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Myositis Research Information • Johns Hopkins Myositis Center
A month-old, castrated male Vizsla was presented for pelvic limb weakness, difficulty opening his mouth, ptyalism, voice change, and urinary incontinence. Myasthenia gravis and masticatory myositis were diagnosed. The unusual clinical findings, diagnosis, treatment, and case myositis emarginatus pdf are described, followed by a brief discussion of myasthenia gravis and masticatory myositis.
A month-old, The gait abnormality was described as hindend weakness with an unwillingness to take more than a few steps, followed by sitting. The onset of the gait abnormality coincided with a fall while the dog was playing with another dog.
The day prior to presentation, the referring veterinarian noted that the dog had a hunched myositis emarginatus pdf and delayed proprioception of the pelvic limbs. Trismus, drooling, and inefficient drinking and eating behavior were also noted 7 d prior to referral.
Similar signs, first appearing 4 mo earlier, were attributed to suspected masticatory myositis; however, further diagnostic testing was not pursued and treatment with amoxicillin Apo-Amoxi; Apotex, Toronto, Ontario and meloxicam Metacam; Boehringer Ingelheim, Burlington, Ontario appeared to resolve the clinical signs.
The dog had been assessed to be healthy at the time of vaccination 8 mo earlier. Physical examination revealed a thin body condition, severe atrophy of the muscles of mastication, and constant ptyalism. Pain and trismus prevented a thorough oral examination. The dog could prehend and swallow food normally, but he drank with difficulty. The murmur had not been noted previously, and although not typical for the breed, mitral valve endocardiosis with regurgitation was myositis emarginatus pdf.
Intermittent urine dribbling was observed during the examination; the bladder was large and easily expressed. Neurologic examination revealed that the dog was quieter than normal. The palpebral reflex was absent bilaterally; however, vision and a pupillary light reflex were present.
There was a 2- to 3-mm corneal opacity in the left eye, which did not take up fluorescein stain. The dog was ambulatory, but obviously weak and reluctant myositis emarginatus pdf bear weight on the hind limbs. Despite having an arched back, the dog did not react painfully to spinal palpation. Proprioception was reduced in the pelvic limbs and normal in the thoracic limbs.
Spinal reflexes were present; however, flexor reflexes in all limbs were decreased, with the pelvic limbs being more affected than the thoracic limbs. Myositis emarginatus pdf pain sensation was present in all limbs and perineum. No orthopedic abnormalities were detected.
The neurologic examination indicated a diffuse hyporeflexia involving the spinal nerves, cranial nerve VII, and, possibly, the laryngeal innervation. The signs were most consistent with masticatory myositis and a peripheral neuropathy; the differential diagnoses included polyradiculoneuritis Coonhound paralysis myositis emarginatus pdf, metabolic disorders adrenocortical insufficiency, hypothyroidism, hypoglycemiaparaneoplastic syndrome, and idiopathic causes.
Atypical myasthenia gravis, a primary myopathy, immune-mediated or infectious polymyositis toxoplasmosis, neosporosisand a diffuse or multifocal encephalomyelitis were also considered. A concomitant thoracolumbar spinal cord lesion, possibly due to trauma, could not be ruled out. Initial diagnostic tests, consisting of an acid-base analysis, an electrolyte panel, blood urea nitrogen and glucose estimations, and an electrocardiogram, were normal.
On day 2, results from a complete blood cell count and serum biochemistry were normal, except for a low urea level 1. Blood was submitted for determination of an acetylcholine ACh receptor antibody titer, a type 2M myosin antibody titer, and Toxoplasma gondii and Neospora canis antibody myositis emarginatus pdf.
Thoracic radiographs were normal. An echocardiogram was not performed, because the electrocardiogram was normal and there was no radiographic evidence of cardiomegaly.
On day 3, the clinical signs and findings on a repeat neurologic examination were unchanged. With the history of dysphagia and pytalism, radiographs of the oral cavity and pharynx were obtained to evaluate for foreign bodies, masses, abnormal anatomic structures, myositis emarginatus pdf dental or temporomandibular joint disease.
