The motor effort of jaw-closing muscles during maximal voluntary clenching (MVC) was compared between individuals with spastic cerebral palsy (CP) and nondisabled control subjects (CG). Bilateral electromyographic (EMG) activity of the anterior temporalis (AT) and masseter (MS) muscles was obtained during MVC in 22 subjects with CP and 29 nondisabled subjects. The oral functional status of the group with CP was evaluated using the Orofacial Motor Function Assessment Scale. The group with CP presented lower bilateral EMG activity during MVC compared to the control group for both AT and MS muscles. Comparisons of AT and MS EMG activity showed no difference in muscular effort. Subgroups with CP who were only slightly and very slightly orally impaired had a higher bilateral AT EMG activity compared to individuals with CP who were severely and moderately compromised. A statistically significant positive correlation was found between the oral motor function and EMG activity of the group with CP in all the muscles evaluated. Individuals with CP had motor weakness in the jaw-closing muscles, a condition that may compromise their masticatory function.

The Roman emperor Claudius suffered from a wide range of physical tics and disabilities. Many scholars have explained these symptoms by hypothesizing that Claudius suffered from cerebral palsy. However, this hypothesis is dated and does not take into account all of the evidence available. This article re-examines ancient historical sources in the light of modern medical knowledge in an attempt to retrospectively diagnose the cause of Claudius’s problems. In doing so, it suggests that Claudius may have suffered from Tourette’s syndrome.

We demonstrate the utility of functional near-infrared spectroscopy (fNIRS) as a tool for physicians to study cortical plasticity in children with cerebral palsy (CP). Motor cortex activation patterns were studied in five healthy children and five children with CP (8.4+/-2.3 years old in both groups) performing a finger-tapping protocol. Spatial (distance from center and area difference) and temporal (duration and time-to-peak) image metrics are proposed as potential biomarkers for differentiating abnormal cortical activation in children with CP from healthy pediatric controls. In addition, a similarity image-analysis concept is presented that unveils areas that have similar activation patterns as that of the maximum activation area, but are not discernible by visual inspection of standard activation images. Metrics derived from the images presenting areas of similarity are shown to be sensitive identifiers of abnormal activation patterns in children with CP. Importantly, the proposed similarity concept and related metrics may be applicable to other studies for the identification of cortical activation patterns by fNIRS.

INTRODUCTION: Cerebral palsy is the most common cause of physical disability in children. Spasticity is a disabling clinical symptom that is prevalent among patients suffering from cerebral palsy. The treatment of spasticity with botulinum toxin type A (BTX-A) is a well-established option in the interdisciplinary management of spasticity, providing focal reductions in muscle tone in cerebral palsy patients. OBJECTIVE: The aim of this retrospective study was to describe the effect of multilevel BTX-A injections in the lower extremities, focusing mainly on gross motor function and functional status in cerebral palsy patients. METHODS: Data from 71 cerebral palsy patients (64% male, 36% female, mean age 6.7 +/-3.2 years) were analyzed retrospectively. We used the Ashworth and Tardieu scales to evaluate the degree of spasticity. Motor function was measured by the Gross Motor Function Measure (GMFM-88), and functional status was classified by the Gross Motor Function Classification System (GMFCS I-V). Multilevel BTX-A injections were applied after sedation and with electrostimulation guidance. The evaluations were repeated every three months, and the patients were followed for six months. RESULTS: We found that the Ashworth and Tardieu scores decreased significantly at the three-month evaluation (p<0.05) but not at the six-month evaluation (p>0.05). Although the improvement in spasticity was not maintained at the six-month evaluation, GMFM-88 scores increased significantly at the three- and six-month assessments. GMFSC levels showed no change in the three- and six-month assessments. CONCLUSION: We believe that a single multilevel BTX-A injection reduces spasticity and improves motor function in children with cerebral palsy.

