Motor Neuron Disease Flashcards
1
Q
Outline the pathology of motor neuron disease
A
Abnormal ubiquination produces collections of abnormal proteins
Abnormal glutamate signalling mediates excitotoxic damage motor neurons
Neurofilaments and microfilaments damaged by oxidative stress
2
Q
How does MND present?
A
UMN and LMN signs
Starts in a single limb and spread to other limbs
3
Q
Which kind of MND has a poor prognosis?
A
Pseudobulbar
4
Q
How is MND investigated?
A
MRI to exclude lesions
Fasciculations on EEG
Nerve condition studies (normal)
5
Q
How is MND treated?
A
Riluzole alters disease progression for a few months
