Neuromuscular disorders Flashcards

1
Q

List the non-specific signs of generalised neuromuscular disease

A
  • Tetraparesis and exercise intolerance
  • Stiff/stilted gait with reduced stride length, bunny hopping
  • Narrow based stance
  • Tremors/fasciculations
  • Regurgitation/altered oesophageal motility
  • Dysphonia (disorders of the voice)
  • Reduced reflexes and tone
  • Muscle atrophy
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2
Q

Describe a ‘typical neuromuscular gait’

A

Short strided, appears stiff/lame, difficulty supporting weight, bunny hopping, sensation/proprioception is usually normal

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3
Q

How does a junctionopathy present?

A
  • Generalised
  • Classically exercise intolerance with fatigue
  • Normal sensory function
  • Often intact tendon reflexes unless severe weakness
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4
Q

What are the generalised clinical signs of a myopathy?

A
  • Atrophy or hypertrophy
  • Specific features: dimple contractures (myotonia); myalgia; restricted joint movement (contracture)
  • Normal sensory function
  • Often normal tendon reflexes but exceptions
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5
Q

Name 3 causes of junctionpathies

A

Myasthenia gravis
Botulism
Organophosphate toxicity

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6
Q

Name 2 causes of myopathies

A
  1. Polymyositis - immune mediated or infectious
  2. Electrolyte abnormalities - Addisons, Hypokalaemia
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7
Q

What neuropathies present with chronic and progressive neuromuscular signs?

A
  • Inflammatory/infectious e.g. Protozoal, Viral
  • Toxic e.g. lead
  • Metabolic e.g. Diabetes, Cushings, Hypothyroidism
  • Idiopathic
  • Degenerative
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8
Q

Which myopathies present with chronic and progressive neuromuscular signs?

A
  • Inflammatory polymyositis
  • Infectious polymyositis
  • Metabolic/endocrine
  • Paraneoplastic
  • Degenerative e.g. muscular dystrophies
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9
Q

List the initial tests for neuromuscular diseases

A
  • Haem/biochem
  • Urinalysis
  • Chest radiographs
  • Abdo imaging
  • Endocrine tests
  • Serology/PCR
  • Genetic testing
  • CSF
  • Biopsies
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10
Q

Increases levels of … in blood indicate muscle damage?

A

Creatinine kinase

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11
Q

Increased levels of … in urine indicate muscle damage?

A

Myoglobinuria

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12
Q

Which 2 conditions linked to neuromuscular disease could be observed on chest radiographs?

A

Megaoesophagus
Aspiration pneumonia

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13
Q

Where would you take a muscle biopsy?

A
  • Area where muscle fibres are oriented in a single direction
  • Distant from tendons and aponeuroses
  • Disease requirements (e.g. congenital myasthenia gravis biopsy motor point or whole muscle for quantification)
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14
Q

Which muscle is a masticatory muscle biopsy taken from?

A

Temporalis muscle

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15
Q

What is polyradiculoneuritis?

A

Inflammation of nerves and roots
- Recent association with Campylobacter and raw feeding
- Most pathology is in ventral spinal roots

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16
Q

Polyradiculoneuritis is predisposed in which breed?

A

Bengal cats

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17
Q

How does polyradiculoneuritis present?

A
  • Acute onset, rapidly progressive
  • Tetra/paraparesis
  • Flaccid, markedly reduced motor function (inc. absent reflexes)
  • Can affect respiratory muscles
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18
Q

How is polyradiculoneuritis diagnosed?

A

CS
History
CSF analysis
Electrophysiology

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19
Q

Describe the natural disease progression of polyradiculoneuritis

A
  • Inflammation is of short duration
  • Recovery depends on severity of damage
  • Remyelination over days-weeks
  • More prolonged if axonal damage
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20
Q

How is polyradiculoneuritis treated?

A

Supportive
- Recumbency care
- Physiotherapy
- May have to consider ventilatory support

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21
Q

What is myasthenia gravis?

A

Reduced neuromuscular transmission - grave muscle weakness

22
Q

What are the two types of myasthenia gravia?

A

Congenital – genetic defect
Acquired: immune-mediated

23
Q

How does acquired myasthenia gravis present in dogs?

A
  • Generalised (most common)
  • Fulminant: acute onset, rapidly progressive
  • Focal (~40%): facial, pharyngeal, laryngeal
  • Megaoesophagus in ~90% of cases
24
Q

How does acquired myasthenia gravis present in cats?

