Page 317 - Color Atlas Of Pathophysiology (S Silbernagl Et Al, Thieme 2000)
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A.  Diseases of the Motor Unit
         Amyotrophic                Death of supra-  Hyperreflexia,
         lateral sclerosis          spinal neurons  spasticity
          Spinal                                           Paralysis
       1  muscular atrophy          Death of      Fasciculation
                                    α-motoneurons
             Poliomyelitis
              Autoimmune  α-motoneuron
          Metabolic                                                    I
          (B 1 , B 12  deficiency,        Slowing of conduction velocity,
          diabetes mellitus)              disorders of sensory functions
        2    Toxic                        (sensory loss, paresthesias)  Paralysis  and Muscles
             (lead, alcohol)
                                     Axon
              Genetic (Charcot-
              Marie-Tooth syndrome)    Muscle
          Autoimmune                                                   Motor Unit
          3   Genetic                              Fibrillations  Paralysis
        B.  Myotonias                  C.  Muscular Dystrophies
                                  1
        Action potential               Xp21          Sarcolemma
                          Repolarization  defect                       Plate 10.5
                                                     Muscle cell
         Na +      Intra-                                       Actin
                  cellular                           Dystrophin
         Ca 2+                                   Normal dystrophin molecule
        Postsynaptic  infolding  +  Cl –  Cl –  +  2

          K
                  Ca 2+        K         1        Defective dystrophin
                                                   Disordered gait
               Contraction    Relaxation  Nonfunctioning
       Muscle                           molecule
        cell  Depolarization  Repolarization
            normal        normal                    Becker’s dystrophy
                                                    milder course of disease
                                       Severe
       Action potential           2    muscle dystrophy
                       +
                      Na  channel myotonia,
                 Na +  congenital paramyotonia,  Respiratory
                      periodic hyperkalemic
                      paralysis         muscle
                 Ca 2+  Periodic hypokalemic
                      paralysis                      Lordosis
                                        failure,
                 Cl –  Congenital myotonia  Respiratory
                      (Thomsen, Becker)  heart failure
                 K +  Periodic hyperkalemic
                      paralysis                     Calf muscle
                                                    hypertrophy
             Halothane  Ca 2+  Malignant                          (Drawing by Duchenne)
                          hyperthermia  Duchenne’s dystrophy
                                          fatal course within         307
                                          20 years
       Silbernagl/Lang, Color Atlas of Pathophysiology © 2000 Thieme
       All rights reserved. Usage subject to terms and conditions of license.
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