The Experts below are selected from a list of 81 Experts worldwide ranked by ideXlab platform

Sunil Pradhan - One of the best experts on this subject based on the ideXlab platform.

  • Clinical study Shank sign in myotonic dystrophy type-1 (DM-1)
    2007
    Co-Authors: Sunil Pradhan
    Abstract:

    This study aimed to examine the specific shape of the shoulders and arms as a result of selective changes in muscle bulk, which could be diagnostic of myotonic dystrophy type-1 (DM-1). Thirty-two DM-1 patients from 18 families were asked to abduct their arms to about 90 with elbows flexed to 90 in such a manner that the hands were directed upwards. Examination from behind revealed a normal appearance of the shoulder Girdle and proximal upper arm musculature, and sudden tapering thereafter, due to wasting of biceps, triceps and forearm muscles. The upper Limbs looked like the shanks of an animal. ‘Shank sign’ was visible in 25 (78%) patients with DM-1 but not in any with DM-2 or other common muscular dystrophies such as Becker’s dystrophy, Limb-Girdle Syndrome or facioscapulohumeral dystrophy. Patients with positive signs included nine with very mild myotonia, suggesting its clinical utility in difficult cases. 2006 Elsevier Ltd. All rights reserved.

  • Shank sign in myotonic dystrophy type-1 (DM-1).
    Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia, 2006
    Co-Authors: Sunil Pradhan
    Abstract:

    This study aimed to examine the specific shape of the shoulders and arms as a result of selective changes in muscle bulk, which could be diagnostic of myotonic dystrophy type-1 (DM-1). Thirty-two DM-1 patients from 18 families were asked to abduct their arms to about 90 degrees with elbows flexed to 90 degrees in such a manner that the hands were directed upwards. Examination from behind revealed a normal appearance of the shoulder Girdle and proximal upper arm musculature, and sudden tapering thereafter, due to wasting of biceps, triceps and forearm muscles. The upper Limbs looked like the shanks of an animal. 'Shank sign' was visible in 25 (78%) patients with DM-1 but not in any with DM-2 or other common muscular dystrophies such as Becker's dystrophy, Limb-Girdle Syndrome or facioscapulohumeral dystrophy. Patients with positive signs included nine with very mild myotonia, suggesting its clinical utility in difficult cases.

D D Kilmer - One of the best experts on this subject based on the ideXlab platform.

  • Profiles of neuromuscular diseases. Design of the protocol.
    American journal of physical medicine & rehabilitation, 1995
    Co-Authors: W M Fowler, R T Abresch, S Aitkens, G T Carter, E R Johnson, D D Kilmer, M A Mccrory, N C Wright
    Abstract:

    The purpose of this 10-yr investigation was to develop comprehensive impairment and disability profiles of the clinical characteristics of seven neuromuscular diseases: spinal muscular atrophy, hereditary motor sensory neuropathy, Duchenne muscular dystrophy, Becker's muscular dystrophy, facioscapulohumeral muscular dystrophy, Limb-Girdle Syndrome, and myotonic dystrophy. Based on the World Health Organization's classification of disablement, as applied to neuromuscular diseases, impairment was evaluated by measurements of strength, range of motion, spine deformity, cardiac and pulmonary function, and intellectual capacity. Disability was evaluated by measures of mobility and upper extremity function, cardiopulmonary adaptations, cardiac and pulmonary complications, and psychosocial adjustment.

  • Profiles of neuromuscular diseases. Limb-Girdle Syndromes.
    American journal of physical medicine & rehabilitation, 1995
    Co-Authors: C M Mcdonald, W M Fowler, R T Abresch, G T Carter, E R Johnson, D D Kilmer
    Abstract:

