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Dyskinetic cerebral palsy and dystonia

Elegast Monbaliu, Paul De Cock, Lisa Mailleux, Bernard Dan, Hilde Feys
AIM: To relate dystonia and choreoathetosis with activity, participation and quality of life (QOL) in children and youth with dyskinetic Cerebral Palsy (CP). METHODS: Fifty-four participants with dyskinetic CP (mean age 14y6m, SD 4y2m, range 6-22y) were included. The Dyskinesia Impairment Scale (DIS) was used to evaluate dystonia and choreoathetosis. Activity, participation and quality of life (QOL) were assessed with the Gross Motor Function Measure (GMFM), the Functional Mobility Scale (FMS), the Jebsen-Taylor Hand Function Test (JTT), the ABILHAND-Kids Questionnaire (ABIL-K), the Life Habits Kids (LIFE-H) and the Quality of Life Questionnaire for children with CP (CP-QOL)...
September 23, 2016: European Journal of Paediatric Neurology: EJPN
Hortensia Gimeno, Jean-Pierre Lin
The multidisciplinary team (MDT) approach illustrates how motor classification systems, assessments and outcome measures currently available have been applied to a national cohort of children and young people with dystonia and other hyperkinetic movement disorders (HMD) particularly with a focus on dyskinetic cerebral palsy (CP). The paper is divided in 3 sections. Firstly, we describe the service model adopted by the Complex Motor Disorders Service (CMDS) at Evelina London Children's Hospital and King's College Hospital (ELCH-KCH) for deep brain stimulation...
September 8, 2016: European Journal of Paediatric Neurology: EJPN
Anne Koy, Lars Timmermann
Cerebral palsy (CP) is the most common cause for acquired dystonia in childhood. Pharmacological treatment is often unsatisfactory and side effects are frequently dose-limiting. Data on outcome of DBS in paediatric patients with dyskinetic CP is very limited and heterogeneous. Reasons for the variability in responses are not entirely known yet. Interestingly, some CP-patients seem to improve subjectively on pallidal stimulation but without measurable changes in impairment scales. Besides dystonia scales, the use of sensitive age-dependent assessments tools is therefore reasonable to capture the full effect...
May 27, 2016: European Journal of Paediatric Neurology: EJPN
Bhooma R Aravamuthan, Jeff L Waugh
BACKGROUND: Dyskinetic cerebral palsy affects 15%-20% of patients with cerebral palsy. Basal ganglia injury is associated with dyskinetic cerebral palsy, but the patterns of injury within the basal ganglia predisposing to dyskinetic cerebral palsy are unknown, making treatment difficult. For example, deep brain stimulation of the globus pallidus interna improves dystonia in only 40% of patients with dyskinetic cerebral palsy. Basal ganglia injury heterogeneity may explain this variability...
January 2016: Pediatric Neurology
Elegast Monbaliu, Paul de Cock, Els Ortibus, Lieve Heyrman, Katrijn Klingels, Hilde Feys
AIM: The aim of the study was to map clinical patterns of dystonia and choreoathetosis and to assess the relation between functional classifications and basal ganglia and thalamus lesions in participants with dyskinetic cerebral palsy (CP). METHODS: In this cross-sectional study, 55 participants with dyskinetic CP (mean age 14y 6mo, SD 4y 1mo; range 6-22y) were assessed with the Dyskinesia Impairment Scale and classified with the Gross Motor Function Classification System (GMFCS), Manual Ability Classification System (MACS), and Communication Function Classification System (CFCS)...
February 2016: Developmental Medicine and Child Neurology
Katherine A Benfer, Rachel Jordan, Sasaka Bandaranayake, Christine Finn, Robert S Ware, Roslyn N Boyd
OBJECTIVES: To compare the patterns of motor type and gross motor functional severity in preschool-aged children with cerebral palsy (CP) in Bangladesh and Australia. METHODS: We used comparison of 2 prospective studies. A total of 300 children with CP were aged 18 to 36 months, 219 Australian children (mean age, 26.6 months; 141 males) recruited through tertiary and community services, and 81 clinic-attendees born in Bangladesh (mean age, 27.5 months; 50 males)...
