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1.
Saori Miyagishima Tadayoshi Asaka Kaori Kamatsuka Naoki Kozuka Masaki Kobayashi Lisa Igarashi Tsukasa Hori Hiroyuki Tsutsumi 《Brain & development》2018,40(8):627-633
Aims
We conducted a longitudinal cohort study to analyze the relationship between outcome of gross motor development in preterm infants and factors that might affect their development.Methods
Preterm infants with a birth weight of <1500?g were recruited. We measured spontaneous antigravity limbs movements by 3D motion capture system at 3?months corrected age. Gross motor developmental outcomes at 6 and 12?months corrected age were evaluated using the Alberta Infant Motor Scale (AIMS). Statistical analysis was carried out by canonical correlation analysis.Results
Eighteen preterm infants were included. In the 6?months corrected age analysis, spontaneous movement had a major effect on Prone and Sitting at 6?months corrected age of AIMS. In the 12?months corrected age analysis, spontaneous movement had a major effect on Sitting and Standing at 12?months corrected age of AIMS.Conclusions
In preterm infants, better antigravity spontaneous movements at 3?months corrected age were significantly correlated with better gross motor development at 6 or 12?months corrected age. 相似文献2.
Mayumi Matsuda Kazuhide Tomita Arito Yozu Tomohiro Nakayama Jyunko Nakayama Haruka Ohguro Nobuaki Iwasaki 《Brain & development》2018,40(6):452-457
Purpose
This study investigated the sequential physical changes after botulinum toxin type A (BTX-A) injected in children with cerebral palsy.Methods
Nine children with cerebral palsy were included. Measurements were performed before treatment and 4?weeks, 8?weeks, and 12?weeks after treatment. We used video-recorded gait in the sagittal plane. The maximum flexion and extension angles of the hip, knee and ankle joints, step length, gait speed, and observational gait were measured using the Foot Contact Scale (FCS) and the Physician’s Rating Scale (PRS). We also measured the lower limb range of motion (ROM), Modified Tardieu Scale (MTS), knee joint extension torque, and Gross Motor Function Measure-66 (GMFM-66).Results
The ankle dorsiflexion ROM, GMFM-66, and the maximum dorsiflexion angle of the ankle during gait were significantly increased at 8?weeks after treatment, and knee joint extension torque was significantly increased at 12?weeks after treatment.Conclusion
Maximum effects of BTX-A treatment do not occur during the early stage after treatment. Therefore, long-term intervention with rehabilitation between BTX-A treatment may be more effective than implementing rehabilitation for only a brief period. 相似文献3.
Tatsuji Hasegawa Kei Yamada Takenori Tozawa Tomohiro Chiyonobu Sachiko Tokuda Akira Nishimura Hajime Hosoi Masafumi Morimoto 《Brain & development》2018,40(9):743-752
Purpose
Cerebellar injury is well established as an important finding in preterm infants with cerebral palsy (CP). In this study, we investigated associations between injury to the cerebellar peduncles and motor impairments in preterm infants using quantitative tractography at term-equivalent age, which represents an early phase before the onset of motor impairments.Methods
We studied 64 preterm infants who were born at <33?weeks gestational age. These infants were divided into three groups: CP, Non-CP (defined as infants with periventricular leukomalacia but having normal motor function), and a Normal group. Diffusion tensor imaging was performed at term-equivalent age and motor function was assessed no earlier than a corrected age of 2?years. Using tractography, we measured fractional anisotropy (FA) and apparent diffusion coefficient (ADC) of the superior cerebellar peduncles (SCP) and middle cerebellar peduncles (MCP), as well as the motor/sensory tracts.Results
The infants in the CP group had significantly lower FA of the SCP and sensory tract than those in the other groups. There was no significant difference in FA and ADC of the motor tract among the three groups. Severity of CP had a significant correlation with FA of the MCP, but not with the FA of other white matter tracts.Conclusion
Our results suggested that the infants with CP had injuries of the ascending tracts (e.g. the SCP and sensory tract), and that additional MCP injury might increase the severity of CP. Quantitative tractography assessment at term-equivalent age may be useful for screening preterm infants for prediction of future motor impairments. 相似文献4.
