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1.

Background

Confusional migraine is a rare type of migraine presenting as an acute confusional state. However, the mechanism of this confusional state remains unclear.

Subject and methods

We examined an 11-year-old girl with confusional migraine, using electroencephalography, brain magnetic resonance imaging, cerebrovascular magnetic resonance angiography, and single-photon emission computed tomography to investigate cerebral blood flow changes.

Results

Our findings revealed vessel narrowing in the left middle and posterior cerebral artery territory, indicating vasospasm and suggesting that the confusion was caused by hypoperfusion. However, abnormal increased cerebral blood flow in the left middle and posterior cerebral artery territory was observed during the non-confusional state.

Conclusion

The recorded cerebral blood flow changes are similar to those associated with migraine attacks, gradually changing from abnormally low to abnormally high during the confusional and post-confusional state.  相似文献   

2.

Aim

To clarify the morphologic characteristics of the brain, which are the foundation of the emergence of general movements (GMs) in very-low-birth-weight infants.

Study design

Prospective cohort study. GMs were scored according to a semiquantitative scoring system: the GMs optimality score (GMOS) at preterm and term ages. Brain magnetic resonance imaging (MRI) at term-equivalent age was scored using a validated scoring system (MRI score). We examined the relationship between the two scores by multiple regression analysis with relevant clinical background.

Subjects

We included 50 very-low-birth-weight infants cared for at Oita University Hospital from August 2012 to August 2018 who underwent MRI and GMs assessment. Their median gestational age and birth weight were 29w2d and 1145?g, respectively.

Results

The MRI score and systemic steroid administration were related to preterm GMOS, and the MRI score was related to term GMOS. The component cerebellum score and cortical grey matter score of the MRI score were associated with preterm GMOS, and the cerebellum and the cerebral white matter scores were associated with term GMOS.

Conclusion

The quality of GMs was associated with brain morphological development. The co-evaluation of GMs and brain morphology leads to accurate developmental prediction.  相似文献   

3.

Objective

This study measured the serum carnitine levels in patients with epilepsy and determined the factors contributing to low carnitine levels.

Methods

We measured the serum carnitine levels in 94 consecutive patients with epilepsy, including the free carnitine (FC) and acylcarnitine fractions, using an enzyme cycling method. We defined a low FC as a serum FC level?<?36?μmol/L. Age, body mass index (BMI), standard deviation score of BMI (BMI-SDS), use of valproate, cognitive disorder, and feeding problems differed between patients with low and normal FC. In patients taking valproate, the associations of the serum FC level with the platelet count and serum ammonia and amylase levels were analyzed.

Results

Univariate analysis showed that a low BMI and BMI-SDS, the use of valproate, and cognitive disorder were more frequent in patients with a low FC. Logistic regression analysis revealed that a low BMI-SDS and cognitive disorders were independently associated with a low FC. Among the patients taking valproate, a low BMI-SDS and age were associated with a low FC. The serum FC and ammonia levels were inversely correlated, whereas no correlation was observed between the serum FC level and platelet count or serum amylase level.

Conclusion

A low BMI and cognitive disorders were related to a low FC in patients with epilepsy and the serum carnitine levels should be monitored in these patients.  相似文献   

4.

Purpose

Motor skill screening tools are essential for the early detection of developmental coordination disorder (DCD). The present study aimed to examine any cultural and rater effects on these tools. This then enabled us to judge the validity of the original cut-off values for identifying diagnosable children.

Methods

A community sample survey was performed in Japan; 3852 children aged 6–9?years were recruited. Both parents and teachers evaluated the motor skills of their children using the Movement Assessment Battery for Children – Second Edition Checklist. The psychometric properties were evaluated and the scoring characteristics examined based on the type of rater and country of origin, as compared to data originally sampled in the UK.

Results

High reliability and validity of the Japanese samples were confirmed. The Japanese adults evaluated their children’s motor skills more rigorously than the Europeans. Additionally, there was a large disagreement between parent and teacher rating scores; the degree of agreement varied depending on the severity of motor deficits in the child.

Conclusion

The first findings from a Japanese sample suggest that the assessment of motor skills in children is significantly affected by culture and rater. These cultural characteristics and rater biases strongly suggest that new cut-off values, reflecting country and rater type, be introduced for identifying children at risk of DCD.  相似文献   

5.

