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  3. University College London (UCL)
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  5. NEUR0010 Neurobiology of Brain Injury and Disease (NEUR0010)

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Myasthenia Gravis Exam/Essay Summary
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  • Vak
  • NEUR0010 Neurobiology of Brain Injury and Disease (NEUR0010)
  • Instelling
  • University College London (UCL)

Summary of notes structured in exam and essay format complete with point, evidence, analysis, and critical thinking. Structured based on marking criteria.

Voorbeeld 2 van de 15  pagina's

Document informatie

  • 10 oktober 2022
  • 15
  • 2022/2023
  • Samenvatting

Onderwerpen

  • myasthenia gravis

Geschreven voor

  • University College London (UCL)
  • Onbekend
  • NEUR0010 Neurobiology of Brain Injury and Disease (NEUR0010)
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Voorbeeld van de inhoud

Contents
Part 1: Introduction + Background Information...............................................................................................1
Part 2: Pathogenesis..................................................................................................................................... 3
Part 3: Role of the Thymus.......................................................................................................................... 11
Part 4: Treatment and Management of MG.................................................................................................12
Part 5: Biggest Challenges and Unanswered Questions..............................................................................14
Part 6: Lambert-Eaton Myasthenic Syndrome.............................................................................................14
Part 7: Botulism........................................................................................................................................... 15

Part 1: Introduction + Background Information
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2730933/pdf/nihms132899.pdf = good review
https://www.nature.com/articles/s41572-019-0079-y.pdf = has links to recent papers and evidence


Description/summary:
 Myasthenia gravis is a prototypical immune-mediated disorder of NMJ where antibodies are directed
towards proteins of the NMJ, particularly the AchRs
 Non AChR components of the NMJ can also be attacked such as tyrosine kinases resulting in
endplate dysfunction and fatigable muscle weakness
 As such, MG is divided into AChR-positive and AChR-negative individuals
o In most cases MG is caused by autoantibodies directed towards the skeletal muscle
acetylcholine receptor (AChR)  AChR-positive
o In other causes, MG is caused by autoantibodies directed towards muscle-specific receptor
tyrosine kinase (MUSK)
 Patients experience skeletal muscle weakness and worsening of receptive activity as a result of this
autoimmune attack

Subgroups
1. Early-onset MG: ocular, ocular-bulbar or generalized
a. HLA DR3, B8
b. Thymic hyperplasia, thymoma, other organ-specific autoimmune diseases
c. F>M
d. AChR ab in 50 – 90%
2. Late-onset MG: ocular, ocular-bulbar or generalized
a. HLA DR2, B7, DRB1 15.01
b. Thymic atrophy
c. M>F
d. AChR ab in 50 – 90%
3. Thymomatous MG: ocular, ocular-bulbar or generalized
a. Usually AChR ab positive
b. Titin, ryanodine ab
c. Other paraneoplastic associations (autoimmune neuromyotonia…
4. MuSK MG: bulbar or generalised (~5% of MG)  muscle-specific receptor tyrosine kinase (MUSK)
is the target for the autoimmune attack
a. HLA DQ5, DR14, DR16
b. F>>M
5. LRP4 MG: mild (very rare)
6. Triple-seronegative MG
a. Clustered receptor assay picks up AChR ab in ~50%
b. Agrin, collagen Q
c. Variable association with thymus pathology
7. Drug-induced
a. Immune checkpoint inhibitors (nivolumab, ipilmumab)
i. Often with myositis, sometimes neuropathy, cardiomyositis, hepatitis
ii. Elevated CK, positive AChR labs, anti-titin labs
iii. Also can exacerbate pre-existing MG

, b. Other drugs
8. Transient neonatal MG
a. Passive transfer
b. Self-limiting

Epidemiology
 Prevalence ~14 per 100,000
 Early peak in incidence F>M
o Historically thought of as a disease of young women
 Late-onset MG: M>F
o Raises possibility that causation is different in older age group
o Myasthenia may be underdiagnosed in elderly people – prone to frailness, unfitness –
symptoms of MG

Symptoms and signs
 Fatigable weakness
 Diurnal fluctuation – much worse in evening than in morning (fluctuates also with exertion)
 Affects the eyes – ocular
o Ptosis, varial opthalmoplegia
o Normal pupils – pupils are innervated by muscarinic, not nicotinic receptors
o If pupil is involved, it is not MG
 Does NOT affect sensation

Typical presentation: ocular MG
 Presenting feature in 50-80%
 Variable, fatigable, complex opthalmoplegia
 Ptosis + frontalis hyperactivity
 Generalises in ~50% within 2 years
o Higher levels of antibodies as they progress
o No clear understanding as to why this happens
 Remains ocular in ~15%
 Prisms rarely helpful in long term

Typical presentation: bulbar/respiratory
 Dysphagia: nasal regurgitation, choking, fatigue on chewing
 Fatigable dysarthria
 Exertional dyspnoea, nocturnal hyperventilation
 Especially common in MuSK MG
 Respiratory assessment: Forced Vital Capacity (not peak flow or arterial blood gases) = how much,
not how fast
 Some patients experience difficulty swallowing tablets like pyridostigmine

Differential diagnosis
 Dysthyroid eye disease
 Chronic progressive external opthalmoplegia
 Pupil-sparing 3rd nerve lesion
 Lambert-Eaton Myasthenic Syndrome
 Botulism
 Amyotrophic lateral sclerosis
 Myopathies
 Congenital myasthenic syndromes

Tests
 Serology
o Looking for the antibodies that block the AChRs
o Blood test
o Also check thiopurine methyltransferase and thyroid function
 Determines ability to metabolise drug used to treat MG
 Thyroid antibodies is common and treatable
 Clinical neurophysiology

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