Motor Neuron Disease

Question 1

Definition of MND

Answer 1

Upper and lower motor neurons affected

Question 2

How to distinguish MND from MS or polyneuropathy

Answer 2

MND has no sensory loss and no sphincter disturbance

Question 3

How to distinguish MND from myasthenia gravis

Answer 3

MND never affects eye movements

Question 4

Inheritance of MND

Answer 4

Autosomal dominant

Question 5

Types of MND

Answer 5

Amyotrophic lateral sclerosis (ALS)

Progressive bulbar palsy

Progressive muscular atrophy

Primary lateral sclerosis

Question 6

ALS definition

Answer 6

Loss of motor neurons in motor cortex and anterior horn of cord

Question 7

Sx of ALS

Answer 7

Weakness

UMN signs (upgoing plantars)

LMN signs (fasciculations)

Question 8

Progressive bulbar palsy definition

Answer 8

Only affects cranial nerves

Question 9

Types of progressive blubar palsy

Answer 9

Bulbar palsy

Corticobulbar palsy (pseudobulbar palsy)

Question 10

Bulbar palsy definition

Answer 10

Disease of nuclei of cranial nerves 9-12 in medulla

Question 11

Signs of bulbar palsy

Answer 11

LMN lesions of cranial nerves 9-12

Flaccid fasciculating tongue

Jaw jerk normal or absent

Quiet hoarse voice

Question 12

Causes of bulbar palsy

Answer 12

MND

Guillain Barre

Myaesthenia gravis

Question 13

Corticobulbar palsy definition

Answer 13

UMN lesion of muscles of swallowing and talking

Due to bilateral lesions above mid-pons (corticobulbar tracts)

Question 14

Causes of corticobulbar palsy

Answer 14

MS

MND

Stoke

Central pontine myelinolysis

Question 15

Signs of corticobulbar palsy

Answer 15

Slow tongue movements

Slow deliberate speech

Jaw jerk hyperreflexia

Pseudobulbar affect

Question 16

Pseudobulbar affect

Answer 16

Emotional incontinence without mood change

Eg. weeping w/o sorrow

Eg. incongruent giggling

Question 17

Progressive muscular atrophy definition

Answer 17

Anterior horn cell lesion only

Question 18

Signs of progressive muscular atrophy

Answer 18

No UMN signs

Affects distal muscles before proximal

Question 19

Primary lateral sclerosis definition

Answer 19

Loss of Betz cells in motor cortex

Question 20

Signs of primary lateral sclerosis

Answer 20

UMN signs

Spastic leg weakness

Pseudobulbar palsy

No cognitive decline

Question 21

Association to MND

Answer 21

Fronto-temporal dementia

Question 22

Classic MND patient

Answer 22

> 40 yrs

Stumbling spastic gait

Foot drop

Proximal myopathy

Question 23

Diagnostic criteria for MND

Answer 23

Definite

Probable

Probably with lab support

Possible

Suspected

Question 24

Definite diagnostic criteria for MND

Answer 24

LMN + UMN signs in 3 regions

Question 25

Probable diagnostic criteria for MND

Answer 25

LMN + UMN signs in 2 regions

Question 26

Probably with lab support diagnostic criteria for MND

Answer 26

LMN + UMN signs in 1 region OR UMN signs in 1 or more regions + EMG shows acute denervation in 2 or more limbs

Question 27

Possible diagnostic criteria for MND

Answer 27

LMN + UMN signs in 1 region

Question 28

Suspected diagnostic criteria for MND

Answer 28

UMN or LMN signs only 1 or more regions

Question 29

Prognosis of MND

Answer 29

Often fatal in 2-4 yrs

Question 30

Mx of MND

Answer 30

MDT Riluzole Propantheline Blend food/NG tube/PEG for dysphagia Non-invasive ventilation

Question 31

Riluzole

Answer 31

Antiglutamatergic drug Prolongs life ~ 3 months Expensive

Question 32

SEs of riluzole

Answer 32

Raises LFTs Vomiting Tachycardia Headache

Question 33

Propantheline use

Answer 33

For drooling in MND