Masticatory Muscle Myositis in
the Cavalier King Charles Spaniel


What It Is

Masticatory muscle myositis (MMM) is a neuromuscular disease in which the muscles used by the dog to chew – the jaw and temporal muscles, which are called masticatory muscles – are inflamed, and it is painful and difficult or impossible for the dog to open its mouth.

MMM is an immune-mediated disease which is reportedly hereditary in the cavalier King Charles spaniel. Other breeds in which MMM is most common are German shepherds, Labrador retrievers, Doberman pinschers, and golden retrievers.

“Myositis” is a general term for inflammation of the muscles. “Immune-mediated” diseases are conditions in which the immune system may over-react or start attacking the body. MMM has also been known as “eosinophilic myositis” or “atrophic myositis”. The inability to open the mouth is called “trismus”.

This condition is limited to the masticatory muscles because they have a molecular structure, called 2M muscle fibers, which are found nowhere else in the dog’s body. Masticatory myositis results when the immune system’s antibodies specifically target these 2M muscle fibers.

The disorder is known to have an early onset in cavalier King Charles spaniels, having occurred in cavalier littermates as young as twelve weeks of age (see 2007 study report), and several other CKCSs before six months. The reported typical age of onset is about three years

In a 2005 study, Dr. Diane Shelton, board certified in veterinary internal medicine, concluded:

"In a search of the database of the Comparative Neuromuscular Laboratory over the years 2001-2005, masticatory muscle myositis was confirmed in 11 young Cavalier King Charles Spaniels with onset at less than 6 months of age. In several cases, onset followed within 10 days of vaccinations. Although long-term information is not yet complete, there was resolution of clinical signs of masticatory muscle myositis in most cases. Of interest, other complications such as early onset hypothyroidism and allergies were reported. Additional information will be provided as more is learned about this early-onset masticatory muscle myositis in this breed."

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Symptoms

Masticatory muscle myositis may start suddenly or appear gradually. The main clinical sign is difficulty in opening the mouth (called “trismus”). In the initial (acute) stage, there may also be swelling of the jaw and temporal muscles, pain in the jaw, and bulging eyes due to the swollen muscles behind them. There may be a fever and a swollen local lymph node. The dog will seem to in pain when he tries to open his mouth or attempts to chew and will be reluctant to eat.

Cavalier's jaw barely opened under anesthesiaIf MMM is not noticed early enough, it develops into the chronic stage, in which damaged muscle fibers are replaced with fibrotic connective tissue which results in further restriction of opening the mouth. In the chronic stage, there is noticeable atrophy of the masticatory muscles and inability to open the mouth due to the fibrosis. Instead of appearing swollen, the dog’s jaw and temple muscles will hollow. The fibrosis can become so severe that the mouth cannot be opened, even under general anesthesia (see cavalier's jaw barely opened, at right) – a very serious form of lockjaw. Unfortunately, many owners do not recognize that their dogs are having a problem until they reach the chronic phase.

Some mouth symptoms are similar to those of muscular dystrophy, another neuromuscular disease affecting the mouth muscles.

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Diagnosis

You may expect your cavalier to endure a battery of diagnostic tests, including complete physical and neurologic examinations, general and specialized blood tests – there is a unique blood test for masticatory muscle myositis called the “2M antibody” test (see box) – as well as a muscle biopsy, x-rays, urinalysis, and electromyography.

The Most Accurate Blood Test for MMM

The 2M antibody blood test detects circulating antibodies against masticatory muscles’ 2M fibers. Only the masticatory muscles have the molecular structure called 2M fibers. Masticatory myositis results when the immune system’s antibodies specifically target these 2M fibers.

The 2M antibody test is an “immunocytochemical” test, in which positives for MMM reportedly have been proven to be 100% accurate, and false negatives for MMM have occurred only 10% to 15% of the time.

USDA Comparative Neuromuscular LaboratoryThe test is conducted by the Comparative Neuromuscular Laboratory at the University of California, San Diego. Serum samples should be sent to the laboratory; its turn around time for reporting results is five to seven week days. The laboratory’s director is Dr. G. Diane Shelton, ACVIM board certified in internal medicine. Contact information is on this website: Comparative Neuromuscular Laboratory.

