Frontal N30 of median nerve SSEPs for evaluation of movement disorders with destructive basal ganglia deficits

This study was performed to show the usefulness of N30 and N20 of median nerve SSEPs for evaluating the pathogenesis of central nervous system movement disorders. The subjects were 14 patients, consisting of 7 patients with an extrapyramidal lesion, 3 patients with suspicion of hypoxic-ischemic cerebral injury before birth period (H-I group), 3 patients with kernicterus during the neonatal period (kernicterus group), and 1 patient having Wilson's disease. Seven patients had spastic hemiplegia. Patients with athetotic CP had the ability to handle an electric wheelchair alone, and those with spastic hemiplegia could walk alone. Thirty control subjects were included in this study for clinical investigation. The characteristic finding of a predominant absence or amplitude reduction of frontal N30 compared with that of parietal N20 (4 and 1, respectively, out of 6 examinations) was obtained in athetotic CP patients of the H-I group. The predominant absence of N30 compared with N20 did not appear in the kernicterus group, hemiplegic patients or a patient with Wilson's disease. Therefore, frontal N30 might sensitively reflect destructive damage in the frontal basal ganglia in children.     

Higher amount of MyHC IIX in a wrist flexor in tetraplegic compared to hemiplegic cerebral palsy

Spastic cerebral palsy can be divided into diagnostic groups by the relative severity of the arm impairment. This study investigates if hemiplegic, tetraplegic or diplegic cerebral palsy (CP) results in different patterns of myosin heavy chain (MyHC) expression in the flexor carpi ulnaris muscle from 17 young patients with CP. Using enzyme-immunohistochemistry and gel electrophoresis techniques we found a higher percentage of fibers expressing fast MyHC IIx (52%) in tetraplegic CP compared to hemiplegic patients (32%), (p<0.05). Tetraplegic CP also resulted in a lower amount of fibers expressing slow MyHC I (18%) compared to hemiplegic CP (40%), (p<0.005). The proportion of muscle fibers containing fetal MyHC was higher in tetraplegic CP compared to other groups, (p<0.005). Taken together theses results indicate that tetraplegic CP is associated with a shift from slow to fast myosins and that regenerative events are more prominent in tetraplegic CP compared with milder brain damage.     

Visual impairment in children with spastic cerebral palsy measured by psychophysical and electrophysiological grating acuity tests

Background: This study measured grating visual acuity in 173 children between 6–48 months of age who had different types of spastic cerebral palsy (CP).

Method: Behavioural acuity was measured with the Teller Acuity Cards (TAC) using a staircase psychophysical procedure. Electrophysiological visual acuity was estimated using the sweep VEP (sVEP).

Results: The percentage of children outside the superior tolerance limits was 44 of 63 (69%) and 50 of 55 (91%) of tetraplegic, 36 of 56 (64%) and 42 of 53 (79%) of diplegic, 10 of 48 (21%) and 12 of 40 (30%) of hemiplegic for sVEP and TAC, respectively. For the sVEP, the greater visual acuity deficit found in the tetraplegic group was significantly different from that of the hemiplegic group (p?<?0.001). In the TAC procedure the mean visual acuity deficits of the tetraplegic and diplegic groups were significantly different from that of hemiplegic group (p?<?0.001). The differences between sVEP and TAC means of visual acuity difference were statistically significant for the tetraplegic (p?<?0.001), diplegic (p?<?0.001), and hemiplegic group (p?=?0.004).

Discussion: Better visual acuities were obtained in both procedures for hemiplegic children compared to diplegic or tetraplegic. Tetraplegic and diplegic children showed greater discrepancies between the TAC and sVEP results. Inter-ocular acuity difference was more frequent in sVEP measurements.

Conclusions: Electrophysiologically measured visual acuity is better than behavioural visual acuity in children with CP.     