Matteo Bologna Kelly Bertram Giulia Paparella Claudia Papi Daniele Belvisi Antonella Conte Antonio Suppa David R. Williams Alfredo Berardelli 《Clinical neurophysiology》2017,128(9):1547-1552
Objective
Abnormal primary motor cortex plasticity might be involved in the pathophysiology of progressive supranuclear palsy. In the present study we aimed to investigate possible abnormalities of depotentiation, a mechanism involved in plasticity regulation, in this condition.Methods
Primary motor cortex excitability, investigated with single and paired-pulse transcranial magnetic stimulation, as well as long-term potentiation-like plasticity and its reversibility, were studied using theta burst stimulation in 15 patients with progressive supranuclear palsy and 11 healthy controls. Participants underwent two sessions using (1) the intermittent theta-burst stimulation (potentiation protocol) and (2) intermittent theta-burst stimulation combined with a depotentiation protocol (a short continuous theta-burst stimulation).Results
Patients with PSP had higher corticospinal excitability and lower intracortical inhibition than healthy controls. Intermittent theta-burst stimulation elicited an abnormally increased long term potentiation-like effect in patients in comparison to healthy subjects. However, the depotentiation protocol was able to reverse the effects intermittent theta-burst stimulation on motor cortex excitability both in patients and in healthy controls.Conclusions
Altered primary motor cortex plasticity in patients with PSP does not reflect an abnormality of depotentiation.Significance
This study provides information for a deeper understanding of the possible pathophysiological mechanisms underlying the altered M1 plasticity in PSP. 相似文献5.
Mayumi Matsuda Nobuaki Iwasaki Yuki Mataki Hirotaka Mutsuzaki Kenichi Yoshikawa Kazushi Takahashi Keiko Enomoto Kumiko Sano Aoi Kubota Tomohiro Nakayama Junko Nakayama Haruka Ohguro Masafumi Mizukami Kazuhide Tomita 《Brain & development》2018,40(8):642-648
Purpose
The Hybrid Assistive Limb® (HAL®, CYBERDYNE) is a wearable robot that provides assistance to a patient while they are walking, standing, and performing leg movements based on the wearer’s intended movement. The effect of robot-assisted training using HAL® for cerebral palsy (CP) is unknown. Therefore, we assessed the effect of robot-assisted training using HAL® on patients with CP, and compared walking and gross motor abilities between pre-intervention and post-intervention.Methods
Six subjects with CP were included (mean age: 16.8?years; range: 13–24?years; Gross Motor Function Classification System levels II–IV: n?=?1, 4, 1). Robot-assisted training using HAL® were performed 2–4 sessions per week, 20?min per session, within a 4?weeks period, 12 times in total. Outcome measures included gait speed, step length, cadence, single-leg support per gait cycle, hip and knee joint angle in stance, and swing phase per gait cycle, 6-minute walking distance (6?MD), physiological cost index (PCI), knee-extension strength, and Gross Motor Function Measure (GMFM).Results
There were significant increases in self-selected walking speed (SWS), cadence during SWS and maximum walking speed (MWS), single-leg support per gait cycle, hip joint angle in the swing phase, 6?MD, and GMFM. In contrast, gait speed during MWS, step length during SWS and MWS, hip and knee joint angle in the stance phase, knee joint angle in the swing phase, PCI, and knee-extension strength generally improved, but not significantly.Conclusion
Robot-assisted training using HAL® may improve walking and gross motor abilities of patients with CP. 相似文献6.
