Motor neurone disease Flashcards Preview

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Flashcards in Motor neurone disease Deck (14):
1

What is motor neurone disease?

untreatable and rapidly progressive neurodegenerative condition
-progressive motor poblems with both UMN and LMN features
-no sensory problems
-focal onset and continuous spread

2

What is the average survival time for motor neurone disease?

3 years

3

What is the genetic inheritance of motor neurone disorder

90% sporadic
10% inherited

4

When does MND usually present and what clinical symptoms and signs should make you think of motor neuron disease?

It rarely presents before 40 years

S/S
-fasciculation
-the absence of sensory signs/symptoms*
-the mixture of lower motor neurone and upper motor neurone signs
-wasting of the small hand muscles/tibialis anterior is common


Other features
-doesn't affect external ocular muscles
-no cerebellar signs
-abdominal reflexes are usually preserved and sphincter dysfunction if present is a late feature

5

What are the five different types of MDN?

amyotrophic lateral sclerosis,

primary lateral sclerosis,

progressive (or spinal) muscular atrophy,

progressive bulbar palsy

pseudobulbar palsy

6

Amyotrophic lateral sclerosis - is this common? what signs are seen? In familial cases where is the gene responsible located?

50% patients

typically LMN signs in arms and UMN signs in legs

in familial cases the gene responsible lies on chromosome 21 and codes for superoxide dismutase

7

Primary lateral sclerosis - what signs are seen?

UMN signs only
-leg weakness and is found to have a relatively pure spastic parapesis

8

Progressive muscular atrophy:
What signs are seen?
Which muscles are affected?
prognosis?

LMN signs only
affects distal muscles before proximal
carries best prognosis

9

Progressive bulbar palsy
-what is a bulbar palsy?
-what are the signs/symptoms?
-F:M? what age

bulbar palsy is a lower motor neuron lesion of cranial nerve IX, X and XII
-slurring dysarthria
-dysphagia
-F>M (60-80yrs)

10

Psuedobulbar palsy:
-what is a pseudobulbar palsy?

pseudobulbar palsy = upper motor neurone lesion of cranial nerve IX, X and XII
-increased gag/jaw jerk reflex
-spastic tongue
- palatal movement is absent
-spastic speech (monotonous/slurred/high pitch = donald duck)

11

How is MND diagnosed?
-clinically definitive
-clinically probable
-clinically possible

clinically - diagnosis of exclusion (after ix have ruled out other DDx)

Definitive:
-UMN and LMN signs in bulbar region + 2 or more spinal regions
-OR UMN and LMN signs in 3 spinal regions

Probable:
-UMN and LMN in 2 or more spinal regions with some UMN signs rostral to LMN

Possible:
-UMN and LMN in 1 spinal region
-LMN signs and 2 or more spinal regions
-LMN signs are found rostral to UMN signs

12

What UMN signs exist?

-pseudobulbar affect
-moderate weakness
-spasticity
-hyperreflexia
-extensor (up going) plantar reflexes - babinski sign

13

What LMN signs exist?

-severe weakness
-fasciculations
-muscle cramps
-muscle hypotonicity
-muscle atrophy
-hyporeflexia

14

What is the management of MND?

specialist services: key worker, S+L, nutrition, respiratory needs

Riluzole: prevents stimulation of glutamate receptors, used mainly in amyotrophic lateral sclerosis, prolongs life by about 3 months


Respiratory care:
non-invasive ventilation (usually BIPAP) is used at night
studies have shown a survival benefit of around 7 months