A
  • Abyssinians predisposed
  • Generalised in 90%
  • Megaoesophagus less likely (15%)
  • Cranial mediastinal mass (thymoma) in ~26-52%
  • Can arise in cats on thiourylene medication for hyperthyroidism
25
How is myasthenia gravis diagnosed?
- Clinical signs - nAChR Ab test is gold standard (small percentage of false –ves) - Electrodiagnostic tests (repetitive nerve stimulation) - Response to cholinesterase inhibitor (edrophonium or neostigmine)
26
How is myasthenia gravis treated?
- Anti-cholinesterase: Pyridostigmine p/o or neostigmine i/m - Supportive: postural feeding for megaoesophagus
27
Which Clostridial disease causes neuromuscular disease?
Clostridium botulinum
28
How does infection with clostridium botulinum occur?
- Ingestion of contaminated food - esp. animal carcasses - Rare in dogs and cats - Toxin is absorbed from the gut
29
What is the main effect of exotoxin from clostridium botulinum?
Acts to block vesicle fusion with the presynaptic membrane and ACh release
30
Describe the clinical signs of clostridium botulinum infection
1. Nicotinic ACh synapses (junctionopathy) = Acute onset rapidly progressive tetraparesis (2-3 d). May affect cranial nerves: jaw tone, facial paralysis, gag reflex, megaoesophagus. May affect respiratory muscles 2.Muscarinic ACh synapses (dysautonomia) = Urinary dysfunction, GI dysmotility, mydriasis, reduced tear production
31
How is clostridium botulinum diagnosed?
- Clinical signs - Electrophysiology - Demonstrate: Toxin (feed, faeces) – usually not possible - Serology (retrospective exposure)
32
How is botulism treated?
Supportive care: - Bladder management - Recumbency care - Megaoesophagus management - Physiotherapy
33
Who is most commonly affected by immune mediated polymyositis
More common in dogs than cats, particularly large breeds - Newfoundland, Boxer, GSD, Labrador, Golden retriever
34
What are the clinical signs of immune mediated polymyositis?
- Pyrexia, stiffness, non-ambulatory tetraparesis, reluctance to move, lowered head carriage, myalgia, muscle swelling - Generalised weakness, muscle atrophy, exercise intolerance, fatigue +/- oesophageal involvement – regurgitation
35
How is immune mediated polymyositis diagnosed?
- H+B: Inflammatory leucogram, elevated CK/AST (acute typically +++) - Electrodiagnostics: marked EMG abnormalities, normal MNCV - Muscle biopsy for diagnosis - Rule out infectious diseases and tumours
36
How is immune mediated polymyositis treated?
Prednisolone
37
What is the cause of infectious myositis?
Protozoal - Neospora caninum/Toxoplasma gondii
38
What are the clinical signs of neosporosis in puppies?
- Radiculoneuritis and polymyositis - Pelvic limb hyperextension (usually starts in one limb and progresses to other) - Ascending paralysis of pelvic limbs with muscle contracture and arthrogryposis
39
How is neosporosis diagnosed?
Clinical signs, CK/AST, EMG, biopsy SEROLOGY PCR on CSF, blood
40
How is neosporosis treated?
Clindamycin/TMPS + pyrimethamine
41
In which breed is there a juvenile form of masticatory myositis?
CKCS
42
What are the clinical signs of masticatory myositis?
Swollen/painful MM, exophthalmos Trismus (pain/fibrosis), MM atrophy
43
How is masticatory myositis treated?
Prednisolone +/- other IM drugs Physiotherapy
44
Is trigeminal neuritis more commonly seen in dogs or cats?
Dogs
45
What is trigeminal neuritis?
Inflammation throughout trigeminal nerves including ganglia bilaterally Demyelination>axon degeneration
46
What are the clinical signs of trigeminal neuritis?
- Acute onset paresis/plegia of masticatory muscles -> dropped jaw - Normal gag and tongue tone and movements (Horner’s or facial paresis can be seen) - Sensory deficits in ~30% of cases - Masticatory muscle atrophy
47
How is trigeminal neuritis diagnosed?
MRI: swollen, inflamed nerves CSF
48
How is trigeminal neuritis treated?
Resolves usually within 3 weeks Supportive care
49
What are the cases of unilateral temporal muscle atrophy?
~50% of cases due to trigeminal nerve sheath tumour ~25% due to other neoplasia Other possibilities – infectious/inflammatory, idiopathic, traumatic
50
What are the clinical signs of unilateral temporal muscle atrophy?
- Neurogenic atrophy of masticatory muscles, otherwise may be normal at onset - May be abnormal sensation (face rubbing, absent palpebral and corneal reflexes) - Progression to involve other cranial nerve and brainstem functions if neoplastic