Yuri Matsubara Hitoshi Osaka Takanori Yamagata Ryusuke Ae Jun Shimizu Noriko Oguro 《Brain & development》2018,40(9):807-812
Background
Acute encephalopathy causes various sequelae, including motor disabilities and intellectual delays. Previous studies reported that cognitive impairments can also occur after acute encephalitis. Although the incidence of acute encephalopathy is high in Japan, there have been few reports on its sequelae.Objective
To characterize the neurological outcomes of pediatric patients who sought motor rehabilitation for motor dysfunction after acute encephalopathy.Method
Subjects were 26 children who were healthy before suffering from motor dysfunction following acute encephalopathy and were referred to our pediatric rehabilitation institute during a 9-year period (August 2007–April 2017). We examined subjects’ neurological status and followed sequelae for at least 8?months.Results
Of 26 individuals, 21 became ambulatory after several months or years during the observation period. Patients who could sit without support within 5?months after the onset of acute encephalopathy were able to walk within several months or years. Patients showing high intensity on T2-weighted sequences or “bright tree appearance” in the frontal region took an average of 7?months to develop walking, which was longer than other patients. Among ambulatory subjects, 16(76%) exhibited mild to moderate intellectual delay with a developmental quotient (DQ) under 70, and 20 (95%) exhibited cognitive impairment. There was a significant correlation between DQ scores and motor disability (p?=?0.013, r?=??0.481).Conclusions
Although 80% of patients who had motor dysfunction caused by acute encephalopathy and visited out motor rehabilitation outpatient clinic were eventually able to walk, the time taken to develop walking ability depended on which region exhibited magnetic resonance imaging abnormalities. DQ scores and motor disability were significantly correlated. 相似文献7.
J.F. Vázquez-Costa M. Campins-Romeu J.J. Martínez-Payá J.I. Tembl M.E. del Baño-Aledo J. Ríos-Díaz V. Fornés-Ferrer M.J. Chumillas T Sevilla 《Clinical neurophysiology》2018,129(12):2650-2657
Objective
To describe the fasciculation pattern in ALS and to analyse its clinical and pathophysiological significance.Methods
Ultrasound of 19 muscles was performed in 44 patients with a recent diagnosis (<90?days) of ALS. The number of fasciculations was recorded in each muscle and the muscle thickness and strength were additionally measured in limb muscles. A subgroup of patients were electromyographically assessed.Results
US was performed in 835 muscles and EMG was available in 263 muscles. US detected fasciculations more frequently than EMG. Fasciculations were widespread, especially in upper limbs onset patients and in the cervical region. Fasciculations’ number inversely associated with ALSFR-R and body mass index (BMI) and directly with BMI loss and upper motor neuron (UMN) impairment. Our statistical model suggest that fasciculations increase with the initial lower motor neuron (LMN) degeneration, reach their peak when the muscle became mildly to moderately weak, decreasing afterwards with increasing muscle weakness and atrophy.Conclusions
Our study suggests that both UMN and LMN degeneration trigger fasciculations causing BMI loss. The degree of LMN impairment could account for differences in fasciculations’ rates within and between muscles.Significance
In ALS, fasciculations could explain the link between hyperexcitability and BMI loss. 相似文献8.
Daisuke Sawada Katsunori Fujii Sonoko Misawa Tadashi Shiohama Tomoyuki Fukuhara Mayuko Fujita Satoshi Kuwabara Naoki Shimojo 《Brain & development》2018,40(9):830-832
Background
Guillain-Barré syndrome is an acute immune-mediated peripheral polyneuropathy. Neuroimaging findings from patients with this syndrome have revealed gadolinium enhancement in the cauda equina and in the anterior and posterior nerve roots, but intra-spinal lesions have never been described.Aim
Herein, we report, for the first time, bilateral spinal anterior horn lesions in a patient with an acute motor axonal neuropathy form of Guillain-Barré syndrome.Case
The patient was a previously healthy 13-year-old Japanese girl, who exhibited acute-onset flaccid tetraplegia and loss of tendon reflexes.Results
Nerve conduction studies revealed motor axonal damage, leading to the diagnosis of acute motor axonal neuropathy. Notably, spinal magnetic resonance imaging revealed bilateral anterior horn lesions on T2-weighted imaging at the Th11–12 levels, as well as gadolinium enhancement of the cauda equina and anterior and posterior nerve roots. The anterior horn lesions were most prominent on day 18, and their signal intensity declined thereafter. Although intravenous treatment with immunoglobulins was immediately administered, the motor function was not completely regained.Conclusion
We propose that anterior spinal lesions might be responsible for the prolonged neurological disability of patients with Guillain-Barré syndrome, possibly produced by retrograde progression from the affected anterior nerve roots to the intramedullary roots, and the anterior horn motor neurons. 相似文献9.