The purpose of some of the tests is to rule out other possible causes of the symptoms. For example, the physical and neurologic examinations are to confirm that clinical signs are limited to the masticatory muscles. Polymyositis is a more generalized muscle inflammation of the masticatory muscles and other muscles but otherwise is difficult to distinguish from masticatory myositis. Other possible causes of such symptoms include temporomandibular joint disorders, and endocrine disorders, such as Cushing's syndrome and hypothyroidism.

General anesthesia may be called for to get the dog to open its mouth to check for other possible causes of the pain, such as broken teeth or bones or a dislocated jaw. In advanced chronic cases, the dog’s jaws may not open even under anesthesia.

In a September 2013 journal article, veterinary researchers reported that magnetic resonance imaging (MRI) scanning detected "widespread, symmetrical, and inhomogeneously hyperintense areas in the masticatory muscle", enabling early diagnosis and therapeutic treatment."

If the dog’s jaw or temporal muscles have started to atrophy (a symptom in the chronic stage of MMM), the examining veterinarian should find out if the dog has been treated with lengthy (seven days or longer) corticosteroid therapy for another disorder, since corticosteroids have been known to cause these muscles to atrophy.

The owner should tell the veterinarian about any recent vaccinations of the dog. Dr. Diane Shelton, board certified in veterinary internal medicine, recently reported that several young cavaliers developed MMM within ten days of vaccinations.

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Treatment

Tricolor PupIt is important to begin therapy of the MMM-affected dog as early as possible, so that the acute stage does not progress to the chronic stage.  However, treatment should not begin before thorough testing and diagnosis.

The standard course of treatment consists of heavy, lengthy doses of corticosteroids, such as prednisone, until the dog’s jaw seems to open normally. Then, the doses may be lowered gradually over six to nine months. If the medication is discontinued too early, the dog may be expected to relapse. So, sufficient dosages of corticosteroids need to be given and for a sufficient period of time. MMM is known to respond well initially to corticosteroid therapy, but a relapse usually will occur if the treatment is discontinued prematurely. In some cases, the drug cannot ever be completely stopped.

Prednisone is a synthetic corticosteroid which serves as an “immunosuppressant” intended to suppress the dog’s immune system. Prednisone may have some harmful side effects. Patients on long term prednisone will drink and urinate excessively. For dogs not able to tolerate the side effects of corticosteroids, another immunosuppressive drug, azathioprine (Azasan, Imuran, Azamun, Imurel) may be prescribed in addition to corticosteroid treatment.

Other drugs which have been considered for treatment of MMM include dexamethasone, which is approximately ten times stronger than prednisone, and cyclosporine, which is another immunosuppressive drug, and colchicine, which is used for its anti-fibrotic properties in liver disease.

The dog may require a semi-liquid diet during initial recovery. Its mouth should not be forced open, but the dog should be encouraged to chew its toys, as a form of physical therapy.

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Prognosis

Prognosis of recovery from masticatory muscle myositis is determined by the degree of fibrosis present and the dog’s response to treatment. The prognosis is good if: (a) MMM is detected early, in the initial “acute” stage; and (b) the treatment is appropriate, meaning mainly aggressive immunosuppressive therapy comprised of high doses of medication given long enough to avoid a relapse. “Good” prognosis means that a full or significant partial range of jaw motion is regained.

Dogs which are not treated until they are in the “chronic” stage of masticatory muscle myositis may be expected to have a less favorable prognosis.

However, with MMM, the treatment may turn out to be worse than the disease itself.  Some dogs cannot tolerate the prescribed drugs, particularly the corticosteroids. At least one cavalier being treated for MMM is known to have suffered a slow death from the medications.*

*Read Butter's experience with MMM and its treatment.

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What You Can Do

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Current Research

August 2013: Italian researchers find MRI can detect early masticatory muscle myositis. In a September 2013 journal article, a team of Italian veterinary researchers, Alberto Cauduro, Favole Paolo, Roberto M. Asperio, Valeria Rossini, Maurizio Dondi, Lucia A. Simonetto, Carlo Cantile, and Valentina Lorenzo, report that MRIs detected "widespread, symmetrical, and inhomogeneously hyperintense areas in the masticatory muscle", enabling early diagnosis and therapeutic treatment."

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Related Links

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Veterinary Resources

Canine muscle fiber types and susceptibility of masticatory muscles to myositis. Julia Stephens Orvis, Dr. George H. Cardinet III. Muscle & Nerve 1981; 4(4):354-359.