    Sixty-six individuals with Limb-Girdle Syndrome (LGS) were evaluated over a 10-yr period and classified into three types: 19 severe autosomal recessive muscular dystrophy of childhood (ARMDC), alternatively referred to by some as SCARMD, 18 autosomal dominant late onset (ADLO), and 29 pelvifemoral (PF) individuals. ARMDC subjects showed the greatest weakness, 2.5 +/- 1.0, mean Manual Muscle Test (MMT) grade for all muscles combined, and the only significant progression of loss of strength, -0.59 MMT unit decline per decade. Strength loss in ADLO and PF types was about the same, 3.7 +/- 0.7 and 4.0 +/- 0.7 grades, respectively. Quantitative strength measurements in ADLO and PF types were more sensitive than MMTs, showing losses of 30-40% strength in muscle groups with MMT grades of 4 or higher. All three types showed greater proximal and lower extremity weakness but usually no difference between flexor and extensor strength. There was a high percentage (44%) of mild very slowly progressive scoliosis in ARMDC, but spine deformity was unusual in ADLO and PF (11%) LGS. Contractures were few, slowly progressive, and usually mild in severity in all types, although more frequent in ARMDC. There also was a low frequency of severe restrictive lung disease in all types (10%) but a high percentage of electrocardiogram abnormalities (62-73%). The most common electrocardiogram abnormalities were increased R/S ratio in V1 and infranodal conduction defects. Intellectual and cognitive functions were within normal limits. Mobility and extremity function reflected the strength differences between the ARMDC and other types of LGS. Eight-five percent of ARMDC individuals relied on a wheelchair for all or part of their mobility, and all were unable to complete timed motor performance tests within the 99th percentile range for controls.

R T Abresch - One of the best experts on this subject based on the ideXlab platform.

  • Employment profiles in neuromuscular diseases.
    American journal of physical medicine & rehabilitation, 1997
    Co-Authors: William M. Fowler, R T Abresch, Todd R. Koch, Mary Louise Brewer, Russell K. Bowden, Richard L. Wanlass
    Abstract:

    Consumer and rehabilitation provider factors that might limit employment opportunities for 154 individuals with six slowly progressive neuromuscular diseases (NMD) were investigated. The NMDs were spinal muscular atrophy (SMA), hereditary motor sensory neuropathy (HMSN), Becker's muscular dystrophy (BMD), facioscapulohumeral muscular dystrophy (FSHD), myotonic muscular dystrophy (MMD), and Limb-Girdle Syndrome (LGS). Forty percent were employed in the competitive labor market at the time of the study, 50% had been employed in the past, and 10% had never been employed. The major consumer barrier to employment was education. Other important factors were type of occupation, intellectual capacity, psychosocial adjustment, and the belief by most individuals that their physical disability was the only or major barrier to obtaining a job. Psychological characteristics were associated with level of unemployment. However, physical impairment and disability were not associated with level of unemployment. There also were differences among the types of NMDs. Compared with the SMA, HMSN, BMD, and FSHD groups, the MMD and LGS groups had significantly higher levels of unemployment, lower educational levels, and fewer employed professional, management, and technical workers. Nonphysical impairment factors such as a low percentage of college graduates, impaired intellectual function in some individuals, and poor psychological adjustment were correlated with higher unemployment levels in the MMD group. Unemployment in the LGS group was correlated with a failure to complete high school. Major provider barriers to employment were the low level of referrals to Department of Rehabilitation by physicians and the low percentage of acceptance into the State Department of Rehabilitation. The low rate of acceptance was primarily attributable to the low number of referrals compounded by a lack of counselor experience with individuals with NMD. Both consumer and provider barriers may contribute to the lack of interest in obtaining a job.

  • Profiles of neuromuscular diseases. Design of the protocol.
    American journal of physical medicine & rehabilitation, 1995
    Co-Authors: W M Fowler, R T Abresch, S Aitkens, G T Carter, E R Johnson, D D Kilmer, M A Mccrory, N C Wright
    Abstract:

    The purpose of this 10-yr investigation was to develop comprehensive impairment and disability profiles of the clinical characteristics of seven neuromuscular diseases: spinal muscular atrophy, hereditary motor sensory neuropathy, Duchenne muscular dystrophy, Becker's muscular dystrophy, facioscapulohumeral muscular dystrophy, Limb-Girdle Syndrome, and myotonic dystrophy. Based on the World Health Organization's classification of disablement, as applied to neuromuscular diseases, impairment was evaluated by measurements of strength, range of motion, spine deformity, cardiac and pulmonary function, and intellectual capacity. Disability was evaluated by measures of mobility and upper extremity function, cardiopulmonary adaptations, cardiac and pulmonary complications, and psychosocial adjustment.

