• 대한유전성대사질환학회지
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• 제14권1호
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• pp.29-36
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• 2014

Mucopolysaccharidoses (MPSs) are a group of rare inherited metabolic diseases caused by deficiency of lysosomal enzymes. MPSs are clinically heterogeneous and characterized by progressive deterioration in visceral, skeletal and neurological functions. The aim of this article is to review the treatment of MPSs, the unmet needs of current treatments and vision for the future including recent clinical trials. Until recently, supportive care was the only option available for the management of MPSs. Hematopoietic stem cell transplantation (HSCT), another potentially curative treatment, is not routinely advocated in clinical practice due to its high risk profile and lack of evidence for efficacy. From the early 2000s, enzyme replacement therapy (ERT) was approved and available for the treatment of MPS I, II and VI. ERT is effective for the treatment of many somatic symptoms, particularly walking ability and respiratory function, and remains the mainstay of MPS treatment. However, no benefit was found in the neurological symptoms because the enzymes do not readily cross the blood-brain barrier (BBB). In recent years, intrathecal (IT) ERT, substrate reduction therapy (SRT) and gene therapy have been rapidly gaining greater recognition as potential therapeutic avenues. Although still under investigation, IT ERT, SRT and gene therapy are promising MPS treatments that may prevent the neurodegeneration not improved by ERT.

• 대한물리치료과학회지
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• 제18권3호
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• pp.33-39
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• 2011

Background : The purpose of this study was to apply cervical orthosis for temporal parameters of gait was to evaluate the effect. Methods : Seven normal adults participated in this study. Before and after applying a cervical orthosis compared to gait. Outcome measure were: general characteristics, temporal parameters of gait. General chacteristics included age, gender, height, weight. Temporal parameters included the Velocity cycle, Stride length, Step length, Cadence cycle, Initial double support time. Temporal parameters of gait, using the motion analysis system for cervical orthosis were evaluated before and after applying. The data was analyzed using SPSS 12.0 software and the Wilcoxon's signed-ranks test. Results : Velocity cycle and Step length were no significant differences(p>0.05). But Stride length, Cadence cycle, Initial double support time were significant(p<0.05). After apply Cervical orthosis in gait, Stride length and Initial double support time was decreased and Cadence cycle was increased. Conclusion : Changes in temporal of temporal parameters of gait was apply a cervical orthosis with the limitations of vision due to take effect. Therefore, Cervical orthosis does not interfere with the normal gait pattern by limiting the Range of Motion so that we consider to apply.

• The Journal of Korean Physical Therapy
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• 제13권3호
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• pp.799-814
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• 2001

This study was performed to investigate the clinical pictures and rehabilitation service patterns of stroke patients in cities of kimchon(kumi), jinju. pohwang, jeonju and jeju from January 1,2001 to march. Authors developed structured questionnaire, and distributed it to each physical therapist. Total number of distributed questionnaire was 400, and 301 questionnaire were col looted and analysed finally.1. The occurrence rate of hemorrhagic stroke(60.5%) was higher than that of ischemic stroke(39.5%). The highest incidence of the stroke was noted in the group of 50 years and ratio of male to female 1.3 : 1. 2. The several warning sign is motor deficit(42.5%). headache. dizziness, vomitting(31.9%) and difficulty speaking or understanding(10.6%). 3. The most important contributing factor of stroke was hypertension both hemorrhagic(50.0%) and ischemic stroke(40.3%). 4. In the painful stroke patients(53.2%). the major problems were shoulder pain(51.3%) and shoulder-hand syndrom(31.3%). 5. In hemorrhagic stroke. the seasonal preference was autumn and summer. In ischemic stroke. the seasonal preference was autumn and winter. 6. In the surgery, hemorrhgic stroke(44.5%) was higher than that of ischemic stroke(20.2%). 7 The major impairment were motor deficit(95.3%), psychological deficits(24.4%), hearing and speech deficit(23.4%). vision deficit(14.7%) and perception deficit(12.7%). 8. The rehabilitation services for stroke patients were physical therapy(98.0%), occupational therapy(41.5%), oriental therapy(35.1%), speech therapy(17.5%) and psychological therapy(7.4%). 9. The start of rehabilitation service was different according to surgery. 36.5% of patients started physical therapy in 4 week and 32.0% of patients started in 8 week later in surgery and 53.1% of patients started physical therapy in 2 week in not surgery( P<0.05). 10. The highest frequency of rehabilitation service was six times per week.

