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SS - Exam 1 > NMJ & Muscle Disease > Flashcards

NMJ & Muscle Disease Flashcards

1
Q

Duchenne muscular dystrophy (DMD)

A

Common human disease, occurs in dogs/cats - Goldens
X-linked muscular dystrophy (females are carriers)
- loss of dystrophin

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2
Q

Onset for DMD

A

Onset 8-12 wks of age
Stiffness & bunny hopping

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3
Q

Diagnosing DMD

A

Clinical signs
CK markedly elevated
Possible genetic testing
Special stains - will show decreased dystrophin on muscle biopsy

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4
Q

Treatment for DMD

A

L-carnitine & CoQ10
Gene therapy for prevention?
Long term prognosis is very poor

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5
Q

Centronuclear myopathy

A

Reported in border collies, labs & Great Danes
Clinical signs vary but are consistent with LMN disease

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6
Q

Clinical signs of CNM

A

Short strided gait, stiff gait, exercise intolerance

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7
Q

Diagnosing and treatment of CNM

A

Electro diagnosis
Muscle biopsy
Genetic testing
CoQ10
L-carnitine

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8
Q

Congenital myotonia

A

Myotonia congenita is an inherited neuromuscular disorder characterized by the inability of muscles to quickly relax after a voluntary contraction

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9
Q

Clinical signs for congenital myotonia

A

Hypertrophied muscle, stiff gait, difficulty rising, usually see improvement with exercise

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10
Q

Diagnosing congenital myotonia

A

Compatible clinical signs
CK - normal to mild elevation
Genetic testing - CLCN1 mutation
EMG - electro testing for myotonic discharges (classic)

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11
Q

Treating & prognosis for congenital myotonia

A

Mexiletine, procainamide
Not usually progressive but can affect quality of lifed

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12
Q

Steroid myopathy

A

Usually from chronic corticosteroid usage
Profound weakness & muscle atrophy
Type 2 myofibers are primarily affected

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13
Q

Clinical signs for diffuse motor unit disease

A

Paresis
Weakness
Gait abnormalities
Exercise intolerance
Reduced reflexes
Postural alterations
Dyphonia
Regurgitation
Dysphagia
Dyspnea

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14
Q

Junctionpathies

A

Degenerative
Anomalous - congenital MG
Metabolic
Nutritional
Neoplastic - paraneoplastic MG
Idiopathic
Inflammatory - myasthenia gravis
Infectious - tick paralysis, botulism
Trauma
Toxin
Vascular

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15
Q

Myasthenia gravis

A

Myasthenia gravis is a chronic autoimmune, neuromuscular disease that causes weakness in the skeletal muscles (the muscles that connect to your bones and contract to allow body movement in the arms and legs, and allow for breathing)

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16
Q

classifying myasthenia gravis

A

Location & timing - focal, generalized, acute
Pathophys - acquired or congenital
Paraneoplastic - thymoma vs no thymoma
Testing - seropositive or seronegative

17
Q

Signalment/history for myasthenia gravis

A

Any signalment - either young or older dogs
Acute or chronic, generalized weakness, exercise intolerance, regurgitation, Dysphagia

18
Q

Diagnosing myasthenia gravis

A

History/exam
Radiographs
Tensilon /neostigmine testing - false pos or negs
**ACh antibody titers - doesn’t work for congenital, could be negative in some acquired cases

19
Q

Treatment of MG

A

Remove thymoma if present
Pyridostigmine
Could use steroids
Supportive care
Prognosis is variable - usually better for cats

20
Q

Mechanism of congenital MG

A

Multiple genes are implicated

21
Q

Acute MG

A

Worse case - sudden onset & rapid progression
Present with lateral recumbency
Respiratory distress due to aspiration pneumonia

22
Q

Treating acute MG

A

IV cholinesterase inhibitors
Mechanical ventilation if needed
IVIG
Prognosis is very grave for acute

23
Q

Differentials for flaccid tetraparetic animals

A

Acute polyradiculoneuritis
Botulism
Tick paralysis
Fulminant MG

24
Q

Botulism

A

Caused by C. Botulinum - ingested in spoiled food
Binds SNARE proteins to prevent release of neurotransmitter can lead to flaccid paralysis

25
Treating botulism
Polyvalent anti-toxin in severe cases Antibiotics are not necessary- usually bacterial infection isn’t active or live
26
Tick paralysis
Dermacentor in North America, Ixodes in Australia Toxins are present in tick saliva and are presynaptic blocking neurotransmitter release Occur 5-9 days after tick attachment Dogs & cats, more severe cases in Australia Acute, flaccid paralysis is outcome
27
Treatment and prognosis for tick paralysis
Supportive care if necessary US tick - 8-72 hours for full recovery Australian tick 5% mortality
28
Muscle biopsy
Various locations - triceps, cranial tibial, gastroc Must ship on ice in formalin
29
Non inflammatory myopathy
Like a sniper taking out specific targets
30
Inflammatory myopathy
Like an atom bomb taking out the whole town
31
Inflammatory myopathies
Polymyositis Masticatory muscle myositis Extraocular myositis Dermatomyositis
32
Polymyositis - infectious
Parasitic - toxo, neospara, babesia, Hepatozoon, sarco Rickettsial - ehrlicia Viral - FeL, FIV Bacterial - clostridial Fungal - sporotrichosis *good idea to submit for titers
33
Immune mediated Polymyositis
Paraneoplastic Newfoundlands & vizsla are common breeds Generalized weakness, stiff gait, megaesophagus, Dysphagia, muscle atrophy, myocarditis, gloss it is
34
Diagnosing IM Polymyositis
CK elevated but not 100% reliable Electrodiagnosis Multiple muscles affected Treat w immunosuppression Prognosis depends
35
MMM
Masticatory muscle myositits Primarily in dogs, 2 forms Acute - swelling of muscles, pain, fever Chronic - masticatory muscle atrophy, trismus (can’t open mouth)
36
Diagnosing MMM
2M titer antibody, temporalis biopsy Treat early EMG markedly abnormal esp in acute
37
Extraocular myositis
Rare condition - in young female golden retrievers 21/37 dogs Exophthalmos without 3rd eyelid protrusion Diagnosis most commonly by clinical presentation Orbital ultrasound may be helpful
38
Dermatomyositis
Canine family Dermatomyositis Ischemic dermatopathy and myopathy Collies, shetland sheepdogs, juveniles Alopecia on face & extremities EMG - fibs, sharps Muscle biopsy may be helpful Genetic risk factors
39
Non inflammatory myopathy
Duchenne muscular dystrophy Centronuclear myopathy Congenital myopathy Steroid myopathy

SS - Exam 1 (33 decks)

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