Motor Neuron Disease

Question 1

9/10 MND diagnoses are familial and 1/10 are sporadic. True/false?

Answer 1

False - 9/10 are sporadic and 1/10 familial

Question 2

At what age range does sporadic MND tend to occur?

Answer 2

50-75 years

Question 3

ALS is less common in caucasian/non-caucasian populations?

Answer 3

Less common in non-caucasian

Question 4

PLS is a very ____ type of MND. It presents with ______ signs and without muscle ______.

Answer 4

PLS is a very rare type of MND. It presents with UMN signs and without muscle wasting.

Question 5

Progressive Muscular Atrophy is a very ____ type of MND. It presents with ____ signs and with muscle ____ and ____.

Answer 5

Progressive Muscular Atrophy is a very rare type of MND. It presents with LMN signs and with muscle wasting and fasciculations.

Question 6

ALS is the ____ ______ type of MND. It presents with ____ and ____ signs.

Answer 6

ALS is the most common type of MND. It presents with UMN and LMN signs.

Question 7

Describe presentation of ALS patient.

Answer 7

muscle wasting, 
fasciculations,
increased tone,
brisk reflexes,
So UMN & LMN

Question 8

Outline the presentation of progressive bulbar palsy MND?

Answer 8

wasted, fasciculated tongue,
brisk reflex, 
hypotonic nasal speech,
mixed UMN and LMN in mouth,
normal peripheries

Question 9

Which type of MND is fastest killer?

Answer 9

ALS

Question 10

What is PLS and is it common/rare?

Answer 10

Primary lateral sclerosis - is rare

Question 11

List 6 signs of UMN disease.

Answer 11

• spasticity
• stronger reflexes
• increased plantar response (Babinski sign)
• spastic gait
• slow movements
• exaggerated jaw-jerk

Question 12

List 4 signs of LMN disease.

Answer 12

• muscle wasting
• fasciculations
• weakness
• absent/reduced reflexes

Question 13

What is the difference between bulbar and pseudobulbar palsy?

Answer 13

Bulbar is an LMN lesion of CN IX, X and XII and pseudobulbar is UMN lesion of CN IX, X and XII

Question 14

What is MND Catabolism?

Answer 14

sudden weight loss that occurs in 50% patients with MND

Question 15

Bulbar onset occurs in approx. 2%/25%/70% patients?
Limb onset occurs in approx. 2%/25%/70% patients?
Thoracic onset occurs in approx. 2%/25%/70% patients?

Answer 15

Bulbar - 25%
Limb - 70%
Thoracic - 2%

Question 16

Is bulbar onset MND more common in men or women?

Answer 16

Women

Question 17

What are the 3 types of less fatal MND?

Answer 17

• Flail arm syndrome
• Flail leg syndrome
• PLS

Question 18

What is the investigation for MND?

Answer 18

• electrophysiology

- diagnosis of exclusion

Question 19

What type of drug is an option for MND patients and why do most patients refuse it?

Answer 19

Riluzole - it only extends life by 3 months and lots side effects (esp. liver)

Question 20

What is the management for MND patients with dysphagia?

Answer 20

ensuring hydration and nutrition,
saliva,
gastrostomy

Question 21

Name 4 management options for sialorrhoea.

Answer 21

hyoscine/buscopan,
glycopyrronium (especially if cognitively impaired),
botox,
suction

Question 22

Name 2 drugs for muscle cramps.

Answer 22

• quinine

- baclofen

Question 23

Name 4 drugs for muscle spasms.

Answer 23

• baclofen
• tizanidine
• dantrolene
• gabapentin

Question 24

What non-invasive treatment can be offered for respiratory problems?

Answer 24

biPAP mask

Question 25

Approx. what percentage of patients have cognitive impairment with MND?

Answer 25

50%