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Objective : Cervical surgery in patients with cervical spondylotic myelopathy (CSM) and cerebral palsy (CP) is challenging owing to the complexities of the deformity. We assessed factors affecting postoperative complications and outcomes after CSM surgery in patients with CP.Methods : Thirty-five consecutive patients with CP and CSM who underwent cervical operations between January 2006 and January 2014 were matched to 35 non-cerebral palsy (NCP) control patients. Postoperative complications and radiologic outcomes were compared between the groups. In the CP group, the Japanese Orthopaedic Association score; Oswestry neck disability index; modified Barthel index; and values for the grip and pinch, Box and Block, and Jebsen-Taylor hand function tests were obtained preand postoperatively and compared between those with and without postoperative complications.Results : Sixteen patients (16/35%) in the CP group and seven (7/35%) in the NCP group (p=0.021) had postoperative complications. Adjacent segment degeneration (p=0.021), postoperative motor weakness (p=0.037), and revisions (p=0.003) were significantly more frequent in the CP group than in the NCP group; however, instrument-related complications were not significantly higher in the CP group (7/35 vs. 5/35, p=0.280). The number of preoperative fixed cervical deformities were significantly higher in CP with postoperative complications (5/16 vs. 1/19, p=0.037). In the CP group, clinical outcomes were almost similar between those with and without postoperative complications.Conclusion : The occurrence of complications during the follow-up period was high in patients with CP. However, postoperative complications did not significantly affect clinical outcomes.

Background Cognitive and emotional disturbances are common serious issues in patients with traumatic brain injury (TBI). However, predictors associated with neuropsychological functions were not consistent. Objective To investigate factors affecting cognition and emotion in patients with TBI, we evaluated executive function, memory, and emotion based on injury severity and lesion location. Methods Neuropsychological outcomes of 80 TBI patients were evaluated via Wisconsin Card Sorting Test (WCST), Color Trail Test (CTT), Controlled Oral Word Association Test (COWAT), Everyday Memory Questionnaire (EMQ), Geriatric Depression Scale (GDS), State-Trait Anxiety Inventory (STAI), and Agitated Behavior Scale (ABS). WCST, CTT, and COWAT assessed executive function; EMQ assessed everyday memory; and GDS, STAI, and ABS assessed emotion. Patients were categorized according to lateralization of lesion and existence of frontal lobe injury. Results Patients with longer duration of loss of consciousness (LOC) showed more severe deficits in everyday memory and agitated behaviors. The frontal lesion group showed poorer performance in executive function and higher agitation than the non-frontal lesion group. Patients with bilateral frontal lesion showed greater deficits in executive function and were more depressed than unilateral frontal lesion groups. Especially in those unilateral frontal lesion groups, right side frontal lesion group was worse on executive function than left side frontal lesion group. Conclusions Duration of LOC and lesion location are main parameters affecting executive function, everyday memory, and emotion in neuropsychological outcomes following TBI, suggesting that these parameters need to be considered for cognitive rehabilitation interventions.

This study aimed to investigate the differences in gait outcomes of young adults with cerebral palsy (CP) following rhythmic auditory stimulation (RAS) with different types of cueing. A total of 13 ambulatory adults with CP were recruited. The participants were assigned to receive either RAS with simple chords or RAS with complex chords. Each participant received 30-min individual sessions three times per week for 4 weeks. In the simple RAS group, basic chords were used for cueing. In the complex RAS group, the diversified chords were adopted from patients' preferred music. At pre- and posttest, spatiotemporal and kinematic parameters and the range of motion (ROM) for each joint during a gait cycle were collected. After RAS, cadence, velocity, and stride length significantly increased, but no significant group effect was found. Meanwhile, regarding kinematic parameters, a significant interaction effect between time and group was observed with the angle of plantar flexion in the preswing phase and ROM in the ankle. The complex RAS group showed increased maximal ankle plantar flexion in the preswing phase. These results demonstrated that the primary agent for gait control is rhythm, while perception of music facilitates patient engagement in walking differently depending on the level of musical elements.

Lateral cephalometry has been widely used for skeletal classification in orthodontic diagnosis and treatment planning. However, this conventional system, requiring manual tracing of individual landmarks, contains possible errors of inter- and intravariability and is highly time-consuming. This study aims to provide an accurate and robust skeletal diagnostic system by incorporating a convolutional neural network (CNN) into a 1-step, end-to-end diagnostic system with lateral cephalograms. A multimodal CNN model was constructed on the basis of 5,890 lateral cephalograms and demographic data as an input. The model was optimized with transfer learning and data augmentation techniques. Diagnostic performance was evaluated with statistical analysis. The proposed system exhibited >90% sensitivity, specificity, and accuracy for vertical and sagittal skeletal diagnosis. Clinical performance of the vertical classification showed the highest accuracy at 96.40 (95% CI, 93.06 to 98.39; model III). The receiver operating characteristic curve and the area under the curve both demonstrated the excellent performance of the system, with a mean area under the curve >95%. The heat maps of cephalograms were also provided for deeper understanding of the quality of the learned model by visually representing the region of the cephalogram that is most informative in distinguishing skeletal classes. In addition, we present broad applicability of this system through subtasks. The proposed CNN-incorporated system showed potential for skeletal orthodontic diagnosis without the need for intermediary steps requiring complicated diagnostic procedures.

Swallowing difficulties are a common complaint among patients with a variety of diseases. To address these concerns, a singing-enhanced swallowing protocol was constructed, and its differential benefits for two patient populations were investigated. Two patients with Parkinson’s disease (PD) and two patients with head and neck cancer (HNC) participated in this study. Each patient participated in 30-min individual sessions of a singing-enhanced swallowing protocol two times per week for 12 weeks. Following the intervention, laryngeal diadochokinesis and quality-of-life measurements were found to be higher in all four patients. However, the Videofluoroscopic Dysphagia Scale showed this improvement was associated with different swallowing tasks for each patient group. In addition, the maximum phonation time decreased for patients with HNC, while it increased for patients with PD. The findings support the use of a singing-enhanced swallowing protocol for patients whose swallowing difficulties are due to neurological or structural impairment. In addition, the study results suggest that different intervention components should be considered depending on the etiology of the patient’s swallowing difficulties.