Differences in standing balance between patients with diplegic and hemiplegic cerebral palsy

Maintaining standing postural balance is important for walking and handling abilities in patients with cerebral palsy.This study included 23 patients with cerebral palsy(seven with spastic diplegia and16 with spastic hemiplegia),aged from 7 to 16 years of age.Standing posture balance measurements were performed using an AMTI model OR6-7 force platform with the eyes open and closed.Patients with diplegic cerebral palsy exhibited greater center of pressure displacement areas with the eyes open and greater center of pressure sway in the medial-lateral direction with the eyes open and closed compared with hemiplegic patients.Thus,diplegic patients exhibited weaker postural balance control ability and less standing stability compared with hemiplegic cerebral palsy patients.     

Correlation between ICIDH handicap code and Gross Motor Function Classification System in children with cerebral palsy

The aim of this study was to apply the International Classification of Impairments, Disabilities and Handicap (ICIDH; WHO 1980) parallel to the Gross Motor Function Classification System (GMFCS; Palisano et al. 1997) in a population-based series of children with cerebral palsy (CP). Of the 116 children studied, birth characteristics, data on gross motor function, and level of handicap at 5 to 6 years of age, were retrospectively collected from medical records and documentation made by rehabilitation team members. Low handicap scores and mild levels of gross motor disability were present in children with hemiplegic CP, moderate scores in children with diplegic CP, simple ataxia, and athetotic CP, and high scores in children with dystonic CP and tetraplegic CP. A significant correlation was found between high handicap scores as well as high levels on the GMFCS and the presence of learning disability, epilepsy, and obvious aetiology of CP. A strong correlation was found between the handicap code and the GMFCS, the strongest concerning the dimension of mobility (r = 0.95,p<0.0001). A striking similarity in the grading of disability was present between the ICIDH handicap code and the GMFCS. The GMFCS is considerably less time-consuming and can be evaluated retrospectively. The handicap code requires more detailed information and is more useful for a comprehensive profile of the child.     

Pituitary reserve of growth hormone in adolescent patients with cerebral palsy and mental retardation

The pituitary reserve of growth hormone (GH) was studied in 11 severely handicapped boys (14 approximately 18 years old) with mental retardation and cerebral palsy (CP); athetotic type 4 cases, and spastic type 7 cases. Serum GH was determined before and after intravenous injection of 1 ng/kg growth hormone releasing factor (GRF) or oral administration of 0.1 mg/m2 clonidine. The athetotic type patients showed low somatomedin C (Sm C) concentrations (0.31 +/- 0.11 U/ml), poor responses to clonidine, and good responses to GRF. The spastic type patients showed subnormal responses to GRF although their Sm C was normal (0.91 +/- 0.43 U/ml). Clinically, the stature was small, and the puberty was delayed in the former. The levels of plasma testosterone were significantly lower than those in the latter. It is concluded that athetotic type CP was associated with hypothalamic GRF deficiency and spastic type CP was associated with reduced pituitary reserve of GH.     

Pervasive developmental disorders in individuals with cerebral palsy

The aim of the present study was to describe the prevalence and associated factors of pervasive developmental disorders (PDD), including autistic disorder and PDD not otherwise specified (NOS), in a clinical sample of 126 children and adolescents (75 males, 51 females; age range 4–18y, mean 8y 8mo, SD 3y 8mo) with tetraplegic, hemiplegic, diplegic, dyskinetic, or mixed types of cerebral palsy (CP); 28% could not crawl or walk even with support, 29% could move with support, and 43% walked independently. Participants were examined for PDD in two stages. In the first stage, probable participants were determined by direct observation, Autism Behavior Checklist score, and medical reports. In the second stage, those with 'probable' symptoms underwent psychiatric examination and their autistic symptoms were scored on the Childhood Autism Rating Scale. The final diagnosis of autistic disorder or PDD-NOS was given according to DSM-IV criteria. Fourteen (11%) and five (4%) of the participants met the criteria for autistic disorder and PDD-NOS respectively. Children with CP and PDD differed from those without PDD in terms of type of CP ( p =0.02), presence of epilepsy ( p <0.001), intellectual level ( p <0.001), and level of speech ( p <0.001). PDD was more common in children with tetraplegic, mixed, and hemiplegic CP, and in children with epilepsy, learning disability, * and low level of speech. The findings corroborate the notion that CP is a complex disorder, often associated with additional impairments. PDD is not rare in CP and should be considered in patients with comorbid conditions such as epilepsy, learning disability, and language delay and in the presence of tetraplegic, mixed, and hemiplegic CP types.     

