New quantitative method for evaluation of motor functions applicable to spinal muscular atrophy.

Abstract:

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.

journal_name

Brain Dev

journal_title

Brain & development

authors

Matsumaru N,Hattori R,Ichinomiya T,Tsukamoto K,Kato Z

doi

10.1016/j.braindev.2017.12.003

subject

Has Abstract

pub_date

2018-03-01 00:00:00

pages

172-180

issue

3

eissn

0387-7604

issn

1872-7131

pii

S0387-7604(17)30309-1

journal_volume

40

pub_type

杂志文章
  • An extra-axial hemangioma mimicking a large prenatal brain tumor.

    abstract::A rare case of a congenital brain tumor was diagnosed by sonography in a fetus at 37weeks' gestation. The ultrasound examination showed a large area of both increased echogenicity and echolucency in one hemisphere suggestive of brain tumor or hemorrhage. Extensive surgical removal of the tumor was performed and reveal...

    journal_title:Brain & development

    pub_type: 杂志文章

    doi:10.1016/j.braindev.2010.02.006

    authors: Yang CY,Hsu JF,Lin KL,Jung SM,Lien R,Chang YL

    更新日期:2010-11-01 00:00:00

  • Widespread pH abnormalities in patients with malformations of cortical development and epilepsy: a phosphorus-31 brain MR spectroscopy study.

    abstract:INTRODUCTION:Neuroimaging studies demonstrate that not only the lesions of malformations of cortical development (MCD) but also the normal-appearing parenchyma (NAP) present metabolic impairments, as revealed with (1)H-MRS. We have previously detected biochemical disturbances in MCD lesions with phosphorus-31 magnetic ...

    journal_title:Brain & development

    pub_type: 杂志文章

    doi:10.1016/j.braindev.2013.12.010

    authors: Andrade CS,Otaduy MC,Valente KD,Park EJ,Kanas AF,Silva Filho MR,Tsunemi MH,Leite CC

    更新日期:2014-11-01 00:00:00

  • Rituximab was effective for acute disseminated encephalomyelitis followed by recurrent optic neuritis with anti-myelin oligodendrocyte glycoprotein antibodies.

    abstract:BACKGROUND:The effect of rituximab on acute disseminated encephalomyelitis (ADEM) followed by recurrent optic neuritis (ON) is not yet known. PATIENT:We are reporting the case of a 4-year-old Japanese girl who was diagnosed with anti-myelin oligodendrocyte glycoprotein (MOG) antibody positive ADEM followed by recurren...

    journal_title:Brain & development

    pub_type: 杂志文章

    doi:10.1016/j.braindev.2018.03.011

    authors: Nagashima M,Osaka H,Ikeda T,Matsumoto A,Miyauchi A,Kaneko K,Nakashima I,Nakano Y,Wakabayashi K,Monden Y,Yamagata T

    更新日期:2018-08-01 00:00:00

  • Prognosis of infants with ankle clonus within the first year of life.

    abstract::The present study was undertaken to clarify how we should assess the necessity of close follow-up in each case, when we first examine an infant with ankle clonus within the first year of life. The neurologic prognoses of 169 infants who had exhibited ankle clonus at least once during the first year of life were review...

    journal_title:Brain & development

    pub_type: 杂志文章

    doi:10.1016/s0387-7604(96)00069-1

    authors: Futagi Y,Otani K,Goto M

    更新日期:1997-01-01 00:00:00

  • Theophylline impairs memory/learning in developing mice.

    abstract::We studied the relationship between theophylline and memory/learning using an elevated plus-maze test and measuring spontaneous locomotor activity in developing mice. There were no significant differences in transfer latency (TL) in 21-, 30- and 42-day-old mice in the acquisition trial, but theophylline significantly ...

    journal_title:Brain & development

    pub_type: 杂志文章

    doi:10.1016/j.braindev.2003.12.008

    authors: Hirose M,Yokoyama H,Iinuma K

    更新日期:2004-10-01 00:00:00

  • Epileptic facial metamorphopsia.

