Master of Science / Most studies of condylar movement are based on the movement of an arbitrary condylar point. As the condyle is a 3-dimensional body which undergoes complex rotations and translations in function, the movement of one point in the vicinity of the condyle may not accurately represent condylar movement. The aims of this investigation were to determine in human subjects, during open-close and excursive jaw movements, the movement patterns of arbitrary and anatomical condylar points; and whether the trajectory of a single selected point can accurately reflect the movement of the condyle. In 44 subjects, condylar point movements were recorded with an opto-electronic tracking system (JAWS3D), which recoded the position of three light-emitting diodes attached to each dental arch. The primary point, selected to represent movement of the condyle, was 15 mm medial to the palpated lateral condylar pole, parallel to the Frankfort horizontal plane. Additionally, four points were selected along orthogonal axes in the sagittal plane, and four in the horizontal plane: each was 5 mm from the primary point. In two subjects, the mandibular condyles were imaged by computerised tomography (CT) and the lateral and medial poles, most superior, anterior and posterior points of their condyles were selected. The trajectories of each point were compared for each subject for the mandibular movements listed above. Variability in both path form and dimension was noted between the subjects for all mandibular movements. For example, in an open-close mandibular movement the condylar point translation varied in the antero-posterior direction between 1.8-22.8 mm, and in the supero-inferior direction between 4.5-12.1 mm. For each subject, the pathway of each point was different in form and dimension from that subject’s other condylar points for the open-close, and ipsilateral lateral mandibular movements. For the open-close movement, in only four of the 44 subjects were the arbitrary point traces similar in form within a subject; and the tracings of each subject’s condylar points showed, on average, a 3.2 mm difference in maximal horizontal (i.e. antero-posterior) translation and 2.9 mm in maximal vertical (i.e. supereo-inferior) translation. For contralateral lateral mandibular movements, the path form and dimension in the sagittal plane of the condylar points were similar within a subject; however the lateral component showed variability in path length for the different points within a subject. The pathways of the condylar points for a protrusive movement displayed the most similarity within a subject, with an average of 0.4 mm variation in maximal horizontal or vertical displacement between each subject’s arbitrary condylar points’ tracings. The anatomical condylar points of the two subjects showed variability between and within each subject. For these two subjects the trajectories of the arbitrary condylar points moved in directions similar to the anatomical points of all movements except for the ipsilateral lateral mandibular movement, where in one subject, the arbitrary condylar points moved posteriorly, inferiorly and laterally whereas the anatomical points moved anteriorly, inferiorly and laterally. There is much variability in both form and dimension for mandibular condylar movement between human subjects. There is also considerable variability within subjects in the form and dimension of condylar point movement, whether arbitrary or anatomical, depending on the point selected. By inference therefore, a single condylar point cannot accurately reflect the movement of the mandibular condyle, except perhaps for a protrusive mandibular movement. Multiple mandibular points are therefore required to describe the motion of the condyle. In an ipsilateral lateral mandibular movement, for example, an arbitrary point may move in a completely different direction to the mandibular condyle, and so anatomically derived condylar points should be utilised to assess accurately condylar movement.

