Motor Neurone Disease

Question 1

Are fasciculations present in LMN disease?

Answer 1

YES

Question 2

How do reflexes compare in UMN and LMN?

Answer 2

UMN - hyperreflexia

LMN - hyporeflexia

Question 3

Babinski’s sign is not present in UMN - T/F?

Answer 3

FALSE - it is present

Question 4

Genetic background on ALS

Answer 4

the protein superoxide dismutase has been seen on chromosome 21

Question 5

most likely individual group to get ALS

Answer 5

50-75
M>F
Caucasians

Question 6

Amyotrophic lateral sclerosis clinical features

Answer 6

upper & lower motor symptoms

(Late) - dysarthria, dysphagia, dyspnoea & recurrent pneumonia

Question 7

is the distribution of upper & lower motor symptoms in ALS symmetrical?

Answer 7

No, it’s asymmetrical

Question 8

Name all the different types of motor neurone diseases

Answer 8

Amyotrophic lateral sclerosis (ALS)
Progressive muscular atrophy
Primary lateral sclerosis
Progressive bulbar/pseudobulbar palsy

Question 9

what type of motor neurone disease presents with purely UMN signs?

Answer 9

primary lateral sclerosis

Question 10

what is the difference in bulbar and pseudobulbar?

Answer 10

bulbar = LMN affecting CN 9, 10, 12 
pseudobulbar = UMN affecting CN9, 10, 12

Question 11

what conditions could lead to bulbar palsy?

Answer 11

Guilian -Barre
Myasthenia Gravis
ALS

Question 12

what are the symptoms of pseudobulbar palsy?

Answer 12

dysphagia, slow speech, brisk jaw jerk reflex

Question 13

Management of motor neurone disease

Answer 13

Communication needs (SALT)

Nutritional needs (dieticians)

Respiratory needs (BIPAP VENTILATION AT NIGHT)

Question 14

Pharmacological management of MND

Answer 14

Riluzole

Question 15

MOA of riluzole

Answer 15

blocks glutaminergic neurotransmission in the CNS

Question 16

complications of the management of MND

Answer 16

depression
weight loss & malnutrition (due to dysphagia)
respiratory failure