Hypoxic–ischemic encephalopathy (HIE) is a devastating neonatal brain condition caused by lack of oxygen and limited blood flow. Environmental enrichment (EE) is a classic paradigm with a complex stimulation of physical, cognitive, and social components. EE can exert neuroplasticity and neuroprotective effects in immature brains. However, the exact mechanism of EE on the chronic condition of HIE remains unclear. HIE was induced by a permanent ligation of the right carotid artery, followed by an 8% O2 hypoxic condition for 1 h. At 6 weeks of age, HIE mice were randomly assigned to either standard cages or EE cages. In the behavioral assessments, EE mice showed significantly improved motor performances in rotarod tests, ladder walking tests, and hanging wire tests, compared with HIE control mice. EE mice also significantly enhanced cognitive performances in Y-maze tests. Particularly, EE mice showed a significant increase in Cav 2.1 (P/Q type) and presynaptic proteins by molecular assessments, and a significant increase of Cav 2.1 in histological assessments of the cerebral cortex and hippocampus. These results indicate that EE can upregulate the expression of the Cav 2.1 channel and presynaptic proteins related to the synaptic vesicle cycle and neurotransmitter release, which may be responsible for motor and cognitive improvements in HIE.

Parkinson’s disease (PD) is a prevalent motor disease caused by the accumulation of mutated α-synuclein (α-Syn), however, its early stages are also characterized by non-motor symptoms, such as olfactory loss, cognitive decline, depression, and anxiety. The therapeutic effects of environmental enrichment (EE) on motor recovery have been reported, but its effects on non-motor symptoms remain unclear. Herein, we reveal the beneficial effects of EE on PD-related non-motor symptoms and changes in synaptic plasticity in the nucleus accumbens. To investigate its therapeutic effects in the early phase of PD, we randomly assigned eight-month-old mice overexpressing human A53T (hA53T) α-Syn to either the EE or standard condition groups for two months. Next, we performed behavioral tests and biochemical and histological analyses at 10 months of age. EE significantly alleviated locomotor hyperactivity and anxiety during the early stages of PD. It normalized the levels of tyrosine hydroxylase, phosphorylated and oligomeric α-Syn, and soluble N-ethylmaleimide-sensitive factor attachment protein receptor complex-forming proteins, including synaptosomal-associated protein, 25 kDa, syntaxin1, and vesicle-associated membrane protein 2 (VAMP2). Moreover, the interactions between VAMP2 and pSer129 α-Syn were markedly reduced following EE. The restoration of synaptic vesicle transportation status may underlie the neuroprotective effects of EE in hA53T α-Syn mice.

BackgroundAlthough Parkinson’s disease (PD) is one of the prevalent motor diseases caused by the accumulation of mutated α-Synuclein (α-Syn), it is characterized by non-motor symptoms such as olfactory loss, cognitive decline, depression and anxiety in the early stage of the disease. Environmental enrichment (EE) provides a complex environment comprising physical, cognitive, and social stimuli that improve synaptic plasticity and behavioral functions. The therapeutic effects of EE on behavioral recovery of motor function in a mouse model of PD pathology have been reported. However, the effects of EE on non-motor symptoms in human A53T α-Syn overexpressing transgenic (hA53T α-Syn) mice have yet to be determined. In this study, we revealed the beneficial effect of EE on changes in synaptic plasticity in the striatum and nucleus accumbens (NAc) during the initial phase of PD.MethodTo investigate therapeutic effects of EE in abnormalities during the early phase of PD, we randomly assigned eight-month-old hA53T α-Syn mice to either EE (PD-EE) or standard conditions (PD-SC) for two months. Next, we performed behavioral tests, biochemical and histological analysis at 10 months of age.ResultsEE significantly alleviated locomotor hyperactivity and anxiety in the early stage of PD. EE normalized the level of tyrosine hydroxylase (TH), phosphorylated and oligomeric forms of α-Syn (pSer129 α-Syn), and SNARE complex-forming proteins including SNAP-25, Syntaxin1 and VAMP2. Moreover, the close link between VAMP2 and pSer129 α-Syn was significantly reduced after exposure to EE.ConclusionsOur results showed that EE attenuates aversive mood state on the early stage of PD mouse model. These results were parallel with the reduced expression of pathological α-Syn and the increased expression of neurotransmitter transporter proteins in EE. Interestingly, the interaction between pSer129 α-Syn and VAMP2 also decreased in EE. The restoration of synaptic vesicle transportation status may be responsible for the neuroprotective effects of EE in hA53T α-Syn mice.

Purpose Severe sepsis is associated with functional disability among patients surviving an acute phase of infection. Efforts to improve functional impairment are important. We assessed the effects of early exercise rehabilitation on functional outcomes in patients with severe sepsis. Materials and methods A prospective, single-center, case-control study was conducted between January 2013 and May 2014 at a tertiary care center in Korea. Patients with severe sepsis and septic shock were enrolled and randomized to receive standard sepsis treatment or intervention. Intervention involved early targeted physical rehabilitation with sepsis treatment during hospitalization. Participants were assessed at enrollment, hospital discharge, and 6 months after enrollment. Functional recovery was measured using the Modified Barthel Index (MBI), Functional Independence Measure (FIM), and Instrumental Activities of Daily Living (IADL). Results Forty participants (21 intervention patients) were included in an intention-to-treat analysis. There were no significant differences in baseline MBI, FIM, and IADL between groups. Intervention yielded greater improvement of MBI, FIM, and IADL in the intervention group at hospital discharge, but not significantly. Subgroup analysis of patients with APACHE II scores ≥10 showed significantly greater improvement of physical function at hospital discharge (MBI and FIM) in the intervention group, compared to the control group (55.13 vs. 31.75, p=0.048; 52.40 vs. 31.25, p=0.045). Intervention was significantly associated with improvement of MBI in multiple linear regression analysis (standardized coefficient 0.358, p=0.048). Conclusion Early physical rehabilitation may improve functional recovery at hospital discharge, especially in patients with high initial severity scores.

배경 및 목적: 말장애지수(Speech Handicap Index, SHI)는 말 평가에 필수적으로 실시되는 자기보고 평가로서, 구강이나 구인두암 환자의 말 기능과 심리사회적 기능을 평가하는 데 특화되어 개발된 도구이다. SHI가 말 문제를 평가하기에 적합하게 개발되었음에도 불구하고 30개라는 전체 문항 수는 시간적인 제약을 받는다. 따라서 이 연구에서는...

배경 및 목적: 대부분의 파킨슨병 환자들은 본인들의 말 문제를 스스로 인식하므로, 그로 인해 좌절감, 무능감을 느끼고 삶의 질이 저하된다. 국내외적으로 파킨슨병 환자의 일반적인 삶의 질에 관한 다양한 연구가 이루어지고 있지만 파킨슨병 환자의 말 문제에 특화된 삶의 질 연구는 그리 많지 않다. 방법: 본 연구는 파킨슨병 환자 48명을 대상으로...

