Myasthenia Gravis Myasthenia Gravis Examination Statement

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Myasthenia Gravis Myasthenia Gravis Examination Statement Powered By Docstoc
					Myasthenia Gravis
 Examine face, CNs eyes
 Approach to Ptosis (See Ptosis) and weakness

 Eyes
    Ptosis with fatigability
    Variable strabismus and diplopia that occurs after some time
    Check for hyperthyroid and thyroid eye disease
    Check for anaemia
    Check for malar rash of SLE
 Face
    VII – show your teeth : snarls
    Assessment of speech : Yeeee or count 1 to 20, nasal voice (bulbar palsy)
    Masseter weakness but pterygoids normal\
    Check neck for goitre and scars
 ULs
    Normal deep tendon reflexes (Eaton Lambert and Miller Fisher are reduced)
    Normal sensation
    Fatigability with weakness
    RA and SLE features
 Thymectomy scar and plasmapheresis line
 Associations
    Endo: Thyroid, DM, Pernicious anaemia
    CTD: RA, SLE, Polymyositis
 Requests
    Drug Hx
       D-penicillamine usage for RA or Wilson’s
       Antibiotics and CVS drugs that can worsen MG
    Temperature chart for fever – precipitate weakness

Sir, this patient has myasthenia gravis as evidenced by presence of muscle weakness
with fatigability. The patient’s deep tendon reflexes and sensation are normal.

On examination of the face, there is presence of bilateral facial muscle weakness
producing a mask-like facies with ptosis. I noticed furrowing of her forehead
musculature in an attempt to compensate for the ptosis. There is presence of variable
strabismus and diplopia after sustained gaze. I also noticed the presence of a nasal
voice as well as a reduction in volume of his speech when asked to count from 1 to
20. The patient does not require a nasogastric tube.

I did notice mid line sternotomy scar which suggest previous thymectomy.

There is no goitre or features of hyperthyroidism. Patient also does not have
symmetrical deforming polyarthropathy to suggest RA and has no cutaneous features
of SLE. I also did not notice any diabetic dermopathy.
I would like to complete my examination by
 Checking his negative inspiratory force
 Temperature chart
 Drug history

In summary, this patient has myasthenia gravis with mild weakness of which a
thymectomy has been done previously.

What is Myasthenia Gravis?
 Autoimmune condition with antibodies targeting the post-synaptic Ach receptors
  of the neuromuscular junction
 Resulting in progressive muscle weakness with use of the muscle and recovery of
  strength after a period of rest
 Weakness experienced once number of receptors is 30% or less

How common is the thymus involved?
 75% of cases of which 15% are thymomas and 85% are thymic hyperplasia

What are the common presentations?
  Age
     2 peaks
        20 to 30 years old with female predominance
        >50 years old with male predominance
 Ptosis, diplopia
 Dysarthria, difficulty swallowing (isolated bulbar muscles involvement occurs in
 Generalised weakness or reduced exercise tolerance
 Respiratory failure in 1%
 Tends to occur extraocular muscles first, then to facial to bulbar and to limbs and

What can exacerbate MG or precipitate crisis?
 Non compliance to medications
 Infection
 Emotions
 Drugs
   Antibiotics: aminoglycosides, tetracyclines, macrolides and fluoroquinolones
   CVS : Beta blockers, Calcium channel blockers (verapamil)
   Others : Chloroquine, quinidine, procainamide, Li, Mg, Prednisolone,
      quinine(in gin tonic drinks), penicillamine

What is cholinergic crisis?
 Can cause confusion between myasthenic crisis from cholinergic crisis
 Results from excess of cholinesterase inhibitors such as neostigmine and
 Causes flaccid paralysis and SLUDGE (Miosis, salivation, lacrimation, urinary
  incontinence, diarrhea, gastrointestinal hypermotility and emesis)
How would you investigate?
 Blood Ix
   AchR Ab
       Positive in 80% with generalised MG
       Positive in only 50% with ocular involvement only
       also present in 90% of patients with penicillamine induced MG
   Antistriated musce Ab
   Anti Muscle specific kinase Ab (Anti MuSK Ab – positive in patients with
      AchR Ab –ve)
   FBC to rule out infection
 Imaging
   CXR – thymus (anterior mediastinal mass), aspiration pneumonia
   CT for thymus
 Tensilon test
   Dx and distinguishing from cholinergic crisis
       Edrophonium (T1/2 10 mins)
       Look for objective improvement in ptosis (require observer)
       Cardiac monitoring for bradycardia and asystole (Rx with atropine)
       1 mg test dose and up to 10 mg
       In cholinergic crisis, will get increased salivation etc
       Note that in ALS, improvement in muscle weakness also occurs
 Ice Pack test
   Ice applied with glove to eyelids for 2 mins
   Improvement in ptosis is dx (positive in 80%)
 Electrodiagnostic studies
   Repetitive nerve stimulation test – shows a decrease in the compound muscle
      action potential by 10% in the 4th or 5th response to a train of nerve stimuli
   Single fibre nerve electromyography – evidence of neuromuscular blockade
      with increased jitter

How do you grade the severity of the weakness?
 Myasthenia Gravis Foundation of America
         o Grade 1 – affects the ocular muscles only
         o Grade 2 – mild weakness affecting muscles other than ocular muscles
                 2A – Affects the limb and axial muscles
                 2B - Affects the respiratory and bulbar muscles
         o Grade 3 – moderate weakness (3A AND 3B)
         o Grade 4 – Severe weakness (4A and 4B)
         o Grade 5 – Intubation required
 Osserman’s grading
         o I: Ocular
         o II A: Mild generalised with slow progression
         o II B: Moderate generalised
         o III: Acute fulminant MG
         o IV: late severe MG (takes 2 yrs to progress from I to II)

How would you manage?
 Emergencies in crisis (ABC)
 Treat exacerbating factors
   Stop medications that can exacerbate
     Treat fever with antipyretics
     Treat infections
   Oral pyridostigmine, neostigmine
   Steroids, azathioprine, cyclosporine
   Plasmapheresis
   IVIG
   Thymectomy

What are the complications?
 Myasthenic crisis
   Severe exacerbation of MG
   10% require intubation
 Treatment complications
   Cholinergic crisis
   Cx of medications

What is Eaton-Lambert syndrome?
 Myasthenic disorder associated with malignancy such as small cell ca of lung
 Affects the proximal (especially the pelvic girdle and thigh) and truncal
   musculature; bulbar muscles is rarely involved
 Improves with exercise
 Presence of Abs to calcium channels

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