    abstract::A 14-year-old patient with a right parietal arteriovenous malformation presented with seizures characterized by metamorphopsia of faces. Unlike adults with right hemisphere pathology she performed like an age matched control on a task requiring recognition of unfamiliar faces. This likely reflects maturational changes...

    journal_title:Brain & development

    pub_type: 杂志文章

    doi:10.1016/s0387-7604(85)80059-0

    authors: Nass R,Sinha S,Solomon G

    更新日期:1985-01-01 00:00:00

  • Neurophysiological studies in the Leigh syndrome.

    abstract::We performed neurophysiological studies in 12 patients with the Leigh syndrome (6 pathologically confirmed and 6 clinically diagnosed). The results are compared with data derived from a literature survey of 173 Leigh syndrome patients. We found no positive contribution of neurophysiological studies towards the diagnos...

    journal_title:Brain & development

    pub_type: 杂志文章

    doi:10.1016/s0387-7604(86)80004-3

    authors: Van Erven PM,Colon EJ,Gabreëls FJ,Renier WO,Vingerhoets DM

    更新日期:1986-01-01 00:00:00

  • Lower brainstem dysfunction in an infant with persistent primitive trigeminal artery.

    abstract::A 6-month-old boy with persistent primitive trigeminal artery (PPTA) presented with stridor, dysphagia, delayed motor development and postural neck and shoulder dystonia. Magnetic resonance imaging/angiography and ultrasonography revealed PPTA, with flow from the dilated basilar artery to the right internal carotid ar...

    journal_title:Brain & development

    pub_type: 杂志文章

    doi:10.1016/j.braindev.2006.08.008

    authors: Okanishi T,Saito Y,Miki S,Nagaishi J,Hanaki K,Tomita Y,Fukuda C,Fujii S,Fujiwara K,Kawamoto K,Hata F,Maegaki Y,Ohno K

    更新日期:2007-04-01 00:00:00

  • Complex regional pain syndrome in childhood: report of three cases.

    abstract::We describe three patients with the limb pain of complex regional pain syndrome (CRPS) in childhood with autonomic nervous system function involvement. Their autonomic nerve abnormality was non-invasively examined by means of laser doppler flowmetry (LDF) and a sympathetic skin response (SSR) test. In one it was resol...

    journal_title:Brain & development

    pub_type: 杂志文章

    doi:10.1016/s0387-7604(00)00174-1

    authors: Matsui M,Ito M,Tomoda A,Miike T

    更新日期:2000-10-01 00:00:00

  • Epidemiology of West syndrome in Singapore.

    abstract::The incidence of West syndrome (WS) was determined by a search of reports of electroencephalograms (EEG) recorded in 1998 and 1999 in all public hospitals in Singapore. Amongst records of patients born in 1998, nine were found with EEG features of hypsarrhythmia or modified hypsarrhythmia with onset of seizures betwee...

    journal_title:Brain & development

    pub_type: 杂志文章

    doi:10.1016/s0387-7604(01)00277-7

    authors: Lee WL,Ong HT

    更新日期:2001-11-01 00:00:00

  • Longitudinal developmental course of electrical activity of brain.

    abstract::Different phases of brain growth precede maturation as indicated in the developmental course of brain electrical activity. This can be illustrated by EEG and evoked potentials recorded from the scalp from a postmenstrual age of 24 weeks. A description of electrical patterns according to postmenstrual age is valid beca...

    journal_title:Brain & development

    pub_type: 杂志文章

    doi:10.1016/s0387-7604(80)80006-4

    authors: Eeg-Olofsson O

    更新日期:1980-01-01 00:00:00

  • Clinical profile of a male with Rett syndrome.

    abstract::We describe a clinical profile of a male with Rett syndrome who presented initially with significant axial and peripheral hypotonia, head and truncal titubation and global delay. He is non-ambulatory, lost the few words he had learned and gradually developed hand stereotypes, breathing difficulties, seizures, scoliosi...