The purpose of this study was to characterize glenohumeral joint laxity and stiffness using instrumented arthrometry. To evaluate the validity of an instrumented measurement system we compared cutaneous and bone-pinned measures of laxity and stiffness that replicate previously reported in vivo methodology. Characterization of capsular laxity was achieved through determination of the sagittal plane translational area at increasing levels of quantified force. Finally, a method for increasing the objectivity of the standard manual laxity examination was developed for the orthopaedic clinician to quantify humeral head translation and capsular volume in vivo. We hypothesized that: 1) cutaneous measures could accurately predict bone-pinned measures, 2) capsular laxity would increase with increasing levels of applied force, and 3) manual cutaneous, manual bone-pinned, and force-displacement bone-pinned measures of translation would be equal. Thirty fresh frozen cadaveric shoulder specimens (mean age=70��14 years) were tested. The shoulders were thawed and mounted to a custom-made shoulder-testing apparatus. Displacement was measured using an electromagnetic tracking system. Sensors were secured cutaneously and with bone-pins to the scapula and humerus. Force-displacement testing was performed using a load applicator and manual displacement testing utilized the anterior/posterior drawer and inferior sulcus tests. A comparison of cutaneous and bone-pinned measures of laxity and stiffness revealed good to excellent criterion validity (r=0.68 to 0.79). Examination of displacement measures at increasing levels of force revealed increasing capsular laxity with symmetric directional compliance. No significant difference was observed between anterior and posterior translation (0.4 mm, p=.55), with significant differences between inferior and anterior (4.6 mm, p<.0001) and between inferior and posterior (5.1 mm, p<.0001). A comparison of manual cutaneous to bone-pinned manual and kinetic measures of translation revealed a significant difference between methods (p=.0024) and between directions (p<.0001) with no significant interaction (p=.0948). Estimations of the force required to achieve clinical end-point suggest that greater force is required in the anterior (173 N) direction compared to posterior (123 N) and inferior (121 N). We have developed two new methods to measure glenohumeral joint kinematics and reported new information regarding normal kinematics of the glenohumeral joint. / Graduation date: 2001

Master of Science / Most studies of condylar movement are based on the movement of an arbitrary condylar point. As the condyle is a 3-dimensional body which undergoes complex rotations and translations in function, the movement of one point in the vicinity of the condyle may not accurately represent condylar movement. The aims of this investigation were to determine in human subjects, during open-close and excursive jaw movements, the movement patterns of arbitrary and anatomical condylar points; and whether the trajectory of a single selected point can accurately reflect the movement of the condyle. In 44 subjects, condylar point movements were recorded with an opto-electronic tracking system (JAWS3D), which recoded the position of three light-emitting diodes attached to each dental arch. The primary point, selected to represent movement of the condyle, was 15 mm medial to the palpated lateral condylar pole, parallel to the Frankfort horizontal plane. Additionally, four points were selected along orthogonal axes in the sagittal plane, and four in the horizontal plane: each was 5 mm from the primary point. In two subjects, the mandibular condyles were imaged by computerised tomography (CT) and the lateral and medial poles, most superior, anterior and posterior points of their condyles were selected. The trajectories of each point were compared for each subject for the mandibular movements listed above. Variability in both path form and dimension was noted between the subjects for all mandibular movements. For example, in an open-close mandibular movement the condylar point translation varied in the antero-posterior direction between 1.8-22.8 mm, and in the supero-inferior direction between 4.5-12.1 mm. For each subject, the pathway of each point was different in form and dimension from that subject’s other condylar points for the open-close, and ipsilateral lateral mandibular movements. For the open-close movement, in only four of the 44 subjects were the arbitrary point traces similar in form within a subject; and the tracings of each subject’s condylar points showed, on average, a 3.2 mm difference in maximal horizontal (i.e. antero-posterior) translation and 2.9 mm in maximal vertical (i.e. supereo-inferior) translation. For contralateral lateral mandibular movements, the path form and dimension in the sagittal plane of the condylar points were similar within a subject; however the lateral component showed variability in path length for the different points within a subject. The pathways of the condylar points for a protrusive movement displayed the most similarity within a subject, with an average of 0.4 mm variation in maximal horizontal or vertical displacement between each subject’s arbitrary condylar points’ tracings. The anatomical condylar points of the two subjects showed variability between and within each subject. For these two subjects the trajectories of the arbitrary condylar points moved in directions similar to the anatomical points of all movements except for the ipsilateral lateral mandibular movement, where in one subject, the arbitrary condylar points moved posteriorly, inferiorly and laterally whereas the anatomical points moved anteriorly, inferiorly and laterally. There is much variability in both form and dimension for mandibular condylar movement between human subjects. There is also considerable variability within subjects in the form and dimension of condylar point movement, whether arbitrary or anatomical, depending on the point selected. By inference therefore, a single condylar point cannot accurately reflect the movement of the mandibular condyle, except perhaps for a protrusive mandibular movement. Multiple mandibular points are therefore required to describe the motion of the condyle. In an ipsilateral lateral mandibular movement, for example, an arbitrary point may move in a completely different direction to the mandibular condyle, and so anatomically derived condylar points should be utilised to assess accurately condylar movement.