Background

Sensory processing difficulties, which commonly occur in autism spectrum disorder (ASD), are expected to have negative effects on the primary caregiver’s mental health. The aim of this study was to examine the association between sensory processing difficulties in children with ASD and the mental health of primary caregivers.

Methods

A total of 707 primary caregivers (mothers in the present study) and their children with ASD (4–18?years of age) participated in this study. Sensory processing difficulties were indexed using the Short Sensory Profile (SSP). The mental health of primary caregivers was indexed using the General Health Questionnaire (GHQ12).

Results

Higher scores on Auditory Filtering as measured with the SSP were associated with poorer mental health of primary caregivers, even after an adjustment for ASD symptom severity. Analyses of two age sub-groups, a young (4–10?years) and an old age group (11–18?years), revealed that higher scores on Tactile Sensitivity and Auditory Filtering were associated with poorer mental health of primary caregivers in younger children, whereas only higher scores on Auditory Filtering were associated with poorer mental health of primary caregivers in older children.

Conclusions

Our findings suggest that practitioners who support primary caregivers of children with ASD need to focus not only on the social and communication-related symptoms of the child but also on their specific sensory processing difficulties.  相似文献   

6.

Background

Attention-deficit hyperactivity disorder (ADHD) is a common childhood neuropsychiatric disorder. Diagnosis of ADHD is based on core symptoms or checklists; however, practitioner subjectivity inevitably results in instances of over- or under-diagnosis. Although an elevated theta/beta ratio (TBR) of the electroencephalography (EEG) band has been approved by the Food and Drug Administration as a factor that may be used in diagnosis of ADHD, several studies have reported no significant differences between the TBR of patients with ADHD and controls.

Purpose

In this study, a method was developed based on Hjorth Mobility (M) analysis of EEG to compare patients with ADHD and controls.

Methods

Differences in the presentations of ADHD between boys and girls are well established; therefore, separate investigations are required. The present study enrolled 30 girls with ADHD and 30 age-matched controls.

Results

The results revealed that the control group had significantly higher Hjorth M values in most brain areas in EEG readings compared with the values for the ADHD group. Compared with TBR, our method revealed a greater number of more significant differences between the girls in the ADHD group and the controls. Moreover, our method can produce the higher average sensitivity (0.796), average specificity (0.796), average accuracy (0.792), and average area under the curve of receiver operating characteristic curve (AUC) value (0.885). Therefore, compared with TBR, Hjorth M possessed the better potential for differentiating between girls with ADHD and controls.

Conclusion

The proposed method was more accurate than the TBR in diagnosing ADHD. Therefore, Hjorth M may be a promising tool for differentiating between children with ADHD and controls.  相似文献   

7.

Objective

We aimed to clarify the current status of pharmacotherapy for tic disorders and comorbidities in Japan. We used a systematic survey to collate the consensus of Japanese experts and compare it with the recent international evidence.

Methods

We devised a questionnaire on pharmacotherapy for tics and comorbidities and sent it to Japanese experts on tic disorders. Based on the response to the first survey, we revised the questionnaire and conducted a second survey to determine the consensus among the experts on a 4-point Likert scale by the Delphi method.

Results

The first survey revealed variability in preferred medications and dosages among the experts in Japan. However, we were able to build a general consensus on pharmacotherapy for tic disorders and comorbidities based on the second survey. Aripiprazole and risperidone were the first- and second-line medication for tic disorders, respectively. Agonists of α-2 adrenergic receptors were seldom prescribed. Fluvoxamine was the first-line medication for comorbid obsessive-compulsive disorder, and atomoxetine for comorbid attention deficit/hyperactivity disorder.

Conclusions

This study will help Japanese physicians choose medications for tic disorders more judiciously and will improve the quality of tic pharmacotherapy in Japan.  相似文献   

8.

Objective

To examine the relationship between the catch-up growth of preterm, SGA children and their behavioral development.

Methods

We analyzed data from a large Japanese, nationwide, population-based, longitudinal survey that started in 2001. We restricted the study participants to preterm children with information on height at 2?years of age (n?=?1667). Catch-up growth for SGA infants was defined as achieving a height at 2?years of age above ?2.0 standard deviations for chronological age. We then used logistic regression to estimate odds ratios (ORs) and 95% confidence intervals (95% CIs) for the associations of SGA/catch-up status with neurobehavioral development both at 5.5 and 8?years of age, adjusting for potential infant- and parent-related confounding factors.