    journal_title:Brain & development

    pub_type: 杂志文章

    doi:10.1016/j.braindev.2005.03.018

    authors: Budden SS,Dorsey HC,Steiner RD

    更新日期:2005-11-01 00:00:00

  • A case of infantile spasms: epileptic apnea as partial seizures at onset.

    abstract::We report a 2-month-old boy who presented with apneic attacks as a manifestation of epileptic seizures at onset and eventually progressed to infantile spasms. At onset, at 2 months of age, apneic attacks were the sole symptom of epileptic fits. Although these seizures were accompanied by cyanosis, bradycardia was not ...

    journal_title:Brain & development

    pub_type: 杂志文章

    doi:10.1016/0387-7604(95)00148-4

    authors: Kamei A,Ichinohe S,Ito M,Fujiwara T

    更新日期:1996-05-01 00:00:00

  • L-arginine is effective in stroke-like episodes of MELAS associated with the G13513A mutation.

    abstract::We report a case involving a 15-year-old boy with MELAS (G13513A mutation) who developed several stroke-like episodes in a short period of time. Intravenous administration of l-arginine during the acute phase of the stroke-like episodes reduced symptoms immediately, and oral supplementation of l-arginine successfully ...

    journal_title:Brain & development

    pub_type: 杂志文章

    doi:10.1016/j.braindev.2010.07.013

    authors: Shigemi R,Fukuda M,Suzuki Y,Morimoto T,Ishii E

    更新日期:2011-06-01 00:00:00

  • Neurodevelopment evaluation in children with congenital hypothyroidism by Bayley-III.

    abstract:BACKGROUND:Congenital hypothyroidism is the most common reason of mental retardation, and normal neurological development can be provided by early and effective treatment. In this present study, it is aimed to compare neurological developments of patients in 6-42 months of age with congenital hypothyroidism and healthy...

    journal_title:Brain & development

    pub_type: 杂志文章

    doi:10.1016/j.braindev.2012.07.003

    authors: Komur M,Ozen S,Okuyaz C,Makharoblıdze K,Erdogan S

    更新日期:2013-05-01 00:00:00

  • Founder mutation causes classical Fukuyama congenital muscular dystrophy (FCMD) in Chinese patients.

    abstract:PURPOSE:Fukuyama congenital muscular dystrophy (FCMD) is a congenital muscular dystrophy rarely reported outside Japan. Here, we report three patients with Fukuyama congenital muscular dystrophy (FCMD) in China who shared a similar clinical phenotype and 3-kb insertion in the FKTN 3' untranslated region. METHODS:Immun...

    journal_title:Brain & development

    pub_type: 杂志文章

    doi:10.1016/j.braindev.2015.02.010

    authors: Yang H,Kobayashi K,Wang S,Jiao H,Xiao J,Toda T,Wu X,Xiong H

    更新日期:2015-10-01 00:00:00

  • Thiamine-deficient encephalopathy due to excessive intake of isotonic drink or overstrict diet therapy in Japanese children.

    abstract:AIM:To report on two children with encephalopathy caused by dietary thiamine deficiency due to newly developing nutritional problems in contemporary Japan. SUBJECTS:A 1-year-old boy who had consumed 1l of isotonic drinks per day for 4 months after an episode of diarrhea, and presented with ocular movement disorder, dy...

    journal_title:Brain & development

    pub_type: 杂志文章

    doi:10.1016/j.braindev.2009.08.004

    authors: Saeki K,Saito Y,Komaki H,Sakakibra T,Nakagawa E,Sugai K,Sakuma H,Sasaki M,Honda T,Hayashi H,Katori N,Miyahara Y

    更新日期:2010-08-01 00:00:00

  • Genetically engineered human neural stem cells for brain repair in neurological diseases.

    abstract::Neural stem cells (NSCs)of the central nervous system (CNS) have recently received a great deal of attention and interest for their therapeutic potential for neurological disorders. NSCs are defined as CNS progenitor cells that have the capacity for self-renewal and multipotent potential to become neurons or glial cel...