Sawa Yasumoto Yoko Ohtsuka Katsuaki Sato Atsuyo Kurata Yotaro Numachi Masahiro Shimizu 《Brain & development》2018,40(9):786-791
Purpose
To investigate the efficacy and safety of long-term lamotrigine (LTG) monotherapy in Japanese and South Korean pediatric patients with newly diagnosed typical absence seizures.Methods
Six Japanese patients and one South Korean patient were enrolled in the extension phase of the study after completing the 12-week maintenance phase of an open-label clinical study of LTG monotherapy. During the extension phase, patients underwent efficacy and safety evaluation every 12?weeks.Results
Of the seven patients, six patients completed the extension phase. The seizure-free rate confirmed by hyperventilation (HV)-electroencephalography ranged from 71.4% to 100.0% at each visit up to Week 168 of the extension phase. Similar effects were confirmed by HV-clinical signs and seizure diaries. Although no unexpected adverse events were observed, one Japanese patient was withdrawn from the extension phase due to mild drug-related rash developed 842?days after the start of LTG.Conclusion
Although the number of patients is limited, long-term LTG monotherapy appeared to be effective and generally well tolerated in Japanese and South Korean pediatric patients with typical absence seizures. 相似文献10.
Lin-Mei Chiang Go-Shine Huang Chi-Chin Sun Ying-Li Hsiao Chung Kun Hui Mei-Hua Hu 《Brain & development》2018,40(9):775-780
Purpose
Epilepsy is an important neurological condition that frequently associated with neurobehavioral disorders in childhood. Our aim was to identify the risk of developing epilepsy subsequent to febrile seizure and the association between epilepsy risk factors and neurobehavioral disorders.Subjects and methods
This longitudinal population-based cohort data included 952 patients with a febrile seizure diagnosis and 3808 age- and sex-matched controls. Participants were recruited for the study from 1996 to 2011, and all patients were followed up for maximum 12.34?years.Results
The association of epilepsy was significantly higher (18.76-fold) in individuals that experienced febrile seizure compared to controls. Further, of those individuals who experienced febrile seizure, the frequency of subsequent development of epilepsy was 2.15-fold greater in females, 4.846-fold greater in patients with recurrent febrile seizure, and 11.26-fold greater patients with comorbid autism.Conclusions
Our study showed that being female, comorbid autism with febrile seizure and recurrent febrile seizure had an increased association with development of epilepsy. Increased recognition the association for epilepsy might be warranted in those febrile seizure children with certain characteristics. 相似文献11.
Naoki Matsumaru Ryo Hattori Takashi Ichinomiya Katsura Tsukamoto Zenichiro Kato 《Brain & development》2018,40(3):172-180
Objective
The aim of this study was to develop and introduce new method to quantify motor functions of the upper extremity.Methods
The movement was recorded using a three-dimensional motion capture system, and the movement trajectory was analyzed using newly developed two indices, which measure precise repeatability and directional smoothness. Our target task was shoulder flexion repeated ten times. We applied our method to a healthy adult without and with a weight, simulating muscle impairment. We also applied our method to assess the efficacy of a drug therapy for amelioration of motor functions in a non-ambulatory patient with spinal muscular atrophy. Movement trajectories before and after thyrotropin-releasing hormone therapy were analyzed.Results
In the healthy adult, we found the values of both indices increased significantly when holding a weight so that the weight-induced deterioration in motor function was successfully detected. From the efficacy assessment of drug therapy in the patient, the directional smoothness index successfully detected improvements in motor function, which were also clinically observed by the patient’s doctors.Conclusion
We have developed a new quantitative evaluation method of motor functions of the upper extremity. Clinical usability of this method is also greatly enhanced by reducing the required number of body-attached markers to only one. This simple but universal approach to quantify motor functions will provide additional insights into the clinical phenotypes of various neuromuscular diseases and developmental disorders. 相似文献12.
