MND

Question 1

What is it?

What type of motor neurone is affected - UMN/LMN?

What distinguishes it from MS and polyneuropathies?

What distinguishes it from myasthenia gravis?

Answer 1

A cluster of neurodegenerative diseases, characterised by selective loss of neurons in motor cortex, cranial nerve nuclei and anterior horn cells (spinal cord - that’s why LMN).

Both

There is no sensory loss or sphincter disturbances.

MND doesn’t affect eye movement?

Question 2

Common symptoms:

Upper limb and spine effects:

• Grip
• Wrist
• Neck

Lower limb effects:

• Foot
• Gait
• What may they have difficulty doing?

Respiratory and bulbar:

• Speech
• Swallowing
• Breathing
• What type of pneumonia are they likely to have?
• Why do they get headaches in the morning?

What type of dementia does 10-20% of them get?

Answer 2

Weak/clumsy grip
Wrist drop
Neck flexion weakness
-----
Foot drop
Unsteady spastic gait 
Balance problems and difficulty rising from chairs
----
Slow/blurred speech 
Dysphagia 
SOB 
Aspiration pneumonia 

Fronto temporal dementia

Question 3

Mixed upper and lower motor neuron signs:

UMN?
LMN?

Answer 3

Hyperreflexia
Hypertonia

Fasciculations
Muscle atrophy

Low power in both UMN and LMN pathology

Question 4

Types:

(ALS) Amyotrophic lateral sclerosis - most common type:

• Where is the degeneration? - 2
• What is usually affected first?

(PBP) Progressive bulbar palsy - RARE:
- What nerves are affected specifically? - think bulbar

Answer 4

Motor cortex and anterior horn cells

UMN and LMN of cranial nerves 9-12

Question 5

Types:

(PLS) Primary lateral sclerosis:

• Where is the degeneration:
• It only affects 1 type of MN. Which one is it?
• Symptoms

(PMA) Progressive muscular atrophy:

• Where is the degeneration?
• It only affects 1 type of MN. Which one is it?
• Symptoms

Answer 5

Motor cortex

UMN only - spastic paraparesis, pseudobulbar palsy

Anterior horn cells

LMN only - weakness and wasting

Affects distal muscles first (e.g. small muscles in hands and feet)

Question 6

DDx:

Neck flexion weakness - 2 - READ

Foot drop is more commonly from peripheral causes. Name 2?

Answer 6

Myasthenia gravis
Guillain Barre syndrome

Peroneal nerve compression
Sciatica

Question 7

DDx:

What may happen in the cervical spine causing mixed upper/lower motor neuron signs?

What vitamin deficiency can cause this?

Answer 7

Cervical radiculomyelopathy

B12 deficiency

Question 8

Investigations:

What type of diagnosis is made?

What is done to exclude other causes?

What imaging can be done to look for subclinical denervation?

Answer 8

Clinical diagnosis - using signs without evidence of other causes

MRI and LP

Electromyography (EMG) measures muscle response or electrical activity in response to a nerve’s stimulation of the muscle. The test is used to help detect neuromuscular abnormalities. During the test, one or more small needles (also called electrodes) are inserted through the skin into the muscle.

Question 9

What can be used for diagnosis and to monitor disease progression?

Answer 9

Respiratory testing:

• Pulse oximetry (+/- ABG)
• Vital capacity
• Sniff nasal inspiratory pressure (SNIP)

Question 10

Management:

Who should be involved in their care? - think about symptoms

Answer 10

Neurologist 
Physio
OT - adaption for home 
Dietician - aspiration pneumonia 
Speech and language 
Psychology - mind

Question 11

Management:

Limb symptom relief:

• What can be given to relax the muscles? - B, D, D
• What can be given for spasticity?
• What can be given for muscle cramps?
• What can be given for joint pain?

Answer 11

Baclofen
Dantrolene
Diazepam

Gabapentin

Quinine

Analgesia

Question 12

Management:

Bulbar symptom relief:

• What can be done for dysphagia?
• What is glycopyrrolate used for?

Answer 12

Soft food
NG tube
PEG

Drooling

Question 13

Management:

Respiratory symptom relief:

• What type of ventilation be used for respiratory failure or sleep apnoea?
• What are opioids and benzodiazepines used for?

Answer 13

NIV - non-invasive ventilation

SOB

Question 14

Life-prolonging treatment:

What does Riluzole do?

What is done if NIV is no longer sufficient?

Answer 14

Adds 3 months to life - used in ALS - delays the onset of ventilator-dependence or tracheostomy in some people.

NIV or invasive ventilation via tracheostomy

Question 15

Prognosis:

How does death usually occur?

What is the prognosis?

Answer 15

Resp failure

3-5 yrs