    journal_title:Brain & development

    pub_type: 杂志文章,评审

    doi:10.1016/j.braindev.2006.07.012

    authors: Kim SU

    更新日期:2007-05-01 00:00:00

  • Recent advances in the biochemistry and genetics of sphingolipidoses.

    abstract::Sphingolipidoses are a subgroup of lysosomal storage diseases. They are defined as disorders caused by a genetic defect in catabolism of sphingosine-containing lipids. Catabolism of these lipids involves enzymes and activator proteins. After the discovery of lysosomes by de Duve and the demonstration of the first defe...

    journal_title:Brain & development

    pub_type: 杂志文章,评审

    doi:10.1016/j.braindev.2004.01.005

    authors: Ozkara HA

    更新日期:2004-12-01 00:00:00

  • Epidemiology of acute encephalopathy in Japan, with emphasis on the association of viruses and syndromes.

    abstract::A research committee supported by the Japanese government conducted a nationwide survey on the epidemiology of acute encephalopathy in Japan using a questionnaire. A total of 983 cases reportedly had acute encephalopathy during the past 3 years, 2007-2010. Among the pathogens of the preceding infection, influenza viru...

    journal_title:Brain & development

    pub_type: 杂志文章

    doi:10.1016/j.braindev.2011.07.012

    authors: Hoshino A,Saitoh M,Oka A,Okumura A,Kubota M,Saito Y,Takanashi J,Hirose S,Yamagata T,Yamanouchi H,Mizuguchi M

    更新日期:2012-05-01 00:00:00

  • Peripheral nerve involvement in Werdnig-Hoffmann disease.

    abstract::The number of large myelinated axons was markedly decreased in almost all the intramuscular nerve bundles included in 32 muscle biopsies from patients with Werdnig-Hoffmann disease compared to that in normals. The morphometric analysis of peripheral nerves in 5 epon-embedded sections also showed a selective loss of la...

    journal_title:Brain & development

    pub_type: 杂志文章

    doi:10.1016/s0387-7604(89)80040-3

    authors: Chien YY,Nonaka I

    更新日期:1989-01-01 00:00:00

  • Idiopathic hypereosinophilic syndrome complicated by central sinovenous thrombosis.

    abstract::Idiopathic hypereosinophilic syndrome (IHES) is a rare disorder affecting cardiac, pulmonary and nervous systems with peripheral neuropathy, encephalopathy and cerebral thromboembolism. We report a 7-year-old boy with IHES who developed central sinovenous thrombosis and cerebral hemorrhage. Although he had hypereosino...

    journal_title:Brain & development

    pub_type: 杂志文章

    doi:10.1016/j.braindev.2006.08.004

    authors: Sakuta R,Tomita Y,Ohashi M,Nagai T,Murakami N

    更新日期:2007-04-01 00:00:00

  • Clinicopathological differences between juvenile and late infantile metachromatic leukodystrophy.

    abstract::The autopsy report of the juvenile type of metachromatic leukodystrophy is rare. The clinical and pathological difference between the juvenile and the late infantile type of metachromatic leukodystrophy was described. Loss of myelin sheaths was much less in the brain stem and spinal cord in the juvenile type than in t...

    journal_title:Brain & development

    pub_type: 杂志文章

    doi:10.1016/s0387-7604(81)80065-4

    authors: Takashima S,Matsui A,Fujii Y,Nakamura H

    更新日期:1981-01-01 00:00:00

  • Evaluation of ADL in patients with Hunter disease using FIM score.

    abstract::MPS type II (Hunter disease) results from deficiency of the lysosomal enzyme iduronate-2-sulfate sulfatase. Two forms of the disease have been recognized, based on absence or presence of progressive intellectual deterioration. This study aimed to assess activities of daily life (ADL) in 27 Hunter disease-affected Japa...

    journal_title:Brain & development

    pub_type: 杂志文章

    doi:10.1016/j.braindev.2006.08.015

    authors: Kato T,Kato Z,Kuratsubo I,Ota T,Orii T,Kondo N,Suzuki Y

    更新日期:2007-06-01 00:00:00

  • A clinical study of attention-deficit/hyperactivity disorder in preschool children--prevalence and differential diagnoses.