Purpose Pain Scrambler therapy is a patient-specific electrocutaneous nerve stimulation device. Burn pruritus is a common form of chronic and disabling neuropathic pain that is often difficult to treat effectively. Pruritus is mediated by histamines, which are effector molecules stored in mast cells and released locally during injury or inflammation. Burn pruritus may be accompanied by peripheral neuropathic pain, which may result from injury to sensory nerves that hampers conductance of neuronal messages along the large A and small C afferent fibers to the spinal cord. In this study, we investigated the effect of pain Scrambler therapy on burn scar pruritus. Methods Sixteen subjects were recruited to participate in this study. The subjects complained of severe pruritus that was rated at least 5 on the visual analogue scale (VAS), despite treatments with antihistamines, gabapentin medication, and other physical modalities. Each Scrambler Therapy with the MC-5A Pain Scrambler Therapy® technology device was performed for 40 min daily (Monday through Friday) for 10 consecutive days. The stimulus was increased to the maximum intensity bearable by the individual patient without causing any additional pain or discomfort. The numerical rating scale (NRS), 5-D Itch Scale, and Leuven Itch Scale were administered and evaluated immediately before Scrambler therapy, and then immediately after 5 and 10 therapy sessions. Results For all 16 patients, NRS showed mean values of 6.75 ± 1.13 before therapy, 5.06 ± 1.53 after 5 sessions, and 4.13 ± 1.45 after 10 sessions. The NRS values before therapy and after 10 sessions were significantly different (p

배경 및 목적: 독립적인 생활이 여전히 가능한 치매 환자조차도 인지 기능의 저하로 인해 숫자 처리와 계산에서 어려움을 보일 수 있다. 본 연구에서는 치매 환자군이 정상군에 비해 숫자 처리와 계산 수행 시 오류율과 오류 유형에서 어떤 차이를 보이는지 살펴보았다. 방법: 알츠하이머성 치매 환자 및 일반 노인 각 16명씩을 대상으로 숫자 표기법(기수법,...

Difficulties with speech and swallowing occur in patients with Parkinsonism. Lee Silverman Voice Treatment (LSVT) is proven as an effective treatment for speech and swallowing function in idiopathic Parkinson’s disease (IPD). The effect of LSVT on swallowing function in multiple system atrophy-cerebellar type (MSA-C) is unknown. We sought to determine LSVT’s effect on swallowing function in MSA-C patients compared to IPD patients. LSVT-LOUD was performed on 13 patients with Parkinsonism (6 IPD and 7 MSA-C). Maximum phonation time (MPT), voice intensity, Speech Handicap Index-15 (SHI-15), Swallowing-Quality of Life (SWAL-QOL), National Institutes of Health-swallowing safety scale (NIH-SSS), and videofluoroscopic dysphagia scale (VDS) before and after LSVT were analyzed and reevaluated three months after treatment. The IPD and MSA-C groups showed significant improvements in overall speech and swallowing measures after LSVT. In particular, pharyngeal phase score and total score of VDS improved significantly in both groups. A two-way repeated-measure ANOVA revealed a significant main effect for time in the MPT, voice intensity, NIH-SSS, pharyngeal phase score and total score of VDS, psychosocial subdomain of SHI-15, and SWAL-QOL. The MSA-C group experienced less overall improvement in swallowing function, but the two groups had an analogous pattern of improvement. In conclusion, LSVT is effective for enhancing swallowing function, particularly in the pharyngeal phase, in both IPD and MSA-C patients. This study demonstrated that LSVT elicits significant improvements in MSA-C patients. We deemed LSVT to be an effective treatment for IPD and MSA-C patients who suffer from dysphagia.

Background Individuals with cerebral palsy (CP) experience bone loss due to impaired weight bearing. Despite serious complications, there is no standard medication. Objective To develop a new pharmacological agent, we performed a series of studies. The primary aim was to develop an animal model of CP to use our target medication based on transcriptome analysis of individuals with CP. The secondary aim was to show the therapeutic capability of collagen-binding peptide (CBP) in reversing bone loss in the CP mouse model. Methods A total of 119 people with CP and 13 healthy adults participated in the study and 140 mice were used for the behavioral analysis and discovery of therapeutic effects in the preclinical study. The mouse model of CP was induced by hypoxic-ischemic brain injury. Inclusion and exclusion criteria were established for CBP medication in the CP mouse model with bone loss. Results On the basis of clinical outcomes showing insufficient mechanical loading from non-ambulatory function and that underweight mainly affects bone loss in adults with CP, we developed a mouse model of CP with bone loss. Injury severity and body weight mainly affected bone loss in the CP mouse model. Transcriptome analysis showed SPP1 expression downregulated in adults with CP who showed lower bone density than healthy controls. Therefore, a synthesized CBP was administered to the mouse model. Trabecular thickness, total collagen and bone turnover activity increased with CBP treatment as compared with the saline control. Immunohistochemistry showed increased immunoreactivity of runt-related transcription factor 2 and osteocalcin, so the CBP participated in osteoblast differentiation. Conclusions This study can provide a scientific basis for a promising translational approach for developing new anabolic CBP medication to treat bone loss in individuals with CP.

The present study aimed to investigate gene expression changes related to cell cycle activation in patients with spinal cord injury (SCI) and to further evaluate the difference between the upper and lower limbs of SCI patients. Fibroblasts were obtained from the upper and lower limbs of SCI patients and healthy subjects. To investigate gene expression profiling in the fibroblasts from SCI patients compared to the healthy subjects, RNA-Seq transcriptome analysis was performed. To validate the parasympathetic effects on cell cycle activation, fibroblasts from upper or lower limbs of SCI patients were treated with the anticholinergic agents tiotropium or acetylcholine, and quantitative RT-PCR and Western blot were conducted. Cell proliferation was significantly increased in the upper limbs of SCI patients compared with the lower limbs of SCI patients and healthy subjects. The pathway and genes involved in cell cycle were identified by RNA-Seq transcriptome analysis. Expression of cell-cycle-related genes CCNB1, CCNB2, PLK1, BUB1, and CDC20 were significantly higher in the upper limbs of SCI patients compared with the lower limbs of SCI patients and healthy subjects. When the fibroblasts were treated with tiotropium the upper limbs and acetylcholine in the lower limbs, the expression of cell-cycle-related genes and cell proliferation were significantly modulated. This study provided the insight that cell proliferation and cell cycle activation were observed to be significantly increased in the upper limbs of SCI patients via the parasympathetic effect.

