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Biomedical subjects

W K Engel

Publications and source records attributed to W K Engel.

At least 235 records · Page 13Linked to original sources

Erythrocyte shape in Duchenne muscular dystrophy.

In a controlled and blind study we were not able to identify any abnormality of erythrocytes of eight patients with Duchenne muscular dystrophy compared with seven approximately age-matched unaffected siblings (six males, one female step-sibling). Erythrocyte morphology was found to be very sensitive to various types of cell treatment. At this time, erythrocyte morphology, evaluated by the techniques used, should not be considered an established diagnostic test for the Duchenne muscular dystrophy patient or carrier.

Adolescent↗

The acetylcholine receptor in normal and pathologic states. Immunoperoxidase visualization of alpha-bungarotoxin binding at a light and electron-microscopic level.

Acetylcholine receptor now can be visualized in the muscle sarcolemmal membrane with the use of an immunoperoxidase staining of alpha-bungarotoxin (alpha BT), a substance that binds specifically to the acetylcholine receptor. This technique has allowed new observations in various neuromuscular diseases in which the acetylcholine receptor is affected. In normal muscle, the acetylcholine receptor is confined to the neuromuscular junction. In both experimental denervation and human denervating illnesses, the acetylcholine receptor becomes present diffusely along the muscle sarcolemmal membrane in denervated fibers. In myasthenia gravis, a circulating factor that blocks alpha BT binding to the acetylcholine receptor of either normal neuromuscular junctions or denervated sarcolemmal membranes is present in 68 percent of serums tested.

Animals↗

Remote effects of focal cancer on the neuromuscular system.

The remote effects of cancer on the neuromuscular system include type II muscle fiber atrophy, dermatomyositis/polymyositis, myasthenia gravis, the facilitating myasthenic syndrome, peripheral neuropathy (including amyloid neuropathy), and possibly amyotrophic lateral sclerosis. The clinical and pathological findings and a number of possible pathokinetic mechanisms of these disorders are discussed. In none is the pathokinetic mechanism known. Hence, much work remains in therapeutically oriented research of the mechanisms in all of the remote effects of cancer on the neuromuscular system.

Amyotrophic Lateral Sclerosis↗

Iatrogenic muscle fibrosis. Arm levitation as an initial sign.

Three patients had iatrogenic muscle fibrosis, without weakness or sensory loss. Deltoid muscle fibrosis produced the unique clinical sign of gradual, involuntary, and irreducible arm levitation. The third patient had both levitated arms and levitated legs, a result of injections in the rectus femoris muscles. Repeated intramuscular injections apparently resulted in muscle fibrosis and intramuscular nerve-twig damage. Pentazocine (Talwin) is a particularly offending agent. One patient showed an unusual tissue reaction to foreign material, which may have played a role in her muscle fibrosis. Nevertheless, dramatic recovery of muscle function followed surgical sectioning of the fibrous deltoid bands.

Adult↗

Chick muscle in tissue culture: the ubiquity of viral infection.

In chick skeletal muscle fibers cultured from embryos of commercially obtained "normal" eggs we have demonstrated numerous C-particles and large amounts of avian leucosis/sarcoma envelope antigen, especially when cultured in the presence of dinitrophenol. C-particles were present in t-tubules, which were possible intracellular viaducts of infection or dissemination and perhaps were the loci of receptors of viral invasion of the cytoplasm and sites of egress. The abundant lace-like membraneous proliferations, probably of t-tubules, usually had C-particles adjacent to or within them and perhaps were virus-provoked. Questioned is the validity of using cultured muscle, or extract, of embryos from ordinary chicken eggs for analysing normal biological phenomena--or conversely, is viral influence "normal" in chick development?

Alpharetrovirus↗

Abundant nuclear rods in adult-onset rod disease.

Abundant, highly organized, rod-shaped particles have been found in skeletal-muscle nuclei of two patients with adult-onset rod disease. They were usually single in affected nuclei. Like myofibrillar rods, the nuclear rods consisted of bundles of long, parallel, apparently cross-linked filaments. On longitudinal section the rods had an axial periodicity and in transverse section a wire-mesh appearance. Average periodicities were 189A (nuclear) and 178A (myofibrillar) axially, 180A (both) transversely between the longitudinal rod-filaments on longitudinal-section and cross-section of the rods. Minor differences were that the nuclear rods were slightly lighter stained after osmium-uranyl acetate-lead citrate, lacked thin filaments protruding from their ends, and often were broader. It is proposed that nuclear rods may have a contractile-protein composition and pathokinesis similar to that of myofibrillar rods. Their formation may reflect an epitomization of a newly recognized general biological capability of exogenous, or perhaps endogeneous, nuclear protein alteration.

Adult↗