MND

Question 1

Outline the pathophysiology of MND?

Answer 1

Degeneration of upper and lower motor neurones

Extremely poor prognosis

Question 2

What are the signs and symptoms of MND?

Answer 2

• weakness
• wasting
• increasing loss of mobility
• difficulties with speech, swallowing, breathing
• cognitive impairment

DOES NOT affect the senses or bladder/bowel

Question 3

How should MND be fully investigated?

Answer 3

Clinical dx = presence upper/lower neuron signs, disease progression, absence of any other explanation

EMG (electromyography - muscle response to electricity) = diffuse, ongoing, chronic denervation

Neuroimaging = normal, rule out ALS

Question 4

Outline the management of MND

Answer 4

Riluzole = antiglutamate that slows progression

Dysphagia = diet modification, percutaneous endoscopic gastrostomy (PEG) tube

Respiratory failure = bilevel +ve airway pressure (BiPAP)

No cure, poor prognosis

Question 5

Outline the 3 patterns of MND

Answer 5

Amyotrophic lateral sclerosis = UMN + LMN, limb weakness and wasting, rapidly changing emotions, most common type

Progressive muscular atrophy = LMN disorder, progressive weakness, atrophy, fasciculation, slow rates of progression, longer survival

Progressive bulbar palsy = UMN + LMN, speech and swallowing muscle weakness and wasting, limbs affected later