Joint Position Sense (JPS) of the shoulder as determined by repeated repositioning tasks has been performed under different constrained testing conditions. The variability in the testing protocols for JPS testing of the gleno-humeral joint may incorporate different movement patterns, numbers of trials used to derived a specific JPS variable and range of motion. All of these aspects may play an important role in the assessment of G-H JPS testing. When using a new instrument for assessing JPS all of these issues need to be examined to document the optimal testing protocols for subsequent clinical assessments. By undertaking these studies future clinical trials may be more optimally assessed to determine if there are differences between dominant and non-dominant arms as well as the presence of JPS changes in performance associated with pathology and rehabilitation. This study used a 3-dimensional tracking system to examined gleno-humeral JPS using 2 open kinetic chain movement patterns. The 'conventional' 90 degree abducted, externally rotated movement was compared to the hypothetically more functional D2 movement pattern used in proprioceptive neuromuscular facilitatory techniques. These two patterns were tested at different ranges (low and high). Two cohorts (n=12, n=16) of normal healthy athletic males aged 17-35 years, performed matching tasks of both left and right arms. The second cohort (n=16) were assessed with and without strapping the gleno-humeral joint with sports tape. Accuracy (overall bias) and precision (variability) scores were determined for progressively greater numbers of trials. The findings of the study show that estimates of JPS accuracy and precision become more stable from data derived from 5 to 6 matching trials. There were no statistical differences between sides [95%CI ± 1.5cm]. The accuracy but not precision improved as subjects approximated the 'high' end of range in the 'conventional' or D2 pattern. Furthermore, no systematic differences were detected at different ranges of movement or movement patterns with or without the application of sports tape. These findings provide a guide to the number of trials that optimise the testing of the gleno-humeral joint and also suggest that in normal controls the magnitude of differences between sides and movement patterns is similar. These findings also iii indicate that sports tape applied to the shoulder may not significantly change the JPS performance in healthy, athletic males.

Function of the shoulder complex is highly dependent on the relationship between the scapula and the humerus. Etiologies for the disruption of the glenohumeral relationship include impaired or abnormal scapular function, motion, or position. The lateral scapular slide test (LSST) has been developed as a clinical tool to assess this phenomenon, also known as scapular dyskinesis. The primary purpose of this study was to determine the validity of the LSST by comparing the clinical measurements on the skin surface to the actual anatomical distance between the scapula and the spine as seen on radiographic images. The secondary purpose of this study was to determine the intra-rater and inter-rater reliability of the LSST. Nine subjects (18 shoulders) were assessed with the clinical LSST and radiographic images in three test positions (0��, 45��, and 90�� of glenohumeral abduction). Comparison of the clinical LSST measurements with the radiographs revealed the LSST to be valid (>0.80) in only the 0�� and 45�� test positions with respective Pearson correlation values of 0.91 and 0.98. Excellent (>0.75) intra-rater ICC (2,1) reliability (0.91-0.97) was found for all three test positions. Inter-rater ICC (2,1) reliability values were excellent for the 0�� (0.87) and 45�� (0.83) test positions, and fair to good for the 90�� position (0.71). This study demonstrated that the LSST is an accurate and consistent measure of scapular movement and position for the 0�� and 45�� test positions. Clinicians should exercise caution when interpreting measurements obtained at the 90�� test position because the validity and reliability values did not reach established standards. / Graduation date: 2002

Thesis (M.S.)--University of Tennessee Health Sciences Center, 2005.

The purpose of this study was to further investigate the relationship between patterns of shoulder movement and muscular response. Thirteen females were tested against maximal manual resistance in twelve different patterns, eight straight plane, and four diagonal. Five of the six subjects who met established kinematic criteria were used for electromyographic (EMG) analysis of the anterior deltoid (AD), the middle deltoid, the posteroir deltoid (PD), and the pectoralis major. No significant differences were found between number of muscles solicited or duration of muscular effort during the different movements. Maximal EMG was significantly higher for the AD in abduction and in flexion than in the other patterns, and for the PD in diagonal flexion with abduction and in transverse abduction.

