Neurology - Neuromuscular Junction

Question 1

Lambert Eaton syndrome

• action against which channel
• characteristic/differentiating character
• diagnosis
• association with
• treatment

Answer 1

• action against calcium channels
• has autonomic features such as erectile dysfunction, dry mouth, constipation, Ortho static hypotension
• temporary improvement of power and reflexes post exercise
• investigate with nerve conduction studies (decreased amplitude with increase post exercise), VGCC antibodies
• associated with SCLC (small cell)
• treatment with 3-4 diaminopyridine, IVIG and pred

Question 2

Myasthenia Gravis - musk positive

• demographics
• treatment
• respiratory and bulbar involvement

Answer 2

Usually in non white young female, early onset, not related to thymoma. Generalised MG. Severe disease with respiratory and bulbar involvement. Poor response to treatment but good response with rituximab.