Manual skill, hand skill asymmetry, and neuropsychological test performance in schoolchildren with spastic cerebral palsy

Bilateral hand skill assessment with a computerised version of the Peg Moving Task, and neuropsychological testing, were performed in 30 children aged 7 to 8 years with spastic cerebral palsy (CP) and without mental retardation, diplegia (n = 10), right hemiplegia (n = 10), or left hemiplegia (n = 10), and in 30 controls. Compared to controls: (i) 30% of the hemiplegic children showed impairment of the unaffected hand and 70% of the diplegic children showed impairment in both hands; (ii) children with CP were impaired only in oral repetition and in visual-motor tasks. Results of neuropsychological testing were not significantly different between the three groups of children with CP. Right minus left asymmetry in hand skill was not related to neuropsychological testing; however, degree of impairment of the right hand was associated with phonological and metaphonological skills, and of the left hand with visuospatial and counting performance. Hand skill was related to the ability to perform many daily living manual activities. It is concluded that impairment of hand function, rather than the side of the more affected hand, is related to neuropsychological deficits in children with cerebral palsy.     

Z蛋白与小儿脑性瘫痪的关系

目的研究Z蛋白(PZ)和小儿脑性瘫痪之间的关系。方法随机选取脑瘫患儿40例(脑瘫组)、缺氧缺血性脑病患儿30例(HIE组)、脑瘫高危新生儿20例(脐血组)以及各组正常对照健康儿童20例,用酶联免疫吸附试验法(ELISA法)测定各组血浆中PZ的含量(脐血组测定脐血浆PZ含量)。结果脐血组PZ水平高于脐血对照组(P0.05);脑瘫组和HIE组血浆PZ水平均明显高于各自对照组(P0.01);HIE组血浆PZ水平显著高于脐血组(P0.05);脑瘫组血浆PZ水平明显高于HIE组(P0.05)。结论 PZ在小儿脑瘫的演变过程中呈现动态变化的规律,可在一定程度上为临床早期诊断、治疗提供参考依据。     

Manual skill,hand skill asymmetry,and neuropsychological test performance in schoolchildren with spastic cerebral palsy

Bilateral hand skill assessment with a computerised version of the Peg Moving Task, and neuropsychological testing, were performed in 30 children aged 7 to 8 years with spastic cerebral palsy (CP) and without mental retardation, diplegia (n = 10), right hemiplegia (n = 10), or left hemiplegia (n = 10), and in 30 controls. Compared to controls: (i) 30% of the hemiplegic children showed impairment of the unaffected hand and 70% of the diplegic children showed impairment in both hands; (ii) children with CP were impaired only in oral repetition and in visual‐motor tasks. Results of neuropsychological testing were not significantly different between the three groups of children with CP. Right minus left asymmetry in hand skill was not related to neuropsychological testing; however, degree of impairment of the right hand was associated with phonological and metaphonological skills, and of the left hand with visuospatial and counting performance. Hand skill was related to the ability to perform many daily living manual activities. It is concluded that impairment of hand function, rather than the side of the more affected hand, is related to neuropsychological deficits in children with cerebral palsy.     