Introdução: As mucopolissacaridoses (MPS) são doenças genéticas raras causadas pela atividade deficiente de enzimas lisossômicas que afetam o catabolismo de glicosaminoglicanos, o que leva ao seu acúmulo no organismo e a um quadro clínico multisistêmico. As manifestações clínicas geram limitações nas tarefas cotidianas. Objetivos: Avaliar a capacidade funcional e a amplitude de movimento articular (ADM), e o efeito da Terapia de Reposição Enzimática (TRE) em ambas as variáveis, em um grupo de pacientes com MPS acompanhados por um centro de referência em doenças lisossômicas do Hospital de Clínicas de Porto Alegre, Brasil. Métodos: Estudo prospectivo, longitudinal, com amostragem por conveniência. Utilizou-se o Pediatric Evaluation of Disability Inventory (PEDI) e a Medida de Independência Funcional (MIF) para avaliar funcionalidade, e a goniometria para avaliar ADM. Foram realizadas três avaliações em 0, 6 e 12 meses após inclusão no estudo (Momento 1, Momento 2 e Momento 3). Para fins de análise, os pacientes foram divididos em rês grupos: Grupo 1: pacientes sem TRE; Grupo 2: pacientes em TRE antes e após inclusão no estudo; Grupo 3: pacientes em TRE após inclusão no estudo. Resultados: 21 pacientes foram incluídos: Grupo 1=7 (MPS II, MPS III-B, MPS IV-A); Grupo 2=6 (MPS I; MPS IV) e Grupo 3=8 (MPS I, MPS II, VI), mediana de idade de 10,5 anos, 18,5 anos e 2 anos; e intervalo interquartil de 9-14,5 anos, 11,5-21,75 anos e 1,5-5 anos, respectivamente. Não houve diferença estatisticamente significativa entre os grupos para ADM. Encontrou-se diferença para a área de autocuidado do PEDI para o Grupo 3 (p=0,05), a melhora clínica na ADM foi observada somente para este grupo. No teste MIF o Grupo 2 apresentou melhores escores em todos os domínios avaliados. Houve correlação positiva entre a área de autocuidado do PEDI e flexão de punho (r=,718). Discussão/Conclusão: A TRE parece promover a manutenção da ADM e funcionalidade. No entanto, é difícil avaliar se isso decorre da TRE, da melhora clínica geral proporcionada pelo tratamento, ou da combinação destes fatores. A preservação da funcionalidade é um desafio no tratamento clínico destes pacientes e a manutenção do desempenho ocupacional deve ser definida como objetivo a ser alcançado. / Introduction: The mucopolysaccharidoses (MPS) are rare genetic disorders caused by a deficiency in lysosomal enzymes that affect the catabolism of glycosaminoglycans and cause their accumulation, resulting in a multisystemic clinical picture. Their clinical manifestations result in limitations to perform daily life tasks. Objectives: To evaluate functional capacity, joint range of motion (ROM), and the effect of enzyme replacement therapy (ERT) in both variables in patients with MPS followed at the reference center for lysosomal disorders at Hospital de Clínicas de Porto Alegre, Brazil. Methods: The present was a prospective, longitudinal study with convenience samples. The Pediatric Evaluation of Disability Inventory (PEDI) and the Functional Independence Measure (FIM) were used to evaluate functionality, and goniometry was used to evaluate ROM, at three moments (study allocation, and 6 and 12 months after study inclusion). For the analysis, three groups were formed, as follows: Group 1 (patients without ERT); Group 2 (patients on ERT before and after study inclusion), and Group 3 (patients that initiated ERT after study inclusion). Results: 21 patients were included: 7 in Group 1 (MPS II: 3, MPS III-B: 2, MPS IV-A: 2); 6 in Group 2 (MPS I: 3; MPS VI: 3), and 8 in Group 3 (MPS I: 3, MPS II: 4, MPS VI: 1). A statistically significant difference was found in the area of self-care of the PEDI for Group 3 (p=0,05), and clinical improvement in ROM was seen only in Group 3. Group 2 showed higher scores in all domains evaluated by the FIM. No statistically significant difference was found between the groups for ROM in the three moments evaluated. There was a positive correlation between the area of self-care of the PEDI and wrist flexion (r=0.718). Discussion/Conclusion: ERT seems to promote maintenance of ROM and functionality. However, it is difficult to evaluate whether or not this is due to ERT, to the general clinical improvement resulting from the treatment, or the combination of both. The preservation of functionality is an increasing challenge in the treatment of these patients, and maintenance of occupational performance should be defined as an objective to be reached by therapies used.

