Reading Time:
Pathology
Autoimmune mediated neuromuscular disease characterized by fatigable muscle weakness resulting in various symptoms
Aetiology
Autoantibodies to post synaptic acetylcholine receptors or muscle specific tyrosine kinase ris.
Signs
Ptosis, ophthalmoplegia, dysarthria and fatigable weakness
Symptoms
Dysphagia, dysarthria, facial weakness, limb and respiratory muscle weakness. Weakness worse with exercise
Investigations
Bloods: Acetylcholine receptor antibodies, anti-MUSK antibodies
Imaging: Chest X-Ray and CT thorax: presence of thymoma
Tensilon test: May produce a transient improvement in certain symptoms.
Repetitive Nerve Stimulation: Decrease in muscle response
Pulmonary Function Tests: Assess for respiratory muscle weakness
Treatment
Medical: Corticosteroids, and acetylcholinesterase inhibitors (neostigmine)
IV immunoglobulins and plasma exchange for myasthenic crisis.
Patients with respiratory decompensation should be managed in ITU as
they may require respiratory support and FVC monitoring.
Steroid sparing agents used for long-term immunosuppression
Surgery: Thymectomy
Prognosis
Patients usually have a normal life expectancy with treatment
Respiratory failure, aspiration pneumonia
Key Facts
Lorem ipsum dolor sit amet, sapien platea morbi dolor lacus nunc, nunc ullamcorper. Felis aliquet egestas vitae, nibh ante quis quis dolor sed mauris.
Key Images
Lorem ipsum dolor sit amet, sapien platea morbi dolor lacus nunc, nunc ullamcorper. Felis aliquet egestas vitae, nibh ante quis quis dolor sed mauris.
Key References
Lorem ipsum dolor sit amet, sapien platea morbi dolor lacus nunc, nunc ullamcorper. Felis aliquet egestas vitae, nibh ante quis quis dolor sed mauris.