Motor Neurone Disease Flashcards
UMN location
CNS
- brain and SC
LMN location
PNS
- from spinal nerve roots and moving peripherally
What is the positive babinski sign?
Plantars upgoing (extensor plantar response)
UMN signs
- tone
- atrophy
- fasciculations
- reflexes
- plantars
- clonus
- tone = INCREASED
- atrophy = ABSENT
- fasciculations = ABSENT
- reflexes = BRISK
- plantars = UPGOING
- clonus = PRESENT
LMN signs
- tone
- atrophy
- fasciculations
- reflexes
- plantars
- clonus
- tone = DECREASED (flaccid)
- atrophy = PRESENT EARLY
- fasciculations = PRESENT
- reflexes = DECREASED
- plantars = DOWNGOING
- clonus = ABSENT
UMN pattern of weakness
Weak extensors in the arm
Weak flexors in the leg
MND - definition
Rapidly progressive motor neuron degeneration
Variety of different subtypes
Where does MND usually stare? the extremities/core ?
Extremities
e.g. lower or upper limb
MND is usually a mixture of UMN and LMN abnormalities together with sensory disturbance. True or false?
False
- there is NO sensory abnormalities in MND
MND - genetic component
TDP-43
MND - there is a spectrum of different clinical phenotypes with MND at one end of the spectrum and ___ at the other end of the spectrum
Fronto-temporal dementia (FTD)
Classic MND
Amyotrophic lateral sclerosis (ALS)
Classic MND (ALS) - diagnostic criteria
UMN + LMN signs in bulbar and at least 2 of the following spinal regions:
- cervical
- thoracic
- lumbosacral
MND - primary bulbar onset always generalises into amyotrophic lateral sclerosis. True or false?
True
MND - management of bulbar symptoms
Nutritional support
SALT
MND - respiratory onset is common. True or false?
False
- rare
MND - clinical features (broad)
UMN and/or LMN signs with NO sensory problems
There may be a link with cognitive impairment Muscle weakness Speech problems Swallowing difficulty Breathing problems
MND - pattern of muscle weakness
Usually focal onset and then continuous spread.
Usually starts at extremities
What is spasticity and is it a UMN or LMN sign?
UMN sign
Increased tone
What is split hand syndrome?
Typical pattern of atrophy where there is preferential wasting of thinner group
Which presents with more weakness, UMN or LMN dysfunction?
LMN dysfunction
What is the role of imaging in MND?
To rule out other (potentially curable) diseases
MND is incurable. True or false?
True
MND - management
There is no treatment
Which type of MN presents with UMN signs only?
Primary lateral sclerosis
Which type of MND is likely to present with early complications such as pneumonia?
Progressive bulbar palsy
Which type of dementia is MND associated with?
Fronto-temporal dementia
Which type of MND presents with LMN signs only in distal muscles?
Progressive muscular atrophy
Which type of MND presents with LMN signs in the arms and UMN signs in the legs?
Amyotrophic lateral sclerosis