The present study investigated the distribution and antimicrobial susceptibility patterns of Vibrio parahaemolyticus in water samples and aquatic animals (fish and shrimp) from major aquaculture farms along the Korean coast in 2018. V. parahaemolyticus is the most common pathogen causing seafood-borne illness. The strain was detected in 34.7% of all samples tested, and was detected at higher levels during summer to autumn when the water temperature is higher. Although more than 90.0% of V. parahaemolyticus isolates were sensitive to 13 of the 15 antimicrobials tested, which is useful for treating V. parahaemolyticus infectious disease, the isolates exhibited higher resistance to two antibiotics (colistin and ampicillin), which should be excluded as treatment options for these infections. Koreans typically enjoy consuming raw seafood. To reduce the potential human health risk of raw seafood consumption, the prevalence and antimicrobial resistance of V. parahaemolyticus in aquaculture environments should be continuously valuated.

Amyotrophic lateral sclerosis (ALS) is a severe disease causing motor neuron death, but a complete cure has not been developed and related genes have not been defined in more than 80% of cases. Here we compared whole genome sequencing results from a male ALS patient and his healthy parents to identify relevant variants, and chose one variant in the X-linked ATP7A gene, M1311V, as a strong disease-linked candidate after profound examination. Although this variant is not rare in the Ashkenazi Jewish population according to results in the genome aggregation database (gnomAD), CRISPR-mediated gene correction of this mutation in patient-derived and re-differentiated motor neurons drastically rescued neuronal activities and functions. These results suggest that the ATP7A M1311V mutation has a potential responsibility for ALS in this patient and might be a potential therapeutic target, revealed here by a personalized medicine strategy., Yeomin Yun, Sung-Ah Hong et al. compare the whole-genome sequence from a male with amyotrophic lateral sclerosis (ALS) to his healthy parents, identifying the M1311V variant in the X-lined ATP7A gene. They show that CRISPR-mediated gene correction in patient-derived neurons rescues neuronal activity.

Objective There is no consensus on a standardized approach to spasticity or dystonia management of cerebral palsy (CP). This study aimed to investigate clinical outcomes and compare therapeutic responses for pallidal stimulation versus intrathecal baclofen (ITB) therapy in adult patients with severe CP. Methods We retrospectively reviewed patients with CP treated with deep brain stimulation (DBS) of the globus pallidus internus (GPi) or implantation of ITB pump between June 2003 and April 2017. Patients were included if they were clinically diagnosed with medically intractable CP and had >12 months of postprocedural follow-up data. Patients were assessed before and 12 months post-treatment using the visual analogue scale, Burke–Fahn–Marsden Dystonia Rating Scale, self-rating improvement scale, and 36-Item Short-Form General Health Survey Questionnaire. Results Patients (n = 22) were divided into GPi DBS (n = 12) and ITB therapy (n = 10) groups. For the Burke–Fahn–Marsden Dystonia Rating Scale, DBS group movement scores and ITB group disability scores were significantly improved post-treatment. Although visual analogue scales did not differ between groups, self-rating improvement scores differed significantly between groups. For quality of life, physical functioning, body pain, vitality, social functioning, and mental health significantly improved in ITB group 12 months post-treatment compared with those of preoperative period. Only mental health differed significantly between groups. Conclusions Despite retrospective design and relatively low number of cases, this study indicated that ITB therapy was less invasive and more effective in improving the quality of life compared with GPi DBS. ITB therapy should be considered an alternative treatment for patients with severe CP.

Osteoarthritis (OA) is degenerative disease, leading to pain and functional disability. It is reported that polydeoxyribonucleotide (PDRN) is a suitable therapy for OA. However, the therapeutic mechanisms of PDRN in OA are not fully understood. To investigate the effect of PDRN in an in vitro model of OA, interleukin (IL)-1β or phosphate-buffered saline (PBS) was used to treat a human chondrocytic cell line in hypoxic conditions for 24 h (IL-1β group or control group). PDRN was then used to treat IL-1β group cells for 24 h (PDRN group). By Label-Based Human Antibody Array 1000, angiopoietin-2 (ANG-2), platelet-derived growth factor (PDGF), angiostatin, and endostatin, which were related to angiogenesis, were chosen for further validation studies. Quantitative real-time reverse transcription polymerase chain reaction and western blot analysis validated that the levels of PDGF and ANG-2, which were related to pro-angiogenesis, were significantly increased in the PDRN group compared with those in the control group or the IL-1β group. However, the levels of endostatin and angiostatin, which were related in anti-angiogenesis, were significantly decreased in the PDRN group compared with those in the control group or the IL-1β group. In the same manner, vascular endothelial growth factor, which was a mediator of angiogenesis, was significantly increased in the PDRN group compared with those in the control group or the IL-1β group. Furthermore, wound closure was significantly increased in the PDRN group compared with the control group or the IL-1β group by in vitro scratch assay. Moreover, PDRN decreased expression of metalloproteinase 13, as a catabolic factor for OA, but increased expression of aggrecan, which was an anabolic factor for OA. These data suggest that PDRN may promote angiogenesis and wound healing via down-regulation of catabolism and up-regulation of anabolism in an in vitro model of OA.

Purpose This study was designed to investigate the characteristics of Korean patients with calpainopathy. Materials and methods Thirteen patients from ten unrelated families were diagnosed with calpainopathy via direct or targeted sequencing of the CAPN3 gene. Clinical, mutational, and pathological spectra were then analyzed. Results Nine different mutations, including four novel mutations (NM_000070: c.1524+1G>T, c.1789_1790inA, c.2184+1G>T, and c.2384C>T) were identified. The median age at symptom onset was 22 (interquartile range: 15-28). Common clinical findings were joint contracture in nine patients, winged scapula in four, and lordosis in one. However, we also found highly variable clinical features including early onset joint contractures, asymptomatic hyperCKemia, and heterogeneous clinical severity in three members of the same family. Four of nine muscle specimens revealed lobulated fibers, but three showed normal skeletal muscle histology. Conclusion We identified four novel CAPN3 mutations and demonstrated clinical and pathological heterogeneity in Korean patients with calpainopathy.