Results

Twenty-six percent of preterm SGA infants failed to catch up. SGA children without catch-up growth were more likely to be unable to listen without fidgeting (OR 2.51, 95% CI: 1.06–5.93) and unable to focus on one task (OR 2.66, 95% CI: 1.09–6.48) compared with non-SGA children at 5.5?years of age. Furthermore, SGA children without catch-up growth were at significant risk for inattention at 8?years of age.

Conclusions

SGA infants with poor postnatal growth were at risk for attention problems throughout preschool-age to school-age among preterm infants. Early detection and intervention for attention problems among these infants is warranted.  相似文献   

9.

Background

Super-refractory status epilepticus (SRSE) is a seizure that continues >24?h after anesthesia, or recurs on the reduction of anesthesia. SRSE is extremely difficult-to-control and associated with poor outcome. To date, optimal therapy and outcome data in children is limited.

Objective

To assess etiology, treatment options and outcome in pediatric SRSE patients.

Method

We reviewed medical records of children <15?years old with SRSE during 2007–2017 at King Chulalongkorn Memorial Hospital. Demographic data, etiology, treatment, complications and discharge outcome were recorded.

Results

Seventeen patients, aged 1?month–13?years were included. The leading etiology was immune-mediated encephalitis (29.4%) and epilepsy (29.4%). The most common anesthetic agents were midazolam (94.1%) and propofol (52.9%) with the average maximal dose of 1.3 and 6.9?mg/kg/h respectively. Other treatments included immunological therapy (76.5%), ketogenic diet (76.5%), pyridoxine/pyridoxal-5-phosphate (70.5%). The most common complications were hypotension (61.5%), drug hypersensitivity (32.5%). Median length of anesthetic and intensive care were 9 and 23?days. The mortality rate was 17.6%, and 2 of 3 febrile infection-related epilepsy syndrome cases died. At discharge, all survivors were seizure free.

Conclusion

The majority of pediatric SRSE does not have epilepsy and the etiology is various. Treatment should expand from antiepileptic drugs to other modalities targeting different possible mechanisms such as immunomodulation or specific metabolic treatment. Multiple anesthetic drugs could be tolerated with close monitoring. Ketogenic diet, via enteral or parenteral route, could be considered early if requiring multiple anesthetic drugs. Initial outcome in children is relatively better than in adults.  相似文献   

10.

Background

When single pulse transcranial magnetic stimulation (TMS) is applied over the primary motor cortex (M1) with sufficient intensity, it evokes muscular contractions (motor-evoked potentials, MEPs) and muscle twitches (TMS-evoked movements). Participants may also report various hand sensations related to TMS, but the perception elicited by TMS and its relationship to MEPs and evoked movements has not been systematically studied.

Objective

The main aim of this work is to evaluate participants' kinesthetic and somatosensory hand perceptions elicited by single-pulse TMS over M1-hand area at different intensities of stimulation and their relation with MEPs and TMS-evoked movements.

Methods

We compared the number of MEPs (measured by electromyography), TMS-evoked movements (measured by an accelerometer) and participants' hand perception (measured by verbal report) elicited by TMS at different intensity of stimulation. This way, we estimated the amplitude of MEPs and the acceleration of TMS-evoked movements sufficient to trigger TMS evoked hand perceptions.

Results

We found that TMS-evoked hand perceptions are induced at 105% of the individual resting motor threshold, a value significantly different from the threshold inducing MEPs (about 100%) and TMS-evoked movements (about 110%). Our data indicate that only MEPs with an amplitude higher than 0.62 mV and TMS-evoked movements with acceleration higher than 0.42 m/s2 were associated with hand perceptions at threshold.

Conclusions

Our data reveal the main features of TMS-evoked hand perception and show that in addition to MEPs and TMS-evoked movements, this is a separate discernible response associated to single-pulse TMS over M1.  相似文献   

11.

Background

Myelin oligodendrocyte glycoprotein antibodies (MOG Abs) are frequently detected in pediatric acquired demyelinating syndrome (ADS), and MOG-Ab-positive ADS differs from multiple sclerosis (MS) and aquaporin-4 (AQP4)-Ab-positive neuromyelitis optica spectrum disorder (NMOSD) in terms of age distribution, therapeutic response, and prognosis.