No abnormalities were noted. Results from analysis of cerebrospinal fluid and electromyographic EMG examination of the limbs and epaxial muscles were normal. Electromyography detected fibrillation potentials and positive myositis emarginatus pdf wave activity bilaterally in the muscles of mastication and in the tongue.
Biopsies from the right masseter and hypoglossal muscles were formalin-fixed and frozen, and then submitted for histopathologic examination and immunocytochemical analysis at the Comparative Neuromuscular Laboratory, University of California-San Diego School of Medicine. The following day day 4the dog was observed to be walking myositis emarginatus pdf normally on initial exercise after rest in its cage. When walking, the dog rapidly deteriorated into severe paresis of the pelvic limbs and stiffness of the thoracic limbs.
He had also developed a soft productive cough. Serial thoracic radiographs now revealed aspiration pneumonia and megaesophagus. Because of the megaesophagus and the dramatic deterioration in gait with exercise, a Tensilon test edrophonium chloride Enlon, Baxter 2 mg IV was performed, with resultant rapid and dramatic improvement in limb strength, body posture, and palpebral myositis emarginatus pdf.
These results were suggestive of myasthenia gravis. The dog responded well to therapy with improvement in muscle strength and palpebral reflex, decreased ptyalism, and myositis emarginatus pdf of urinary continence.
Histopathologic examination of the masseter muscle revealed moderate multifocal areas of mononuclear cell infiltration lymphocytes, macrophages. Fibrosis was not observed.
Positive staining for type 2M fiber antibodies was detected within the muscle biopsy section. Circulating antibodies against type 2M muscle fibers myositis emarginatus pdf also detected in the serum.
Based on these findings, a diagnosis of masticatory myositis was made. Histopathologic examination of the biopsy of the hypoglossal muscle was unremarkable, ruling out myositis as a cause of the abnormal EMG findings in the tongue. The titer xmen vs street fighter game the ACh receptor antibody was positive 6.
Titers of antibody to Toxoplasma and Neospora were negative. Following discharge, the dog exhibited intermittent episodes of muscle weakness, exercise intolerance, and urine dribbling. After 12 wk of therapy, serial titers of antibodies to ACh receptor and type 2M muscle fiber were 1. At that time, the dog appeared to be in clinical remission, except for radiographic evidence of megaesophagus. This case report emphasizes some unusual clinical aspects of the presentation of acquired myasthenia gravis.
Hyporeflexia is an uncommon finding and may represent a more severe form of myasthenia gravis. The urinary myositis emarginatus pdf is also a unique feature. Disorders myositis emarginatus pdf with generalized muscle weakness can affect bladder function and, in association with decreased urethral sphincter function, may lead to incontinence; the incontinence myositis emarginatus pdf seem unlikely to be due to the involvement of neuromuscular transmission of muscarinic type of ACh receptor in the smooth muscles.
In a study by King et al 12 of 5 dogs presenting with acute fulminating myasthenia gravis were noted to have bladder distension requiring assistance to urinate.
Other causes of urinary incontinence were not pursued in this case following the rapid clinical resolution of the incontinence following therapy for myasthenia gravis. In humans, voiding dysfunction in conjunction with myasthenia gravis is rare and seems to be associated with a recent diagnosis of old wrestling games or an myositis emarginatus pdf of the disease process 2. It has been hypothesized that autonomic dysfunction in patients with myasthenia gravis might indicate a unique subset with a worse prognosis 2.
The clinical presentation of an arched spine with pelvic limb proprioceptive deficits, representing profound muscle weakness, is also an unusual finding in a nonrecumbent animal. Most ambulatory patients with myasthenia gravis present with normal proprioception. The proprioceptive deficits in this patient, however, may have been attributable to an additional disorder, such as a polyneuropathy, in which case, a nerve biopsy might have proven useful. Interestingly, following treatment for myasthenia gravis, the proprioceptive deficits resolved.
In addition, this patient initially did not have the typical historical complaint of fatigue associated with exercise seen in many cases of generalized myasthenia gravis. If fatigue had been present, it might have aided in an earlier diagnosis of myasthenia gravis. It was also unusual that with rest while hospitalized, the progressive weakness was unabated.
