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Path 27 PNS


One slow fat red ox Type 1 fiber
fast glycolytic enzyme rich fibers Type 2 fiber
weakness beginning in the distal limbs, rapidly advancing to affect prox muscle fnx (ascending paralysis). w inflamm and segmental demyelination of spinal n. roots and periph n. (radiculoneuropathy). immune mediated. preceding infx by Camp. jejuni. Guillan-Barre - high CSF prot
symmetric polyneuropathy affecting cool extremities. infx involves pain fibers w loss of sensation. large traumatic ulcers. Leprosy (Hansen Disease) - Mycobac. leprae
progressive muscle atrophy of leg below knee (peroneal muscular atrophy) and pes cavus. demyel/remyel with onion bulbs in distal nerves. degen post columns of spinal cord. autosom dominant Charcot-Marie-Tooth (Hereditary Motor/Sensory Neuropathy Type 1 [HMSN 1])
slowly progressive recessive disorder begins in early kids, delay in develop milestones. both trunk and limb muscle involved. enlarged periph n. (onion bulbs). Dejerine-Sottas Neuropathy (HMSN 3)
distal symmetric sensory/sensorimotor neuropathy, autonomic neuropath, and focal/multifocal asymm neuropath. decreased sense in distal extremities-> ulcers. Periph Neuropath in Adult-Onset DM
axonal degen->periph distal symmetric neuropath, maybe associated w muscle cramps, distal dyesthesias, decreased DTRs. uremic neuropath Neuropath d/t renal failure
Recessive motor neuron degen of infancy d/t homozygous deletions of SMN1. panfascicular atrophy->hypotonia (floppiness) Spinal Muscular Atrophy (Type 1=Werdnig-Hoffmann, Type 2, Type 3 [survive to adult])
Most severe and common form of musc dystrophy. X-linked mutant DMD gene (encodes dystrophin). at risk for cardomyopathy. weakness begins in young boys in pelvic girdle->shoulder girdle w enlarged weak legs (pseudohypertrophy). Duchenne Musc Dystrophy - Becker is the other one (much less common and severe)
sustained involuntary contraction of group of muscles. elicited by percussing thenar eminence. autosom dominant CTG repeat expansion affecting mRNA for DMPK. changes in intrafusal fibers of muscle spindles, fiber splitting, necrosis, and regen. Mytonic Dystrophy -child abnorm gait w weak foot dorsiflexors-> weak intrinsic and extensor hand muscles->ptosis/typical facial appearance. And, CATARACTS, balding, gonad atrophy, caridomyopath, decreased plasma IgG, abnorm glucose tolerance
muscle pain, tightness, and myoglobinuria following exercise d/t blocks in fatty acid oxidation and accumulation of lipid drops w/in muscle Lipid Myopathies
ragged red fibers, increased mitochondria w irreg shapes, parking lot inclusions. ophthalmoplegias and stuff Mitochondrial Myopathies
inflamm of skin and skel muscle. lilac discoloration of upper eyelids w periorbital edema. ducky red patches over knuckles, elbows, knees (Grotton lesions). proximal muscle weakness first. perifasicular atrophy. and capillary injury Dermatomyositis
symm prox muscle but lack of skin involvement. Adults. CD8+ Lymphos Polymyositis
begins w distal muscle involvement, may be asymm. >50 y/o. rimmed vacuoles. Immunosuppressive therapy not helpful Inclusion Body Myositis
immune mediated loss of AChR (autoantibods). Young adult women. thymic hyperplasia. Begins w ptosis and diplopia->generalized weakness. Myasthenia Gravis
often a paraneoplastic prob w small-cell carcinoma of lung. prox muscle weakness and autonom dysfnx but no improvement w anticholinesterase drugs. autoimmunity to Ca channels Lambert-Eaton Myasthenic Synd
Created by: Jakphooey