Methods

Based on medical records, we retrospectively evaluated patients with MOG-Ab-positive NMOSD treated in the acute phase who were followed up in the chronic phase at our hospital from January 2011 to December 2017.

Results

The patients comprised two boys and two girls aged 3–12 (median, 8) years. Peak MOG-Ab titers were 1:2048 to 1:32768 (median, 1:10240), and the relapse rate ranged from 0 to 1.25 times/year (median, 0.59 times/year); no sequelae were observed in any cases. Lesions other than those of optic neuritis were distributed at the cortex in one patient, subcortical white matter in four, deep white matter in three, and brainstem in one, all of which were disseminated lesions. No lesions were found in the corpus callosum, periventricular white matter, diencephalon, and regions adjacent to the third and fourth ventricles. The lesions tended to be asymptomatic, and two patients aged >5?years had well-demarcated lesions.

Conclusion

All the patients showed disseminated lesions in the subcortical region to deep white matter, which were different from those found in MS and AQP4-Ab-positive NMOSD and were consistent with the characteristics of brain lesions in MOG-Ab-positive ADS, including other disease types.  相似文献   

12.

Background

Cardio-facio-cutaneous syndrome (CFCS) is a rare genetic disorder characterized by cardiovascular anomalies, dysmorphic faces, ectodermal abnormalities and developmental delays. Mutations in BRAF and other RAS-MAPK pathway-associated genes are commonly identified in patients with CFCS. While this molecular pathway is known to be associated with neuro-inflammatory conditions, only one case with CFCS has been reported thus far to develop acute encephalopathy in childhood.

Case report

A 3-year-old boy with dysmorphic features and mild psychomotor delay developed acute encephalopathy. After a 45-min long, generalized seizure, the magnetic resonance imaging revealed that the restricted diffusion signals spread to the bilateral subcortical white matters on day 1 of illness. Despite the 14?days of intensive care, the acute symptoms of encephalopathy left him intractable epilepsy and severe neurocognitive impairments. The whole-exome sequencing analysis identified a de novo heterozygous mutation of BRAF (NM_004333:p.Thr241Met) in this case.

Conclusion

The present case suggests that the hyperactive condition of ERK signals might augment the development of acute encephalopathy and post-encephalopathic epilepsy in childhood.  相似文献   

13.

Background

Nitrous oxide (N2O) is a commonly used inhaled anesthetic in outpatient dental procedures. However, the increasing recreational use of N2O may result in vitamin B12 deficiency-related neurologic and psychiatric symptoms. The aim of this study was to demonstrate the clinical features of chronic N2O abuse in pediatric patients.

Methods

Patients under 20?years of age who were diagnosed with N2O-induced subacute combined degeneration of the spinal cord from 2012 to 2018 were enrolled in this study. Clinical presentations, laboratory, imaging, ancillary studies, treatments and outcomes were analyzed.

Results

Nine patients were included, all of whom presented with symptoms of myeloneuropathy including limb numbness, limb weakness or unsteady gait. Six patients had low or low-normal vitamin B12 (cyanocobalamin) levels. Eight patients had evidence of subacute combined degeneration of the spinal cord via neuroimaging studies. All of the patients received vitamin B12 supplementation as treatment. All had full recovery of muscle power within 2?months. Five patients had persistent sensory deficits.

Conclusion

Chronic N2O abuse can cause permanent neurological damage if not treated promptly. Clinical staff should be aware of the various presentations of neurotoxicity related to N2O abuse.  相似文献   

14.

Objective

This study investigated the relationship between motor and cognitive/language development in children with Down syndrome (DS). We also tested the hypothesis that acquisition of walking skills facilitates later cognitive/language development.

Methods

Participants were 156 children with DS who were less than 48?months old and had undergone a health checkup by medical doctors and received rehabilitation treatment between April 2013 and March 2017 in Yokohama, Japan. To assess their development, the Kyoto Scale of Psychological Development (KSPD) 2001 was used, which measures development in three subdomains: Posture-Motor (P-M), Cognitive-Adaptive (C-A), and Language-Social (L-S). To investigate the relationship between motor and cognitive/language development, partial correlation analyses were conducted that controlled for participants’ age. To test the effect of achieving walking skills, regression analyses were conducted using only data from participants who took the KSPD at least twice and could not walk at the initial test.

