Motor neurone disease Flashcards
Would you expect brisk or slow reflexes in MND?
Brisk
What is motor neurone disease also called?
Amyotrophic lateral sclerolsis
Is ALS a disease of upper motor neurones, lower motor neurones or both?
Both
What type of dementia is associated with MND?
Fronto temporal dementia
Is primary lateral sclerosis a disease of upper motor neurones, lower motor neurones or both?
Upper
is progressive muscular atrophy a disease of upper motor neurones, lower motor neurones or both?
Lower
In progressive bulbar palsy a disease of upper motor neurones, lower motor neurones or both?
Lower; bulbar regions
Is pseudobulbar palsy a disease of upper motor neurones, lower motor neurones or both?
Upper; bulbar region
A 76 year old man presents with weakness. he said it first started in his left hand but now he is struggling to move his right hand. His wife reports that he has been acting strangely lately; becoming more rude than usual. One examination he has brisk reflexes and muscle wasting in his hands.
Motor neurone disease
How does progressive muscular atrophy present?
Since it is a pure lower motor neurone problem it presents with
- Weakness
- Wasting
- Fasiculations
Usually starting in one limb and then moving to adjacent spinal segments.
A 65 year old lady presents with difficulty swallowing. She also seems to be having problems speaking. What type of MND could this be?
Progressive bulbar/pseudobulbar palsy
What nerves does progressive bulbar palsy affect?
Cranial nerves only
What is the average survival time in MND?
3 years
Where is the most common starting point for MND?
Extremities (more often the upper limbs)
Does bulbar onset MND remain confined to the bulbar region?
No. It will always generalize into ALS
What is primary lateral sclerosis?
Progressive muscle weakness in vouluntary muscles
A rare neuromuscular disorder that causes progressive weakening and wasting of the muscles in the arms and legs in particular. It only affects males and is slowly progressive meaning life expectancy is near normal.
Kennedys disease
A rare neuromuscular disease that causes progressive muscle weakness in voluntary muscles. It only affects upper motor neurones and there is no degeneration so spinal motor neurones or muscle wasting. Patients will also have spasticity and hyperreflexia. Life expectancy is near normal.
Primary lateral sclerosis
What other neurological disease is often seen with motor neurone disease?
Fronto - temporal dementia
A lady presents with her husband. He says she has started showing some behavioural abnormalities: dysinhibition, decreased impulse control, increased food intake, weight gain, socially inadequate, positive mood, no insight
Fronto temporal dementia
18 months prior to onset of motor symptoms behavioural abnormalities: dysinhibition, decreased impulse control, increased food intake, weight gain, socially inadequate, positive mood, no insight. She has now developed right thigh weakness and a left sided foot drop.
Fronto temporal dementia and motor neurone disease
A rare type of motor neurone disease that only affects the lower motor neuorones. Symptoms are usually of atrophy, fasiculations and muscle weakness. No spasticity or hyperreflexia.
Progressive muscular atrophy
A rare subtype of MND is which you get atrophy, fasiculation and muscle weakness in the arms only
Flail arm syndrome
A rare subtype of MND is which you get atrophy, fasiculation and muscle weakness in the legs only
Flail leg syndrome
Is spasticity an upper or lower motor neurone sign?
Upper
Is the babinski sign an upper or lower motor neurone sign?
Upper
Is hyperreflexia an upper or lower motor neurone sign?
Upper
Is a clonic jaw jerk an upper or lower motor neurone sign?
Upper
Are pseudobulbar signs due to upper or lower motor neurone problems?
Upper
Is severe muscle weakness an upper or lower motor neurone sign?
Lower (moderate weakness seen in upper motor neurone problems)
Is hyporeflexia seen in upper or lower motor neurone problems?
Lower
Are fasiculations seen in upper or lower motor neurone problems?
Lower
Is muscle hypotonicity seen in upper or lower motor neurone problems?
Lower
What is the average age of diagnosis in MND?
65