Boudewijn T.H.M. Sleutjes Judith Drenthen Ernest Boskovic Leonard J. van Schelven Maria O. Kovalchuk Paul G.E. Lumens Leonard H. van den Berg Hessel Franssen 《Clinical neurophysiology》2018,129(8):1634-1641
Objective
To study excitability of single motor units (MUs) using high-density surface-EMG.Methods
Motor unit action potentials (MUAPs) were evoked by submaximal stimulation of the median nerve at the wrist and recorded with a 9?×?14 electrode grid on the skin overlying the thenar muscles. For excitability tests of single MUs, the most optimal specific single-channel surface-EMG signal was selected based on the spatiotemporal profile of single MUs.Results
Axonal excitability measures were successfully obtained from 14 single MUs derived from ten healthy subjects. Selecting the optimal single-channel surface-EMG signals minimized interference from other single MUs and improved signal-to-noise ratio. The muscle fiber conduction velocity (MFCV) could also be derived from the unique spatiotemporal profile of single MUs.Conclusion
High-density surface-EMG helps to isolate single MUAP responses, making it a suitable technique for assessing excitability in multiple single motor axons per nerve.Significance
Our method enables the reliable study of ion-channel dysfunction in single motor axons of nerves without any requirement for specific conditions, such as prominent MU loss or enlarged MUAPs due to collateral sprouting. 相似文献13.
Hongfei Tai Liying Cui Mingsheng Liu Yuzhou Guan Xiaoguang Li Dongchao Shen Kang Zhang Shuangwu Liu Shuang Wu Qingyun Ding Youfang Hu 《Clinical neurophysiology》2018,129(5):926-930
Objective
To explore the relationship between serum creatine kinase (CK) level and electromyographic characteristics in patients with amyotrophic lateral sclerosis (ALS).Methods
Two hundred thirty-eight consecutive ALS patients were enrolled. All patients underwent electrophysiological study with a consistent approach. We calculated a compound muscle action potential (CMAP) sum score, and spontaneous potentials were graded from 0 to 4 depending on their density and distribution. We tested for any independent correlation of the CK levels with CMAP sum score, mean spontaneous potential (MSP) score, F wave persistence or conduction velocity.Results
The median serum CK level was 151 U/L. Log CK was independently correlated with MSP score (β?=?0.07, 95% CI: 0.01–0.14, p?=?0.032) and F persistence (β?=??0.0013, 95% CI: ?0.00251 to ?0.0002, p?=?0.02) but not with CMAP sum score or F wave conduction velocity. When stratified by sex, the correlation of log CK with MSP score and F persistence was significant in male but not female patients.Conclusions
The results support that lower motor neuron loss and muscle denervation are associated with elevated CK levels of ALS patients.Significance
The severity of lower motor neuron loss and denervation might be involved in pathophysiological mechanisms of CK elevation in ALS patients. 相似文献14.
Hiroo Tani Nobutsune Ishikawa Yoshiyuki Kobayashi Shohei Yamaoka Yuji Fujii Kimihiko Kaneko Toshiyuki Takahashi Masao Kobayashi 《Brain & development》2018,40(10):943-946
Background
Rett syndrome (RTT) is a neurodevelopmental disorder primarily caused by mutations in the methyl-CpG-binding protein 2 (MECP2) gene, resulting in developmental regression after normal development during infancy. Transient presentation of many autistic features is also commonly seen in RTT. Anti-myelin oligodendrocyte glycoprotein (MOG)-antibody encephalitis is an acquired relapsing demyelinating syndrome characterized by a variety of neuroinflammatory symptoms. Here, we report a case of anti-MOG antibody encephalitis in a patient with genetically confirmed RTT, which mimicked many of the features of RTT.Case report
A three-year-old girl presented with subacute verbal and motor dysfunction, along with involuntary movements and marked irritability. Magnetic resonance imaging (MRI) revealed extensive white matter lesions, with anti-MOG antibodies detected in the serum and cerebrospinal fluid, resulting in an initial diagnosis of anti-MOG antibody encephalitis. However, additional testing of the MECP2 gene was performed in response to persistent involuntary hand movements in combination with progressive verbal and motor deterioration. Sequencing analysis revealed a known pathogenic mutation in MEPC2, indicating a concurrent diagnosis of RTT.Conclusion
Both RTT and anti-MOG antibody encephalitis are rare conditions. Similarities in disease presentation suggest that anti-MOG antibody encephalitis may mimic many of the symptoms of RTT. 相似文献15.