    abstract:OBJECTIVE:We aimed to examine (1) the prevalence and characteristics of ADHD in preschool children, and (2) differential diagnoses among children who display symptoms of inattention and hyperactivity-impulsivity in early childhood. METHODS:The participants were children living in Kanie-cho, in Japan's Aichi Prefecture...

    journal_title:Brain & development

    pub_type: 杂志文章

    doi:10.1016/j.braindev.2013.11.004

    authors: Nomura K,Okada K,Noujima Y,Kojima S,Mori Y,Amano M,Ogura M,Hatagaki C,Shibata Y,Fukumoto R

    更新日期:2014-10-01 00:00:00

  • Magnetic resonance studies of brain lesions in patients with Kawasaki disease.

    abstract::We evaluated brain lesions in patients with coronary arterial lesions (CAL) as a complication of Kawasaki disease (KD) by magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA). Among 47 patients who underwent coronary angiography for the evaluation of CAL due to KD at Kyushu University Hospital fro...

    journal_title:Brain & development

    pub_type: 杂志文章

    doi:10.1016/j.braindev.2005.04.003

    authors: Muneuchi J,Kusuhara K,Kanaya Y,Ohno T,Furuno K,Kira R,Mihara F,Hara T

    更新日期:2006-01-01 00:00:00

  • Abnormalities of joint mobility and gait in children with autism spectrum disorders.

    abstract:AIMS:Abnormalities of gross motor function in children with autism are well known to clinicians but have not received much empirical documentation and, with the exception of stereotypies, are not among its diagnostic criteria. We recorded the characteristics of gait and prevalence of toe walking, the range of passive j...

    journal_title:Brain & development

    pub_type: 杂志文章

    doi:10.1016/j.braindev.2012.02.005

    authors: Shetreat-Klein M,Shinnar S,Rapin I

    更新日期:2014-02-01 00:00:00

  • X-linked adrenoleukodystrophy: clinical course and minimal incidence in South Brazil.

    abstract:UNLABELLED:X-linked adenoleukodystrophy is a genetic disease that affects the degradation of very long-chain fatty acids. In male patients, common pictures are the cerebral form (CALD), myeloneuropathy (AMN), and Addison-only. OBJECTIVE:To describe the clinical course of affected male patients from South Brazil betwee...

    journal_title:Brain & development

    pub_type: 杂志文章

    doi:10.1016/j.braindev.2009.02.002

    authors: Jardim LB,da Silva AC,Blank D,Villanueva MM,Renck L,Costa ML,Vargas CR,Deon M,Coelho Dl,Vedolin L,de Castro CG Jr,Gregianin L,Bonfim C,Giugliani R

    更新日期:2010-03-01 00:00:00

  • Pathophysiology of Rett syndrome from the stand point of clinical characteristics.

    abstract::In this report, we reviewed the characteristics of motor development and motor symptoms of Rett Syndrome (RTT) and demarcated the early and pathognomonic motor symptom which correlates to the impairment of the higher cortical function (HCF) assessed by the ability of language. It is suggested that failure of locomotio...

    journal_title:Brain & development

    pub_type: 杂志文章,评审

    doi:10.1016/s0387-7604(01)00352-7

    authors: Segawa M

    更新日期:2001-12-01 00:00:00

  • Progressive diffuse brain atrophy in West syndrome with marked hypomyelination due to SPTAN1 gene mutation.

    abstract::A 1-year-old male began suffering from West syndrome at 3 months of age, when electroencephalography revealed hypsarrhythmia accompanied by a periodic, brief suppression phase. The administration of adrenocorticotropic hormone was partially effective for stopping the condition, and the seizure type evolved into brief ...

    journal_title:Brain & development

    pub_type: 杂志文章

    doi:10.1016/j.braindev.2012.05.002

    authors: Nonoda Y,Saito Y,Nagai S,Sasaki M,Iwasaki T,Matsumoto N,Ishii M,Saitsu H

    更新日期:2013-03-01 00:00:00