Cerebral Palsy

We describe a 78-year-old Japanese woman with early-stage progressive supranuclear palsy (PSP). She had a 3-week history of postural instability and gait disturbance. On examination, upper vertical gaze palsy, akinesia, hyperreflexia with pathological reflexes, hesitation, and postural instability were observed. Rigidity and resting tremors were not apparent. Brain MRI revealed atrophy of the frontotemporal lobes and dilatation of the third ventricle. A month later, she died of cerebral infarction. The total duration of her clinical course was approximately 2 months. The brain weighed 1180 g after fixation. Macroscopically, mild atrophy of the frontal lobes and mild depigmentation of the substantia nigra were observed. The conspicuous findings included degeneration confined to the subthalamic nucleus and substantia nigra and widespread but infrequent tau-positive neurofibrillary tangles/pretangles and glial fibrillary tangles (tuft-shaped astrocytes, coiled bodies and argyrophilic threads) in the brain. It has been reported that the most affected areas in PSP are the globus pallidus, subthalamic nucleus and substantia nigra. We suggest that degeneration in PSP would start with involvement of the substantia nigra and subthalamic nucleus.

Intrauterine infection is a unique pathologic process that raises the risk for early-onset neonatal sepsis (EONS). By acting synergistically with prematurity, EONS increases the risk for adverse neonatal outcomes, including intraventricular hemorrhage and cerebral palsy. Although several pathways for the pathogenesis of fetal damage have been proposed, the basic molecular mechanisms that modulate these events remain incompletely understood. Discovery of clinically and biologically relevant biomarkers able to reveal key pathogenic pathways and predict pregnancies at risk for antenatal fetal damage is a priority. Proteomics provides a unique opportunity to fill this gap.

We studied the effect of botulinum toxin A injections to the lower extremities of spastic cerebral palsy children on upper limb body function and occupational performance. A total of 16 children with spastic cerebral palsy, aged 2 to 8 years, Gross Motor Function Classification System levels I-IV, referred to a child neurology outpatient clinic for botulinum toxin A injections to the lower limbs, underwent 4 assessments: 1 month prior to injection, immediate pre injection, and at 1 and 5 to 6 months post injection. Three tools were used to test everyday function (Canadian Occupational Performance Measure and the Pediatric Evaluation of Disability Inventory) and upper extremity body function (Quality of Upper Extremity Skills Test). Significant improvement was documented between the immediate preinjection and the 2 postinjection assessments, indicating that improvement lasted for at least 6 months. Botulinum toxin A injections to the lower extremities in children with cerebral palsy improve upper limb body function and occupational performance.

BACKGROUND: During late pregnancy and the puerperium cerebral venous and sinus thrombosis (CVST) is a rare but important cause of stroke. Despite adequate anticoagulation some patients deteriorate, which may warrant the use of more aggressive treatment modalities. CASE REPORT: A 29-year-old pregnant woman (29th week of pregnancy) presented with diffuse headaches and a progressive left hemiparesis. MRI revealed a CVST involving the superior sagittal sinus, the left lateral sinus, and the rectal sinus. Furthermore, it showed a space occupying brain edema and a congestional bleeding within the frontal and parietal lobes on the right side. Despite immediate intravenous anticoagulation and treatment with mannitol the patient developed a progressive loss of consciousness and unilateral third nerve palsy as a result of a beginning transtentorial herniation. Due to the severe course of the CVST an urgent decompressive craniectomy and shortly thereafter a cesarean section were performed. The patient made an excellent recovery. CONCLUSION: While previous reports have demonstrated the feasibility of decompressive hemicraniectomy in selected patients with CVST and beginning herniation due to space occupying brain edema, venous infarction and congestional bleeding with mass effect, our rare case supports the notion that this procedure can also be life-saving in pregnant women.

