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Segmental distribution of muscle weakness in SMA III: implications for deterioration in muscle strength with time.

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We examined 26 spinal muscular atrophy type III (SMA III) patients with SMNt deletions, searching for possible segmental distribution of muscle weakness. In those with disease duration of < or = 11 years, the weakest muscles were upper lumbar innervated ones in the lower extremities. In the upper

Muscle weakness correlates with muscle atrophy and precedes the development of inclusion body or rimmed vacuoles in the mouse model of DMRV/hIBM.

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Distal myopathy with rimmed vacuoles (DMRV), also called hereditary inclusion body myopathy (hIBM), is characterized clinically by weakness and atrophy that initially involves the distal muscles and pathologically by the presence of rimmed vacuoles (RVs) or intracellular protein deposits in

POLG mutation in a patient with cataracts, early-onset distal muscle weakness and atrophy, ovarian dysgenesis and 3-methylglutaconic aciduria.

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Mutations in POLG account for one of the most frequent nuclear encoded causes of mitochondrial disorders to date. Individuals harboring POLG mutations exhibit fairly heterogeneous clinical presentations leading to increasing difficulties in classifying these patients into defined clinical

Histopathologic findings in the sacrocaudalis dorsalis medialis muscle of horses with vitamin E-responsive muscle atrophy and weakness.

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OBJECTIVE To characterize clinical findings, outcomes, muscle characteristics, and serum or muscle concentrations of α-tocopherol for horses with vitamin E-responsive signs of muscle atrophy and weakness consistent with signs of equine motor neuron disease (EMND). METHODS Retrospective case-control

Clinical and pathologic findings in two draft horses with progressive muscle atrophy, neuromuscular weakness, and abnormal gait characteristic of shivers syndrome.

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Two Belgian geldings, 4 and 14 years old, respectively, with muscle atrophy, weakness, and abnormal gait characteristic of severe advanced shivers were examined clinically and on necropsy. Neurologic examination revealed no evidence of ataxia, and the clinical diagnosis was neuromuscular weakness

Unilateral thigh atrophy and weakness in hip osteoarthritis.

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Osteoarthritis is a common condition that affects a significant percentage of people in the United States. The case and discussion will serve to introduce to the neurologist the issue of hip osteoarthritis causing thigh atrophy and weakness. The pathogeneses of muscular atrophy in the setting of

Diaphragmatic spinal muscular atrophy with bulbar weakness.

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We present the clinical and histopathological features of a child affected by diaphragmatic spinal muscular atrophy. The child was born with mild distal arthrogryposis, mild hypotonia and developed marked diaphragmatic and bulbar muscle weakness in the first week of life. Electrophysiological and

Respiratory muscle weakness associated with cerebellar atrophy.

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Transdiaphragmatic pressures were measured in 3 patients with cerebellar atrophy. Recordings were made during 3 types of voluntary maneuver--maximal sniffs, full inspirations, and maximal static inspiratory efforts, and during bilateral supramaximal phrenic nerve stimulation at 1 Hz. Although

The MRL/MpJ mouse strain is not protected from muscle atrophy and weakness after rotator cuff tear.

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Chronic rotator cuff tears are a common source of shoulder pain and disability. Patients with rotator cuff tears often have substantial weakness, fibrosis, and fat accumulation which limit successful surgical repair and postoperative rehabilitation. The Murphy Roths Large (MRL) strain of mice have

Association between muscle atrophy/weakness and health care costs and utilization among patients receiving total knee replacement surgery: a retrospective cohort study.

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OBJECTIVE The aim of the study reported here was to examine health care resource utilization, costs, and risk of rehospitalization for total knee replacement (TKR) patients with and without muscle atrophy/weakness (MAW). METHODS Individuals aged 50-64 years with commercial insurance or 65+ years

Clinical and economic characteristics of hip fracture patients with and without muscle atrophy/weakness in the United States.

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This retrospective analysis of hip fracture patients with and without muscle atrophy/weakness (MAW) revealed that those with MAW had significantly higher healthcare utilization and costs compared with hip fracture patients without MAW. OBJECTIVE Examine the demographics, clinical characteristics,

Muscle weakness and atrophy are associated with decreased regenerative capacity and changes in mTOR signaling in skeletal muscles of venerable (18-24-month-old) dystrophic mdx mice.

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The muscles of mdx mice progressively deteriorate with age. We wanted to know whether this is associated with a decrease in regenerative capacity and/or changes in the mammalian target of rapamycin complex (mTOR) signaling pathway. Muscles of mdx mice aged 5 weeks, 5, 12, and 18-24 months were

Dynamic MRI testing of the cervical spine has prognostic significance in patients with progressive upper-limb distal weakness and atrophy.

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BACKGROUND The syndrome of isolated progressive upper-limb distal weakness and atrophy results from disease processes affecting lower motor neurons originating in the cervical anterior horn gray matter. Lower motor neuron dysfunction restricted to the C7-T1 myotomes in the absence of neuropathy,

Demographics and clinical and economic characteristics of patients receiving total hip arthroplasty with and without muscle atrophy/weakness.

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This study analyzed administrative claims by a US population with commercial or Medicare supplemental insurance to compare demographics, comorbid medical conditions, and health care utilization and costs among patients undergoing total hip arthroplasty (THA) with and without muscle atrophy/weakness

[Two brothers with very late onset of muscle weakness in X-linked recessive spinal and bulbar muscular atrophy].

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Spinal and bulbar muscular atrophy (SBMA) is a motor neuron disease characterized by slowly progressive spinal and bulbar muscular atrophy associated with signs of androgen insensitivity including gynecomastia. This disease becomes prominent clinically in the fourth and fifth decades of life.

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