Objective To investigate the effect of treadmill walking exercise as a treatment method to improve gait efficiency in adults with cerebral palsy (CP) and to determine gait efficiency during overground walking after the treadmill walking exercise. Methods Fourteen adults with CP were recruited in the experimental group of treadmill walking exercise. A control group of 7 adults with CP who attended conventional physical therapy were also recruited. The treadmill walking exercise protocol consisted of 3-5 training sessions per week for 1-2 months (total 20 sessions). Gait distance, velocity, VO2, VCO2, O2 rate (mL/kg·min), and O2 cost (mL/kg·m) were assessed at the beginning and at the end of the treadmill walking exercise. The parameters were measured by KB1-C oximeter. Results After the treadmill walking exercise, gait distance during overground walking up to 6 minutes significantly increased from 151.29±91.79 to 193.93±79.01 m, and gait velocity increased from 28.09±14.29 to 33.49±12.69 m/ min (p

We performed unilateral carotid artery occlusion on CD-1 mice to create a neonatal hypoxic-ischemic (HI) model and investigated the effects of neonatal HI brain injury by studying neurobehavioral functions in these mice compared to non-operated (i.e., normal) mice. During the study, Rice-Vannucci's method was used to induce neonatal HI brain damage in postnatal day 7-10 (P7-10) mice. The HI operation was performed on the pups by unilateral carotid artery ligation and exposure to hypoxia (8% O(2 )and 92% N(2 )for 90 min). One week after the operation, the damaged brains were evaluated with the naked eye through the semi-transparent skull and were categorized into subgroups based on the absence ("no cortical injury" group) or presence ("cortical injury" group) of cortical injury, such as a lesion in the right hemisphere. On week 6, the following neurobehavioral tests were performed to evaluate the cognitive and motor functions: passive avoidance task (PAT), ladder walking test, and grip strength test. These behavioral tests are helpful in determining the effects of neonatal HI brain injury and are used in other mouse models of neurodegenerative diseases. In this study, neonatal HI brain injury mice showed motor deficits that corresponded to right hemisphere damage. The behavioral test results are relevant to the deficits observed in human neonatal HI patients, such as cerebral palsy or neonatal stroke patients. In this study, a mouse model of neonatal HI brain injury was established and showed different degrees of motor deficits and cognitive impairment compared to non-operated mice. This work provides basic information on the HI mouse model. MRI images demonstrate the different phenotypes, separated according to the severity of brain damage by motor and cognitive tests.

Intrathecal baclofen (ITB) has been known to reduce spasticity which did not respond to oral medications and botulinum toxin treatment. However, few results have been reported comparing the effects of ITB therapy in patients with cerebral palsy (CP) and acquired brain injury. This study aimed to investigate beneficial and adverse effects of ITB bolus injection and pump therapy in patients with CP and to compare outcomes to patients with acquired brain injury such as traumatic brain injury and hypoxic brain injury. ITB test trials were performed in 37 patients (19 CP and 18 acquired brain injury). Based on ambulatory function, CP patients were divided into 2 groups: 11 patients with nonambulatory CP and 8 patients with ambulatory CP. Change of spasticity was evaluated using the Modified Ashworth Scale. Additional positive or negative effects were also evaluated after ITB bolus injection. In patients who received ITB pump implantation, outcomes of spasticity, subjective satisfaction and adverse events were evaluated until 12 months post-treatment. After ITB bolus injection, 32 patients (86.5%) (CP 84.2% versus acquired brain injury 88.9%) showed a positive response of reducing spasticity. However, 8 patients with CP had negative adverse effects. Particularly, 3 ambulatory CP patients showed standing impairment and 1 ambulatory CP patient showed impaired gait pattern such as foot drop because of excessive reduction of lower extremity muscle tone. Ambulatory CP patients received ITB pump implantation less than patients with acquired brain injury after ITB test trials (P = .003 by a chi-squared test). After the pump implantation, spasticity was significantly reduced within 1 month and the effect maintained for 12 months. Seventeen patients or their caregivers (73.9%) were very satisfied, whereas 5 patients (21.7%) suffered from adverse events showed no subjective satisfaction. In conclusion, ITB therapy was effective in reducing spasticity in patients with CP and acquired brain injury. Before ITB pump implantation, it seems necessary to perform the ITB bolus injection to verify beneficial effects and adverse effects especially in ambulatory CP.

Aims/Introduction To assess the current status of glycemic control in patients with type 2 diabetes treated with a combination of metformin and sulfonylurea for >3 months, as measured by glycosylated hemoglobin (HbA1c). Materials and Methods Data on patient demographics, diabetic complications, HbA1c, fasting plasma glucose (FPG) and type of treatment were collected in this multicenter, cross-sectional, non-interventional study. Results From April 2008 to February 2009, 5,628 patients were recruited from 299 centers in Korea. Patients characteristics (mean ± SD) were as follows: age 58.4 ± 10.8 years, duration of diabetes 6.1 ± 4.7 years, body mass index 24.7 ± 2.9 kg/m2, HbA1c 7.77 ± 1.22%, FBG 147.4 ± 46.5 mmol/L and FPG 164.0 ± 54.3 mmol/L. The most common diabetic complication was neuropathy (22.5%), followed by retinopathy (18.3%) and microalbuminuria (16.1%). Just 1,524 (27.1%) patients achieved HbA1c ≤7%. A higher number of patients (32.6%) treated by endocrinologists achieved HbA1c ≤7% than those treated by internists (24.4%) and primary care physicians (23.2%). In multivariate analyses, diabetic retinopathy (odds ratio 0.455, 95% confidence interval 0.341–0.606), nephropathy (odds ratio 0.639, 95% confidence interval 0.43–0.949), diabetes for ≥5 years (odds ratio 0.493, 95% confidence interval 0.4–0.606) and older age added by 1 year (odds ratio 1.019, 95% confidence interval 1.01–1.029) was significantly associated with achieving target HbA1c. In addition, treatment by endocrinologists rather than internists significantly increased chances of achieving target HbA1c (odds ratio 1.417, 95% confidence interval 1.146–1.751). Conclusions The majority of patients with type 2 diabetes in Korea had inadequate glycemic control, despite receiving a combination of metformin and sulfonylurea.

Objective: Cerebral palsy (CP) is a disabling condition characterized by the motor impairment, which is difficult to be ameliorated. In the brain of infants with CP, there are persistent pathomechanisms including accentuated neuroinflammation. Since erythropoietin was demonstrated to have neuroprotective effect via anti-inflammatory and anti-apoptotic properties, we hypothesized that the administration of recombinant human EPO (rhEPO) could help children with CP, especially young infants. Materials and method: We investigated the therapeutic efficacy of rhEPO for infants with CP, who had been undergoing active rehabilitation in hospitalized setting to eliminate treatment bias. Twenty infants with CP were randomly divided into EPO or control group equally. We compared the changes in the Gross Motor Function Measure (GMFM) and the Bayley Scales of Infant Development-II (BSID-II) scores during one month of hospitalization between two groups. Results: The improvements after 1 month on the GMFM A and GMFM total scores differed significantly between the groups (p = 0.003, p = 0.04, respectively). However, the changes after 6 months were not different between the two groups. The scores of BSID-II did not show any differences at 1-month and 6-months post-treatment. Conclusion: These results indicated that rhEPO could have therapeutic efficacy for infants with CP during the active rehabilitation and anti-inflammation was suggested to be one of its therapeutic mechanisms.