  • Profiles of neuromuscular diseases. Limb-Girdle Syndromes.
    American journal of physical medicine & rehabilitation, 1995
    Co-Authors: C M Mcdonald, W M Fowler, R T Abresch, G T Carter, E R Johnson, D D Kilmer
    Abstract:

    Sixty-six individuals with Limb-Girdle Syndrome (LGS) were evaluated over a 10-yr period and classified into three types: 19 severe autosomal recessive muscular dystrophy of childhood (ARMDC), alternatively referred to by some as SCARMD, 18 autosomal dominant late onset (ADLO), and 29 pelvifemoral (PF) individuals. ARMDC subjects showed the greatest weakness, 2.5 +/- 1.0, mean Manual Muscle Test (MMT) grade for all muscles combined, and the only significant progression of loss of strength, -0.59 MMT unit decline per decade. Strength loss in ADLO and PF types was about the same, 3.7 +/- 0.7 and 4.0 +/- 0.7 grades, respectively. Quantitative strength measurements in ADLO and PF types were more sensitive than MMTs, showing losses of 30-40% strength in muscle groups with MMT grades of 4 or higher. All three types showed greater proximal and lower extremity weakness but usually no difference between flexor and extensor strength. There was a high percentage (44%) of mild very slowly progressive scoliosis in ARMDC, but spine deformity was unusual in ADLO and PF (11%) LGS. Contractures were few, slowly progressive, and usually mild in severity in all types, although more frequent in ARMDC. There also was a low frequency of severe restrictive lung disease in all types (10%) but a high percentage of electrocardiogram abnormalities (62-73%). The most common electrocardiogram abnormalities were increased R/S ratio in V1 and infranodal conduction defects. Intellectual and cognitive functions were within normal limits. Mobility and extremity function reflected the strength differences between the ARMDC and other types of LGS. Eight-five percent of ARMDC individuals relied on a wheelchair for all or part of their mobility, and all were unable to complete timed motor performance tests within the 99th percentile range for controls.

W M Fowler - One of the best experts on this subject based on the ideXlab platform.

  • Profiles of neuromuscular diseases. Design of the protocol.
    American journal of physical medicine & rehabilitation, 1995
    Co-Authors: W M Fowler, R T Abresch, S Aitkens, G T Carter, E R Johnson, D D Kilmer, M A Mccrory, N C Wright
    Abstract:

    The purpose of this 10-yr investigation was to develop comprehensive impairment and disability profiles of the clinical characteristics of seven neuromuscular diseases: spinal muscular atrophy, hereditary motor sensory neuropathy, Duchenne muscular dystrophy, Becker's muscular dystrophy, facioscapulohumeral muscular dystrophy, Limb-Girdle Syndrome, and myotonic dystrophy. Based on the World Health Organization's classification of disablement, as applied to neuromuscular diseases, impairment was evaluated by measurements of strength, range of motion, spine deformity, cardiac and pulmonary function, and intellectual capacity. Disability was evaluated by measures of mobility and upper extremity function, cardiopulmonary adaptations, cardiac and pulmonary complications, and psychosocial adjustment.

  • Profiles of neuromuscular diseases. Limb-Girdle Syndromes.
    American journal of physical medicine & rehabilitation, 1995
    Co-Authors: C M Mcdonald, W M Fowler, R T Abresch, G T Carter, E R Johnson, D D Kilmer
    Abstract:

    Sixty-six individuals with Limb-Girdle Syndrome (LGS) were evaluated over a 10-yr period and classified into three types: 19 severe autosomal recessive muscular dystrophy of childhood (ARMDC), alternatively referred to by some as SCARMD, 18 autosomal dominant late onset (ADLO), and 29 pelvifemoral (PF) individuals. ARMDC subjects showed the greatest weakness, 2.5 +/- 1.0, mean Manual Muscle Test (MMT) grade for all muscles combined, and the only significant progression of loss of strength, -0.59 MMT unit decline per decade. Strength loss in ADLO and PF types was about the same, 3.7 +/- 0.7 and 4.0 +/- 0.7 grades, respectively. Quantitative strength measurements in ADLO and PF types were more sensitive than MMTs, showing losses of 30-40% strength in muscle groups with MMT grades of 4 or higher. All three types showed greater proximal and lower extremity weakness but usually no difference between flexor and extensor strength. There was a high percentage (44%) of mild very slowly progressive scoliosis in ARMDC, but spine deformity was unusual in ADLO and PF (11%) LGS. Contractures were few, slowly progressive, and usually mild in severity in all types, although more frequent in ARMDC. There also was a low frequency of severe restrictive lung disease in all types (10%) but a high percentage of electrocardiogram abnormalities (62-73%). The most common electrocardiogram abnormalities were increased R/S ratio in V1 and infranodal conduction defects. Intellectual and cognitive functions were within normal limits. Mobility and extremity function reflected the strength differences between the ARMDC and other types of LGS. Eight-five percent of ARMDC individuals relied on a wheelchair for all or part of their mobility, and all were unable to complete timed motor performance tests within the 99th percentile range for controls.

E R Johnson - One of the best experts on this subject based on the ideXlab platform.

  • Profiles of neuromuscular diseases. Design of the protocol.
    American journal of physical medicine & rehabilitation, 1995
    Co-Authors: W M Fowler, R T Abresch, S Aitkens, G T Carter, E R Johnson, D D Kilmer, M A Mccrory, N C Wright
    Abstract:

    The purpose of this 10-yr investigation was to develop comprehensive impairment and disability profiles of the clinical characteristics of seven neuromuscular diseases: spinal muscular atrophy, hereditary motor sensory neuropathy, Duchenne muscular dystrophy, Becker's muscular dystrophy, facioscapulohumeral muscular dystrophy, Limb-Girdle Syndrome, and myotonic dystrophy. Based on the World Health Organization's classification of disablement, as applied to neuromuscular diseases, impairment was evaluated by measurements of strength, range of motion, spine deformity, cardiac and pulmonary function, and intellectual capacity. Disability was evaluated by measures of mobility and upper extremity function, cardiopulmonary adaptations, cardiac and pulmonary complications, and psychosocial adjustment.

  • Profiles of neuromuscular diseases. Limb-Girdle Syndromes.
    American journal of physical medicine & rehabilitation, 1995
    Co-Authors: C M Mcdonald, W M Fowler, R T Abresch, G T Carter, E R Johnson, D D Kilmer
    Abstract:

    Sixty-six individuals with Limb-Girdle Syndrome (LGS) were evaluated over a 10-yr period and classified into three types: 19 severe autosomal recessive muscular dystrophy of childhood (ARMDC), alternatively referred to by some as SCARMD, 18 autosomal dominant late onset (ADLO), and 29 pelvifemoral (PF) individuals. ARMDC subjects showed the greatest weakness, 2.5 +/- 1.0, mean Manual Muscle Test (MMT) grade for all muscles combined, and the only significant progression of loss of strength, -0.59 MMT unit decline per decade. Strength loss in ADLO and PF types was about the same, 3.7 +/- 0.7 and 4.0 +/- 0.7 grades, respectively. Quantitative strength measurements in ADLO and PF types were more sensitive than MMTs, showing losses of 30-40% strength in muscle groups with MMT grades of 4 or higher. All three types showed greater proximal and lower extremity weakness but usually no difference between flexor and extensor strength. There was a high percentage (44%) of mild very slowly progressive scoliosis in ARMDC, but spine deformity was unusual in ADLO and PF (11%) LGS. Contractures were few, slowly progressive, and usually mild in severity in all types, although more frequent in ARMDC. There also was a low frequency of severe restrictive lung disease in all types (10%) but a high percentage of electrocardiogram abnormalities (62-73%). The most common electrocardiogram abnormalities were increased R/S ratio in V1 and infranodal conduction defects. Intellectual and cognitive functions were within normal limits. Mobility and extremity function reflected the strength differences between the ARMDC and other types of LGS. Eight-five percent of ARMDC individuals relied on a wheelchair for all or part of their mobility, and all were unable to complete timed motor performance tests within the 99th percentile range for controls.