Results

P-M developmental age (DA) was significantly and positively correlated with both C-A DA and L-S DA in children 1–3?years old. The relationship strengthened with increased age. Acquisition of walking skills had a significant positive effect on both the C-A DA and L-S DA at the second test when controlling for the C-A DA and L-S DA at the first test and age at the second test.

Conclusion

Motor development was correlated with both cognitive and language development in young children with DS. Results also suggested that achievement of walking could facilitate later cognitive/language development in children with DS.  相似文献   

15.

Background

Transcranial magnetic stimulation (TMS) is a non-invasive method to stimulate localized brain regions. Despite widespread use in motor cortex, TMS is seldom performed in sensory areas due to variable, qualitative metrics.

Objective

Assess the reliability and validity of tracing phosphenes, and to investigate the stimulation parameters necessary to elicit decreased visual cortex excitability with paired-pulse TMS at short inter-stimulus intervals.

Methods

Across two sessions, single and paired-pulse recruitment curves were derived by having participants outline elicited phosphenes and calculating resulting average phosphene sizes.

Results

Phosphene size scaled with stimulus intensity, similar to motor cortex. Paired-pulse recruitment curves demonstrated inhibition at lower conditioning stimulus intensities than observed in motor cortex. Reliability was high across sessions.

Conclusions

TMS-induced phosphenes are a valid and reliable tool for measuring cortical excitability and inhibition in early visual areas. Our results also provide appropriate stimulation parameters for measuring short-latency intracortical inhibition in visual cortex.  相似文献   

16.

Objective

To evaluate the efficacy and safety of pyridoxal for treating West syndrome.

Methods

We retrospectively investigated pyridoxal’s efficacy and safety in 117 patients with West syndrome at Saitama Children’s Medical Center from July 1993 to May 2016. Pyridoxal was administered at doses of 10–50?mg/kg/day. We evaluated seizure outcomes and electroencephalographic findings at 4?weeks after pyridoxal therapy. The responders were those with complete cessation of spasms for more than 4?weeks and those with resolution of hypsarrhythmia on EEG at 1–4?weeks after pyridoxal therapy.

Results

Five of the 117 patients (4.3%) were responders. The median duration between pyridoxal therapy to spasm cessation was 6 (5–13) days. Among the responders, four had hypsarrhythmia resolution, no spasm relapse, and no other seizure types more than 2?years after pyridoxal therapy. One responder had partial seizures and spasm relapse. No serious adverse effects occurred. There were no significant differences in sex, etiologies, complication, other seizure types preceding the spasms, onset age of spasms, age of pyridoxal therapy, treatment lag, initial and maintenance doses of pyridoxal, and adverse effects between pyridoxal responders and non-responders.

Conclusions

The efficacy rate of pyridoxal monotherapy as first-line treatment for West syndrome was low. However, pyridoxal therapy showed a rapid response within 1?week and was safe. We consider pyridoxal therapy as a kind of challenge therapy during the evaluation period concerning differential diagnosis and etiologies of West syndrome and immunological risks before adrenocorticotrophic hormone therapy or vigabatrin therapy.  相似文献   

17.

Objective

The present work aims to evaluate the significance of sleep disturbance and electroencephalogram (EEG) alteration in the early stage of Alzheimer's disease (AD).

Background and Rationale

Sleep disturbance is common in patients with AD. It is not known if it can occur at the early stage of AD and if EEG recording may help identify the early sign of the disease.

Historical Evolution

Sleep disturbance in AD has generally been considered as late consequence of the neurodegenerative process. A growing body of evidence has suggested that the sleep disturbance may occur at the early stage of AD.

Updated Hypothesis

Based on the previous epidemiologic studies and our recent findings, we propose that sleep disturbance may play an important role in the development of AD. Sleep EEG changes may serve as a valuable early sign for AD in the prepathological stage.

Early Experimental Data

Our data suggested that AβPPswe/PS1ΔE9 transgenic AD mice at preplaque stage (3 and 4 months of age) exhibited different profile of sleep architecture and sleep EEG, which preceded the cognitive deficit and AD neuropathology.