OBJECTIVE: To probe into an effective therapy for treatment of cerebral palsy of involuntary movement. METHODS: Sixty cases were randomly divided into two groups, the control group was treated with routine rehabilitation method (Bobath + Tuina + scalp acupuncture), while the observation group was treated with plum-blossom needle on Governor Vessel and point Jiaji (EX-B 2) on the basis of routine rehabilitation method. After 3 months of treatment, therapeutic effect, total percentage of Gross Motor Function Measurement (GMFM), incurvation reflex and muscular tension fluctuation were compared. RESULTS: The obvious effective rate of 53.3% (16/30) in the observation group was superior to that of 20.0% (6/30) in the control group (P < 0.05); the total percentage of GMFM increased, incurvation reflex disappeared, muscular tension fluctuation relieved in both groups after treatment (P < 0.05, P < 0.01), but the indices above all improved more significantly in the observation group than those in the control group (P < 0.05, P < 0.01). CONCLUSION: Plum-blossom needle on Governor Vessel and point Jiaji (EX-B 2) on the basis of routine rehabilitation method for treatment of cerebral palsy of involuntary movement can enhance the gross motor function, make the incurvation reflex disappear effectively, relieve the muscular tension fluctuation.

Objective: The purpose of this study was to determine the effect of a new physiotherapy concept on bone density, muscle force and motor function in bilateral spastic cerebral palsy children. Methods: In a retrospective data analysis 78 children were analysed. The concept included whole body vibration, physiotherapy, resistance training and treadmill training. The concept is structured in two in-patient stays and two periods of three months home-based vibration training. Outcome measures were dual-energy x-ray absorption (DXA), Leonardo Tilt Table and a modified Gross Motor Function Measure before and after six months of training. Results: Percent changes were highly significant for bone mineral density, -content, muscle mass and significant for angle of verticalisation, muscle force and modified Gross Motor Function Measure after six months training. Conclusions: The new physiotherapy concept had a significant effect on bone mineral density, muscle force and gross motor function in bilateral spastic cerebral palsy children. This implicates an amelioration in all International Classification of Functioning, Disability and Health levels. The study serves as a basis for future research on evidence based paediatric physiotherapy taking into account developmental implications.

INTRODUCTION: Spasticity is an important medical and social problem with a high rate of incidence both in childhood, where it is mainly a result of infantile cerebral palsy, and in adults, where it is frequently brought about by a cerebrovascular accident, traumatic brain injury, spinal cord injury or multiple sclerosis, among other pathologies. It gives rise to important problems that have a significant effect on the quality of life of patients and their caregivers. Clinical practice guidelines (CPG) are instruments that help professionals, administrators and planners to make decisions that can improve clinical outcomes. DEVELOPMENT: Due to the complexity of spasticity, its evaluation and treatment should be carried out by specialised units using a multidisciplinary approach. One of the first-line treatment options is botulinum toxin type A (BTA). For this treatment to be successful and to be able to evaluate the patient’s progress, it is advisable to have a work protocol that includes the knowledge and tools necessary to use BTA in the most suitable way and in agreement with the available scientific evidence. All these factors led the Spasticity Unit to consider the need to draw up a set of CPG. CONCLUSIONS: These CPG have been in use at the Hospital Universitari Mutua de Terrassa for 15 years, and are updated on a regular basis. This has enabled us to carry out a standardised selection of spastic patients and to establish a clear-cut set of treatment goals. It has also allowed us to implement a suitable plan regarding follow-up, the adjuvant therapies needed for each of the patients and an evaluation of results that is as objective as possible.

