- Lower extremity strength profile in ambulatory adults with cerebral palsy and spastic diplegia; norm values and reliability for hand-held dynamometry. [Journal Article]PM R 2019PM R
- CONCLUSIONS: No subtypes other than adults with CP and spastic diplegia were included.Lower extremity strength profiles demonstrate substantial muscle weakness in ambulatory adults with CP compared to TD adults, which highlights the importance to address muscle weakness in this population group. These strength profiles may serve as norm values for clinicians and researchers. In addition, HHD showed to be reliable to assess lower extremity strength in adults with CP.
- Comparison of Gait Ability of a Child with Cerebral Palsy According to the Difference of Dorsiflexion Angle of Hinged Ankle-Foot Orthosis: A Case Report. [Journal Article]Am J Case Rep 2019; 20:1454-1459AJ
- CONCLUSIONS: The results of this study demonstrated that wearing a dorsiflexion 10° hinged AFO would have a positive effect on improving gait ability of a child with cerebral palsy rather than wearing a bare foot and a general hinged AFO.
- Functional Outcome of Adulthood Selective Dorsal Rhizotomy for Spastic Diplegia. [Journal Article]Cureus 2019; 11(7):e5184C
- Objective The medical evidence supporting the efficacy of selective dorsal rhizotomy (SDR) on children with spastic diplegia is strong. However, the outcome of SDR on adults with spastic diplegia remains undetermined. The aim is to study the effectiveness and morbidities of SDR performed on adults for the treatment of spastic diplegia. Methods Patients who received SDR in adulthood for the treatm…
Objective The medical evidence supporting the efficacy of selective dorsal rhizotomy (SDR) on children with spastic diplegia is strong. However, the outcome of SDR on adults with spastic diplegia remains undetermined. The aim is to study the effectiveness and morbidities of SDR performed on adults for the treatment of spastic diplegia. Methods Patients who received SDR in adulthood for the treatment of spastic diplegia were surveyed. The survey questionnaire addressed the living situation, education level, employment, health outcomes, postoperative changes of symptoms, changes in ambulatory function, adverse effects of SDR and orthopedic surgery after SDR. Results The study included 64 adults, who received SDR for spastic diplegia. The age at the time of surgery was between 18 and 50 years. The age at the time of the survey was between 20 and 52 years. The follow-up period ranged from one to 28 years. The study participants reported post-SDR improvements of the quality of walking in 91%, standing in 81%, sitting in 57%, balance while walking 75%, ability to exercise in 88%, endurance in 77%, and recreational sports in 43%. Muscle and joint pain present before surgery improved in 64% after surgery. Concerning the level of ambulatory function, all patients who walked independently in all environments maintained the same level of ambulatory function. Eighteen percent of the patients who walked independently in some environments improved to the independent walking in all environments. All patients who walked with an assistive device before SDR maintained the assistive walking after SDR. Concerning adverse effects of SDR, 50% (32 of 64 patients) developed numbness in the various parts of the legs. Two patients reported a complete loss of sensation in parts of the legs, and one patient reported numbness and constant pain in the bilateral lower extremities. Ten patients (16%) reported recurrent spasticity after SDR, and three patients (5%) reported ankle clonus, which is an objective sign of spasticity. Tendon lengthening surgery after SDR was needed in 27% and hip and knee surgery in 2% and 6%, respectively. Conclusions The great majority of our 64 patients, who received adulthood SDR for spastic diplegia, improved the quality of ambulation and abated signs of early aging. Numbness and diminished sensation in the lower extremity was the most common adverse effect of the adulthood SDR.