Fiber type-specific autoantibodies in a dog with eosinophilic myositis. Dr. G. Diane Shelton, Dr. George H. Cardinet III, Dr. Everett Bandman, Dr. Paul Cuddon. Muscle & Nerve 1985; 8(9): 783-790.

Canine masticatory muscle disorders: A study of 29 cases. Dr. G. Diane Shelton, Dr. George H. Cardinet III, Dr. Everett Bandman. Muscle & Nerve 1987; 10(8):753-766.

Masticatory myopathy in the dog: a retrospective study of 18 cases. Gilmour MA, Morgan RV, Moore FM. J Am Anim Hosp Assoc. Jul/Aug 1992; 28(4):300-306.

An Investigation into the Cause and Most Successful Treatment of a Group of Symptoms, Particularly a Stiff Jaw and Swelling Head, in 32 Cases of CKCS. Vera Darby, Anne East. Copyright Vera A. Darby 1995.

Masticatory Muscle Myositis: Pathogenesis, Diagnosis, and Treatment. Caeley Melmed, G. Diane Shelton, Robert Bergman, Claudia Barton. Compendium. Aug. 2004. Quote: "Cavalier King Charles spaniels appear to have a genetic predisposition to masticatory muscle myositis."

Canine Inflammatory Myopathies: A Clinicopathologic Review of 200 Cases. Jason Evans, Donald Levesque, and G. Diane Shelton. J Vet Intern Med 2004;18:679–691.

Masticatory muscle myositis in a young male Cavalier King Charles Spaniel Masticatory Muscle Myositis in Young Male CKCSDr. Dana Graham. Comparative Neuromuscular Laboratory at the University of California, San Diego; July 2005. Quote: "The pup was born 3/15/05. An initial DHPP vaccination was given by the breeder, and booster DHPP and intranasal Bordatella vaccines were given at 9 weeks of age. At that time, physical examination was with normal limits with the exception of a small, reducible umbilical hernia. The dog was also dewormed and placed on preventative treatment for fleas. Ten days following the second vaccination, the dog presented for lethargy and anorexia. ... Conclusion by Dr. Diane Shelton: In a search of the database of the Comparative Neuromuscular Laboratory over the years 2001-2005, masticatory muscle myositis was confirmed in 11 young Cavalier King Charles Spaniels with onset at less than 6 months of age. In several cases, onset followed within 10 days of vaccinations. Although long-term information is not yet complete, there was resolution of clinical signs of masticatory muscle myositis in most cases. Of interest, other complications such as early onset hypothyroidism and allergies were reported. Additional information will be provided as more is learned about this early-onset masticatory muscle myositis in this breed."

4 month old CKCS with MMMMasticatory muscle myositis and Neospora infection in a 4 month old female Cavalier King Charles Spaniel. Dr. Simon Tappin and Dr. Kate Murphy. Comparative Neuromuscular Laboratory at the University of California, San Diego; March 2006. Quote: "A 4 month old female Cavalier King Charles Spaniel [right]... Outcome: The dog has now completed the 6 month tapering prednisolone course, and although a slight head tilt and the absence menace response are still present, the dog has a very good quality of life. The dog’s jaw opens close to a normal range with normal prehension and chewing. ... A severe and early onset form of masticatory myositis is seen in Cavalier King Charles Spaniels, although this is yet to be fully characterized."

Atypical masticatory muscle myositis in three cavalier King Charles spaniel littermates. Pitcher GD, Hahn CN. J Small Anim Pract. 2007 Apr;48(4):226-8. Quote: “This case report describes a novel manifestation of the immune-mediated disease, masticatory muscle myositis. Clinical signs, including difficulty in opening the mouth (trismus), were seen in three of four 12-week-old cavalier King Charles spaniel littermates. Diagnosis was established by 2M immuno-histochemistry, supported by characteristic histopathological changes in affected temporal muscle. Treatment using corticosteroids at immune-modifying doses resulted in resolution of clinical signs in all the affected animals. Masticatory muscle myositis should be considered as a differential diagnosis in groups of young dogs with clinical signs of myositis localised to the head.”

A Practical Guide to Canine and Feline Neurology. Curtis W. Dewey. John Wiley & Sons; 2008; 491. Quote: "Most dogs with masticatory myositis are young adults. However, juvenile onset of masticatory myositis has been reported in 12-week-old Cavalier King Charles Spaniel dogs."