PURPOSE OF REVIEW: This review focuses on new insights in deep brain stimulation (DBS) for patients with hyperkinetic movement disorders: dystonia, tardive dyskinesia and Gille de la Tourette’s syndrome, during the last 18 months. RECENT FINDINGS: The recent literature confirms the efficacy of high-frequency stimulation of the globus pallidus internus (GPi) for primary dystonia, generalized or not, with a stable effect over time. The benefit of DBS in other forms of localized dystonia remains to be demonstrated in larger studies. Some clinical and radiological predictive factors have been determined with a predominant influence of the disease duration. Tardive dystonia and myoclonus-dystonia are also improved by GPi stimulation. Encouraging results obtained in cerebral palsy may pave the way for the application of DBS in other secondary dystonia. In Gilles de la Tourette’s syndrome, both stimulation of the centre-median/parafascicular nucleus of the thalamus and GPi stimulation (ventromedial) have demonstrated efficacy with stable long-term effect. Thalamic stimulation failed to improve obsessions and compulsions in some patients. Stimulation of the nucleus accumbens has been tested in few cases with contradictory efficacy. In both diseases, complications are rare with no major side effects. SUMMARY: The few controlled studies showed that bilateral GPi stimulation is a well tolerated and a long-term effective treatment for hyperkinetic disorders. However, recent published data of DBS applied in different targets or patients (especially secondary dystonia) are mainly uncontrolled case reports, precluding the clear determination of the efficacy of this procedure and the choice of the ‘good’ target for the ‘good’ patient.

The plantar grasp reflex can be elicited in all normal infants from 25 weeks of postconceptional age until the end of 6 months of corrected age according to the expected birth date. This reflex in human infants can be regarded as a rudiment of responses that were once essential for ape infants in arboreal life. The spinal center for this reflex is probably located at the L(5)-S(2) levels, which, however, are controlled by higher brain structures. Nonprimary motor areas may exert regulatory control of the spinal reflex mechanism through interneurons. In infants, this reflex can be elicited as the result of insufficient control of the spinal mechanism by the immature brain. In adults, lesions in nonprimary motor areas may cause a release of inhibitory control by spinal interneurons, leading to a reappearance of the reflex. The plantar grasp reflex in infants is of high clinical significance. A negative or diminished reflex during early infancy is often a sensitive indicator of spasticity. Infants with athetoid type cerebral palsy exhibit an extremely strong retention of the reflex, and infants with mental retardation also exhibit a tendency toward prolonged retention of the reflex. Copyright © 2010 Elsevier Inc. All rights reserved.

While intraventricular administration of epidermal growth factor (EGF) expands the proliferation of neural stem/progenitor cells in the subventricular zone (SVZ), overexpression of brain-derived neurotrophic factor (BDNF) is particularly effective in enhancing striatal neurogenesis. We assessed the induction of striatal neurogenesis and consequent functional recovery after chronic infusion of BDNF and EGF in an adult animal model of neonatal hypoxic-ischemic (HI) brain injury. Permanent brain damage was induced in CD-1 (ICR) mice (P7) by applying the ligation of unilateral carotid artery and hypoxic condition. At 6 weeks of age, the mice were randomly assigned to groups receiving a continuous 2-week infusion of one of the following treatments into the ventricle: BDNF, EGF, BDNF/EGF, or phosphate buffered saline (PBS). Two weeks after treatment, immunohistochemical analysis revealed an increase in the number of BrdU(+) cells in the SVZ and striata of BDNF/EGF-treated mice. The number of new neurons co-stained with BrdU and betaIII-tubulin was also significantly increased in the neostriata of BDNF/EGF-treated mice, compared with PBS group. In addition, the newly generated cells were expressed as migrating neuroblasts labeled with PSA-NCAM or doublecortin in the SVZ and the ventricular side of neostriata. The new striatal neurons were also differentiated as mature neurons co-labeled with BrdU(+)/NeuN(+). When evaluated post-surgical 8 weeks, BDNF/EGF-treated mice exhibited significantly longer rotarod latencies at constant speed (48 rpm) and under accelerating condition (4-80 rpm), relative to PBS and untreated controls. In the forelimb-use asymmetry test, BDNF/EGF-treated mice showed significant improvement in the use of the contralateral forelimb. In contrast, this BDNF/EGF-associated functional recovery was abolished in mice receiving a co-infusion of 2% cytosine-b-d-arabinofuranoside (Ara-C), a mitotic inhibitor. Induction of striatal neurogenesis by the intraventricular administration of BDNF and EGF promoted functional recovery in an adult animal model of neonatal HI brain injury. The effect of Ara-C to completely block functional recovery indicates that the effect may be the result of newly generated neurons. Therefore, this treatment may offer a promising strategy for the restoration of motor function for adults with cerebral palsy (CP). Published by Elsevier Ltd.