- Early Brain Damage Affects Body Schema and Person Perception Abilities in Children and Adolescents with Spastic Diplegia. [Journal Article]Neural Plast 2019; 2019:1678984NP
- Early brain damage leading to cerebral palsy is associated to core motor impairments and also affects cognitive and social abilities. In particular, previous studies have documented specific alterations of perceptual body processing and motor cognition that are associated to unilateral motor deficits in hemiplegic patients. However, little is known about spastic diplegia (SpD), which is character…
Early brain damage leading to cerebral palsy is associated to core motor impairments and also affects cognitive and social abilities. In particular, previous studies have documented specific alterations of perceptual body processing and motor cognition that are associated to unilateral motor deficits in hemiplegic patients. However, little is known about spastic diplegia (SpD), which is characterized by motorial deficits involving both sides of the body and is often associated to visuospatial, attentional, and social perception impairments. Here, we compared the performance of a sample of 30 children and adolescents with SpD (aged 7-18 years) and of a group of age-matched controls with typical development (TD) at two different tasks tapping on body representations. In the first task, we tested visual and motor imagery abilities as assessed, respectively, by the object-based mental rotation of letters and by the first-person transformations for whole-body stimuli. In the second task, we administered an inversion effect/composite illusion task to evaluate the use of configural/holistic processing of others' body. Additionally, we assessed social perception abilities in the SpD sample using the NEPSY-II battery. In line with previously reported visuospatial deficits, a general mental imagery impairment was found in SpD patients when they were engaged in both object-centered and first-person mental transformations. Nevertheless, a specific deficit in operating an own-body transformation emerged. As concerns body perception, while more basic configural processing (i.e., inversion effect) was spared, no evidence for holistic (i.e., composite illusion) body processing was found in the SpD group. NEPSY-II assessment revealed that SpD children were impaired in both the theory of mind and affect recognition subtests. Overall, these findings suggested that early brain lesions and biased embodied experience could affect higher-level motor cognition and perceptual body processing, thus pointing to a strict link between motor deficits, body schema alterations, and person processing difficulties.
- The effects of anterior seat inclination on movement time, mechanical work and kinematics during sit-to-stand in children with spastic diplegic cerebral palsy. [Journal Article]Disabil Rehabil Assist Technol 2019; :1-4DR
- CONCLUSIONS: Anterior seat inclination with 5°, 10° and 15° could improve STS performance in children with SDCP. Therefore, applying these seats would be another option for children with SDCP to enhance STS performance. IMPLICATIONS FOR REHABILITATION Anterior seat inclination could improve sitting posture in children with diplegia. Children with diplegia spent less time when STS from the anterior seat inclination. Children with diplegia used less energy when STS from the anterior seat inclination.
- Whether the newly modified rhizotomy protocol is applicable to guide single-level approach SDR to treat spastic quadriplegia and diplegia in pediatric patients with cerebral palsy? [Journal Article]Childs Nerv Syst 2019CN
- CONCLUSIONS: SL-SDR when guided by our newly modified rhizotomy protocol was still feasible to treat pediatric CP cases with spastic quadriplegia and diplegia. Cases in this condition could benefit from such a procedure when followed by our intensive rehabilitation program with regard to their motor function.
- Lipid nanoparticle-targeted mRNA therapy as a treatment for the inherited metabolic liver disorder arginase deficiency. [Journal Article]Proc Natl Acad Sci U S A 2019; 116(42):21150-21159PN
- Arginase deficiency is caused by biallelic mutations in arginase 1 (ARG1), the final step of the urea cycle, and results biochemically in hyperargininemia and the presence of guanidino compounds, while it is clinically notable for developmental delays, spastic diplegia, psychomotor function loss, and (uncommonly) death. There is currently no completely effective medical treatment available. While…
Arginase deficiency is caused by biallelic mutations in arginase 1 (ARG1), the final step of the urea cycle, and results biochemically in hyperargininemia and the presence of guanidino compounds, while it is clinically notable for developmental delays, spastic diplegia, psychomotor function loss, and (uncommonly) death. There is currently no completely effective medical treatment available. While preclinical strategies have been demonstrated, disadvantages with viral-based episomal-expressing gene therapy vectors include the risk of insertional mutagenesis and limited efficacy due to hepatocellular division. Recent advances in messenger RNA (mRNA) codon optimization, synthesis, and encapsulation within biodegradable liver-targeted lipid nanoparticles (LNPs) have potentially enabled a new generation of safer, albeit temporary, treatments to restore liver metabolic function in patients with urea cycle disorders, including ARG1 deficiency. In this study, we applied such technologies to successfully treat an ARG1-deficient murine model. Mice were administered LNPs encapsulating human codon-optimized ARG1 mRNA every 3 d. Mice demonstrated 100% survival with no signs of hyperammonemia or weight loss to beyond 11 wk, compared with controls that perished by day 22. Plasma ammonia, arginine, and glutamine demonstrated good control without elevation of guanidinoacetic acid, a guanidino compound. Evidence of urea cycle activity restoration was demonstrated by the ability to fully metabolize an ammonium challenge and by achieving near-normal ureagenesis; liver arginase activity achieved 54% of wild type. Biochemical and microscopic data showed no evidence of hepatotoxicity. These results suggest that delivery of ARG1 mRNA by liver-targeted nanoparticles may be a viable gene-based therapeutic for the treatment of arginase deficiency.
