charlie Fairhurst, Department of Paediatric Neurodisability, Evelina Children’s Hospital,

Guy’s and Saint Thomas’ Foundation NHS trust, London SE1 9RT, UK; charlie.fairhurst@gstt.nhs.uk.

The descriptive term of cerebral palsy encompasses the largest group of childhood movement disorders. Severity

and pattern of clinical involvement varies widely dependent on the area of the central nervous system

compromised. A multidisciplinary team approach is vital for all the aspects of management to improve function and

minimise disability. From a medical viewpoint, there are two pronged approaches. First a focus on developmental

and clinical comorbidities such as communication, behaviour, epilepsy, feeding problems, gastro-oesophageal

reflux and infections; and second on specifics of muscle tone, motor control and posture. With regards to the latter,

there is an increasing number of available treatments including oral antispasticity and antidystonic medications,

injectable botulinum toxin, multilevel orthopaedic and neurosurgical options and a variety of complementary and

alternative therapies.

PMID: 22868578 [PubMed - in process]

2. Assist Technol. 2012 Summer;24(2):78-86.

Wheelchair components and pulmonary function in children with cerebral palsy.

Barks L, Davenport P.

VA HSR&D/RR&D Research Center of Excellence, Tampa, FL 33544, USA. Lelia.Barks@va.gov

OBJECTIVE: This study investigates the effects of four individual wheelchair components (upper extremity

supports, lateral trunk supports, anterior pelvic belt, and 30 degree posterior seat tilt), on pulmonary function in

prepubertal children with cerebral palsy (CP). METHODS: Participants who range in age from 5-10 years were

evaluated using four wheelchair components in six configurations (conditions 1-6) using a planar seating simulator.

The Respironics Non Invasive Cardiac Output monitor (NICO) and MasterScreen Impulse Oscillometry System

(IOS) measured pulmonary function parameters. Repeated measures ANOVA was used to analyze effect of

Monday 13 August 2012

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Interventions and Management

Cerebral Palsy Alliance is delighted to bring you this free weekly bulletin of the latest published research into

cerebral palsy.

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at www.cpresearch.org.au

Professor Nadia Badawi

Macquarie Group Foundation Chair of Cerebral Palsy

PO Box 560, Darlinghurst, New South Wales 2010 Australia

Cerebral Palsy Alliance

PO Box 184 Brookvale NSW 2100 Australia | T +61 2 9479 7200 | www.cerebralpalsy.org.au 2

wheelchair conditions on total airway resistance (R(AW)). RESULTS: Eight participants completed the protocol. R

(AW) and minute ventilation (MV) varied with wheelchair condition. Lowest R(AW) was seen with two upper

extremity supports or two lateral trunk supports. Differences were not significant (p = 0.253). CONCLUSIONS: The

NICO and IOS, independent of participant effort, measured R(AW) and MV, which varied by wheelchair seating

condition. More research is needed with a larger sample to determine seating components’ impact on pulmonary

function. These methods objectively measured pulmonary function of young children with CP in wheelchairs and

could facilitate further research into benefits of wheelchair postural support components.

PMID: 22876730 [PubMed - in process]

3. BMC Pediatr. 2012 Aug 9;12(1):120. [Epub ahead of print]

Evaluation of the effects of Botulinum toxin A injections when used to improve ease of care and comfort in

children with cerebral palsy whom are non-ambulant: a double blind randomized controlled trial.

Thorley M, Donaghey S, Edwards P, Copeland L, Kentish M, McLennan K, Lindsley J, Gascoigne-Pees L,

Sakzewski L, Boyd

BACKGROUND: Children with cerebral palsy (CP) whom are non-ambulant are at risk of reduced quality of life and

poor health status. Severe spasticity leads to discomfort and pain. Carer burden for families is significant. This

study aims to determine whether intramuscular injections of Botulinum Toxin A (BoNT-A) combined with a regime of

standard therapy has a positive effect on care and comfort for children with CP whom are non-ambulant (GMFCS

IV/V), compared with standard therapy alone (cycle I), and whether repeated injections with the same regime of

adjunctive therapy results in greater benefits compared with a single injecting episode (cycle II). The regime of

therapy will include serial casting, splinting and/or provision of orthoses, as indicated, combined with four sessions

of goal directed occupational therapy or physiotherapy. Method/design This study is a double blind randomized

controlled trial. Forty participants will be recruited. In cycle I, participants will be randomized to either a treatment

group who will receive BoNT-A injections into selected upper and/or lower limb muscles, or a control group who will

undergo sham injections. Both groups will receive occupational therapy and /or physiotherapy following injections.

