I. Alemdaroglu

Different types of upper extremity exercise training in Duchenne muscular dystrophy: Effects on functional performance, strength, endurance, and ambulation

We investigated and compared the effects of 2 different types of upper extremity exercise training on upper extremity function, strength, endurance, and ambulation in patients with early‐stage Duchenne muscular dystrophy (DMD).

Comparison of pain, kinesiophobia and quality of life in patients with low back and neck pain.

[Purpose] The purpose of this study was to compare patients with low back and neck pain with respect to kinesiophobia, pain, and quality of life. [Subjects and Methods] Three-hundred patients with low back (mean age 43.2±11 years) and 300 with neck pain (mean age 42.8±10.2 years) were included in this study. Pain severity was evaluated by using the Short-Form McGill Pain Questionnaire, which includes a Visual Analogue Scale, quality of life by the Nottingham Health Profile, and kinesiophobia by the Tampa Scale for Kinesiophobia. [Results] Pain severity was similar in both groups, with a Visual Analogue Scale score of 6.7±2 in the low back pain and 6.8±2 in the neck pain group. Nottingham Health Profile pain [z=−4.132] and physical activity scores [z=−5.640] in the low back pain group were significantly higher. Kinesiophobia was also more severe in the low back pain group, with a mean 42.05±5.91 versus 39.7±6.0 Tampa Scale for Kinesiophobia score [z=−4.732]. [Conclusion] Patients with low back pain developed more severe kinesiophobia, regardless of the pain severity, and had greater pain perception and lower physical activity levels. Kinesiophobia adversely affects the quality of life and requires effective management of low back pain.

Effect of muscle weakness distribution on balance in neuromuscular disease.

To assess balance and identify the effects of muscle weakness distribution on balance in children with different neuromuscular diseases.

Is there any relationship between orthotic usage and functional activities in children with neuromuscular disorders

Background: Contractures of Achilles tendons and gastrocnemius muscle deteriorate the performance in daily living activities of patients with neuromuscular diseases. Ankle–foot orthoses help to prevent the progression of deformities and to obtain optimal position of the joints to support standing and walking. Objective: To investigate the relationship between orthotic usage and functional activities in pediatric patients with different neuromuscular diseases. Study design: Retrospective study. Methods: A total of 127 subjects’ physical assessment forms were analyzed. Functional level, type of orthoses, falling frequencies, ankle joint range of motion, and timed performance tests were examined in two consecutive dates with an interval of 3 months. Results: A total of 91 patients were using orthoses while 36 patients were not within assessment dates. A total of 64 of 91 (70.3%) patients were diagnosed with Duchenne muscular dystrophy. A total of 81 (89.0%) subjects were using plastic ankle–foot orthoses for positioning at nights and 10 (11%) were using different types of the orthoses (knee–ankle–foot orthoses, dynamic ankle–foot orthoses, and so on) for gait in the study group. Conclusions: Night ankle–foot orthoses were not found to be effective directly on functional performance in children with neuromuscular diseases, although they protect ankle from contractures and may help to correct gait and balance. Clinical relevance This retrospective study shows that the positive effects of using an ankle–foot orthosis at night are not reflected in the functional performance of children with neuromuscular diseases. This may be due to the progressive deteriorating nature of the disease.

Effects of Arm Cycling Exercise in Spinal Muscular Atrophy Type II Patients: A Pilot Study

Exercise studies in neuromuscular diseases like spinal muscular atrophy (SMA), a devastating disease caused by survival of motor neuron 1 (SMN1) gene mutations, are drawing attention due to its beneficial effects. In this study, we presented a constructed arm cycling exercise protocol and evaluated the benefits on SMA patients. Five SMA type II patients performed 12 weeks of supervised arm cycling exercise. The physical functions were evaluated together with the SMN2 copy numbers, SMN protein levels, insulin-like growth factor 1(IGF1) and binding protein 3 (IGFBP3) levels. The active cycling distance and duration of patients significantly improved. Significant changes could not have detected either SMN or IGF1 and IGFBP3 levels in response to exercise. The findings demonstrated that the patients tolerated the exercise protocol and gained a benefit from arm cycling but benefits could not be associated with SMN2 copy number, SMN protein level, IGF1, or IGFBP3 levels.

S.P.16 Egen classification scale (Ek) for Duchenne Muscular Dystrophy and Spinal Muscular Atrophy: Validity and reliability study of the Turkish version

