Myogenic palsies Flashcards
what is meant by myogenic
it is something to do with the muscle itself and the nerves are not affected
list 4 myogenic conditions
- Myasthenia Gravis
- Chronic Progressive External Ophthalmoplegia (CPEO)
- Orbital myositis
- Rhabdomyosarcoma
what type of condition is myasthenia gravis
what does it affect
what is the prevalence
how serious is this condition
- autoimmune condition
- affects the striated muscles in the body e.g. respiratory system
- 1 / 10,000 in the UK
- Life threatening disease
what is myasthenia gravis classified into
- paediatric:
neonatal
congenital
juvenile - adult
ocular
generalised - drug related
how is ocular myasthenia gravis a variable condition and what what age can it come about
- one day the eye can be turning in and he next day it can be turning out etc
- ocular can start at any age, so theres no specific age group
what is meant by neonatal myasthenia gravis
what is the outcome for these patients
- Born to myasthenia mother
- Disease self limiting (in 6 weeks approx.)
what is meant by congenital myasthenia gravis
Infants born to non myasthenic mothers
what is meant by juvenile myasthenia gravis
what is different between this and adult type
- Develop in infancy
- More spontaneous remissions than adults
what is ocular myasthenia limited to in adults
and what sign shows that it is unlikely to become generalised
- Limited to eye muscles
- If still limited to eye muscles after 2 years unlikely to become generalised
what is affected in generalised myasthenia gravis in adults
Excessive fatigability of striated muscle including respiratory muscles
how many % of ocular myasthenia patients go on to become generalised
10%
what happens in the muscles to cause myasthenia gravis
- Myasthenic patients develop antibodies to the receptor sites at the muscle end plate
- Antibodies prevent the acetylcholine from binding
- Reducing the take-up of the neurotransmitter
- Acetylcholine continues to be released until the supply exhausted and the muscle fatigues
what are the 5 systemic signs and symptoms of general myasthenia gravis and explain each one
- General
Fatigue
Lack of energy - Limb girdle
Difficulty getting out of a chair
Difficulty climbing stairs - Facial
Lack of facial expression - Bulbar
Problems with speech - Respiratory
Problems breathing
list the 3 ocular signs of myasthenia gravis
- Ptosis
- Diplopia
- Orbicularis weakness
how will you test if a patient is suffering from general fatigue due to general myasthenia gravis in practice
and how may a patient describe their symptoms of fatigue which may give clues to myasthenia gravis
- ask px to put their arms out, you push down on them and see how much the px can take
- px will say they don’t have the strength to push them selves up off the chair
- if a px says they get breathless but don’t have any asthma or respiratory disorders
when does the symptoms of myasthenia gravis tend to occur which gives clues to the condition
- their symptoms of fatigue occur after exercise or a hot shower
- their symptoms will get worse e.g. their ptosis at the end of the day, they can’t keep their eyes open
what is a ddx of ocular myasthenia gravis
IR palsy
explain how you test is a patient is suffering from ocular myasthenia in practice
by testing their orbiculares weakness
the px with the frontal muscle to try and keep their eyes open
if you want to establish that a px has a ptosis due to myasthenia gravis
in clinic, first thing to do is suspend all over actions of the frontalis muscle but getting your thumb and pressing it onto their forehead, this immediately takes the frontal out of play
the px is to look straight ahead and you want them to elevate their eyes as your looking for the Ach receptors to build up
so you can see when the px is looking up that their lids will start to come down and when looking straight ahead, they will have ptosis
describe 5 signs about the ptosis that describes ocular myasthenia gravis
- Often the initial sign
- Usually bilateral often asymmetrical
- Increases with fatigue
- Frontalis over-action is often seen in attempt to elevate eye
- Lid retraction occasionally in an attempt to elevate the more ptotic eye
what should you ask the px if you detect ptosis
ask about the diurnal variation of the ptosis e.g. what time of day does it get worse
how is cogan’s lid twitch test carried out in patients with ocular myasthenia gravis
- Request patient to depress their eyes for 15 seconds
- Lid movement follows eyes movement on depression
- Return eyes to primary position
- The lid initially moves above its previous level
- Returns to its original ptotic position