배경 및 목적: 자발화에 나타난 품사를 살펴봄으로써 화자의 표현 언어를 문법적으로 분석할 수 있다. 노년층과 청년층의 품사 산출 양상을 비교하여, 노년층의 발화 특성을 분석하고자 하였다. 방법: 노년층 35명, 청년층 35명에게 자발화를 수집하였고, 산출한 품사는 형식, 기능, 의미에 따라 나누었다. 이에 따른 품사의 빈도수와 유형수, 어휘다양도의 수치를...

Background: Brother of the regulator of imprinted sites (BORIS) is a putative new oncogene that is classified as a cancer germline gene; however, its role in the development of cancer is unclear. This study investigated the expression of BORIS in lung cancer and its clinical implications. Methods: The expression of BORIS messenger ribonucleic acid (mRNA) in the sputum of 100 patients with lung cancer (50 with squamous cell carcinoma, 36 with adenocarcinoma, and 14 with small-cell carcinoma) was evaluated by reverse transcription polymerase chain reaction. Results: The overall expression rate of BORIS in patients with lung cancer was 36.0%: 19 of 50 squamous cell carcinomas (38.0%), 13 of 36 adenocarcinomas (36.1%), and 4 of 14 (28.6%) small-cell carcinomas. There was no significant difference in the BORIS expression according to age, gender, or histologic type. However, the mRNA expression of BORIS was significantly related to the pathologic cancer stage (p=0.004) and lymph node metastasis (p=0.001). The expression of the melanoma antigen gene family A1?6 was not associated with the expression of BORIS. Conclusion: Our results suggest that the expression of BORIS might be a negative prognostic factor in lung cancers and implicate BORIS as a molecular target for immunotherapy.

The purpose of this study is to examine the effects of Therapeutic Instrument Music Performance (TIMP) for fine motor exercises in adults with cerebral palsy (CP). Individuals with CP (n = 5) received a total of twelve, 30-min TIMP sessions, two days per week for six to nine weeks. Pre- and post-Music Instrument Digital Interface (MIDI) data were used as a measure of hand function. Pre-velocity was significantly different from the normative data obtained from typical adults (n = 20); however, post-velocity did not yield significance, specifically in the second and fifth fingers, indicating improvement in hand function for the adults with cerebral palsy. The finding implies that TIMP using keyboard playing may effectively improve manual dexterity and velocity of finger movement. Based on these results, future program development of instrumental playing for adults with CP is called for to enhance both their independent living skills and quality of life.

Bone loss is a serious clinical issue in patients with cerebral palsy (CP). Sclerostin has garnered interest as a key mechanosensor in osteocytes, leading to considerations of the therapeutic utilization of anti-sclerostin medications. This study was undertaken to determine associations among mechanical unloading, sclerostin levels, and bone imbalance in patients with CP. A total of 28 patients with CP participated in this cross-sectional study. The following measurements were taken: anthropometrics, clinical diagnosis of CP subtype and ambulatory status, bone mineral density (BMD) z-scores at the lumbar spine and hip, and blood biochemical markers, including sclerostin, parathyroid hormone (PTH), osteocalcin, C-terminal telopeptide, 25-hydroxyvitamin D, 1,25-dihydroxyvitamin D, creatinine, calcium, and phosphorus. In analysis according to CP subtype, patients with spastic CP showed significantly lower BMD z-scores at the lumbar spine and femur neck regions than patients with dyskinetic CP. In analysis according to ambulatory status, patients with non-ambulatory CP showed significantly lower BMD z-scores at all lumbar spine and femoral sites, lower PTH and creatinine levels, and higher plasma sclerostin levels than patients with ambulatory CP. In regression analysis, ambulatory status was a significant determinant of plasma sclerostin levels. This study is the first to report on sclerostin levels and BMD in patients with CP, based on the hypothesis that patients who lack sufficient weight-bearing activities would show increased sclerostin levels and decreased BMD scores, compared with patients who sustain relatively sufficient physical activity. Therefore, this report may provide clinical insights for clinicians considering ambulatory status, sclerostin levels, and bone loss in patients with CP.

This study was designed to investigate functional recovery after the transplantation of mesenchymal stem cells (MSCs) or neurally differentiated MSCs (NMSCs) derived from bone marrow in a rat model of spinal cord injury (SCI). Sprague-Dawley rats were subjected to incomplete SCI using an NYU impactor to create a free drop contusion at the T9 level. The SCI rats were then classified into three groups; MSCs, NMSCs, and phosphate-buffered saline (PBS)-treated groups. The cells or PBS were administrated 1 week after SCI. Basso-Beattie-Bresnahan (BBB) locomotor rating scores were measured at 1-week intervals for 9 weeks. Somatosensory evoked potentials (SSEPs) and motor evoked potentials (MEPs) were also recorded 8 weeks after transplantation. While transplantation of MSCs led to a clear tendency of motor recovery, NMSC-treated rats had significantly improved BBB scores and showed significantly shortened initial latency, N1 latency, and P1 latency of the SSEPs compared to PBS controls. In addition, 5-bromo-2-deoxyuridine (BrdU)-prelabeled MSCs costained for BrdU and glial fibrillary acidic protein (GFAP) or myelin basic protein (MBP) were found rostrally and caudally 5 mm each from the epicenter of the necrotic cavity 4 weeks after transplantation. These results suggest that neurally differentiated cells might be an effective therapeutic source for functional recovery after SCI.

Objective To assess several different cervical alignment parameters to determine the clinical relationship between cerebral palsy (CP) with cervical spondylotic myelopathy (CSM) and cervical deformity. Methods This study included consecutive patients (N = 31) with CP CSM who underwent cervical operation between January 2006 and January 2014 and who had cervical deformities, such as angular and translational deformities. Cervical spine alignment was assessed with the following parameters: C2-7 Cobb angle, C2-7 sagittal vertical axis (SVA), and T1 slope minus C2-7 Cobb angle. Other clinical values were the manual muscle test, spasticity, grip and pinch test, Box and Block test, and Jebsen-Taylor Hand Function Test. Outcome assessments (Oswestry Neck Disability Index [NDI] and modified Barthel Index) were obtained for all patients pre- and postoperatively. Results Mean follow-up duration was 3.5 years. There were 13 patients in the corrected group and 18 in the not corrected group. Angular and translational correction were 19.0° (C2-7 Cobb angle), 19.8° (T1 slope minus C2-7 Cobb angle), and 16 mm (C2-7 SVA). Postoperative NDI scores showed greater improvement in the corrected group than the uncorrected group ( P = 0.049). In the corrected group, grip power increased postoperatively (8.9 ± 8.9 vs 15.5 ± 8.3; P = 0.021). Conclusions Surgical treatment for patients with CP CSM deformity helped alleviate symptoms. Postoperative NDI scores and hand function improved in patients with CP CSM deformity, especially those in the corrected group. Clinicians should consider correcting the deformity in patients with CP CSM.