December 2014: Pediatrics
Anne Koy, K Amande M Pauls, Pia Flossdorf, Johannes Becker, Eckhard Schönau, Mohammad Maarouf, Thomas Liebig, Oliver Fricke, Gereon R Fink, Lars Timmermann
BACKGROUND: Pharmacological treatment of dyskinetic cerebral palsy (CP) is often ineffective. Data about outcome of deep brain stimulation (DBS) in these patients remains scarce. METHODS: Eight patients with dyskinetic CP and DBS of the Globus Pallidus internus were investigated. Using pre- and postoperative videos the severity of dystonia and changes thereof during standardized settings ('on') and after the stimulator had been switched off ('off') were assessed using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS)...
2014: European Neurology
Byung-Hyun Park, Sung-Hee Park, Jeong-Hwan Seo, Myoung-Hwan Ko, Gyung-Ho Chung
OBJECTIVE: To investigate neuroradiological and neurophysiological characteristics of patients with dyskinetic cerebral palsy (CP), by using magnetic resonance imaging (MRI), voxel-based morphometry (VBM), diffusion tensor tractography (DTT), and motor evoked potential (MEP). METHODS: Twenty-three patients with dyskinetic CP (13 males, 10 females; mean age 34 years, range 16-50 years) were participated in this study. Functional evaluation was assessed by the Gross Motor Functional Classification System (GMFCS) and Barry-Albright Dystonia Scale (BADS)...
April 2014: Annals of Rehabilitation Medicine
Anne Koy, Martin Hellmich, K Amande M Pauls, Warren Marks, Jean-Pierre Lin, Oliver Fricke, Lars Timmermann
Secondary dystonia encompasses a heterogeneous group with different etiologies. Cerebral palsy is the most common cause. Pharmacological treatment is often unsatisfactory. There are only limited data on the therapeutic outcomes of deep brain stimulation in dyskinetic cerebral palsy. The published literature regarding deep brain stimulation and secondary dystonia was reviewed in a meta-analysis to reevaluate the effect on cerebral palsy. The Burke-Fahn-Marsden Dystonia Rating Scale movement score was chosen as the primary outcome measure...
May 2013: Movement Disorders: Official Journal of the Movement Disorder Society
Elegast Monbaliu, Els Ortibus, Peter Prinzie, Bernard Dan, Josse De Cat, Paul De Cock, Hilde Feys
BACKGROUND: The Dyskinesia Impairment Scale (DIS) is a new scale for measuring dystonia and choreoathetosis in dyskinetic Cerebral Palsy (CP). Previously, reliability of this scale has only been assessed for raters highly experienced in discriminating between dystonia and choreoathetosis. AIMS: The aims of this study are to examine the reliability of the DIS used by inexperienced raters, new to discriminating between dystonia and choreoathetosis and to determine the effect of clinical expertise on reliability...
May 2013: European Journal of Paediatric Neurology: EJPN
Elegast Monbaliu, Els Ortibus, Jos De Cat, Bernard Dan, Lieve Heyrman, Peter Prinzie, Paul De Cock, Hilde Feys
AIM: The aim of this study was to examine the reliability and validity of the Dyskinesia Impairment Scale (DIS). The DIS consists of two subscales: dystonia and choreoathetosis. It measures both phenomena in dyskinetic cerebral palsy (CP). METHOD: Twenty-five participants with dyskinetic CP (17 males; eight females; age range 5–22y; mean age 13y 6mo; SD 5y 4mo), recruited from special schools for children with motor disorders, were included. Exclusion criteria were changes in muscle relaxant medication within the previous 3 months, orthopaedic or neurosurgical interventions within the previous year, and spinal fusion...
March 2012: Developmental Medicine and Child Neurology
Martin Smith
No abstract text is available yet for this article.
March 2012: Developmental Medicine and Child Neurology
Nicola Specchio, Antonio Carotenuto, Marina Trivisano, Simona Cappelletti, Federico Vigevano, Lucia Fusco
Spasticity is a state of sustained pathological increase in the tension of a muscle. Treatment for spasticity has been revolutionized by the introduction of intrathecal baclofen (ITB) continuous infusion. ITB is associated with a 30% rate of complications mostly as a result of catheter problems that lead to acute ITB withdrawal. We describe a 10-year-old girl with spastic quadriplegia caused by cerebral palsy successfully treated with ITB who developed dystonic-dyskinetic status following acute ITB withdrawal because of a catheter kink resolved by external manipulation...
