DAIM - Diseases of Abnormal Movement Flashcards
Define DAIM
abnormal, involuntary skeletal muscle contractions in the conscious animal
- can arise from skeletal muscle, peripheral nerve or CNS
Are majority of DAIM diseases HYPERkinetic or HYPOkinetic?
HYPERkinetic!
What are the 5 cardinal phenotypes of DAIM?
- Myotonia
- Myokymia/neuromyotonia
- Tetany
- Myoclonus
- Dyskinesia
What is myotonia?
HYPERkinetic – persistent, sustained muscle contraction with delayed muscle relaxation
- disease of Skeletal Muscle
What breeds predisposed to myotonia congenita?
Min. Schnauzer and Chow
What history and exam of myotonia congenita?
Clinical signs in first few months of life
- stiff saw-horse like gait that improves with exercise
- double muscled appearance
- dysphagia from tongue hypertrophy
- percussion dimpling of muscles
- signs get worse in cold weather
What pathophysiology of myotonia congenita?
autosomal recessive mutation in *Voltage-gated Chloride Channel mutation that causes issues with incomplete repolarization, prolonger depolarization and spontaneous depolarization
How do you DX myotonia congenita?
- Signalment (Min Schnauzer or Chow)
- History (CS in first few months of life)
- CS (stiff pelvic limbs with big proximal limb muscles, …)
EMG waxing and waning
- Also, genetic testing
How do you TX myotonia congenita?
Mexilitene, Procainamide, or Regular exercise program
PX and prevention of myotonia congenita
Fair to Good prognosis unless severe dysphagia present
Don’t breed these animals as it is autosomal recessive
What is acquired myotonia/pseudomyotonia?
most commonly a complication of Cushing’s (hyperadrenocorticism) but rare (<1%)
- can be seen with muscular dystrophy and 2,4-D tox
- may be French Poodle genetics
What are clinical signs of acquired myotonia/pseudomyotonia?
Comes with Cushing’s signs
Pathophys it unknown
How do you DX acquired myotonia/pseudomyotonia?
Cushing’s tests (ACTH stim, LDDS, etc.)
CS, HX, Signalment
EMG not waxing and waning but HIGH-frequency
How do you treat acquired myotonia/pseudomyotonia?
Also use, Procainamide
PX of acquired myotonia/pseudomyotonia?
:( does not resolve in most dogs with Cushing’s, even with treatment :(
what is myokymia?
vermicular (undulating, wavelike) muscle contraction that spreads across the surface of the muscle
- focal or generalized
what is neuromyotonia?
persistent muscle stiffness
- ranges from subclinical to intense contracture of distal extremities and face or episodic collapse
How are myokymia/neuromyotonia different from myotonia?
myokymia/neuromyotonia are a disease of motor axons or motor nerve terminals
myotonia is a disease of skeletal muscle
What is typical signalment of myokymia/neuromyotonia?
Jack Russell Terriers. It’s a.k.a. Continuous muscle fiber activity of the Terrier.
- young to adult dogs
- rarely in cats
What breed is predisposed to myokymia/neuromyokymia?
Jack Russell Terriers – Potassium gated channel defect