- Potential effects of traditional massage on spasticity and gross motor function in children with spastic cerebral palsy: A randomized controlled trial. [Journal Article]Pak J Med Sci 2019 Sep-Oct; 35(5):1210-1215PJ
- CONCLUSIONS: It is concluded that TM can effectively reduce the spasticity, does not have harmful effects, so can be administered safely by mothers at home and making it suitable for the management of spastic CP. However, in order to achieve better gross motor function, it should be practiced in conjunction with CPT, functional skills and task oriented approaches.
- Gait Pattern of Adults with Cerebral Palsy and Spastic Diplegia More Than 15 Years after Being Treated with an Interval Surgery Approach: Implications for Low-Resource Settings. [Journal Article]Indian J Orthop 2019 Sep-Oct; 53(5):655-661IJ
- CONCLUSIONS: Although different to TD adults, the gait patterns observed in the adult with CP treated with ISA is in line with other studies. Gait patterns suggest that derotation osteotomies potentially could have improved the long term gait patterns. Although SEMLS might be the preferred treatment method, potentially resulting in better outcomes, ISA can also be used to treat children with CP in developing countries as India and South Africa, where a SEMLS approach is not always feasible.
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- Hepatic arginase deficiency fosters dysmyelination during postnatal CNS development. [Journal Article]JCI Insight 2019; 4(17)JI
- Deficiency of arginase is associated with hyperargininemia, and prominent features include spastic diplegia/tetraplegia, clonus, and hyperreflexia; loss of ambulation, intellectual disability and progressive neurological decline are other signs. To gain greater insight into the unique neuromotor features, we performed gene expression profiling of the motor cortex of a murine model of the disorder…
Deficiency of arginase is associated with hyperargininemia, and prominent features include spastic diplegia/tetraplegia, clonus, and hyperreflexia; loss of ambulation, intellectual disability and progressive neurological decline are other signs. To gain greater insight into the unique neuromotor features, we performed gene expression profiling of the motor cortex of a murine model of the disorder. Coexpression network analysis suggested an abnormality with myelination, which was supported by limited existing human data. Utilizing electron microscopy, marked dysmyelination was detected in 2-week-old homozygous Arg1-KO mice. The corticospinal tract was found to be adversely affected, supporting dysmyelination as the cause of the unique neuromotor features and implicating oligodendrocyte impairment in a deficiency of hepatic Arg1. Following neonatal hepatic gene therapy to express Arg1, the subcortical white matter, pyramidal tract, and corticospinal tract all showed a remarkable recovery in terms of myelinated axon density and ultrastructural integrity with active wrapping of axons by nearby oligodendrocyte processes. These findings support the following conclusions: arginase deficiency is a leukodystrophy affecting the brain and spinal cord while sparing the peripheral nervous system, and neonatal AAV hepatic gene therapy can rescue the defects associated with myelinated axons, strongly implicating the functional recovery of oligodendrocytes after restoration of hepatic arginase activity.