Introdução: As mucopolissacaridoses (MPS) são doenças genéticas raras causadas pela atividade deficiente de enzimas lisossômicas que afetam o catabolismo de glicosaminoglicanos, o que leva ao seu acúmulo no organismo e a um quadro clínico multisistêmico. As manifestações clínicas geram limitações nas tarefas cotidianas. Objetivos: Avaliar a capacidade funcional e a amplitude de movimento articular (ADM), e o efeito da Terapia de Reposição Enzimática (TRE) em ambas as variáveis, em um grupo de pacientes com MPS acompanhados por um centro de referência em doenças lisossômicas do Hospital de Clínicas de Porto Alegre, Brasil. Métodos: Estudo prospectivo, longitudinal, com amostragem por conveniência. Utilizou-se o Pediatric Evaluation of Disability Inventory (PEDI) e a Medida de Independência Funcional (MIF) para avaliar funcionalidade, e a goniometria para avaliar ADM. Foram realizadas três avaliações em 0, 6 e 12 meses após inclusão no estudo (Momento 1, Momento 2 e Momento 3). Para fins de análise, os pacientes foram divididos em rês grupos: Grupo 1: pacientes sem TRE; Grupo 2: pacientes em TRE antes e após inclusão no estudo; Grupo 3: pacientes em TRE após inclusão no estudo. Resultados: 21 pacientes foram incluídos: Grupo 1=7 (MPS II, MPS III-B, MPS IV-A); Grupo 2=6 (MPS I; MPS IV) e Grupo 3=8 (MPS I, MPS II, VI), mediana de idade de 10,5 anos, 18,5 anos e 2 anos; e intervalo interquartil de 9-14,5 anos, 11,5-21,75 anos e 1,5-5 anos, respectivamente. Não houve diferença estatisticamente significativa entre os grupos para ADM. Encontrou-se diferença para a área de autocuidado do PEDI para o Grupo 3 (p=0,05), a melhora clínica na ADM foi observada somente para este grupo. No teste MIF o Grupo 2 apresentou melhores escores em todos os domínios avaliados. Houve correlação positiva entre a área de autocuidado do PEDI e flexão de punho (r=,718). Discussão/Conclusão: A TRE parece promover a manutenção da ADM e funcionalidade. No entanto, é difícil avaliar se isso decorre da TRE, da melhora clínica geral proporcionada pelo tratamento, ou da combinação destes fatores. A preservação da funcionalidade é um desafio no tratamento clínico destes pacientes e a manutenção do desempenho ocupacional deve ser definida como objetivo a ser alcançado. / Introduction: The mucopolysaccharidoses (MPS) are rare genetic disorders caused by a deficiency in lysosomal enzymes that affect the catabolism of glycosaminoglycans and cause their accumulation, resulting in a multisystemic clinical picture. Their clinical manifestations result in limitations to perform daily life tasks. Objectives: To evaluate functional capacity, joint range of motion (ROM), and the effect of enzyme replacement therapy (ERT) in both variables in patients with MPS followed at the reference center for lysosomal disorders at Hospital de Clínicas de Porto Alegre, Brazil. Methods: The present was a prospective, longitudinal study with convenience samples. The Pediatric Evaluation of Disability Inventory (PEDI) and the Functional Independence Measure (FIM) were used to evaluate functionality, and goniometry was used to evaluate ROM, at three moments (study allocation, and 6 and 12 months after study inclusion). For the analysis, three groups were formed, as follows: Group 1 (patients without ERT); Group 2 (patients on ERT before and after study inclusion), and Group 3 (patients that initiated ERT after study inclusion). Results: 21 patients were included: 7 in Group 1 (MPS II: 3, MPS III-B: 2, MPS IV-A: 2); 6 in Group 2 (MPS I: 3; MPS VI: 3), and 8 in Group 3 (MPS I: 3, MPS II: 4, MPS VI: 1). A statistically significant difference was found in the area of self-care of the PEDI for Group 3 (p=0,05), and clinical improvement in ROM was seen only in Group 3. Group 2 showed higher scores in all domains evaluated by the FIM. No statistically significant difference was found between the groups for ROM in the three moments evaluated. There was a positive correlation between the area of self-care of the PEDI and wrist flexion (r=0.718). Discussion/Conclusion: ERT seems to promote maintenance of ROM and functionality. However, it is difficult to evaluate whether or not this is due to ERT, to the general clinical improvement resulting from the treatment, or the combination of both. The preservation of functionality is an increasing challenge in the treatment of these patients, and maintenance of occupational performance should be defined as an objective to be reached by therapies used.

