glycogen and Demyelinating-Diseases

After the long-term exposure to cadmium chloride in drinking water, the Wistar rats developed peripheral polyneuropathy. The main lesion was of myelin degeneration. Ultrastructural examination of the roots and sciatic nerves revealed segmental demyelination beginning from the node of Ranvier. There was the active autophagocytosis of Schwann cells which contained a number of myelin remnants and dense bodies. There was, on the other hand, the evidence of remyelination with toxic damage, in which the thinner myelin sheaths and abnormal myelinations were observed with increase of Schwann cells containing rich ribosomes. Axoplasmic changes were minimal, but consisted of accumulation of glycogen particles which very often produced glycogenosomes in characteristic appearance with axoplasmal dysfunction.

Topics: Animals; Axons; Cadmium Poisoning; Chronic Disease; Demyelinating Diseases; Glycogen; Microscopy, Electron; Myelin Sheath; Peripheral Nervous System Diseases; Phagocytosis; Ranvier's Nodes; Rats; Ribosomes; Schwann Cells; Sciatic Nerve; Spinal Nerve Roots

The authors describe in biopsies from 6 cases of Werdnig-Hoffmann disease, including 2 of the more benign type, the ultrastructural typical aspects of denervation. They compare their findings with those of other workers. The striking points are the great variation in the diameter of the muscle fibres and the myofibrils, the disorganisation of the myofibrils, the sarcomeres and the filaments, with persistance of the relations between thick and thin filaments at various levels, the modifications of the Z-band and the triads in chains. The folds and the basement membrane are examined. Centrioles are present in a muscle fibre and in a satellite. Glycogen is very abundant. The nerves seem normal but some Schwann cells contain pi granules which are not observed usually at the age of the patient. The end plates and a muscle spindle are normal.

Topics: Basement Membrane; Cell Membrane; Child, Preschool; Cytoplasmic Granules; Demyelinating Diseases; Female; Glycogen; Humans; Infant; Male; Motor Endplate; Motor Neurons; Muscles; Muscular Atrophy; Myofibrils; Nerve Degeneration; Peripheral Nerves; Sarcoplasmic Reticulum; Syndrome

Topics: Animals; Demyelinating Diseases; Glycogen; Microscopy, Electron; Myelin Sheath; Nerve Crush; Nerve Degeneration; Nerve Fibers, Myelinated; Neuroglia; Rats; Spinal Cord Injuries; Spinal Nerve Roots; Synaptic Vesicles

Topics: Acute Disease; Adolescent; Adult; Biopsy; Cytoplasm; Demyelinating Diseases; Female; Glycogen; Hodgkin Disease; Humans; Leukemia; Male; Microscopy; Microscopy, Electron; Middle Aged; Nerve Degeneration; Polyneuropathies; Schwann Cells; Sural Nerve; Vinblastine; Vinca Alkaloids; Vincristine

Topics: Adolescent; Adult; Aged; Child; Demyelinating Diseases; Extremities; Female; Glycogen; Humans; Lactates; Male; Middle Aged; Muscles; Muscular Atrophy; Muscular Dystrophies; Myotonia Congenita; Myotonic Dystrophy; Paralysis; Spinal Cord Diseases

Topics: Adult; Aphonia; Child; Child, Preschool; Chronaxy; Citric Acid Cycle; Demyelinating Diseases; Energy Metabolism; Female; Glycogen; Histocytochemistry; Humans; Infant; Leukodystrophy, Metachromatic; Male; Middle Aged; Muscles; Muscular Atrophy; Nerve Degeneration; Syndrome

Topics: Animals; Atrophy; Axons; Cytoplasm; Cytoplasmic Granules; Demyelinating Diseases; Female; Glycogen; Male; Medulla Oblongata; Membranes; Microscopy, Electron; Mitochondria; Mitochondrial Swelling; Rats; Vitamin E Deficiency

Topics: Action Potentials; Aged; Biopsy; Demyelinating Diseases; Female; Glycogen; Glycosaminoglycans; Histocytochemistry; Humans; Hypothyroidism; Male; Microscopy, Electron; Middle Aged; Neural Conduction; Peripheral Nerves; Polyneuropathies; Thyroid Hormones

Topics: Animals; Axons; Cytoplasm; Demyelinating Diseases; Glycogen; Goats; Histocytochemistry; Microscopy, Electron; Mitochondrial Swelling; Nerve Degeneration; Nerve Tissue; Neurons; Peripheral Nerves; Peripheral Nervous System Diseases; Plant Poisoning; Schwann Cells; Sciatic Nerve; Time Factors

Topics: Animals; Animals, Newborn; Cerebellum; Culture Techniques; Cytoplasmic Granules; Demyelinating Diseases; Glycogen; Histocytochemistry; Immune Sera; Microscopy; Microscopy, Electron; Multiple Sclerosis; Myelin Sheath; Nerve Degeneration; Neuroglia; Rats

Topics: Brain; Brain Edema; Carbon Dioxide; Cerebral Cortex; Cerebral Ventricles; Demyelinating Diseases; Encephalomalacia; Female; Glycogen; Histocytochemistry; Humans; Infant; Infant, Newborn; Male; Maple Syrup Urine Disease