Dong Wook Rha

Motor pathway injury in patients with periventricular leucomalacia and spastic diplegia

Periventricular leucomalacia has long been investigated as a leading cause of motor and cognitive dysfunction in patients with spastic diplegic cerebral palsy. However, patients with periventricular leucomalacia on conventional magnetic resonance imaging do not always have motor dysfunction and preterm children without neurological abnormalities may have periventricular leucomalacia. In addition, it is uncertain whether descending motor tract or overlying cortical injury is related to motor impairment. To investigate the relationship between motor pathway injury and motor impairment, we conducted voxelwise correlation analysis using tract-based spatial statistics of white matter diffusion anisotropy and voxel-based-morphometry of grey matter injury in patients with periventricular leucomalacia and spastic diplegia (nu2009=u200943, mean 12.86u2009±u20094.79 years, median 12 years). We also evaluated motor cortical and thalamocortical connectivity at resting state in 11 patients using functional magnetic resonance imaging. The functional connectivity results of patients with spastic diplegic cerebral palsy were compared with those of age-matched normal controls. Since γ-aminobutyric acid(A) receptors play an important role in the remodelling process, we measured neuronal γ-aminobutyric acid(A) receptor binding potential with dynamic positron emission tomography scans (nu2009=u200927) and compared the binding potential map of the patient group with controls (nu2009=u200920). In the current study, white matter volume reduction did not show significant correlation with motor dysfunction. Although fractional anisotropy within most of the major white matter tracts were significantly lower than that of age-matched healthy controls (Pu2009<u20090.05, family wise error corrected), fractional anisotropy mainly within the bilateral corticospinal tracts and posterior body and isthmus of the corpus callosum showed more significant correlation with motor dysfunction (Pu2009<u20090.03) than thalamocortical pathways (Pu2009<u20090.05, family-wise error corrected). Cortical volume of the pre- and post-central gyri and the paracentral lobule tended to be negatively correlated with motor function. The motor cortical connectivity was diminished mainly within the bilateral somatosensory cortex, paracentral lobule, cingulate motor area and visual cortex in the patient group. Thalamovisual connectivity was not diminished despite severe optic radiation injury. γ-Aminobutyric acid(A) receptor binding potential was focally increased within the lower extremity homunculus, cingulate cortex, visual cortex and cerebellum in the patient group (Pu2009<u20090.05, false discovery rate corrected). In conclusion, descending motor tract injury along with overlying cortical volume reduction and reduced functional connectivity appears to be a leading pathophysiological mechanism of motor dysfunction in patients with periventricular leucomalacia. Increased regional γ-aminobutyric acid(A) receptor binding potential appears to result from a compensatory plasticity response after prenatal brain injury.

Short-term effects of combined serial casting and botulinum toxin injection for spastic equinus in ambulatory children with cerebral palsy.

Purpose The purpose of this paper is to test the hypothesis that combination therapy of serial cast and botulinum toxin type A (BTX-A) injection can further enhance the effects of a BTX-A injection in ambulant children with cerebral palsy (CP) who have an equinus foot. Materials and Methods Children in group A (30 legs of 21 children) received a serial casting application after an injection of BTX-A, and children in group B (25 legs of 17 children) received only a BTX-A injection. Assessments were performed before the intervention and 1 month after the intervention. Results After the intervention, there were significant improvements in tone, dynamic spasticity, and passive range of motion (ROM) in both groups. However, the changes were greater in group A than in group B. Dimension D (standing) in Gross Motor Function Measure (GMFM)-66 was significantly improved in group A but not in group B. On the other hand, there were no significant changes in di-mension E (walking, running, jumping) in GMFM-66 in either group. Conclusion The results of our study suggest that a serial casting application after BTX-A injection can enhance the benefits of BTX-A injection in children with cerebral palsy.