Breed Predispositions to Disease in Dogs & Cats (2d Ed.). Alex Gough, Alison Thomas. 2010; Wiley-Blackwell Publ. 52.

Genetic Connection: A Guide to Health Problems in Purebred Dogs, Second Edition. Lowell Ackerman. July 2011; AAHA Press; pg 126. Quote: "Masticatory myositis, also known as eosinphylic myositis and atrophic myositis, is an immune-mediated disease in which antibodies are directed against type II M fibers in masticatory muscles. It is most commonly reported in the German shepherd dog, but other overrepresented breeds include the Cavalier King Charles spaniel ...."

Masticatory Myositis (Eosinophilic Myositis). The Pet Health Care Library. 2013. Quote: "The average patient age is 3 years old. The most common breeds are German shepherds, Labrador retrievers, Doberman pinschers, Golden retrievers, and Cavalier King Charles spaniels. Patients can be of either gender. In the acute phase of the disease, the masticatory muscles are swollen and the eyes appear to bulge due to the swollen pterygoid muscles behind them. There may be a fever and local lymph node swelling at this stage. Results are best if therapy is initiated at this point but unfortunately many owners do not notice the problem until the muscles begin to atrophy and the jaws are rigidly closed, making eating difficult."

Bilateral rastral mandibulectomy for correction of limited mouth opening caused by masticatory muscle myositis: case report in a dog. Arias, M.V.B. et al. MEDVEP. 2013.

Use of MRI for the Early Diagnosis of Masticatory Muscle Myositis. Alberto Cauduro, Favole Paolo, Roberto M. Asperio, Valeria Rossini, Maurizio Dondi, Lucia A. Simonetto, Carlo Cantile, Valentina Lorenzo. J.Am.Anim.Hosp.Assn. Sept. 2013;49(5). Quote: "The medical records of two dogs that were diagnosed with masticatory muscle myositis (MMM) were reviewed. The reported clinical signs included intense pain when opening the mouth and restricted jaw movement. MRI detected widespread, symmetrical, and inhomogeneously hyperintense areas in the masticatory muscle. Electromyography (EMG) demonstrated severe and spontaneous pathologic activity in the temporal and masseter muscles. With early therapeutic treatment, remission of symptoms occurred within 2 mo, and no relapses were observed for the subsequent 2 yr. The gold standard for the diagnosis of MMM is the 2M antibody test, but the purpose of this study was to evaluate the use of MRI as an accurate and efficient diagnostic tool for the initiation of early therapy for the treatment of muscle myositis."

Early Diagnosis of Masticatory Muscle Myositis Is Needed for Treatment Success.   Purina ProClub. Jan. 2014. Quote: "Imagine what it would be like if your Golden Retriever could not open his mouth to eat and drink. That is often what happens with an autoimmune disorder called masticatory muscle myositis (MMM) that affects the jaw muscles, causing pain and dysfunction. Swollen, painful masticatory (chewing) muscles and an inability to open the mouth (trismus) are clinical signs of the disorder. 'These dogs are not able to pick up a ball or eat without experiencing severe pain,' says Brian E. Greenfield, D.V.M., who practices at Animal Clinic Northview in North Ridgeville, Ohio. 'In the early stages of the disease, the muscles that are used for eating and chewing appear swollen. As the disease progresses, these muscles begin to atrophy, or waste away. Sometimes the eyes appear sunken, or, less commonly, they seem to protrude.' Although MMM can occur in any breed, it occurs more commonly in large breeds, such as Golden Retrievers, Doberman Pinschers, German Shepherd Dogs, and Labrador Retrievers. Young Cavalier King Charles Spaniels may be severely affected and are believed to be genetically predisposed to developing MMM. Although the disorder does not occur on a widespread basis in Golden Retrievers, anecdotal data suggest that the incidence may be increasing. Thus, whenever a Golden is suddenly unable to open his mouth, experts recommend immediate veterinary care to determine the cause.  The condition occurs in males and females, with an average age of onset of 3 years, though puppies as young as 4 months have been affected. Fortunately, if MMM is diagnosed early, dogs can be treated to increase the likelihood of a full recovery. Research of this disease at the University of California-San Diego led to the development of a blood test in 2004 that detects the presence of 2M antibodies and accurately identifies affected dogs.

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