Introdução: As mucopolissacaridoses (MPS) são doenças genéticas raras causadas pela atividade deficiente de enzimas lisossômicas que afetam o catabolismo de glicosaminoglicanos, o que leva ao seu acúmulo no organismo e a um quadro clínico multisistêmico. As manifestações clínicas geram limitações nas tarefas cotidianas. Objetivos: Avaliar a capacidade funcional e a amplitude de movimento articular (ADM), e o efeito da Terapia de Reposição Enzimática (TRE) em ambas as variáveis, em um grupo de pacientes com MPS acompanhados por um centro de referência em doenças lisossômicas do Hospital de Clínicas de Porto Alegre, Brasil. Métodos: Estudo prospectivo, longitudinal, com amostragem por conveniência. Utilizou-se o Pediatric Evaluation of Disability Inventory (PEDI) e a Medida de Independência Funcional (MIF) para avaliar funcionalidade, e a goniometria para avaliar ADM. Foram realizadas três avaliações em 0, 6 e 12 meses após inclusão no estudo (Momento 1, Momento 2 e Momento 3). Para fins de análise, os pacientes foram divididos em rês grupos: Grupo 1: pacientes sem TRE; Grupo 2: pacientes em TRE antes e após inclusão no estudo; Grupo 3: pacientes em TRE após inclusão no estudo. Resultados: 21 pacientes foram incluídos: Grupo 1=7 (MPS II, MPS III-B, MPS IV-A); Grupo 2=6 (MPS I; MPS IV) e Grupo 3=8 (MPS I, MPS II, VI), mediana de idade de 10,5 anos, 18,5 anos e 2 anos; e intervalo interquartil de 9-14,5 anos, 11,5-21,75 anos e 1,5-5 anos, respectivamente. Não houve diferença estatisticamente significativa entre os grupos para ADM. Encontrou-se diferença para a área de autocuidado do PEDI para o Grupo 3 (p=0,05), a melhora clínica na ADM foi observada somente para este grupo. No teste MIF o Grupo 2 apresentou melhores escores em todos os domínios avaliados. Houve correlação positiva entre a área de autocuidado do PEDI e flexão de punho (r=,718). Discussão/Conclusão: A TRE parece promover a manutenção da ADM e funcionalidade. No entanto, é difícil avaliar se isso decorre da TRE, da melhora clínica geral proporcionada pelo tratamento, ou da combinação destes fatores. A preservação da funcionalidade é um desafio no tratamento clínico destes pacientes e a manutenção do desempenho ocupacional deve ser definida como objetivo a ser alcançado. / Introduction: The mucopolysaccharidoses (MPS) are rare genetic disorders caused by a deficiency in lysosomal enzymes that affect the catabolism of glycosaminoglycans and cause their accumulation, resulting in a multisystemic clinical picture. Their clinical manifestations result in limitations to perform daily life tasks. Objectives: To evaluate functional capacity, joint range of motion (ROM), and the effect of enzyme replacement therapy (ERT) in both variables in patients with MPS followed at the reference center for lysosomal disorders at Hospital de Clínicas de Porto Alegre, Brazil. Methods: The present was a prospective, longitudinal study with convenience samples. The Pediatric Evaluation of Disability Inventory (PEDI) and the Functional Independence Measure (FIM) were used to evaluate functionality, and goniometry was used to evaluate ROM, at three moments (study allocation, and 6 and 12 months after study inclusion). For the analysis, three groups were formed, as follows: Group 1 (patients without ERT); Group 2 (patients on ERT before and after study inclusion), and Group 3 (patients that initiated ERT after study inclusion). Results: 21 patients were included: 7 in Group 1 (MPS II: 3, MPS III-B: 2, MPS IV-A: 2); 6 in Group 2 (MPS I: 3; MPS VI: 3), and 8 in Group 3 (MPS I: 3, MPS II: 4, MPS VI: 1). A statistically significant difference was found in the area of self-care of the PEDI for Group 3 (p=0,05), and clinical improvement in ROM was seen only in Group 3. Group 2 showed higher scores in all domains evaluated by the FIM. No statistically significant difference was found between the groups for ROM in the three moments evaluated. There was a positive correlation between the area of self-care of the PEDI and wrist flexion (r=0.718). Discussion/Conclusion: ERT seems to promote maintenance of ROM and functionality. However, it is difficult to evaluate whether or not this is due to ERT, to the general clinical improvement resulting from the treatment, or the combination of both. The preservation of functionality is an increasing challenge in the treatment of these patients, and maintenance of occupational performance should be defined as an objective to be reached by therapies used.