Assessment of regional GABAA receptor binding using 18F-fluoroflumazenil positron emission tomography in spastic type cerebral palsy

Periventricular leukomalacia (PVL) due to hypoxic-ischemic insult to the immature brain, chorioamnionitis and maternal infection are the major etiological factors of spastic type cerebral palsy (CP). Despite advances in preventing and treating certain causes of CP, the number of patients has remained essentially unchanged and the pathophysiological mechanisms related to motor dysfunction remain poorly understood. In this study, statistical parametric mapping (SPM) analysis of cerebral gamma-aminobutyric acid (GABA) receptor PET imaging using [18F]-fluoroflumazenil showed increased GABA(A) receptor binding in the bilateral motor and visual cortices in spastic diplegia (SD) type CP patients (n = 20) compared with normal controls (n = 10). As GABA(A) receptor signaling modulates biological perception and production of movement, complex motor skills and use-dependent plasticity in the motor cortex, increased GABA(A) receptor binding in the motor cortex might play a important role in poor motor control. Decreased GABA(A) receptor binding was seen in the brain stem in SD CP patients, which appears to be related to spastic symptom.

Intramuscular nerve distribution pattern of ankle invertor muscles in human cadaver using sihler stain

Introduction: We sought to the ideal sites for botulinum toxin injection by examining the intramuscular nerve patterns of the ankle invertors. Methods: A modified Sihler method was performed on the flexor hallucis longus, tibialis posterior, and flexor digitorum longus muscles (10 specimens each). The muscle origins, nerve entry points, and intramuscular arborization areas were measured as a percentage of the total distance from the most prominent point of the lateral malleolus (0%) to the fibular head (100%). Results: Intramuscular arborization patterns were observed at 20–50% for the flexor hallucis longus, 70–80% for the tibialis posterior, and 30–40% for the flexor digitorum longus. Conclusions: These findings suggest that treatment of muscle spasticity of the ankle invertors involves botulinum toxin injections in specific areas. These areas, corresponding to the areas of maximum arborization, are recommended as the most effective and safest points for injection. Muscle Nerve 53: 742–747, 2016

Novel Influenza A (H1N1)-Associated Acute Necrotizing Encephalopathy: A Case Report

Several cases of acute necrotizing encephalopathy (ANE) with influenza A (H1N1) have been reported to date. The prognosis of ANE associated with H1N1 is variable; some cases resulted in severe neurologic complication, whereas other cases were fatal. Reports mostly focused on the diagnosis of ANE with H1N1 infection, rather than functional recovery. We report a case of ANE with H1N1 infection in a 4-year-old Korean girl who rapidly developed fever, seizure, and altered mentality, as well as had neurologic sequelae of ataxia, intentional tremor, strabismus, and dysarthria. Brain magnetic resonance imaging showed lesions in the bilateral thalami, pons, and left basal ganglia. To our knowledge, this is the first report of ANE caused by H1N1 infection and its long-term functional recovery in Korea.

Change in Pulmonary Function after Incentive Spirometer Exercise in Children with Spastic Cerebral Palsy: A Randomized Controlled Study

Purpose The aim of this study was to investigate the effect of incentive spirometer exercise (ISE) on pulmonary function and maximal phonation time (MPT) in children with spastic cerebral palsy (CP). Materials and Methods Fifty children with CP were randomly assigned to two groups: the experimental group and the control group. Both groups underwent comprehensive rehabilitation therapy. The experimental group underwent additional ISE. The forced vital capacity (FVC), forced expiratory volume at one second (FEV1), FEV1/FVC ratio, peak expiratory flow (PEF), and MPT were assessed as outcome measures before and after 4 weeks of training. Results There were significant improvements in FVC, FEV1, PEF, and MPT in the experimental group, but not in the control group. In addition, the improvements in FVC, FEV1, and MPT were significantly greater in the experimental group than in the control group. Conclusion The results of this randomized controlled study support the use of ISE for enhancing pulmonary function and breath control for speech production in children with CP.

Abdominal ultrasonography findings in patients with spinal cord injury in Korea.

This is a retrospective study of 500 patients with spinal cord injury who underwent abdominal ultrasonography as a routine screening test from 2000 to 2003. We analyzed the results according to the different abdominal organ systems. Among the 500 cases, 226 (45.2%) showed abnormal findings. 98 cases of abnormal findings in the liver included 75 of fatty liver and 13 of mass. The 88 cases of abnormal findings in the bladder included 56 of bladder wall thickening, 14 of cystitis and 10 of urinary stone. The 35 cases of abnormal findings in the kidney included 19 of renal cyst and 6 of pelvic dilatation. The 35 cases with gallbladder abnormalities included 19 with gallstones and 11 with biliary sludge. Excluding the cases with bladder wall thickening, there were still 170 cases with abnormal ultrasonographic findings. Abdominal sonography seems to be a useful tool in detecting hidden intraabdominal pathologies in patients with spinal cord injury.