Abstract EK scale was developed to assess detailed functional ability of non-ambulatory Duchenne Muscular Dystrophy (DMD) patients who have severe physical impairments by Steffensen et al. in 2002. EK total score was found to distinguish the different functional levels and show the prognosis of the nonambulatory DMD and Spinal Muscular Atrophy (SMA) patients. The aim of this study was to make the cultural adaptation and investigate the validity and intra/inter-tester reliability of Turkish Version of EK scale on non-ambulatory DMD and SMA patients The original English version of EK scale was translated into Turkish and necessary arrangements were made after the consensus by the researchers. After the arrangements and cultural adaptation; the pre-test study was made on five patients, inter-tester and intra-tester reliability studies were made on totally 34 patients. Brooke Functional Classification Scale for Upper and Lower Extremities (BFC), Barthels Index for Activities of Daily Living and Pulmonary Dysfunction Test (PDT) were used as gold standards to investigate the validity of the scale in Turkish DMD and SMA population. 11 SMA and 23 DMD patients (Mean age: 13.71±4.25) with non-ambulatory functional status were included in the study. Intra-tester reliability of the EK scale was 0.979 (ICC) with % 95 Confidence Interval [0.930; 0.994] and inter-tester was 0.916 (ICC) with % 95 Confidence Interval [0.407; 0.990]. There were correlations between Turkish version of EK scale and Barthel Index ( r =−0.716, it p

Reliability and validity of the Turkish version of ABILHAND-Kids’ questionnaire in a group of patients with neuromuscular disorders

ABSTRACT This study was performed to examine the reliability and validity of the Turkish version of ABILHAND-Kids questionnaire which assesses manual functions of children with neuromuscular diseases (NMDs). A cross sectional survey study design and Rasch analysis were used to assess the reliability and validity of the Turkish version of scale. Ninety-three children with different neuromuscular disorders and their parents were included in the study. The scale was applied to the parents with face-to-face interview twice; on their first visit and after an interval of 15 days. The test–retest reliability was assessed with intraclass correlation coefficient (ICC), and internal consistency of the multi-item subscales by calculating Cronbach alpha values. Brooke Upper Extremity Functional Classification (BUEFC) and Wee-Functional Independency Measurement (Wee-FIM) were correlated to determine the construct validity. The ICC value for the test/retest reliability was 0.94. The internal consistency was 0.81. Floor (1.1%) and ceiling (11.8%) effects were not significant. There were moderate correlations between the Turkish version of ABILHAND-Kids and Wee-FIM (0.67) and BUEFC (−0.37). Rasch analysis indicated good item fit, unidimensionality, and model fit. The Turkish version of ABILHAND-Kids questionnaire was found to be a reliable and valid scale for the assessment of the manual ability of children with NMDs.

Clinical Characteristics of Oral Structures and Feeding Function in Subacute Sclerosing Panencephalitis

Purpose: The aim was to investigate the characteristics of oral structures and feeding in pa tients with Subacute Sclerosing Panencephalitis (SSPE). Methods: Twenty SSPE patients were included. Clinical evaluation of oral structures and oral hygiene were performed. Each patient was requested to take liquid, pudding and solid food consistencies during feeding evaluation. The Karaduman Chewing Performance Scale (KCPS) was used to determine the level of chewing function. The 3-ounce water swallow test was used to screen patients for aspiration risk. Results: The mean age was 13.76±1.92 years, of which 55% were male. Seven children (35%) had open mouth posture at rest, 5 (25%) had open bite, 12 (60%) had high arched palate, and 8 (40%) had tongue thrust. Eighteen children (90%) had oral hygiene problems. Feeding positions were variable with 1 child fed in supine, 6 in semi-sitting position, and 13 in upright sitting position. Findings during clinical feeding evaluation revealed the following: food acceptance (20, 100%), liquid intake (20, 80%), pudding intake (17, 85%), and solid food intake (5, 25%). Five children (25%) were in level I, 7 children (35%) were in level II, 3 children (15%) were in level III, and 5 children (25%) were in level IV according to the KCPS. Four children (25%) failed in 3 ounce water swallow test. Discussion: Problems with oral structures, oral hygiene, chewing and swallowing function can be seen in SSPE patients. Thus, clinical oral structure and feeding functions should be conside red to evaluate in routine evaluation procedure in patients with SSPE.

T.P.43

The aim of this study was to investigate and compare the performance exposure due to different muscle weakness involvement in proximally and distally affected neuromuscular diseases (NMDs). A total of 40 pediatric patients with different NMDs who were grouped based on proximal (Group 1) and distal (Group 2) muscle weakness involvement were included in the study. The children between Grade 1 and 3 according to Brooke Lower Extremity Functional Classification (BLEFC) were taken under assessments. Lower extremity regional and total muscular strengths were assessed with manual muscle testing. 10m walking time (s) and 6 Minute Walk Test (6MWT) (m) were used to evaluate performance level. The relationship between muscle weakness and performance levels was analyzed. 20 patients with proximal (Duchenne Muscular Dystrophy-DMD) and 20 with distal muscle weakness (Hereditary Sensory Motor Neuropathy) who were respectively 9±3.1 and 12.9±3.3years old were included in the study. Lower extremity total muscle strengths were statistically different with 81.5±18.7 in group 1 and 98.7±16.5 in group 2 ( z =−2.764; p z =−0.122) and 6MWT distances ( z =−0.609) between two groups ( p >0.05). There were positive correlations between lower extremity muscles strength scores except for ankle girdle and 6MWT distance ( p p p >0.05). Muscle weakness distribution is an important performance determinative in NMDs. Performance is more effected in proximal muscle weakness involvement than distal at early stages of the disease.