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Masticatory Myositis   637




            Masticatory Myositis                                                                   Client Education
                                                                                                          Sheet
  VetBooks.ir                                                                                                         Diseases and   Disorders
                                               HISTORY, CHIEF COMPLAINT
            BASIC INFORMATION
                                               •  Dysphagia/jaw pain              •  Tetanus
                                                                                  •  Disease of the temporomandibular joint or
           Definition                          •  Ptyalism                          facial bones
           Masticatory myositis (MM) is a focal inflam-  •  Abnormal facial appearance  ○   Trauma, neoplasia, osteopathy, foreign
           matory myopathy of autoimmune origin that   •  Weight loss                 body
           targets the unique muscle fibers (type 2M) of   •  Decreased activity/lethargy  •  Idiopathic inflammatory polymyopathy in
           the muscles of mastication.                                              Hungarian Vizsla
                                               PHYSICAL EXAM FINDINGS             •  Phenobarbital-responsive hypersalivation
           Synonyms                            Acute stage:                       Chronic disease: other causes of masticatory
           Masticatory muscle myositis (MMM), mastica-  •  Fever,  lymphadenopathy  (mandibular,   muscle atrophy
           tory myopathy, historically: eosinophilic myositis   prescapular)      •  Loss of trigeminal nerve function
           (acute stage), atrophic myositis (chronic stage)  •  Trismus             ○   Peripheral nerve sheath tumor, inflamma-
                                               •  Pain  on  palpation  and  attempted  forced   tion, or other neoplasia
           Epidemiology                         opening of the jaw
           SPECIES, AGE, SEX                   •  Swelling of the muscles of mastication  Initial Database
           •  Canine, all ages, either sex     •  Exophthalmos  ± blindness  ± decreased   •  Biochemistry  profile:  mild  elevations  in
           •  Feline rarely reported            pupillary light responses (PLRs)    muscle enzymes creatine kinase (CK),
                                               Chronic stage:                       alanine  aminotransferase  (ALT),  aspartate
           GENETICS, BREED PREDISPOSITION      •  Atrophy of the muscles of mastication  aminotransferase (AST) in the acute phase
           •  Large-breed, young adult (2-3 year old) to   •  Trismus               (less than elevations associated with general-
             middle-aged (4-9 year old) dogs   •  Enophthalmos                      ized polymyositis); hyperglobulinemia
           •  Overrepresented breeds: German shepherd,   •  Rarely, inability to close the mouth  •  CBC:  leukocytosis  with  neutrophilia  or
             Labrador retriever, Doberman pinscher, and                             eosinophilia
             golden retriever                  Etiology and Pathophysiology       •  Urinalysis: ± proteinuria
           •  Rottweilers  are  more  prone  to  peripheral   •  The  muscles  of  mastication  include  the   •  Muscle or lymph node aspirates can reveal lym-
             eosinophilia and eosinophilic  muscle   temporalis, masseter, pterygoid (medial and   phoplasmacytic or eosinophilic inflammation
             infiltrates due to their propensity for   lateral), and rostral digastricus innervated   •  Thorough head and jaw evaluation (sedation
             hypereosinophilic syndrome         by the mandibular branch of the trigeminal   may be required)
           •  Cavalier  King  Charles  spaniels:  possible   nerve that also innervates the tensor tympani   ○   Measure the extent of jaw opening.
             genetic  predisposition;  can  develop  signs   and tensor veli palatine muscles.  •  Skull radiographs under sedation to rule out
             of disease at a much younger age (12 weeks)  •  They  have  a  unique  embryologic  origin   trauma, neoplasia, or foreign body
                                                (brachial arch vs. paraxial mesoderm),
           ASSOCIATED DISORDERS                 myofiber types (2M vs. 2A), and distinct   Advanced or Confirmatory Testing
           MM has been documented to occur concur-  myosin isoform compared with limb muscles.  •  Serum 2M antibody: high specificity (100%)
           rently with myasthenia gravis, and masticatory   •  Autoantibodies are produced against mastica-  and sensitivity (85%-90%)
           muscle involvement can occur as an overlap   tory myosin-binding protein-C; may involve   ○   Negative  <  1:100,  borderline  1:100,
           syndrome with generalized polymyositis.  molecular mimicry with bacterial antigens;   positive > 1:100
                                                results in necrosis and phagocytosis with   ○   Previous glucocorticoid therapy or the
           Clinical Presentation                fibrotic infiltrate of the muscles    chronic form of the disease can result in
           DISEASE FORMS/SUBTYPES                                                     false-negative
           Signs occur bilaterally in the muscles of mas-   DIAGNOSIS             •  Electromyography (EMG)
           tication but are not always equal in severity                            ○   Fibrillation potentials; positive sharp
           or time of onset from one side to the other.   Diagnostic Overview         waves; bizarre, high-frequency discharges
           Dogs can often be weeks into the acute stage   Diagnosis is often based on clinical presenta-  in the acute phase
           of disease before owners present the dog for   tion and a positive serum 2M antibody titer.   ○   Decreased  insertional  activity  in  the
           diagnosis. Signs can wax and wane during this   However, results of this test can vary according   chronic phase
           time. Patients can present in the chronic stage   to the stage of disease (acute or chronic). It is   ○   Limb  muscles  should  also  be  tested  to
           without owner recognition of the acute phase.  important to consider a multimodal approach   differentiate generalized polymyositis
           Acute stage:                        to the diagnosis of this disease, including serum   ○   Muscle  enzyme levels should  be tested
           •  Trismus:  inability  to  open  the  jaw  (even   testing and biopsy exam.  prior  to  EMG  to  prevent  false  enzyme
             under anesthesia)                                                        elevations
           •  Myalgia:  pain  on  palpation  of  temporalis   Differential Diagnosis  •  Muscle biopsy
             and masseter muscles              Acute  disease:  other  causes  of  exophthalmos   ○   Commonly biopsied muscles include the
           •  Swelling of the muscles of mastication  or pain on jaw opening:         temporalis or masseter.
           •  Ocular                           •  Extraocular myositis (EOM)          ■   Care must be taken not to biopsy the
             ○   Exophthalmos  from  swelling  of  the   •  Retrobulbar abscess        thin frontalis muscle located superficial
               pterygoid muscles; can result in blindness   •  Generalized polymyositis  to the temporalis muscle.
               from stretching of the optic nerve if severe,   ○   Initially can present with only masticatory   ■   Important not to biopsy areas where
               and also exposure conjunctivitis   muscle involvement                   an EMG was performed
           Chronic stage:                      •  Infectious myopathy               ○   Necrosis and phagocytosis of muscle fibers
           •  Atrophy of the muscles of mastication  ○   Toxoplasma gondii, Neospora caninum, Bor-  ○   Perivascular infiltration of mononuclear
           •  Enophthalmos (differs from acute stage)  relia burgdorferi, Ehrlichia canis, Rickettsia   cells
           •  Permanent trismus                   rickettsii, Hepatozoon spp        ○   Perimysial and endomysial fibrosis

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