Groups will be assessed at baseline then compared at 4 and 16 weeks following injections or sham control.

Parents, treating clinicians and assessors will be masked to group allocation. In cycle II, all participants will undergo

intramuscular BoNT-A injections to selected upper and/or lower limb muscles, followed by therapy. The primary

outcome measure will be change in parent ratings in identified areas of concern for their child’s care and comfort,

using the Canadian Occupational Performance Measure (COPM). Secondary measures will include the Care and

Comfort Hypertonicity Scale (ease of care), the Cerebral Palsy Quality of Life Questionnaire (CP QoL-Child) (quality

of life), the Caregiver Priorities and Child Health Index of Life with Disabilities Questionnaire (CPCHILD(c)) (health

status) and the Paediatric Pain Profile (PPP) (pain). Adverse events will be carefully monitored by a clinician

masked to group allocation. DISCUSSION: This paper outlines the theoretical basis, study hypotheses and

outcome measures for a trial of BoNT-A injections and therapy for children with non-ambulant cerebral palsy. Trial

registration Australia New Zealand Clinical Trials Registry:N12609000360213.

PMID: 22873758 [PubMed - as supplied by publisher]

4. Gait Posture. 2012 Aug 4. [Epub ahead of print]

Differences in implementation of gait analysis recommendations based on affiliation with a gait laboratory.

Wren TA, Elihu KJ, Mansour S, Rethlefsen SA, Ryan DD, Smith ML, Kay RM.

Children’s Orthopaedic Center, Children’s Hospital Los Angeles, Los Angeles, CA, United States; Keck School of

Medicine, University of Southern California, Los Angeles, CA, United States.

This study examined the extent to which gait analysis recommendations are followed by orthopedic surgeons with

varying degrees of affiliation with the gait laboratory. Surgical data were retrospectively examined for 95 patients

with cerebral palsy who underwent lower extremity orthopedic surgery following gait analysis. Thirty-three patients

were referred by two surgeons directly affiliated with the gait laboratory (direct affiliation), 44 were referred by five

surgeons from the same institution but not directly affiliated with the gait laboratory (institutional affiliation), and 18

were referred by 10 surgeons from other institutions (no affiliation). Data on specific surgeries were collected from

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the gait analysis referral, gait analysis report, and operative notes. Adherence to the gait analysis recommendations

was calculated by dividing the number of procedures where the surgery followed the gait analysis recommendation

(numerator) by the total number of procedures initially planned, recommended by gait analysis, or done

(denominator). Adherence with the gait analysis recommendations was 97%, 94%, and 77% for the direct,

institutional, and no affiliation groups, respectively. Procedures recommended for additions to the surgical plan

were added 98%, 87%, and 77% of the time. Procedures recommended for elimination were dropped 100%, 89%,

and 88% of the time. Of 81 patients who had specific surgical plans prior to gait analysis, changes were

implemented in 84% (68/81) following gait analysis recommendations. Gait analysis influences the treatment

decisions of surgeons regardless of affiliation with the gait laboratory, although the influence is stronger for

surgeons who practice within the same institution as the gait laboratory.

Copyright © 2012 Elsevier B.V. All rights reserved.

PMID: 22871237 [PubMed - as supplied by publisher]

5. Hip Int. 2012 Aug 7:0. doi: 10.5301/HIP.2012.9453. [Epub ahead of print]

Hip flexion deformity improves without psoas-lengthening after surgical correction of fixed knee flexion

deformity in spastic diplegia.

Rutz E, Gaston MS, Tirosh O, Brunner R.

Paediatric Orthopaedic Department, University Children’s Hospital Basle, Basle – Switzerland; and Murdoch

Children’s Research Institute, The Royal Children’s Hospital, Victoria – Australia.

Background: It is unclear if psoas lengthening surgery is required in the treatment of patients with cerebral palsy

(CP) with hip flexion deformity and previous studies show equivocal results with regard to functional outcome.

Methods: This study retrospectively assessed 12 patients with a diagnosis of spastic diplegia who underwent single

event multilevel surgery in order to correct deformities in the sagittal plane distal to the hip. Both clinical and

instrument gait analysis results were recorded preoperatively, at one year (short term) and at five years (mid term)

postoperatively. Results: Clinically measured hip and knee movement improved at both short and mid term follow

up. Correlations of clinically measured maximum hip and knee extension were significant at all three time points.