Objectives
Brain-computer interfaces (BCIs) allow users to operate a device or application by means of cognitive activity. This technology will ultimately be used in real-world environments which include the presence of distractors. The purpose of the study was to determine the effect of visual distractors on BCI performance.Methods
Sixteen able-bodied participants underwent neurofeedback training to achieve motor imagery-guided BCI control in an online paradigm using electroencephalography (EEG) to measure neural signals. Participants then completed two sessions of the motor imagery EEG-BCI protocol in the presence of infrequent, small visual distractors. BCI performance was determined based on classification accuracy.Results
The presence of distractors was found to affect motor imagery-specific patterns in mu and beta power. However, the distractors did not significantly affect the BCI classification accuracy; across participants, the mean classification accuracy was 81.5?±?14% for non-distractor trials, and 78.3?±?17% for distractor trials.Conclusion
This minimal consequence suggests that the BCI was robust to distractor effects, despite motor imagery-related brain activity being attenuated amid distractors.Significance
A BCI system that mitigates distraction-related effects may improve the ease of its use and ultimately facilitate the effective translation of the technology from the lab to the home. 相似文献16.
Katsuhiro Kobayashi Yuji Ohuchi Takashi Shibata Yoshiyuki Hanaoka Mari Akiyama Makio Oka Fumika Endoh Tomoyuki Akiyama 《Brain & development》2018,40(5):397-405
Objective
We explored fast (40–150?Hz) oscillations (FOs) from the ictal scalp electroencephalogram (EEG) data of myoclonic seizures in pediatric patients to obtain insight into the pathophysiological mechanisms involved in the generation of myoclonic seizures.Subjects and methods
The participants were 21 children (11 boys, 10 girls; age ranging from 5?months to 17?years 2?months) with myoclonic seizures associated with generalized (poly)spike-wave bursts in the ictal EEG data. The patients had heterogeneous etiologies and epilepsy diagnoses. In the ictal data, we detected FOs that clearly showed oscillatory morphology in filtered EEG traces and an outstanding spectral blob in time-frequency analysis.Results
We identified FOs in 61 (88.4%) of all 69 myoclonic seizures. Every patient had at least one myoclonic seizure-associated FO. The observed FOs were embedded in the spike component of (poly)spike-wave discharges, and they had a focal distribution with frontal predominance. They ranged in frequency from 41.0 to 123.0?Hz and involved both the gamma and ripple bands, and their spectral peak frequencies were higher in the group of patients with a genetic background free of apparent fundamental brain pathology than in the group of other patients (p?=?0.019).Conclusion
FOs were found to represent at least part of the cortical pathophysiological process in the generation of myoclonic seizures that should involve the thalamocortical network system. 相似文献17.
Çiğdem Genç Sel Mustafa Kılıç Deniz Yüksel Ayşe Aksoy Çiğdem Seher Kasapkara Serdar Ceylaner Kader Karlı Oğuz 《Brain & development》2018,40(10):865-875
Background
Nonketotic hyperglycinemia (NKH) is an autosomal recessive severe life-threatening catostrophic metabolic disorder.Materials and methods
The present study was conducted in a tertiary reference center in Turkey for six years period. The accurate diagnosis of six NKH patients was based on clinical history of the patients, neurological examinations, seizure semiology, serial electroencephalography (EEG) recordings, neuroimaging findings, metabolic tests and genetic analysis.Results
The common clinical findings were hypotonia with severe head lag, poor feeding, poor sucking, and intractable seizures. The starting age of the symptoms was between birth and 45?days of age (median: 8?days). The starting age of the seizures was between 30?min of age and 45?days of age (median: 18?days). The age of accurate diagnosis was between 1?month of age and 5.5?months of age (mean: 3.75?±?1.69?months). The cerebrospinal fluid (CSF) to plasma GLY ratio of the patients was between 0.031 and 0.21 (median: 0.16). The EEG patterns of the patients were suppression-burst, hypsarrhythmia, multifocal epileptic activity, and right centro-occipital epileptic activity on admission. The neuroimaging findings were diffuse hypomyelination, corpus callosum (CC) hypoplasia, CC agenesis and brainstem hypoplasia on the magnetic resonance imaging and glycine peak was evidenced on magnetic resonance spectroscopy. Four of the patients were mutation-positive.Conclusions
If a child is encephalopathic and/or hypotonic with severe head lag, early evaluation of the EEG records should be made even without a history of clinical seizures. The disease has a heterogenous course and the clinical outcome depends on the mutation type. 相似文献18.
