Motor Neuron Disorders

Question 1

Does MND affect the face?

Answer 1

No

Question 2

Does MND affect sphincters?

Answer 2

NO

Question 3

What is the average age of onset for MND?

Answer 3

50-70

Question 4

What is MND?

Answer 4

Cluster of degenerative disorders with selective loss of neurons in the motor cortex, CN nuclei, ant horn cells.
UMN & LMN symptoms

Question 5

What are the 4 patterns of MND?

Answer 5

1. ALS- Amylotrophic Lateral Schlerosis
2. Progressive Bulbar Palsy
3. Progressive Muscular Atrophy
4. Primary Lateral Schlerosis
   Though they often progress from eachother

Question 6

What are symptoms of ALS?

Answer 6

UMN and LMN in limbs ± bulbar weakness

wasting, brisk reflexes, fasciculations and spasticity

Question 7

Progressive Bulbar Palsy nerves involved?

Answer 7

CN 9, 10 , 11, 12

Question 8

Symptoms of progressive bulbar palsy

Answer 8

LMN tongue weakness and fasciculations.

Palate and pharyngeal weakness- dysphasia and dysarthria

Question 9

Progressive Muscular Atrophy what cells affected?

Answer 9

Anterior horn cell lesion

Question 10

Progressive Muscular Atrophy symptoms

Answer 10

LMN limb weakness and wasting, fasciculations and decreased reflexes.
Particularly small muscles of hand

Question 11

Primary Lateral Sclerosis cells affected

Answer 11

Loss of Betz cells in cortex

Question 12

ALS cells affected

Answer 12

Loss of motor neurons in motor cortex and anterior horn

Question 13

Primary Lateral Sclerosis symptoms

Answer 13

UMN: spastic leg weakness and pseudobulbar palsy

Question 14

How do you diagnose MND?

Answer 14

Clinical
MRI
LP to exclude inflammatory cause
EMG

Question 15

Treatments for MND?

Answer 15

Antiglutaminergic RILUZOLE (life expec increased 3 months)
Maintain QOL with MDT
Ventilation in later stage
Analgesia for joint pain
Baclofen
BUscopan for secretions

Question 16

What is life expec with MND?

Answer 16

2-4 years

Question 17

What to MND sufferers die of?

Answer 17

Aspiration Pneumonia