Neuromuscular diseases can be broken down into
List the specific sites of potential disease for each category.
Neuropathy: Neuron (cell body, axon), Schwann cell, myelin
Junctionopathy: Pre-synaptic (i.e. faulty transmitter synthesis or release), synaptic (acetylcholine problem) or post-synaptic (affectign Ach-R e.g. MG)
Myopathy: sarcolemma, transverse tubules, organelles, myofilaments inclusions.
What is a motorneuron unit?
What is variability of myofibre type within a motor unit?
Single histochemical myofibre type, not a mixture of myofibre types
(not usually contiguous –> mosaic pattern)
Name the principal signs of myopathic syndrome
What are the principal signs of motor neuropathic syndrome
What are the principal signs of sensory neuropathic syndrome
Name general clinical signs of neuromuscular disorders

Outline 7 step diagnostic workup plan for neuromuscular disease

List 3 neuromuscular disorder ddx for large CK elevation (>10,000 IU/L)
What specific test can be run in animals suspeted of having mitochondrial myopathy?
Post-exercise plasma pyruvate
Once haematuria has been ruled out, how is myoglobinuria distinguished from hemoglobinuria?
Ammonium sulfate precipitation test
How is masticatory myositis diagnosed?
2M antibody test
2M myofibre = unique antigen present on muscles of mastication
What are two specific tests for neuromuscluar diseases?
Define electromyography
Electromyography: Detection and characterization of electrical activity recorded from patient’s muscles
Allows accurate determination of distribution of muscles affected by neuromuscular disorder.
Which 3 broad muscle locations shoudl be tested with EMG?
Proximal and distal appendicular muscles as well as axial muscles (usually limit testign to unilateral so that biopsies can be harvested from other side)
What is normal EMG of muscle at rest (eg uGA)
Electrically silent

What are the 4 types of EMG traces considered normal spontaneous activity

What are the 3 types of EMG traces considered abnormal spontaneous activity

If selceting muscle for biopsy is it better to take mildly, moderately or severely affected muscle
moderate
What does spontaneous activity mean in relation for EMG
Potentials that are independent of mechanical stimulation.
What can increased insertional activity on EMG mean?
And decreased?
Increased: neuromuscular disease
Decreased: significant myofibre loss
What causes miniature end plate potentials on EMG?
Proximity to a NMJ
What causes end plate spike on EMG?
Normal spontaneous depolarisation of a single myofibre
What causes motor unit action potential on EMG?
normal muscle that isnt completely at rest e.g. light anaesthesia plane