Somatosensory and visual evoked potentials in children with cerebral palsy: Correlations and discrepancies with MRI findings and clinical picture

Purpose: To determine if there is any association between the findings of visual evoked potentials (VEPs), somatosensory evoked potentials (SEPs), and magnetic resonance imaging (MRI) findings with the neurodevelopment and severity in children with cerebral palsy (CP). Methods: The present study included 15 children with spastic diplegic CP and five children with spastic hemiplegic CP and 42 healthy children as controls. The number of the controls was two-times greater than the study group to increase statistical power of this study. VEPs and SEPs were recorded in the CP children and compared with healthy controls. All MR scans were obtained using a 1.5 T MR scanner. Results: A significant difference was found in the latencies P100 (VEP) between the CP and controls. No correlations between increased P100 latencies and asphyxia, prematurity, the CP severity, MRI findings and mental retardation were noted. A significant difference in N13–N20 conductions (SEPs) between the subjects with CP and the control group was found. SEPs were positively correlated with mental retardation in CP children. The brain lesions in MRI showed a significant correlation with the CP severity scores and mental retardation. Conclusion: The differences in VEPs and SEPs were determined between CP children and healthy children. The MRI findings were positively correlated with the CP severity and mental retardation.     

Health-related quality of life of children and adolescents with cerebral palsy: hearing the voices of the children

The aim of the study was to determine whether pediatric patients with cerebral palsy (CP; 24.3% hemiplegic, 30.4% diplegic, 38.5% quadriplegic) can self-report their health-related quality of life (HRQOL) with the Pediatric Quality of Life Inventory Version 4.0 (PedsQL 4.0) Generic Core Scales. The PedsQL was administered to 148 families (84 males, 64 females; mean 10y, SD 3y 9mo, range 5 to 18y) recruited from a children's hospital clinic and state medical therapy clinics. Sixty-nine children (47%) were able to self-report. Children with CP self-reported a significantly lower HRQOL (mean 65.9) than healthy children (mean 83.8). Children with CP self-reported a similar HRQOL to pediatric cancer patients receiving treatment (mean 68.9). Children diagnosed with quadriplegia (mean 49.4) self-reported more impaired HRQOL than children with diplegia (mean 69.1) and hemiplegia (mean 72.4). Parent-child concordance was lowest for emotional functioning. Providers and parents should obtain the children's HRQOL perceptions whenever possible.     

Spastic cerebral palsy: clinical magnetic resonance imaging correlation of 129 children

A prospective study was undertaken of 129 children with spastic cerebral palsy to clarify the relationship between magnetic resonance imaging (MRI) findings and clinical features of cerebral palsy. Low birth weight, asphyxia, prematurity, seizures, mental development, Gross Motor Function Classification System, and MRI findings were analyzed. Significant abnormalities relevant to the cerebral palsy were evident on imaging in 123 (95.3%). A similar percentage of MRI abnormalities were detected in the groups, 45 (100%) in patients with tetraplegic cerebral palsy, 37 (92.5%) in children with diplegic cerebral palsy, and 42 (95.4%) with hemiplegic cerebral palsy. Periventricular leukomalacia was detected more frequently in the children with spastic diplegia than in the patients with tetraplegia or hemiplegia. Cerebral atrophy was found more often in the tetraplegic group compared to the diplegic patients. Porencephalic cysts were detected more frequently in children with spastic hemiplegia. Congenital brain anomalies were found in a higher proportion in tetraplegic children. Significant correlations between the MRI findings and Gross Motor Function Classification System in the diplegic and tetraplegic patients were found. No correlations between the MRI results and risk factors for cerebral palsy in the tetraplegic patients were noted. Early detection of brain abnormalities in children with cerebral palsy may help in the prognosis and in the initiation of appropriate therapy     

Identification of gait patterns in individuals with cerebral palsy using multiple correspondence analysis