• 한국전문물리치료학회지
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• 제13권4호
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• pp.23-29
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• 2006

The purpose of the present study was to examine gaze effects on spatial and kinematic characteristics during a pointing task. Subjects were asked to watch and point to an aimed target (2 mm in diameter) displayed on a vertically mounted board. Four gaze conditions were developed as combinations of "seeing-aiming" in terms of the eye movements: Focal-Focal (F-F), Focal-Fixing (F-X), Fixing-Focal (X-F), and Fixing-Fixing (X-X). Both the home target and an aimed target were presented for 1 second and then were disappeared in F-F and X-F. In X-F and X-X, only an aimed target disappeared after 1 second. Subjects were asked to point (with index finger tip) to an aimed target accurately as soon as the aimed target was removed. A significant main effect of gaze was found (p<.01) for normalized movement time. Peripheral retina targets had significantly larger absolute error compared to central retina targets on the x (medio-lateral) and z (superior-inferior) axes (p<.01). A significant undershooting to peripheral retina targets on the x axis was found (p<.01). F-F and X-F had larger peak velocities compared to F-X and X-X (p<.01). F-F and X-F were characterized by more time spent in the deceleration phase compared to F-X and X-X (p<.01). The present study demonstrates that central vision utilizes a form of on-line visual processing to reach to an object, and thus increases spatial accuracy. However, peripheral vision utilizes a relatively off-line visual processing with a dependency on proprioceptive information.

• 한국안광학회지
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• 제17권2호
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• pp.159-164
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• 2012

목적: 듀안 화이트(Duane White)에 따른 양성 융합버전스가 부족한 환자에게서 나타나는 기본형 외사위 환자 또는 폭주부족 환자의 일반적인 처치 방법인 양성융합버전스를 직접적으로 늘려 줄 수 있는 BO Prism 훈련 방법으로 프레넬 프리즘렌즈를 이용한 훈련효과에 대해서 알아보고자 하였다. 방법: 안질환이 없는 대전 시내 모 대학의 학생 15명(평균 연령 $22.73{\pm}1.68$세)을 선정하여 굴절검사 및 양안시기능 검사를 진행하여 시기능훈련 전 데이터를 기록하고 완전교정을 한 후에 프레넬 프리즘렌즈를 접착하여 1일 30분, 2주간 시기능훈련을 실시하였다. 이 후 양안 시기능 검사를 재실시하여 양성융합버전스의 변화와 그에 동일하게 모건의 B그룹에 해당하는 조절변화값을 관찰하여 프레넬 프리즘 렌즈의 시기능 훈련 효과를 연구하였다. 결과: 근거리 양성 융합버젼스 평균값이 훈련 후 $22.27{\pm}2.26$ $\Delta$로 7.80 $\Delta$ 만큼의 융합여력이 증가하였고, 근거리 양안조절반응 검사 값의 평균은 훈련 후 $0.55{\pm}0.09$ D로 0.40 D 만큼 증가하여 정상치를 나타내었다. 근거리 음성상대조절값의 평균은 훈련 후 $2.22{\pm}0.08$ D를 나타내 0.42 D 만큼 증가하였으며 폭주근점의 평균은 훈련 후 $6.13{\pm}0.53$ cm로 2.80 cm 만큼 짧아졌다. 폭주부족 환자 및 기본형 외사위 환자의 BO 양성 융합버전스를 늘려 줄 수 있는 프레넬 프리즘을 이용한 근거리 시기능 값을 조사하였고 사위량은 제외한 값이 증가하거나 향상되는 결과를 나타내었다. 결론: 프레넬 프리즘렌즈의 효과는 렌즈 자체의 특성으로 인하여 미용적인 효과와 비용절감, 큰 광학적인 교정 효과를 나타내며 양성융합버전스 값이 증가하는 효과를 나타내는 것을 알 수 있었다. 프레넬 프리즘렌즈를 이용한 양안시 이상의 환자들에게 처치는 보다 대중적이고 많은 추천이 될 수 있음을 판단 할 수 있다.

• 한국안광학회지
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• 제15권2호
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• pp.169-174
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• 2010

목적: 조절이상에 대한 치료가 완료된 어린이에 대해 장기간의 추적 관찰을 함으로써 치료직후에 향상된 눈증상과 조절기능이 지속되는지의 여부를 확인하고자 하였다. 방법: 조절부족이나 조절난이가 있는 조절이상자에 대해 12주 동안 비전세라피 프로그램을 실시한 결과 치료가 성공적으로 이루어진 남여 7명(평균 나이 ${\pm}SD$, $12{\pm}1.41$세)을 대상으로 1년이 경과한 후에 설문지를 사용하여 눈증상을 평가한 후 ${\pm}2.00$ D 플리퍼 렌즈로 단안 및 양안의 조절 용이를 측정하였다. 결과: 치료 프로그램이 완료되고 1년이 경과한 다음 College of Optometrists in Vision Development Quality of Life(COVD-QOL)로 평가한 평균 눈증상은 $15.14{\pm}8.53$점으로 치료 직후의 $11.86{\pm}7.60$점 보다 작은 증가를 보였으나 유의하지 않았다(p=0.176). 그리고 단안(왼쪽 눈) 및 양안의 평균 조절용이 측정값도 각각 $13.86{\pm}3.93cpm$과 $11.14{\pm}3.13cpm$ 으로 치료 직후의 $15.86{\pm}4.14cpm$(p=0.147)과 $13.21{\pm}3.76cpm$(p=0.066)보다 작게 감소하였으나 유의하지는 않았으며, 대상자 모두에서 단안 및 양안 조절용이의 정상값인 7 cpm 이상과 5 cpm 이상을 각각 만족하였다. 결론: 장기간의 추적검사를 통해 치료 프로그램 직후에 향상된 눈증상과 조절기능이 여전히 지속되는 것을 볼 수 있었고, 따라서 조절이상에 대한 비전세라피의 치료효과가 장기적임을 확인하였다.