Relation among the Gross Motor Function, Manual Performance and Upper Limb Functional Measures in Children with Spastic Cerebral Palsy

Purpose The objective of this study was to describe hand function in relation with gross motor function and subtype of spastic cerebral palsy and to investigate the relationships among gross motor function, bimanual performance, unimanual capacity and upper limb functional measures in children with spastic cerebral palsy (CP). Materials and Methods We collected upper extremity data of 140 children with spastic CP. The Gross Motor Functional Classification System (GMFCS) was used to assess gross motor function, Manual Ability Classification System (MACS) for bimanual performance, and Modified House Functional Classification (MHC) for the best capacity of each hand. Upper limb functions were evaluated by using the Upper Limb Physicians Rating Scale and Upper Extremity Rating Scale. Results There was a good correlation between GMFCS and MACS in children with bilateral CP, but the correlation was not strong in children with unilateral CP. No significant difference between GMFCS and MACS was found in children with bilateral CP, but children with unilateral CP scored higher on GMFCS than on MACS. A strong correlation was observed between MACS and MHC in children with bilateral CP, but not in children with unilateral CP. The upper limb functional measures in each hand were highly related with MACS and MHC in bilateral CP, but not in unilateral CP. Conclusion Gross motor function, bimanual performance and the best capacity of each hand are closely related with each other in children with bilateral CP, but not in children with unilateral CP.

Reliability and Validity of the Upper Limb Physician's Rating Scale in Children with Cerebral Palsy

Purpose The Upper Limb Physicians Rating Scale (ULPRS) is a tool that assesses movement quality of the upper limbs. It is used as an outcome measure after botulinum toxin type A injection in children with cerebral palsy (CP). This study aimed to investigate the reliability and validity of the ULPRS in children with spastic CP. Materials and Methods Thirty children with spastic CP (M:F=17:13) aged 5 to 13 years old were recruited. The ULPRS was scored based on recorded videotapes by four physicians on two separate occasions. The Melbourne Assessment of Unilateral Upper Limb Function (MUUL) was scored by an occupational therapist. Intraclass correlation coefficients (ICCs), 95% confidence intervals and weighted kappa statistics were calculated for the scores of ULPRS to obtain interrater and intrarater reliability. The relationship between ULPRS and MUUL was assessed using Pearson correlation coefficients. Results The ICCs for the total ULPRS scores were 0.94 between raters and 0.99 to 1.00 within raters. The weighted kappa statistics for subitem scores for the ULPRS ranged from 0.67 to 1.00 within raters and from 0.46 to 0.86 between raters. The relationship between ULPRS and MUUL was strong (Pearson correlation coefficient=0.751; p<0.05). Conclusion The results demonstrated the high reliability of the total ULPRS score within and between raters. A significant concurrent validity between ULPRS and MUUL also supports the clinical utility of the ULPRS as an outcome measure of spastic upper limb in children with CP.

Clinical Features and Associated Abnormalities in Children and Adolescents With Corpus Callosal Anomalies

Callosal anomalies are frequently associated with other central nervous system (CNS) and/or somatic anomalies. We retrospectively analyzed the clinical features of corpus callosal agenesis/hypoplasia accompanying other CNS and/or somatic anomalies. We reviewed the imaging and clinical information of patients who underwent brain magnetic resonance imaging in our hospital, between 2005 and 2012. Callosal anomalies were isolated in 13 patients, accompanied by other CNS anomalies in 10 patients, associated with only non-CNS somatic anomalies in four patients, and with both CNS and non-CNS abnormalities in four patients. Out of 31 patients, four developed normally, without impairments in motor or cognitive functions. Five of nine patients with cerebral palsy were accompanied by other CNS and/or somatic anomalies, and showed worse Gross Motor Function Classification System scores, compared with the other four patients with isolated callosal anomaly. In addition, patients with other CNS anomalies also had a higher seizure risk.