Angles at terminal stance/toe off for hip and knee from kinematic data also showed significant correlations at all

three time points. Conclusions: Our study demonstrates that the hip flexion deformities encountered in these

patients will improve spontaneously when the distal fixed knee flexion deformity is surgically corrected. Therefore

correction at the knee allows the ground reaction force to assume a more normal position resulting in correction at

the hip over time. This then removes the need for surgery at the hip level. This fact is especially important when

applied to psoas lengthening as this procedure can cause significant reduction in propulsion power.

PMID: 22878968 [PubMed - as supplied by publisher]

6. J Biomech. 2012 Aug 3. [Epub ahead of print]

Medial gastrocnemius muscle fascicle active torque-length and Achilles tendon properties in young adults

with spastic cerebral palsy.

Barber L, Barrett R, Lichtwark G.

School of Physiotherapy and Exercise Science & Centre for Musculoskeletal Research, Griffith University, Gold

Coast campus, Queensland 4222, Australia.

Individuals with spastic cerebral palsy (CP) typically experience muscle weakness. The mechanisms responsible

for muscle weakness in spastic CP are complex and may be influenced by the intrinsic mechanical properties of the

muscle and tendon. The purpose of this study was to investigate the medial gastrocnemius (MG) muscle fascicle

active torque-length and Achilles tendon properties in young adults with spastic CP. Nine relatively high functioning

young adults with spastic CP (GMFCS I, 17±2 years) and 10 typically developing individuals (18±2 years)

participated in the study. Active MG torque-length and Achilles tendon properties were assessed under controlled

conditions on a dynamometer. EMG was recorded from leg muscles and ultrasound was used to measure MG

Cerebral Palsy Research News ~ Monday 13 August 2012

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fascicle length and Achilles tendon length during maximal isometric contractions at five ankle angles throughout the

available range of motion and during passive rotations imposed by the dynamometer. Compared to the typically

developing group, the spastic CP group had 33% lower active ankle plantarflexion torque across the available

range of ankle joint motion, partially explained by 37% smaller MG muscle and 4% greater antagonistic cocontraction.

The Achilles tendon slack length was also 10% longer in the spastic CP group. This study confirms

young adults with mild spastic CP have altered muscle-tendon mechanical properties. The adaptation of a longer

Achilles tendon may facilitate a greater storage and recovery of elastic energy and partially compensate for

decreased force and work production by the small muscles of the triceps surae during activities such as locomotion.

Copyright © 2012 Elsevier Ltd. All rights reserved.

PMID: 22867763 [PubMed - as supplied by publisher]

7. J Neurol. 2012 Aug 10. [Epub ahead of print]

Botulinum toxin therapy: its use for neurological disorders of the autonomic nervous system.

Dressler D.

Movement Disorders Section, Department of Neurology, Hannover Medical School, Carl-Neuberg-Str. 1, 30625,

Hannover, Germany, dressler.dirk@mh-hannover.de.

Botulinum toxin (BoNT) has gained widespread use for the treatment of overactive muscles, overactive exocrine

glands and, most recently, non-muscular pain conditions. Autonomic conditions treated with BoNT include

achalasia, gastroparesis, sphincter of Oddi spasms, and unspecific esophageal spasms in gastroenterology and

prostate disorders in urology. BoNT’s use for autonomic conditions related to neurology includes various forms of

bladder dysfunction (detrusor sphincter dyssynergia, idiopathic detrusor overactivity, neurogenic detrusor

overactivity, urinary retention and bladder pain syndrome), pelvic floor disorders (pelvic floor spasms and anal

fissures), hyperhidrosis (axillary, palmar, and plantar hyperhidrosis, diffuse sweating, Frey’s syndrome) and

hypersalivation (hypersalivation in Parkinsonian syndromes, motor neuron disease, neuroleptic use, and cerebral

palsy). Hyperhidrosis, hypersalivation, some forms of bladder dysfunction and pelvic floor disorders can easily be

treated by neurologists. Most bladder dysfunctions require cooperation with urology departments.

PMID: 22878428 [PubMed - as supplied by publisher]

8. Spine (Phila Pa 1976). 2012 Aug 3. [Epub ahead of print]

Mortality and Morbidity in Early Onset Scoliosis Surgery.

Phillips JH, Knapp DR Jr, Herrera-Soto J.