• Parasites, Hosts and Diseases
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• 제38권1호
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• pp.29-31
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• 2000

A toxoplasmic uveitis case was reported on the focus of impairment of pathological findings and serological antibody titers after chemotherapy. A chief complaint of a 60-year-old male was a decreased and blurred vision in his right eye for 2 weeks after experiencing tremendous stress and fatigue. A steroid therapy for 3 weeks was not effective and the retinal lesion became necrotic. Anti-Toxoplasma gondii antibody titer was checked to be a strong positive by both ELISA and indirect latex agglutination assay (lLA). He was treated with Fansidar F for 8 weeks. His vision improved as the necrotic lesion healed by scarring, but the antibody titers still remained very high without any signs of negative conversion. It is suggested to be a recurrent case of the past asymptomatic infection by presumed immune suppression caused by excessive stress.

• 대한물리치료과학회지
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• 제14권1_4호
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• pp.55-60
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• 2007

The purpose of this case report is to investigate whether an attempt to hold the repeated upright posture under blocking the patient's vision affects the deficits to push away from the paralytic side and the relapse time from down to stand up position without push away in patients with hemiplegia with pusher syndrome. Two hemiplegic patients with pusher syndrome were assessed. The task was performed 4 times per day for 6 weeks. The modified barthel index (MBI) was performed to assess activities of daily living (ADL). For assessing balance, the "balanced sitting" and "sit to stand" are analyzed using by modified motor assessment scale (MMAS). The scale for contraversive pushing (SCP) was used for determination of push away from paralyzed side. MBI, MMAS and SCP were assessed before and after trial of the task. In patient 1, total score of the scale is 0 in sitting posture and standing posture within 3 weeks and 4 weeks, respectively, In patient 2, total score of the scale is 0 in sitting posture and standing posture within 4 weeks and 6 weeks, respectively. These results demonstrated that pusher syndrome was completely resolved in at least 6 weeks. Our findings indicate that this physical therapy seems to be relevant for the hemiplegic patients with pusher syndrome.

• 한국전문물리치료학회지
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• 제14권4호
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• pp.35-43
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• 2007

The purpose of this study was to compare the static balance in a sitting position between a group with adolescent idiopathic scoliosis (AIS) and a normal aged-matched group. Forty-nine subjects were included in this study. Thirty-one healthy subjects and eighteen AIS subjects were participated. Each group was tested with the Lumbar Trunk Muscle Endurance Test (LTMET) and Balance Performance Monitor (BPM). The parameters for static balance were sway area, sway path, mean balance, maximum velocity, anterior-posterior angle, and left-right angle of each group with eyes opened and closed. Results from the LTMET showed significantly more increase in the normal group than in the AIS group in the flexor and extensor endurance. The BPM tested showed significantly difference beteen the groups in parameters of sitting balance such as maximum velocity and anterior-posterior sway angle. For the AIS subjects, there were no significant differences in all parameters of sitting balance between eyes opened and eyes closed. In comparisons of the groups with eyes opened there were no significant differences in all parameters of sitting balance. In comparisons of the groups with eyes closed there were significant differences in the sway area, maximum velocity, anterior-posterior sway angle and left-right sway angle. These results suggest that the AIS group relies much more on proprioception than on vision, and develops compensatory passive postures of the spine. Further study is needed to measure many AIS patients with morphologic and electromyographic data for clinical application.

• Physical Therapy Rehabilitation Science
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• 제11권3호
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• pp.296-303
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• 2022

Objective: In general, macular degeneration, cataracts and glaucoma generally cause visual injury in clinical settings. This study aimed to examine the effects of low visual acuity simulations on hand manual dexterity function and brainwaves in healthy young adults. Design: Cross-sectional study design Methods: This study was an observational, cross-sectional study. Seventy healthy young adults participated in this study. To evaluate the effects of low visual acuity simulations on hand function and brain waves, this study involved four different visual conditions including (1) normal vision, (2) simulated cataracts, (3) simulated glaucoma, and (4) simulated macular degeneration. The hand function was measured to use the Minnesota manual dexterity test (MMDT), and the brainwaves was also measured to use the electroencephalography. Results: In hand function, placing and turning performance on the MMDT in the normal visual condition was significantly different than that in the cataract and macular degeneration conditions (p<0.05), and the placing performance was significantly differred in the normal condition than that in the simulated glaucoma. However, turning was not significantly different in the normal condition than that in the simulated glaucoma. The alpha, beta, and gamma waves did not significantly differ among the four visual conditions (p>0.05). Conclusions: The results suggest that limited visual information negatively affects the ability to perform tasks requiring arm-hand dexterity and eye-hand coordination. However, the effectiveness of low visual acuity on the brainwaves should be further studied for rehabilitative evidence of visual impairment.