Great importance has been placed on the development of gait classification in cerebral palsy (CP) to assist clinicians. Nevertheless, gait classification is challenging within this group because the data is characterized by a high-dimensionality and a high-variability. Thus, the aim of this study was to analyze without a priori, a database of clinical gait analysis (CGA) of CP patients, using multiple correspondence analysis (MCA). A retrospective search, including biomechanical and clinical parameters was done between 2006 and 2012. One hundred and twenty two CP patients were included in this study (51 females and 71 males, mean age ± SD: 14.2 ± 7.5 years). Sixteen biomechanical spatio-temporal and kinematic parameters were included in the analysis. This data was transformed by a fuzzy window coding based on the distribution of each parameter in three modalities: low, average and high. Afterward, a MCA was used to associate parameters and to define classes. From this, seven most explicative gait parameters used to characterize gait of CP patients were identified: maximal hip extension, hip range, knee range, maximal knee flexion at initial contact, time of peak knee flexion, and maximal ankle dorsiflexion in stance phase and in swing phase. Moreover, four main profiles of CP patients have been defined from the multivariate approach: an apparent equinus gait group (the most similar of the control group with diplegic and hemiplegic patients with a GMFCS 1), a true equinus gait group (the youngest group with diplegic and some hemiplegic patients with a GMFCS 1), a crouch gait group (the oldest group with a majority of diplegic and rare hemiplegic patients with a GMFCS 2) and a jump knee gait group (the greatest level of global spasticity of the lower limbs with a majority of diplegic and rare hemiplegic patients with a GMFCS 2). Thus, this study showed the feasibility of the MCA in order to characterize and classify a large database of CP patients.     

Kinematic and dynamic analysis of the ankle joint in children with cerebral palsy

The assessment of walking in children affected by cerebral palsy (CP) is currently based principally on qualitative assessment criteria. Gait analysis, meanwhile, evaluates quantitatively kinematic and dynamic aspects of locomotion. The main aim of this study was to examine (using an optoelectronic system and a force platform) kinematic and dynamic aspects of the ankle joint in hemiplegic and in diplegic children (9 and 11 subjects respectively) and in 10 normal control subjects. Our results reveal, in spastic subjects, "equinus" placement of the foot in the initial contact phase, a lack of dorsiflexion (negative DF peak) in the stance phase and a reduction in push-off capacity (capacity to move the leg forward) in the swing phase. The results furnished by this study represent a useful contribution to the assessment of the walking ability of children affected by CP and to the evaluation of the indications and advantages of the rehabilitative intervention chosen.     

Spastic wrist flexors are more severely affected than wrist extensors in children with cerebral palsy

Morphological properties of skeletal muscle were compared between wrist flexors and extensors within the same children (n = 8, six females, two males; age range 4 to 9y, median age 7 y) with wrist muscle imbalance secondary to spastic cerebral palsy (CP). Five patients had hemiplegic CP, two diplegic CP, and one patient had tetraplegic CP. Muscle biopsies were taken during either tendon transfer or tendon lengthening procedures. Analyses included distribution of muscle fibre types, fibre sizes, and expression of developmental myosins. Extensor fibre area was significantly greater than flexor fibre area for type 2A fibres and type 2B fibres but not for type 1 fibres. Coefficient of variation (CV) of fibre size for all three fibre types was greater for flexors compared with extensors. The greatest CV was observed for the type 2A fibres in flexors (39.5 [3.6%]). A wide variation was observed for expression of developmental myosin with the magnitude of the expression being greater, but not statistically significant, in flexors compared with extensors (5.4/mm2 vs 0.53/mm2). These data demonstrate that significant secondary myopathy of wrist flexor muscles results from CP.     

