Week 8 - GBS, MND, ME

Question 1

What is the underlying pathology of GBS?

Answer 1

Multifocal inflammation and de-myelination throughout the PNS with secondary axonal degeneration in most severe

Question 2

What is the most common disease course?

Answer 2

Ascending (begins in legs often distal to proximal body segments)
Nadir reached in 2-4 wk
Plateau for 4-6 week up to 1y
Recovery can start from wk 4 over several months

Question 3

Explain the medical management of GBS

Answer 3

Steroids alone ineffective
Plasma exchange
Gamma Globulin

Question 4

What is MND?

Answer 4

Progressive degeneration of cortical, brain stem and spinal motor neurone, both upper and lower motor neurones

Question 5

What are the 4 main types of MND and their prognosis?

Answer 5

Amyotrophic lateral sclerosis - 2-5y (UMN+LMN)
Progressive bulbar palsy - <2y (LMN)
Progressive muscular atrophy - 5-10 y (LMN)
Primary lateral sclerosis - >10 y (UMN)

Question 6

What is ME?

Answer 6

Inflammation of the brain and muscles. Defining symptom PEM.

Question 7

Describe the clinical signs of GBS

Answer 7

Rapidly evolving symmetrical limb weakness
Loss of tendon reflexes
Absent or mild sensory signs
Variable autonomic dysfunction

Question 8

Name the 3 main types of GBS

Answer 8

Acute inflammatory demyelinating polyradiculopathy - most common
Miller Fisher Syndrome
Acute motor axonal neuropathy and acute motor sensory axonal neuropathy

Question 9

What are the signs and symptoms of MND?

Answer 9

Dysphagia
Dysarthria
Weakness
Spasticity
Hyperreflexia
Clonus
Muscle atrophy
Fasciculation
Dyspnoea