Neurological conditions Flashcards
(21 cards)
what is Duchenne muscular dystrophy?
inherited disease
gradual loss of functional muscle fibres an those muscle fibres are replaced by fat and connective tissue
which muscles weaken first in DMD?
proximal first, then distal
at what age are you unable to walk if you have DMD?
13
what are contractures?
shortening of muscles, becming very stiff
what do a lot of patients with DMD die of?
respiratory failure due to weakened respiratory muscles
what is guillain barre syndrome?
where the immune response is mistargetted - targetted at hosts own nerve tissues instead of the foreign antigens
what causes guillain barre syndrome?
respiratory tract or gastric infection often precedes the onset of symptoms
immunisation (flu jab) and surgery may be precipitating factors
which muscles are affected first in guillain barre syndrome?
begins with lower limb weakness, progresses proximally
how long does it take from onset to peak disability in GBS?
4 weeks
can you recover from GBS?
yes, recovery has the potential to be complete
how is GBS treated?
immunoglobulin therapy
what is motor neurone disease?
a progressive neurodegenerative disease that attacks the upper and lower motor neurones, leading to weakness, wasting of muscle, functional loss of mobility, difficulties with speech, swallowing and breathing
where do symptoms start in MND?
hands and feet
who is MND most common in?
mean age 58, men > women
what is the mean survival from symptom onset in MND?
3-4 years
what does the ability to sustain spontaneous ventilation depend on?
the trial of CNS drive
the capacity of the respiratory muscles
the load that is imposed upon them
what is the drive (in spontaneous ventilation)?
the drive to breathe (CNS)
what is the capacity needed (in spontaneous ventilation)?
the ability of the respiratory muscles to generate pressure
the ability of the pump to do the pumping
strength, endurance, mechanical ability
what is the load required (in spontaneous ventilation)?
the work performed by the respiratory muscles
resistive load - increases in airways resistance
elastic load - lung compliance
what happens in load is greater than capacity in spontaneous ventilation?
hypoventilation which leads to type 2 respiratory failure
why can intubation and ventilation be used for GBS patients?
rests respiratory muscles to allow spontaneous recovery
allows suction of secretions
prevents aspiration and further complications if unable to protect own airway
only used if there is an element of reversibility