Orlando Health-Arnold Palmer Medical Center, Pediatric Orthopaedics, 83 W. Columbia Street, Orlando, Florida

32806, Tel: 321.841.3060, Fax: 321.843.6304, Email: jonathan.phillips@orlandohealth.com Orlando Health-Arnold

Palmer Medical Center, Pediatric Orthopaedics, 83 W. Columbia Street, Orlando, Florida 32806, Tel: 321.841.3060,

Fax: 321.843.6304, Email: raymond.knapp@orlandohealth.com Orlando Health-Arnold Palmer Medical Center,

Pediatric Orthopaedics, 83 W. Columbia Street, Orlando, Florida 32806, Tel: 321.841.3060, Fax: 321.843.6304,

Email: jose.herrera@orlandohealth.com.

STRUCTURED: Study Design. Retrospective chart review. Objective. To accurately determine complication rates,

particularly mortality rates, in surgically treated Early Onset Scoliosis. Summary of Background Data. The advent of

modern segmental instrumentation for spinal fusion surgery in adolescent scoliosis has allowed for application of

similar non-segmental un-fused techniques aimed at controlling scoliosis in the very young child. The dismal

prognosis for these children without repeated spinal lengthening procedures is unquestioned though no controlled

trials exist. Many if not most of these children need surgery, however the surgical complication rate is very high.

Methods. During the study period all surgically treated children with EOS seen at our institution were identified.

Inclusion criteria were: any patient who presented to our clinic with early onset scoliosis which was surgically

managed. The total number of procedures, type of implants, number and type of complications, geographic origin of

the cases and final outcomes were all assessed. Results. A total of 165 surgical procedures on 28 patients accrued

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PO Box 184 Brookvale NSW 2100 Australia | T +61 2 9479 7200 | www.cerebralpalsy.org.au 5

over the study time period, including index implantation of instrumentation, lengthening and definitive fusion as well

as operations performed for complications such as wound debridement and revision of failed implants. Clinical

diagnoses included congenital scoliosis, syndromic and chromosomal abnormalities, cerebral palsy and spinal

muscular atrophy. There was a complication rate of 84% overall with a mortality rate of almost 18%. The only

patients with no complications were those whose entire surgical course had been at our institution only. The

mortality rate was equal in patients whose treatment was performed elsewhere versus exclusively in our center.

Conclusion. This study underlines the grave severity of these scolioses particularly in syndromic children. The high

mortality rate is alarming, suggesting that further study is needed in this area.

PMID: 22869061 [PubMed - as supplied by publisher]

9. Clin Linguist Phon. 2012 Sep;26(9):806-22.

Relationship between kinematics, F2 slope and speech intelligibility in dysarthria due to cerebral palsy.

Rong P, Loucks T, Kim H, Hasegawa-Johnson M.

Department of Speech and Hearing Science , University of Illinois , Champaign, IL , USA.

A multimodal approach combining acoustics, intelligibility ratings, articulography and surface electromyography was

used to examine the characteristics of dysarthria due to cerebral palsy (CP). CV syllables were studied by obtaining

the slope of F2 transition during the diphthong, tongue-jaw kinematics during the release of the onset consonant,

and the related submental muscle activities and relating these measures to speech intelligibility. The results show

that larger reductions of F2 slope are correlated with lower intelligibility in CP-related dysarthria. Among the three

speakers with CP, the speaker with the lowest F2 slope and intelligibility showed smallest tongue release

movement and largest jaw opening movement. The other two speakers with CP were comparable in the amplitude

and velocity of tongue movements, but one speaker had abnormally prolonged jaw movement. The tongue-jaw

coordination pattern found in the speakers with CP could be either compensatory or subject to an incompletely

developed oromotor control system.

PMID: 22876770 [PubMed - in process]

10. Oral Surg Oral Med Oral Pathol Oral Radiol. 2012 Sep;114(3):e10-4. Epub 2012 Feb 28.

Oral myiasis: does an indication for surgical treatment still exist? Two case reports.

Ribeiro AL, de Almeida TE, Júnior JS, de Araújo Castro JF, de Jesus Viana Pinheiro J.

Professor, Department of Oral and Maxillofacial Surgery, School of Dentistry, University Center of Pará-CESUPA,

Belém, Brazil; Chairman, Department of Oral and Maxillofacial Surgery, Hospital Metropolitano de Urgência e

Emergência, Belém, Brazil.

OBJECTIVE: Oral myiasis is a rare infection for which treatment protocol has not yet been established. This article

presents 2 cases treated with a combination of topical application of sulfuric ether and surgery. The reasons for the

use of surgical therapy, as well as the possible advantages and disadvantages of drug-based treatments, are

discussed. CASE REPORT: Two cases of oral myiasis are described, the first being observed in a 9-year-old child

with hypotonic cerebral palsy, and the second in a 52-year-old adult, alcohol-dependant, both showing infection in

the gingival sulcus. Both cases were successfully treated in a process that involved topical application of sulfuric

ether, mechanical removal of larvae, and surgical debridement. CONCLUSIONS: Oral myiasis can be treated

effectively with surgery after topical application of sulfuric ether. The use of drugs may suggest a therapeutic

alternative, but still requires further study and experience to be implemented, especially in individuals with

neurological disorders.