Inhibitory and excitatory amino acids in the cerebrospinal fluid of children with two types of cerebral palsy*

BACKGROUND： Under normal conditions, excitatory amino acids are dynamically balanced with inhibitory amino acids. Excitatory amino acids have been implicated in perinatal brain injury. OBJECTIVE： To investigate differences in the levels of the excitatory amino acids glutamic acid and aspartic acid, and the inhibitory amino acid gamma-aminobutyric acid （GABA） in the cerebrospinal fluid （CSF） of children with spastic cerebral palsy or athetotic cerebral palsy. DESIGN, TIME AND SETTING： Case-control exploratory observation of neurotransmitter in patients. The experiment was performed in the Pediatrics Department of the Second Affiliated Hospital of Changsha Medical College, the Cerebral Palsy Center of Xiangtan Affiliated Hospital of South China University and the Pediatrics Department of Xiangya Hospital, between February 2006 and May 2007. PARTICIPANTS： We selected 27 children with cerebral palsy, including 13 with spastic cerebral palsy and 14 with athetotic cerebral palsy. We selected 10 patients who were not affected by any neurological disease as controls. METHODS： Two mL blood-free CSF was harvested between the third and fourth lumbar vertebrae of each patient after anesthesia, and stored at -70℃. One mL CSF was mixed with 10 mg sulfosalicylic acid and placed in ice-bath for 10 minutes, then centrifuged 2 000 g for 10 minutes. The supernatant was collected for amino acid quantitation. MAIN OUTCOME MEASURES： The concentrations of glutamic acid, aspartic acid and GABA in the CSF were determined by high-performance liquid chromatography and fluorometric method. The correlation of glutamic acid, aspartic acid and GABA levels with muscular tension in children with cerebral palsy was analyzed using linear dependence. RESULTS： The concentration of GABA was significantly lower in both spastic cerebral palsy and athetotic cerebral palsy patients than in the control group （P 〈 0.01）. Glutamic acid and aspartic acid were significantly higher in both cerebral palsy groups than in the control     

Dominant use of the left hand by athetotic cerebral palsied children

Hand preference was studied in 57 children with athetotic cerebral palsy. A left-sided preference was seen in 61% of the subjects. In more severely affected children for whom the possible cause was asphyxia, the left-sided preference was especially common. The perinatal brain damage causing athetosis may affect a motor system controlling movement on the right side more severely.     

Deviations in upper-limb function of the less-affected side in congenital hemiparesis

In the present study we examined upper-limb function of the less-affected side in young adolescents with congenital hemiparesis (cerebral palsy: CP). Five participants with hemiparetic CP and five control participants performed a cyclical reach-and-grasp task with the less-affected hand towards targets placed at 60%, 100%, and 140% of the participant's arm-length. Trunk involvement, end-effector kinematics and activation of the biceps and triceps were examined together with several clinical measures. Movements at the less-affected side were slower and peak velocity was reached later in the experimental group. Even though total trunk involvement was identical in both groups, it was selectively limited to forward bending in participants with CP. Elbow amplitudes of these participants were smaller for the 60% and 100% arm-length target distances. Additionally, participants with CP showed weak positive correlations between agonist (triceps) activity and elbow amplitude, suggesting that deficient agonist rather than antagonist innervation was responsible for the decreased elbow involvement. Especially the more severely affected participants with CP proved to compensate their relatively small elbow amplitudes by increased forward bending. Collectively, the findings demonstrate deviations in upper-limb control of the less-affected body side in congenital hemiparesis.     

Is thrombophilia a factor in the development of hemiplegic cerebral palsy?

A population-based study of children with hemiplegic cerebral palsy (CP) was performed to investigate whether thrombophilic tendencies are implicated in the aetiology of the condition. Thirty-eight children (23 males, 15 females; mean age 8.7 years, SD 4.1 years) with hemiplegic CP were ascertained. Twenty-seven children(18 males, nine females; mean age 8.4 years, SD 4.3) gave consent for inclusion. The non-study group comprised five males and six females; mean age 9.4 years, SD 4.1. In six children, seven thrombophilic 'abnormalities' were identified. Five of these abnormalities were of an equivocal nature and probably did not represent true clinical thrombophilia; reasons for this interpretation are discussed. Contrary to other published non-population-based studies, we have not shown an association between thrombophilia and hemiplegic CP. More studies, including maternal studies, are required to explore this complex subject further.