Chapter 27 Neurodiagnostics Flashcards
(48 cards)
Neuromuscular diseases can be broken down into
- Neuropathy
- Junctionopathy
- Myopathy
- (and neuromyopathies)
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?
- Motor neuron (cell body, and its peripheral axon) supported by Schwann cells
- NMJ
- Myofibres innervated by motorneuron
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
- Generalised weakness
- Exercise intolerance
- Stiff/stilted gait
- Tremors
- Muscle atrophy
- Muscle hypertropy
- “Percussion dimple” contracture
- Muscle pain
- Limited joint movement eg contracture
- Regurg
- Flexion of head and neck
- Trismus (=lockjaw)
What are the principal signs of motor neuropathic syndrome
- Flaccid paresis/paralysis of innervated structures
- Neuroenic muscle atrophy
- Reduced reflexes and muscle tone
- Muscle fasciculations
What are the principal signs of sensory neuropathic syndrome
- Decreased response to noxious stimuli
- Proprioceptive defifits
- Abnormal sensation of face /trunks/lims/self mutilation
- Reduced reflexed in absence of muscle atrophy
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)
- Muscular dystrophies
- Necrotising myopathies
- Inflammatory myopathies
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?
- Electrodiagnostic testing
- Muscle/nerve biopsy
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
- Insertional Activity
- Miniature End Plate Potentials
- End Plate Spike
- Motor Unit Action Potential

What are the 3 types of EMG traces considered abnormal spontaneous activity
- Fibs and Sharps
- Complex Repetitive Discharges
- Myotonic Potentials

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