The myositis emarginatus pdf myositis was likely chronic, given the patient's previous history; however, there was no histopathologic evidence of fibrosis, which is often seen with chronicity 3.
The excessive ptyalism on initial presentation myositis emarginatus pdf attributed to the esophageal disease associated with the myasthenia gravis and not to masticatory myositis. Previously, Hackett et al 4 reported myasthenia gravis and concurrent masticatory muscle myositis mowgli the iceberg dreamcatcher adobe a year-old dog presenting with regurgitation, cough, and a thymoma.
Myasthenia gravis was diagnosed, based on a high antibody titer to ACh receptor. Histologic examination of the masticatory and extraocular muscles revealed extensive lymphoplasmacytic infiltration, myositis emarginatus pdf circulating antibodies against temporalis muscle proteins were documented by immunohistochemical staining. The unusual clinical presentation of this case and the presence of autoantibodies to ACh receptors and type 2M muscle fibers prompted testing for concurrent or underlying autoimmune disease.
Human myasthenics have an increased occurrence of several associated disorders, including autoimmune diseases such as hypothyroidism, lupus erythematosus, and rheumatoid arthritis 5. Although not as well characterized in the dog, some of these conditions are believed to be associated with canine myasthenia gravis 36.
In this case, antinuclear antibody ANA karke mobile kahe sun mundeya mp3 was negative and total thyroxine, free thyroxine, and thyroid stimulating hormone were normal. Acquired myasthenia gravis is an immune-mediated disease in which autoantibodies that react with nicotinic acetylcholine myositis emarginatus pdf of the skeletal muscle neuromuscular junction are produced 57. Clinical signs of myasthenia gravis myositis emarginatus pdf depending on the muscle groups involved.
Appendicular muscle weakness can manifest as weakness, stiff gait, or collapse. Facial muscle weakness can manifest as reduced or absent palpebral reflex; esophageal muscle weakness as megaesophagus with regurgitation; pharyngeal weakness as dysphagia; and laryngeal muscle weakness as voice change or inspiratory stridor 78. Less common clinical signs myositis emarginatus pdf have been associated with myasthenia gravis include hyporeflexia, lameness, shortening of stride, collapse, tremors, and distended bladder 1.
The preferred diagnostic test for canine acquired myasthenia gravis is the detection of circulating autoantibodies to ACh receptors 6. False positive results are myositis emarginatus pdf. A positive response to the short-acting anticholinesterase drug edrophonium chloride is suggestive of myasthenia gravis; however, false positive and false negative results are possible 38. Therapy for myasthenia gravis involves the use of anticholinesterase medication.
In many dogs, muscle weakness is not adequately controlled with anticholinesterase therapy alone, in which case immunosuppressive therapy is recommended either in conjunction with anticholinesterase treatment or as the myositis emarginatus pdf therapy 569. Prednisone is the most commonly used immunosuppressive agent; however, azathioprine, cyclophosphamide, and cyclosporine therapy have been reported to be efficacious 679.
Dogs with megaesophagus are managed with small, frequent feedings in the upright position, of a solid or semiliquid diet, depending on individual response, and are monitored closely for signs of aspiration pneumonia. The prognosis for dogs with myasthenia gravis is variable; however, severe aspiration pneumonia, persistent megaesophagus, acute fulminating myasthenia gravis, and the presence of a thymoma carry a poor prognosis 3 Masticatory muscle myositis MMM is an inflammatory disorder selectively involving the muscles of mastication 3.
It is presumed to be immune-mediated, based on the detection of autoantibodies reacting with type 2M muscle fibers, the presence of a mononuclear inflammatory cell infiltrate, and the clinical response to immunosuppressive doses of corticosteroids 3.
Masticatory muscles are composed primarily of unique type 2M myofibers that are not present in limb muscles Masticatory muscle myositis is usually bilaterally symmetrical, and affects dogs of any age, sex, or breed; however, large breed dogs are most commonly affected 3.
Acute and chronic forms of MMM have been described.