Neural stem cells (NSCs) persist in the subventricular zone lining the ventricles of the adult brain. The resident stem/progenitor cells can be stimulated in vivo by neurotrophic factors, hematopoietic growth factors, magnetic stimulation, and/or physical exercise. In both animals and humans, the differentiation and survival of neurons arising from the subventricular zone may also be regulated by the trophic factors. Since stem/progenitor cells present in the adult brain and the production of new neurons occurs at specific sites, there is a possibility for the treatment of incurable neurological diseases. It might be feasible to induce neurogenesis, which would be particularly efficacious in the treatment of striatal neurodegenerative conditions such as Huntington's disease, as well as cerebrovascular diseases such as ischemic stroke and cerebral palsy, conditions that are widely seen in the clinics. Understanding of the molecular control of endogenous NSC activation and progenitor cell mobilization will likely provide many new opportunities as therapeutic strategies. In this review, we focus on endogenous stem/progenitor cell activation that occurs in response to exogenous factors including neurotrophic factors, hematopoietic growth factors, magnetic stimulation, and an enriched environment. Taken together, these findings suggest the possibility that functional brain repair through induced neurorestoration from endogenous stem cells may soon be a clinical reality.

Erythropoietin (EPO) and granulocyte-colony stimulating factor (G-CSF) are known to have neuroprotective actions. Based on previous reports showing the synergistic effects of EPO+G-CSF combination therapy in experimental models, we investigated the safety of EPO+G-CSF combination therapy in patients with chronic stroke. In a pilot study, 3 patients were treated with EPO and G-CSF for 5 consecutive days, with follow-up on day 30. In an exploratory double-blind study, 6 patients were allocated to treatment with either EPO+G-CSF or placebo. Treatment was applied once a day for 5 days per month over 3 months. Participants were followed up for 6 months. To substantiate safety, vital signs, adverse events, and hematological values were measured on days 0, 5, and 30 in each cycle and on day 180. Functional outcomes were determined on day 0 and 180. In the laboratory measurements, EPO+G-CSF combination therapy significantly elevated erythropoietin, CD34(+) hematopoietic stem cells, white blood cells, and neutrophils on day 5 of each cycle. There were no observations of serious adverse events. In the functional outcomes, the grip power of the dominant hand was increased in the EPO+G-CSF treatment group. In conclusion, this exploratory study suggests a novel strategy of EPO+G-CSF combination therapy for stroke patients.

Objective To clarify factors affecting bone mineral density (BMD) in adults with cerebral palsy (CP). Method Thirty-five patients with CP participated in this study. Demographic data including gender, age, body mass index (BMI), subtype according to neuromotor type and topographical distribution, ambulatory function, and functional independence measure (FIM) were investigated. The BMD of the lumbar spine and femur were measured using Dual-energy X-ray absorptiometry, and the factors affecting BMD were analyzed. Results The BMD had no significant association with factors such as gender, age, and subtype in adults with CP. However, BMI was significantly correlated with the BMD of lumbar spine and femur (p

Although intravenous administration of mesenchymal stem cells (MSCs) can enhance functional recovery after spinal cord injury (SCI), the underlying mechanisms have to be elucidated. In this study, we explored the mechanisms for functional recovery in SCI rats after intravenous transplantation of MSCs derived from human umbilical cord blood. Sprague-Dawley rats were randomly assigned to receive either MSCs (1 × 106 cells/0.5 ml) or PBS into the tail vein immediately after SCI. They were then evaluated by the Basso-Beattie-Bresnahan (BBB) locomotor rating scale weekly for 8 weeks and by somatosensory evoked potentials (SSEPs) 8 weeks after transplantation. MSC-treated rats showed a modest but significant improvement in BBB scores and latencies of SSEPs, compared with PBS controls. When human-specific Alu element was measured in the spinal cord, it was detected only 1 h after transplantation, suggesting transient engraftment of MSCs. Inflammatory cytokines were also determined using RT-PCR or Western blot in spinal cord extracts. In MSC-treated rats, the level of proinflammatory cytokine IL-1β was decreased, but that of antiinflammatory cytokine IL-10 was increased. MSCs also immediately suppressed IL-6 at 1 h posttransplantation. However, the response of IL-6, which has an immunoregulatory role, was increased 1–3 days after transplantation. In addition, we quantified microglia/macrophage stained with Iba-1 around the damaged spinal cord using immunohistochemistry. A proportion of activated microglia and macrophages in total Iba-1+ cells was significantly decreased in MSC-treated rats, compared with PBS controls. These results suggest that early immunomodulation by intravenously transplanted MSCs is a potential underlying mechanism for functional recovery after SCI.

Two stents were placed across the right subclavian vein due to stenosis of the right subclavian vein in a 40-year-old patient with chronic renal failure on hemodialysis. During the follow up period, one of stents migrated into the right ventricle inducing tricuspid valve insufficiency. Percutaneous stent removal had failed and the stent was removed by open heart surgery with Tricuspid valve repair with a good result, and then we report the case.

Recently, in automotive industry, there have been many researches related with hardware components and embedded software which controls hardware components. Since most of embedded software is tightly dependent on car manufacturers, there were some problems in reusability and interoperability of automotive software. In order to solve these problems, AUTOSAR standardized the component-based software architecture of automotive software. In AUTOSAR, several modeling diagrams should be described and their dependencies are also checked. Currently, a few company developed the prototypes of tools supporting AUTOSAR. In this paper, a component modeling tool based on AUTOSAR 3.0 is developed for enhancing the usability of existing tools using Eclipse GMF. The tool is composed of a graphical component modeling tool and a graphical network topology tool. Since these tools are generated based on GMF without hard coding, it is relatively easy to customize the tools for adopting company‘s needs and easy to follow the improvement of the standard and development environments.