July 2011: Epilepsy & Behavior: E&B
Allison Przekop, Terence D Sanger
"Athetosis," from the Greek athetos, meaning "without fixed position," is a movement disorder first described by Hammond in 1871. The term described slow, irregular continual movements of the distal extremities. In 1983, Foley defined the athetoid syndrome as "a nonprogressive but evolving disorder due to damage to the basal ganglia of the full-term brain … [with] impairment of postural reflexes, arrhythmical involuntary movements, and dysarthria, [but] sparing … sensation, ocular movements and … intelligence...
2011: Handbook of Clinical Neurology
Johan van Doornik, Sahana Kukke, Terence D Sanger
It is often assumed that co-contraction of antagonist muscles is responsible for increased resistance to passive movement in hypertonic dystonia. Although co-contraction may certainly contribute to hypertonia in some patients, the role of reflex activation has never been investigated. We measured joint torque and surface electromyographic activity during passive flexion and extension movements of the elbow in 8 children with hypertonic arm dystonia due to dyskinetic cerebral palsy. In all cases, we found significant phasic electromyographic activity in the lengthening muscle, consistent with reflex activity...
May 15, 2009: Movement Disorders: Official Journal of the Movement Disorder Society
Thomas Michael O'Shea
Cerebral palsy is the most prevalent cause of persisting motor function impairment with a frequency of about 1/500 births. In developed countries, the prevalence rose after introduction of neonatal intensive care, but in the past decade, this trend has reversed. A recent international workshop defined cerebral palsy as "a group of permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain...
December 2008: Clinical Obstetrics and Gynecology
P Póo, M Galván-Manso, M J Casartelli, J López-Casas, R M Gassió-Subirats, C Blanco, L Terricabras-Carol
INTRODUCTION: A number of studies have proved the effectiveness and safety of botulinum toxin in therapeutic doses. AIM: To analyse the results obtained over a 12-year period in which botulinum toxin type A (BTA) was used to treat infantile cerebral palsy (CP). PATIENTS AND METHODS: Of a total number of 547 patients who were treated, 515 had CP, 464 with spasticity, 46 with mixed CP and 5 with dyskinetic CP with focal dystonia. RESULTS: Overall evaluation of BTA is positive, both as regards its beneficial effects and its safety: tone was mildly improved in 18...
2008: Revista de Neurologia
T B Khoo, M Y Yusniza, P M Chern
A study was carried out to determine the clinical effectiveness of intramuscular botulinum toxin type A (BTX) in the treatment of spasticity or dystonia in 58 consecutive children with cerebral palsy (CP). The effectiveness of the treatment was determined by the reduction of spasticity and global parental perception scale. The mean age of treatment was six years and the most frequent aim of treatment (91.1%) was functional improvement. The median reduction of spasticity as measured by modified Ashworth scale was 1...
March 2007: Medical Journal of Malaysia
R Guerrini, F Moro, M Kato, A J Barkovich, T Shiihara, M A McShane, J Hurst, M Loi, J Tohyama, V Norci, K Hayasaka, U J Kang, S Das, W B Dobyns
BACKGROUND: ARX is a paired-type homeobox gene located on the X chromosome that contains five exons with four polyalanine (PolyA) tracts, a homeodomain, and a conserved C-terminal aristaless domain. Studies in humans have demonstrated remarkable pleiotropy: malformation phenotypes are associated with protein truncation mutations and missense mutations in the homeobox; nonmalformation phenotypes, including X-linked infantile spasms (ISS), are associated with missense mutations outside of the homeobox and expansion of the PolyA tracts...
July 31, 2007: Neurology
Steffen Berweck, A Sebastian Schroeder, Seung-Hee Lee, Hans Bigalke, Florian Heinen
Botulinum toxin (BTX) offers a new treatment option to reduce drooling in adults and children. Antibody formation against BTX is known to be one reason for clinical secondary non-response to this treatment. This is a case report on the development of secondary non-response to BTX type B (BTX-B) in a 15-year-old male, with bilateral dyskinetic cerebral palsy (Gross Motor Function Classification System Level IV) with additional learning disability* and microcephaly, treated for the indication of drooling. After three successful treatment sessions, the fourth and fifth injections showed no clinical response...
January 2007: Developmental Medicine and Child Neurology
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