Future Experiments and Validation Studies

Future experiments should focus on sleep EEG changes in patients with mild cognitive impairment and early stage of AD. Follow-up studies in high-risk population of the elderly are equally important. In addition, the exact molecular mechanism underlying the sleep disturbance should be thoroughly investigated.

Major Challenges for the Hypothesis

Studies on human participants with early stage of AD, especially the follow-up studies on the presymptomatic elderly in a large population, are difficult and time-consuming.

Linkage to Other Major Theories

Our hypothesis may link previous theories to establish a bidirectional relationship between sleep disorders and AD, which may finally form a new schematic mechanism to understand the disease pathogenesis and disease progression.  相似文献   

18.

Objecive

To clarify the neuroimaging findings of children with acute flaccid myelitis during an outbreak of EV-D68 infection.

Methods

We performed a detailed review of the spinal and cranial MRI results of 54 children with acute flaccid myelitis. We focused on the range of longitudinal lesions, the localization and appearance of lesions within a horizontal section, Gadolinium-enhancement, and changes over time.

Results

All children had longitudinal spinal lesions involving central gray matter. Twenty-six children had lesions spanning the entire spine. Six of them had weakness in all limbs, whereas seven had weakness of only one limb. Thirty-eight children had lesions in both gray and white matter and limb weakness tended to be more severe in these children. During the acute period, spinal lesions showed bilateral ill-defined widespread T2 hyperintensity. During the subacute period, lesions were well defined and confined to the anterior horn. The distribution of limb weakness was correlated with the appearance of lesions during the subacute period. Gadolinium enhancement was performed in 37 children, and enhancement was seen in the cauda equina in 29 children. Enhancement was infrequent within 2?days after onset but was seen in almost all children thereafter. Twenty-two children had brainstem lesions continuous with spinal lesions.

Conclusion

Extensive longitudinal spinal lesions were characteristic in children with acute flaccid myelitis. Lesions were usually bilateral and widespread during the acute period, whereas localization to the anterior horn could become obvious. Although enhancement of the cauda equina was often observed, its appearance was sometimes delayed.  相似文献   

19.

Introduction

We measured the pain of children with disabilities before and after their physiotherapy session in a CAMSP. Then we identified all the elements related to the intervention of the therapist whose presence or not of stretching or load of the child during the sessions as well as external events likely to disturb the child.

Materials and methods

This prospective observational prospective study of the tolerance of a treatment technique was conducted in 34 children aged zero to six years. The paired-series Student test was used for the main objective with a significance level of 0.05.

Results

The children present a significant discomfort at the end of physiotherapy session. Stretching or loading is not a significant source of discomfort. The other identified causes could not be significantly demonstrated as uncomfortable.  相似文献   

20.

Introduction

Epilepsy with continuous spike-waves during slow sleep syndrome (CSWSS) is characterized by various seizure types, a characteristic EEG pattern and neuropsychological disorders. The main purpose of this study was to evaluate the long-term outcome of CSWSS occurred in childhood and to evaluate the variables that could influence the quality of social adaptation and the personality profile.

Material and methods

This is a prospective study on 24 young adults with previous CSWSS (median age 24.5?yrs) who were enrolled between January and July 2011 at the G. Gaslini Children’s Hospital, Genoa, Italy. Patients were divided into two groups: twelve with previous spike-wave index (SWI?>?85%) defined as typical CSWSS (T-CSWSS) and twelve with previous SWI?=?50–85% defined as atypical CSWSS (A-CSWSS). All the subjects were submitted to Minnesota Multiphasic Personality Inventory-2 (MMPI-2), Psychological General Well-Being Index (PGWBI), and to a structured interview.

Results

A correlation was observed with the severity of EEG abnormalities expressed by the SWI and outcome. The T-CSWSS group showed a significantly lower perceived well-being. Similarly in the T-CSWSS group the percentage of MMPI-2 clinical scales with T-scores ≥65 was higher than in the A-CSWSS group. Finally, a significant lower schooling in the T-CSWSS group was observed.

Conclusion

There seem to be two forms of the same disease, with similar onset and clinical evolution but a different outcome regarding the social and psychological conditions. The outcome of the social adaptation and of the personality consciousness was related with the severity of the EEG abnormalities: more favorable in patients with less intense SWI activity (A-CSWSS) compared those with a more severe EEG impairment (T-CSWSS).  相似文献   

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