Motor Neuron Disease and other longterm conditions

Question 1

What is muscular dystrophy?

Answer 1

a group of disorders that result in a progressive loss of muscle mass and consequent loss of strength

Question 2

What is Duchenne muscular dystrophy?

Answer 2

shoulders and arms are held back awkwardly when walking
weak glut muscles so the hip straightens
knees may bend back to take weight
thick lower leg muscles
weak muscles in front of leg causing the foot to drop and tip toe

Question 3

What is dystonia?

Answer 3

describes movement disorders causing uncontrollable muscle spasms and contractions

Question 4

What is motor neurone disease?

Answer 4

Progressive degenerative disorder of cortical, brain stem and spinal motor neurones both upper motor neuron and lower motor neuron

Question 5

What are the signs and symptoms?

Answer 5

Patients typically present with wasting and weakness of the upper limbs more commonly than lower limbs.
Cramps and Fasciculations may precede other motor signs
MND Fasciculations
Speech, swallow and emotional problems

Question 6

How to manage the condition

Answer 6

A person-centred service
Early recognition, prompt diagnosis and treatment
Emergency and acute management
Early and specialist rehabilitation
Community rehabilitation and support
Vocational rehabilitation

Question 7

Pathology of Duchenne Muscular Dystrophy

Answer 7

Muscle fibres replaced by fibrous or fatty tissue

Question 8

Pathology of Myotonic Dystrophy

Answer 8

Disruption in RNA production related to muscle

Question 9

Pathology of Fascioscapular Muscular Dystrophy

Answer 9

Disrupted protein assembly in muscles