A.B. Jacobsen R.S. Kristensen A. Witt A.G. Kristensen L. Duez S. Beniczky A. Fuglsang-Frederiksen H. Tankisi 《Clinical neurophysiology》2018,129(3):646-653
Objective
To compare the diagnostic utility of motor unit number estimation (MUNE) methods to motor unit potential (MUP) analysis in amyotrophic lateral sclerosis (ALS).Methods
Twenty-five patients (1 definite, 11 probable, 9 possible ALS and 4 progressive muscular atrophy) and 22 healthy controls were prospectively included. Quantitative MUP analysis and three MUNE methods; Multiple Point Stimulation MUNE (MPS), Motor Unit Number Index (MUNIX) and MScanFit MUNE (MScan) were done in abductor pollicis brevis muscle. The sensitivities were compared by McNemar chi-square test. MUNE, MUP and revised ALS Functional Rating Scale (ALSFRS-R) parameters were correlated by regression analysis.Results
The sensitivities of MPS (76%) and MScan (68%) were higher than MUP duration (36%) and amplitude (40%) in detecting motor unit loss (p?<?0.05). MUNE methods increased the categorical probability from possible to probable ALS in 4 patients (16%). There was only significant correlation between ALSFRS-R and MScan (r?=?0.443, p?=?0.027) among the electrophysiological tests. MUNE methods did not correlate to MUP parameters.Conclusions
MUNE methods are more sensitive in showing abnormality than MUP analysis.Significance
MUNE methods, in particular MScan, may have the potential to be implemented in the clinical practice for diagnosis and follow-up of neuromuscular disorders particularly ALS. 相似文献19.
Yuko Hirata Shin-ichiro Hamano Satoru Ikemoto Atsuko Oba Ryuki Matsuura 《Brain & development》2018,40(10):841-849
Objective
To quantitatively evaluate regional cerebral blood flow (rCBF) and regional developmental changes during childhood using 123I-N-isopropyl-iodoamphetamine single-photon emission computed tomography (SPECT) and autoradiography.Methods
We retrospectively analyzed quantitative values of rCBF in 75 children (29 girls) aged between 16?days and 178?months (median: 12?months), whose brain images, including magnetic resonance imaging and SPECT data, were normal under visual inspection at Saitama Children’s Medical Center between 2005 and 2015. The subjects had normal psychomotor development, no focal neurological abnormalities, and neither respiratory nor cardiac disease at the time of examination. Regions of interest were placed automatically using a three-dimensional stereotactic template.Results
rCBF was lowest in neonates, who had greater rCBF in the lenticular nucleus, thalamus, and cerebellum than the cerebral cortices. rCBF increased rapidly during the first year of life, reaching approximately twice the adult levels at 8?years, and then fell to approximately adult levels in the late teenage years. Cerebral cortex rCBF sequentially increased in the posterior, central, parietal, temporal, and callosomarginal regions during infancy and childhood.Conclusions
rCBF changed dramatically throughout childhood and ranged from lower than adult values to approximately two times higher than adult values. It had different trajectories in each region during brain development. Understanding this dynamic developmental change is necessary for SPECT image evaluation in children. 相似文献20.
Tushar Issar Ria Arnold Natalie C.G. Kwai Bruce A. Pussell Zoltan H. Endre Ann M. Poynten Matthew C. Kiernan Arun V. Krishnan 《Clinical neurophysiology》2018,129(5):889-894