Proximal weakness

Juvenile dermatomyositis is an inflammatory disease of muscles, skin, and blood vessels of unknown cause affecting all age and ethnic groups, with a reported incidence of 1.9–4.1 per million. It manifests with weakness in axial and proximal muscles and typical skin lesions.

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Merosin-deficient congenital muscular dystrophy (MDCMD) is a progressive autosomal recessive disorder caused by the lack of expression of the α2-chain of laminin-211 glycoprotein. The defect results in skeletal muscle dysfunction with severe muscle weakness, hypotonia, proximal joint contractures, facial dysmorphism, and late or failed ambulation.

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Collagen type VI-related disorders consist of Ullrich congenital muscular dystrophies (UCMD) and Bethlem myopathy, in which these entities are at two opposite extremes of the phenotype continuum. Clinical characteristics include proximal joint contracture, distal joint hyperlaxity, generalized muscle weakness, normal cognitive function, and pulmonary insufficiency.

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Statin-induced immune-mediated necrotizing myopathy (IMNM) is a subtype of IMNM linked to exposure to statins and is characterized by positive anti-hydroxymethylglutaryl (HMG) coenzyme A reductase (HMGCR) antibodies. Although rare, this entity has become increasingly recognized as a cause of proximal muscle weakness, especially with the widespread use of statin therapy.

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This paper studies the proximal point algorithm for the class of generalized P0 variational inequalities. By using the upper semicontinuity result establishing the class of weakly univalent operators, we show that the iterative sequence generated by the algorithm is bounded, approaches to the solution set of the initial problem, and each of its accumulation points is a solution to the problem, provided that the solution set is bounded.

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Cushing’s syndrome consists of signs and symptoms related to prolonged exposure to high levels of glucocorticoid, and should be considered in individuals with the discriminatory signs and symptoms. Proximal myopathy is an important discriminatory sign.

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Lambert-Eaton myasthenic syndrome (LEMS) is a rare disorder of neuromuscular junction transmission in which the primary clinical manifestations are proximal muscle weakness and possible autonomic symptoms. We present a clinical case of LEMS with lung tumor diagnosed by clinical and electromyography.

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Dermatomyositis (DM) is a rare autoimmune disease characterized by symmetric proximal muscle weakness and skin rashes. Corticosteroids and methotrexate are mainly prescribed to treat dermatomyositis based on the consensus of experts.

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Dermatomyositis is an autoimmune myopathy characterized by proximal muscle weakness, muscle inflammation, and typical skin findings. It is a rare disease with an incidence of ~1/100 000. About 15–30 % of adult-onset cases are caused by underlying malignancy and dermatomyositis can be the first symptom of undiagnosed cancer, mainly in the case of anti-transcription intermediary factor 1γ (anti-TIF-1γ) antibodies presence.

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part 1 book “neurological clinical examination” has contents: the wasted hand, wrist drop, proximal weakness of the arm, proximal weakness of the leg, foot drop, ataxia and gait disturbance, facial palsy.

78p tieu_vu13 06-08-2018 33 2   Download

Oculopharyngeal muscular dystrophy (OPMD) is an autosomal dominant disease that usually manifests itself within the fifth decade. The most prom-inent symptoms are progressive ptosis, dysphagia, and proximal limb mus-cle weakness. The disorder is caused by trinucleotide (GCG) expansions in the N-terminal part of the poly(A)-binding protein 1 (PABPN1) that result in the extension of a 10-alanine segment by up to seven more alanines.

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Vasculitis of the nerve and muscle causes a painful symmetric or asymmetric distal sensorimotor neuropathy with variable proximal weakness. It predominantly affects elderly men and is associated with an elevated erythrocyte sedimentation rate and increased CSF protein concentration. SCLC and lymphoma are the primary tumors involved. Pathology demonstrates axonal degeneration and T cell infiltrates involving the small vessels of the nerve and muscle. Immunosuppressants (glucocorticoids and cyclophosphamide) often result in neurologic improvement.

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Neuromuscular Disease Patients with neuromuscular disease often have an abnormal gait, occasionally as a presenting feature. With distal weakness (peripheral neuropathy) the step height is increased to compensate for foot drop, and the sole of the foot may slap on the floor during weight acceptance. Neuropathy may be associated with a degree of sensory imbalance, as described above. Patients with myopathy or muscular dystrophy more typically exhibit proximal weakness. Weakness of the hip girdle may result in a degree of excess pelvic sway during locomotion.

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Pathogenesis Upper Motor Neuron Weakness This pattern of weakness results from disorders that affect the upper motor neurons or their axons in the cerebral cortex, subcortical white matter, internal capsule, brainstem, or spinal cord (Fig. 23-1). Such lesions produce weakness through decreased activation of the lower motor neurons. In general, distal muscle groups are affected more severely than proximal ones, and axial movements are spared unless the lesion is severe and bilateral.

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The subject of ingress on the coaxial transport (either accidental or intentional) is of the largest concern when considering the transportation of telephony services on a Hybrid Fiber Coax (HFC) system. With a well-maintained coax plant that is continually checked for compliance with FCC standards for cumulative leakage index, return path ingress should not present a problem. However, it must be assumed that no system is impervious to strong RF fields that are in close proximity to the telephony equipment.

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