glycogen and Dermatomyositis

Topics: Dermatomyositis; Glycogen; Glycogen Storage Disease Type II; Humans; Liver

Topics: Delayed Diagnosis; Dermatomyositis; Erythema; Facial Dermatoses; Fatal Outcome; Female; Glycogen; Humans; Liver Neoplasms; Middle Aged; Muscle, Skeletal; Ovarian Neoplasms; Paraneoplastic Syndromes; Photosensitivity Disorders

We present a case of juvenile dermatomyositis with unusual histopathologic findings. The child presented with a course consistent with dermatomyositis, a diagnosis confirmed by finding reticulotubular aggregates in endothelial cells on electron microscopy. However, histopathology of his muscle biopsy revealed a striking pattern of glycogen accumulation, to an extent similar to that seen in glycogen storage diseases; this degree of accumulation could potentially confound histopathologic diagnosis.

Topics: Biopsy; Child, Preschool; Dermatomyositis; Diagnosis, Differential; Endothelium; Glycogen; Glycogen Storage Disease; Histocytochemistry; Humans; Male; Muscle, Skeletal; Reticular Formation

In 4 patients with clinical signs of dermatomyositis, confirmed by electromyography and muscle biopsy, a form of muscle fatigue was detected which was expressed clinically by predominantly proximal motor deficit, with phonation and deglutition disturbances, slightly influenced by prostigmine. In all patients, stimulation of the ulnar nerve at 3--10 Hz induced a decrement of muscle-evoked potentials in abductor digiti minimi and at 15--50 Hz an increment at the end of the trains (1.2 sec in duration) of repetitive stimulation (preceded in two cases by a decrement in the response to the fifth stimulus in the train). Stimulation at 30 Hz for 10 sec resulted in a transient facilitation, followed (at 3 Hz stimulation) by postactivation exhaustion which disappeared after 5--15 min. The post-tetanic facilitation, the incremental response and the myasthenic symptoms reverted to normal under treatment with corticosteroids, an immunosuppressor agent and guanidine hydrochloride. A mixed, pre- and postsynaptic mechanism is presumed to underlie the muscle fatigue in our patients. Electron microscopy of muscle biopsies disclosed zones of necrosis and, in incipient stages, large agglomerations of glycogen that had disorganized the structure of myofibrils. The end-plates in the biopsies were larger than normal and the cholinesterase reaction was hyperactive. Serum immunoelectrophoretic and electrophoretic data--increase of IgG and IgM, decrease of IgA and hypergammaglobulinaemia -- point to a possible autoimmune mechanism of the neuromuscular disorders in our patients.

Topics: Adolescent; Adult; Biopsy; Dermatomyositis; Electromyography; Evoked Potentials; Fatigue; Female; Glycogen; Guanidines; Humans; Male; Middle Aged; Muscle Contraction; Muscles; Myasthenia Gravis; Neostigmine; Prednisone; Synaptic Transmission; Ulnar Nerve

Topics: Adolescent; Amyotrophic Lateral Sclerosis; Animals; Biopsy; Cats; Dermatomyositis; Esterases; Female; Glucosyltransferases; Glycogen; Guinea Pigs; Histocytochemistry; Humans; Male; Muscular Atrophy; Muscular Diseases; Muscular Dystrophies; Myofibrils; Nervous System Diseases; Oxidoreductases; Paralysis; Rabbits; Schwann Cells; Staining and Labeling; Transferases; Tyrosine

Topics: Adult; Aged; Antibodies; Child; Culture Techniques; Dermatomyositis; Female; Glycogen; Humans; Lymphocyte Activation; Lymphocytes; Male; Metabolic Diseases; Middle Aged; Muscles; Muscular Atrophy; Muscular Diseases; Muscular Dystrophies

Topics: Adolescent; Adult; Aged; Child; Dermatomyositis; Glycogen; Glycolysis; Hexosephosphates; Humans; Lactates; Middle Aged; Motor Neurons; Muscles; Muscular Diseases; Muscular Dystrophies; Myasthenia Gravis; Myositis; Oculomotor Muscles; Phosphotransferases; Polyarteritis Nodosa