Copyright © 2012 Elsevier Inc. All rights reserved.

PMID: 22862986 [PubMed - in process]

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PO Box 184 Brookvale NSW 2100 Australia | T +61 2 9479 7200 | www.cerebralpalsy.org.au 6

11. Int J Rehabil Res. 2012 Aug 3. [Epub ahead of print]

Comparison between utility of the Thai Pediatric Quality of Life Inventory 4.0 Generic Core Scales and 3.0

Cerebral Palsy Module.

Tantilipikorn P, Watter P, Prasertsukdee S.

Division of Physiotherapy, School of Health and Rehabilitation Science, The University of Queensland, St Lucia,

Queensland, Australia bFaculty of Physical Therapy, Mahidol University, Salaya, Nakornprathom, Thailand.

Health-related quality of life (HRQOL) is increasingly being considered in the management of patients with various

conditions. HRQOL instruments can be broadly classified as generic or disease-specific measures. Several generic

HRQOL instruments in different languages have been developed for paediatric populations including the Pediatric

Quality of Life Inventory 4.0 (PedsQL 4.0) Generic Core Scale. This tool and a condition-specific tool, PedsQL 3.0

Cerebral Palsy (CP) Module, are widely used in children with CP. No psychometric properties have been reported

for Thai PedsQL 4.0. Therefore, this study aimed to explore the psychometric properties of the Thai version of the

PedsQL 4.0 Generic Core Scales and compare these with the values for the Thai PedsQL 3.0 CP Module reported

previously. Thai PedsQL 4.0 Generic Core Scales and the PedsQL 3.0 CP Module were completed, respectively,

by children with CP and their parents or caregivers twice within 2-4 weeks. Respondents were 97 parents or

caregivers and 54 children. Minimal missing data were found in most scales. Acceptable internal consistency was

supported, except for Emotional, Social, and School Functioning. Intraclass correlation coefficients for parent-proxy

report and self-report were good to excellent (0.625-0.849). The feasibility and reliability of the Thai PedsQL 4.0

Generic Core Scales were supported. The Thai PedsQL 3.0 CP Module showed higher values for the psychometric

properties. Low-to-good correlations were found among the scales between the PedsQL 4.0 Generic Core Scales

and the 3.0 CP Module. Both instruments could be used to measure HRQOL for children with CP, and may provide

different information.

PMID: 22868944 [PubMed - as supplied by publisher]

12. Soc Work Health Care. 2012;51(4):279-95.

Social workers as transition brokers: facilitating the transition from pediatric to adult medical care.

Shanske S, Arnold J, Carvalho M, Rein J.

Department of Social Work, Children’s Hospital Boston, Boston, Massachusetts 02115, USA.


Transition from pediatric to adult medical care and the significant psychosocial considerations impacting this

developmental process are a primary focus in health care today. Social workers are often the informal brokers of

this complex and nuanced process and are uniquely trained to complete biopsychosocial assessments to

understand the needs of patients and families and address psychosocial factors. Their extensive knowledge of

resources and systems, along with their sophisticated understanding of the relationship issues, family dynamics,

cultural implications, and basic person-in-context approach allow for unique collaboration with the health care team,

family, and community supports to develop successful transition plans and programs.

PMID: 22489554 [PubMed - indexed for MEDLINE]

Cerebral Palsy Research News ~ Monday 13 August 2012

13. Genet Couns. 2012;23(2):215-21.

Pure distal 9p deletion in a female infant with cerebral palsy.

Chen CP, Lin SP, Su YN, Su JW, Chern SR, Town DD, Wang W.

Department of Obstetrics and Gynecology, Mackay Memorial Hospital, Taipei, Taiwan. cpc_mmh@yahoo.com

We report cytogenetic and molecular characterization of a 15.63-Mb pure distal deletion of chromosome 9p (9p22.3

–>pter) in a l 1/2-year-old female infant with cerebral palsy and diffuse cerebral dysfunction. The deletion is of

paternal origin and encompasses the genes of ANKRDS15, DOCK8, FOXD4 and VLDLR. We discuss the genotype

-phenotype correlation in this case with neurological dysfunction and a distal 9p deletion of paternal origin.

PMID: 22876580 [PubMed - in process]

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