Pneumonia

Neuromuscular Junction Diseases

Medical Department of College of Medicine in 21 September University

: Prepared by

Dr . TAREK AL-SHAIBANI
Myasthenia gravis

 An acquired humoral autoimmune disorder

resulting in insufficient functioning
acetylcholine receptors .
Introduction

 Myasthenia is more common in Women .

Definition

 Neuromuscular disease leading to

fluctuating muscle weakness & fatigability .
Auto Antibodies

 Acetylcholine Receptors Auto Antibodies

seen in 90 % of cases .

 These antibodies block Ach receptors at the

post-synaptic neuromuscular junction .
Associations
 Thymomas in 15 % .

 Thymic hyperplasia in 50 – 70 % .

 Autoimmune disorders : Autoimmune

thyroid disorders , RA , SLE , Pernicious
anaemia .
Features
 Muscle fatigability : The key feature .

 Progressively weaker during period of

activity later in the day .

 Slowly improve after periods of rest .

Features

 Proximal muscle weakness : face , neck &

limb girdle .

 Extra ocular muscle weakness : diplopia and

ptosis .
Features

 Dysphagia ( worse with liquids than solids )

 Dysarthria , slurred speech

Ocular myasthenia
 Bilateral Ptosis

 Variable diplopia

 Without proptosis or injection of the eyes

 With normal all pupils reflexes

Exacerbating factors

 Exertion is the most common exacerbating

factors resulting in fatigability .

 Symptoms become more marked during the

day .
Drugs may exacerbate
 Penicillamine
 Procainamide
 Quinidine
 Beta-blockers
 Lithium
 Phenytoin
 Gentamicin
Investigations
 Tensilon test IV Edrophonium reduce
muscle weakness temporarily & used in
diagnosis ( diagnostic & therapeutic test )

 Single fibre electromyography ( EMG ) is the

most sensitive test for myasthenia gravis
Investigations

 CT thorax to exclude thymoma

 CPK normal
Investigations
 Muscle fatigability as bedside by asking the
pt. to count aloud from 1 to 20 slowly often
inducing slurred speech

 Fatigable ptosis by asking the pt. to

maintain upward gaze without blinking for
30 – 60 seconds
Management

 Long-acting anticholinesterase e.g

Pyridostigmine

 Immunosuppression : Prednisolone initially

Management

 Thymectomy : is significantly improve

symptoms of myasthenia and is the next
logical step once diagnosis of myasthenia is
confirmed
Myasthenic crisis Management

 Intravenous immunoglobulins

 Plasmapharesis usually works quicker but

involves more expensive equipment
Conclusion

 Neurogenic dysphagia that is worse with liquids

than solids

 Also associated with dysartheria , nasal

regurgitation , coughing and choking episodes
during meals
Conclusion

 Opinions vary as to whether Plasmapharasis or IVIG

should be given as a first line .

 Gentamicin is the most likely antibiotics to

exacerbate myasthenia gravis .
Thank you