OBJECTIVES: Information obtained from families is of particular importance in the evaluation of functional mobility skills of children with cerebral palsy (CP) after orthopedic interventions and long-term rehabilitation applications. This study was designed to evaluate the interobserver reliability of the Gillette Functional Assessment Questionnaire (FAQ) which was administered to the mothers and the physiotherapist for children with CP. METHODS: The study included 52 spastic diparetic children with CP (22 girls, 30 boys; mean age 7.8+/-4.4 years; range 4 to 12 years) and their mothers. According to the Gross Motor Function Classification System (GMFCS), all the children were in level 1 to 3. The Gillette FAQ was administered to the mother and physiotherapist to determine the functional walking level of the child and the interobserver reliability of the FAQ was calculated. In addition, gross motor performance was evaluated by the standing and walking-running-jumping dimensions of the Gross Motor Performance Measure (GMPM), and functional independence level was evaluated by the transfer and locomotion dimensions of the Functional Independence Measure for Children (WeeFIM). Correlations were sought between the FAQ results of the physiotherapist and mothers and the GMFCS, GMPM, and WeeFIM. RESULTS: The intraclass correlation coefficient for interobserver reliability of the Gillette FAQ was 0.94 (95% CI 0.898-0.966). A highly significant correlation was found between the responses of the mother and physiotherapist to the Gillette FAQ (r=0.882, p<0.01). The responses of the mother and physiotherapist to the Gillette FAQ showed a negative correlation with the GMFCS level, and positive correlations with the dimensions of the GMPM and WeeFIM studied (p<0.01). CONCLUSION: The Gillette FAQ can be used by the physiotherapists to determine the functional changes in spastic diparetic children with CP and can help clinicians derive important information from the families about functional walking of their children.

BACKGROUND: Morbidity attributable to hypoxic-ischaemic injury (HIE) in the perinatal period remains problematic, and timely and accurate assessment of the degree of injury is required for clinical management and prognosis. Conventional MR sequences typically appear normal in the first 48 h post HIE. While diffusion-weighted imaging (DWI) and apparent diffusion coefficient (ADC) maps register the injury earlier, perhaps within the first 24 h, it has been suggested that there may be a propensity at that early stage to underestimate the lesion severity or extent. OBJECTIVE: To assess whether MR imaging that included DWI, measured ADC values and T1- and T2-weighted sequences ultimately correlated with either neurodevelopmental outcome or with late MR imaging at 2 years of age. In addition, we wished to compare the performance of MR imaging with cranial US imaging. MATERIALS AND METHODS: All infants presenting with HIE who had an MRI within 10 days of life were eligible for enrollment and subsequently underwent a full neurodevelopmental assessment at 2 years of age. All children underwent repeat MRI at this time. All neonates had at least one cranial US study. The US findings were categorized as normal, abnormalities confined to the cerebral cortex and subcortical white matter, isolated central grey matter hyperechogenicity, and central hyperechogenicity combined with cerebral cortical/subcortical changes. All MRI studies were retrospectively reviewed by three radiologists. The patterns of injury on the early DWI and ADC maps and early T1- and T2-W studies were recorded as diffuse, central, watershed or atypical. The patterns of signal abnormality were assessed using a scoring system that yielded four separate scores [basal ganglia (BG), watershed (W), BG/W and summation (S)] for the three sets of images, a total of 12 scores in all. The appearance of the posterior limb of the internal capsule (PLIC) on T1-W inversion recovery sequences and of the corpus callosum on all sequences was also documented. After detailed neurodevelopmental assessment at 2 years of age, infants were classified into two groups according to whether they had a favourable or unfavourable outcome. RESULTS: Of the 26 infants, 6 infants died before formal assessment at the age of 2 years. A further 5 infants had moderate to severe cerebral palsy in addition to severe cognitive impairment. The remaining 15 infants were categorized in the favourable outcome group. The US appearance performed well in terms of predicting final outcome (P = 0.005). The pattern of ischaemia seen on early MRI was a significant predictor of outcome (P < 0.0001). The BG, BG/W and S scores of the diffusion imaging were significantly associated with outcome (P < 0.0001, P < 0.0001 and P = 0.0005 respectively). DWI was predictive of outcome group (P < 0.0001), as were the early T1- and T2-W sequences (P = 0.002) and cranial US (P = 0.005). Assessment of the PLIC in infants with watershed or atypical patterns of ischaemia was found to be less reliable in predicting outcome. The measured ADC value in the PLIC was significantly reduced in those children who had an unfavourable outcome (P = 0.03). CONCLUSION: While early MRI performed better than cranial US, the sonography findings were useful. The pattern of ischaemia on early MRI was a good predictor of prognosis. All infants with watershed or atypical patterns had a favourable outcome. The majority of infants with central patterns of ischaemia had an unfavourable outcome and all infants with a diffuse pattern had an unfavourable outcome. DWI was predictive of outcome group, as were early T1- and T2-W sequences and cranial US.