Objective The purpose of this study was to investigate the feasibility of using real-time high-resolution sonography to guide an injection needle into the intra-articular space within the knee. Methods Eighty-nine patients with radiographically confirmed knee osteoarthritis (Kellgren-Lawrence grade 2 or 3) without an effusion were included. After sonographically guided or blind injection of hyaluronic acid (HA) and contrast dye through a medial patellar portal (MPP) into the knee joint, a radiographic image was made to ascertain whether the injected material had reached the intra-articular space. Result Sonographically guided injections of HA into the knee joint had a significantly greater accuracy rate (95.6%) than blind injections (77.3%; P = .01). Conclusions Intra-articular injections via an MPP using sonographic guidance may raise the accuracy rate in knee joint injections.

Umbilical cord blood (UCB) is known to have stem/progenitor cells. We earlier showed that novel progenitors could be isolated from cryopreserved human UCB with high efficiency. The multipotent progenitor cells were induced to differentiate into neural-lineage cells under the appropriate condition. In this study, we confirmed these neurally induced progenitor cells (NPCs), containing higher quantities of nerve growth factor, promoted functional recovery in rats with spinal cord injury (SCI). Sprague-Dawley rats with SCI achieved a modest improvement in locomotor rating scale until 10 weeks after transplantation of the NPCs. SCI rats treated with NPCs also showed somatosensory-evoked potentials were recovered, and grafted cells especially exhibited oligodendrocytic phenotype around the necrotic cavity. These findings suggest that UCB-NPCs might be a therapeutic resource to repair damaged spinal cords.

This study aimed to determine the effects of freeze-dried onion powder (OP) which contains rich in quercetin (22 ㎎/10 g) on hyperlipidemic participants. Fourteen participants (total cholesterol level in plasma 270.8±21.4 ㎎/dL) 40～59 years of age who were diagnosed as hyperlipidemia by a physician at Fatima Hospital in Changwon took part in this study for 12 weeks. We investigated the anthropometric data and dietary habit by using questionnaires. Also, blood samples were collected from the patients in fasting before and after this 12 weeks intervention study. 78.6% of the patients showed interest in health diet management and weight control; however, they also showed some problems in their dietary habit (meal irregularity, food choice, calorie nutrient balance, high fat and cholesterol etc.) and life style (smoking, eating out, alcohol intake) that could have adverse effect on hyperlipidemic therapy. Most participants (85.7%) thought that OP was good for their health. The total cholesterol and LDL-cholesterol significantly decreased with OP intake. The HDL-cholesterol increased and the Atherogenic index (AI) significantly decreased. Regarding the correlations of dietary habit and life style with anthropometric and biochemical factors, the alcohol intake level was significantly correlated with the frequency of eating out and body mass index (BMI). Exercise level showed positive correlation with HDL-cholesterol and negative correlation with atherogenic index (AI). These results suggest that the intake of polyphenol rich onion may decrease the risk of hyperlipidemia by reducing cholesterol level. We consider these hypolipidemic effects of OP as a possible functional food. However, further research such as a well designed longterm clinical study with experimental and placebo groups is needed.

Objective To investigate immediate changes in hyolaryngeal movement and swallowing function after a cycle of neuromuscular electrical stimulation (NMES) on both submental and throat regions and submental placement alone in patients with dysphagia. Methods Fifteen patients with dysphagia were recruited. First, videofluoroscopic swallowing study (VFSS) was performed before NMES. All patients thereafter received a cycle of NMES by 2 methods of electrode placement: 1) both submental and throat regions and 2) submental placement alone concomitant with VFSS. The Penetration- Aspiration Score (PAS) and the NIH-Swallowing Safety Scale (NIH-SSS) were measured for swallowing function. Results During swallowing, hyolaryngeal descent significantly occurred by NMES on both submental and throat regions, and anterior displacement of hyolaryngeal complex was significant on submental placement alone. NMES on submental placement alone did not change the PAS and NIH-SSS. However, NMES on both submental and throat regions significantly reduced the NIH-SSS, although it did not change the PAS. Patients with no brainstem lesion and with dysphagia duration of

Background As a sequential, programmed movement of fingers, keyboard playing is a promising technique for inducing execution and a high level of coordination during finger movements. Also, keyboard playing can be physically and emotionally rewarding for adolescents in rehabilitation settings and thereby motivate continued involvement in treatment. Objective The purpose of this study is to evaluate the effects of keyboard playing using Musical Instrument Digital Interface (MIDI) on finger movement for adolescents with brain damage. Methods Eight adolescents with brain damage, ages 9 to 18 years (M = 13 years, SD = 2.78), in physical rehabilitation settings participated in this study. Measurements included MIDI keyboard playing for pressing force of the fingers and hand function tests (Grip and Pinch Power Test, Box and Block Test of Manual Dexterity [BBT], and the Jebsen Taylor Hand Function Test). Results Results showed increased velocity of all fingers on the MIDI-based test, and statistical significance was found in the velocity of F2 (index finger), F3 (middle finger), and F5 (little finger) between pre- and post-training tests. Correlation analysis between the pressing force of the finger and hand function tests showed a strong positive correlation between the measure of grip power and the pressing force of F2 and F5 on the Grip and Pinch Strength Test. All fingers showed strong correlation between MIDI results and BBT. For the Jebsen Taylor Hand Function Test, only the moving light objects task at post-training yielded strong correlation with MIDI results of all fingers. Conclusions The results support using keyboard playing for hand rehabilitation, especially in the pressing force of individual finger sequential movements. Further investigation is needed to define the feasibility of the MIDI program for valid hand rehabilitation for people with brain damage.

Objective To investigate the effect of deep brain stimulation (DBS) on reducing dystonia and disability in adults with cerebral palsy (CP) and to compare the therapeutic outcomes between primary dystonia patients and CP patients over two years after bilateral pallidal DBS. Methods Five patients with primary dystonia and seven CP patients with dystonia were recruited. All subjects received DBS surgery in both globus pallidus. Burke-Fahn-Marsden dystonia rating scale consisting of dystonia movement score and disability score and subjective satisfaction scale were assessed after 1 month and every 6 months over two years following DBS treatment. Results On the dystonia movement scale, both groups of primary dystonia patients and CP patients showed a significant decrease over time following DBS. On the disability scale, patients with primary dystonia showed a significant decrease over time, whereas the disability score of CP patients did not change over the two years. Comparing the dystonia movement and disability scores of CP patients at each assessment, patients with primary dystonia showed a significant reduction after 6 months. Comparing the satisfaction scores of CP patients after DBS, patients with primary dystonia showed significantly higher subjective satisfaction. Conclusion Whereas dystonia can be significantly reduced in patients with primary dystonia, CP patients showed a modest improvement on the dystonia movement scale, but not on the disability scale. Therefore, DBS may be considered with caution as a treatment modality of CP patients with dystonia.