Motor Neuron Disease

Question 1

What are other names for motor neuron disease?

Answer 1

Lou Gehrig’s Disease and Amyotropic Lateral Sclerosis )(ALS)

Question 2

What is motor neuron disease?

Answer 2

Progressive neurodegenerative disease in which neurons of the cerebellum, brainstem and spinal cord become damage. Leads to muscle weakness, paralysis and respiratory failure

Question 3

What neurons are affected?

Answer 3

cerebellum, brainstem and cerebral hemispheres

Question 4

What pathways are impacted by neuron degeneration?

Answer 4

corticospinal, lower motor neuron and corticobulbar

Question 5

What is the corticospinal pathway?

Answer 5

axons of upper motor neurons go from primary motor cortex and descend into the brainstem and spinal cord and directly synapse with lower motor neurons that control skeletal muscle

Question 6

What is the corticobulbar pathway?

Answer 6

axons synapse with lower motor neurons in the motor nuclei of cranial nerves supplying eyes, jaw, face and some muscles of the neck and pharynx

Question 7

How many people die and are diagnosed with/from motor neuron disease in Australia each day?

Answer 7

2 for each

Question 8

What is the average life expectancy?

Answer 8

27 months

Question 9

What is the prevalence in Australia?

Answer 9

1,900 (approx)

Question 10

What is the incidence rate of motor neuron disease as according to the AIHW?

Answer 10

Increasing over last decade - 592 people died in 2001 compared with 787 in 2013

Question 11

How many people have ALS worldwide?

Answer 11

4 - 7 out of every 100,000

Question 12

What gender is more effected by ALS?

Answer 12

Males (20% more for males)

Question 13

How many cases of ALS are sporadic compared to familial?

Answer 13

90% sporadic, 5-10% familial

Question 14

How many people in the USA have ALS?

Answer 14

30,000

Question 15

What are the general symptoms of ALS?

Answer 15

muscle aches, cramps and twitching; clumsiness and stumbling; weakness or changes in hands, arms, legs and voice; slurred speech, difficulty swallowing or chewing; fatigue; muscle wasting; weight loss; emotional liability; cognitive change and respiratory changes

Question 16

What pattern are symptoms usually displayed?

Answer 16

distal to proximal

Question 17

What are the three areas symptoms can be attributed to?

Answer 17

lower motor neuron, corticospinal and corticobulbar

Question 18

What are some lower motor neuron symptoms?

Answer 18

focal and multifocal weakness, atrophy, cramps and muscle twitching

Question 19

What are the corticospinal symptoms?

Answer 19

spasticity and hyperresponsive reflexes

Question 20

What are the corticobulbar symptoms?

Answer 20

dysphagia and dysarthria

Question 21

What is sialorrhea?

Answer 21

excess drooling

Question 22

What is fasciculation?

Answer 22

muscle twitching

Question 23

What are the early symptoms of ALS?

Answer 23

stumbling, loss of dexterity, weakness of hand muscles, slurring of speech, difficulty swallowing, cramps and muscle twitching, hyperactive reflexes

Question 24

What are the late symptoms of ALS?

Answer 24

paralysis, breathing difficulties, fatigue, insomnia, cognitive skills (mild), behavioural change, fronto-temporal cognitive changes in some cases, excess laughing or crying, pain and discomfort

Question 25

What is unnaffected by ALS?

Answer 25

senses of sight, hearing, smell, taste and touch as well as bowel, bladder and sexual dysfunction

Question 26

What is the aetiology of ALS?

Answer 26

viruses, toxins, chemicals by which nerve cells are controlled and communicate, genetic factors, immune mediated damage, nerve growth factors and growth, repair and ageing of motor neurons

Question 27

What are sporadic cases of ALS hypothesized to be caused by?

Answer 27

o No known cause for sporadic ALS. Viral, retroviral or environmental causes are hypothesized. May also be due to occupational exposure to chemicals or electromagnetic fields, lead exposure, cigarette smoking and dietary consumption of increased fat and glutamate intake associates with risk and fibre has reduced risk

Question 28

What is the life expectancy after diagnosis?

Answer 28

1 - 5 years

Question 29

How many people live for longer than 10 years after diagnosis?

Answer 29

10%

Question 30

How do symptoms present in younger people?

Answer 30

milder symptoms at diagnosis and usually a longer course

Question 31

What is resistance to ALS?

Answer 31

may demonstrate improvement and live longer than ten years

Question 32

How does death usually occur?

Answer 32

respiratory failure

Question 33

What is the cure for ALS?

Answer 33

no known cure

Question 34

How is ALS diagnosed?

Answer 34

blood tests, nerve conduction studies, electromyography and MRI scans

Question 35

What is the medication demonstrating the best efficacy and is the most commonly used?

Answer 35

Riluzole -extends survival 2-3 monthsand may help to remain in milder stage of disease for longer

Question 36

What type of medication is riluzole?

Answer 36

anti-glutamatergic

Question 37

What medications are used to treat symptoms?

Answer 37

antispasmodic medications, nonsteroidal anti-inflammatories and antibiotics

Question 38

What benefits does a multidisciplinary team have on ALS?

Answer 38

longer lives, better quality of life

Question 39

What two procedures are shown to increase quality and (possibly) length of life?

Answer 39

gastrostomy and noninvasive positive-pressure ventilation