Objectives: To identify evidence evaluating the effectiveness of physiotherapy in adolescents (>16 years of age) and adults with cerebral palsy.Data sources: Systematic literature search from the earliest available time until March 2009. Additional studies were identified through reference and citation tracking.Review methods: Two reviewers independently agreed on eligibility, methodological quality and quality of evidence assessment. Standard methods were used for quality assessments.Results: Included were 13 studies, two of which were randomized controlled trials. No article met the criteria for high methodological quality. Evidence of moderate quality was found on gait after strength training. Evidence of low quality was found on balance after strength training and workstation interventions. Low-quality evidence was also found on functionality after strength training in four studies evaluating gross motor capacity. There was very low-quality evidence on increased muscle strength and in outcome measures used to evaluate range of motion.Conclusion: Evidence for the effect of physiotherapy on adolescents and adults with cerebral palsy is sparse, and therefore there is an urgent need for well-designed physiotherapeutic trials for these people.

BACKGROUND: Children born preterm are known to be at risk for neurodevelopmental disorders. The role of perinatal asphyxia in this increased risk is still a matter of debate. AIM: To analyze the contribution of perinatal asphyxia in a population of preterm infants admitted to a secondary paediatric setting to neurological dysfunction in the first months after birth and to the development of cerebral palsy. METHODS: 17 preterm infants with perinatal asphyxia born before 35weeks postmenstrual age (PMA) and 34 carefully matched preterm controls without asphyxia were studied. Neuromotor outcome was examined by means of three assessments of the quality of general movements (GM) at “preterm” (around 34weeks PMA), “writhing” (around term age) and “fidgety” GM age (around 3months post term). Follow-up until at least 18months corrected age focused on the presence of cerebral palsy (CP). RESULTS: GM-quality of infants with asphyxia and of those without did not differ. Multivariate analysis revealed that abnormal GMs at “preterm” age were associated with respiratory problems, those at “writhing” age with none of the assessed risk factors, and those at “fidgety” age with the severity of periventricular leukomalacia (PVL) on neonatal ultrasound scan. Perinatal asphyxia was not associated with the development of CP. CP was associated with PVL and the presence of abnormal GMs at “fidgety” age. CONCLUSION: Perinatal asphyxia in preterm infants is not associated with an increased risk for neurodevelopmental problems including CP. Respiratory problems during the neonatal period are associated with PVL and adverse neurological outcome. Copyright © 2010. Published by Elsevier Ireland Ltd.

The differential diagnosis of parkinsonian syndromes is considered one of the most challenging in neurology, even for movement disorder specialists. Despite published consensus operational criteria for the diagnosis of Parkinson’s disease (PD) and the various atypical parkinsonian disorders (APDs) such as progressive supranuclear palsy, multiple system atrophy, and corticobasal syndrome, the clinical separation of APDs from PD carries a high rate of misdiagnosis. However, an early differentiation between APD and PD, each characterized by a largely different natural history, is crucial for determining the prognosis and choosing a treatment strategy. Despite limitations, the different modern magnetic resonance (MR) techniques have undoubtedly added to the differential diagnosis of neurodegenerative parkinsonism. Conventional MRI with visual assessment of T(2)- and T(1)-weighted imaging as well as various advanced MRI techniques offer objective measures and may therefore be useful tools in the diagnostic workup of PD and APDs. In clinical practice, conventional MRI is a well-established method for the exclusion of symptomatic parkinsonism due to other pathologies such as tumors, cerebral ischemia or inflammatory diseases. Furthermore, over the past two decades, advances in MR techniques have enabled to quantitatively illustrate abnormalities in the basal ganglia and infratentorial structures in APDs by methods such as magnetic resonance volumetry, diffusion-weighted imaging, magnetization transfer imaging and proton magnetic resonance spectroscopy. This article aims to review research findings on the value of MRI techniques in the differential diagnosis of neurodegenerative parkinsonian disorders. Copyright © 2010 S. Karger AG, Basel.