Guillain Barre Syndrome Flashcards
What is Guillain Barre syndrome?
It is defined as an immune mediated demyelination of the peripheral nervous system, resulting in an acute paralytic polyneuropathy
What is the cause of Guillain Barre syndrome?
Infection
What three infections are most commonly associated with Guillain Barre syndrome?
Campylobacter jejuni
Cytomegalovirus
Epstein Barr virus
What is the most common infection that causes Guillain Barre syndrome?
Campylobacter jejuni
Describe the pathophysiology of Guillain Barre syndrome
It is thought to occur due to molecular mimicry, in which immune B-cells create antibodies against infectious antigens that cross-match with the ganglioside proteins located on nerve cells
This means that the anti-ganglioside antibodies (anti-GM1) can target the myelin sheath of the motor nerve cell or the nerve axon itself
What cells are affected in Guillain Barre syndrome?
Schwann cells
What hypersensitivity reaction occurs in Guillain Barre syndrome?
IV
What are the eight clinical features of Guillain Barre syndrome?
Progressive, Ascending, Symmetrical Flaccid Weakness (Legs - Arms)
Hyporeflexia
Neuropathic Back/Leg Pain
Respiratory Muscle Weakness
Distal Paraesthesia
Urinary Retention
Gastroenteritis Features
Cranial Nerve Involvement
What are the two features gastroenteritis in Guillian Barre syndrome?
Diarrhoea
Vomiting
What are the three features of cranial nerve involvement in Guillain Barre syndrome?
Diplopia
Bilateral facial nerve palsy
Oropharyngeal weakness
How long do the clinical features of Guillain Barre syndrome take to occur following infection?
4 weeks
How long does it take for the clinical features of Guillain Barre syndrome to peak?
2-4 weeks
In most cases how is Guillain Barre syndrome diagnosed?
Clinically
What criteria is used to assist in the clinical diagnosis of Guillain Barre syndrome?
Brighton criteria
What two investigations are used to confirm a diagnosis of Guillain Barre syndrome?
Lumbar puncture
Nerve conduction studies
What is a lumbar puncture?
It involves the insertion of a needle into the subarachnoid space of the spinal cord, specifically between L3 and L4 vertebrae, to collect a sample of CSF
What is the sign of Guillain Barre syndrome on lumbar puncture?
Albuminocytologic dissociation
What is albuminocytologic dissociation?
This is when there are increased protein levels, with a normal white blood cell count and glucose levels
What are nerve conduction studies?
They measure how fast an electrical impulse is transmitted through the nerve
What are the three signs of Guillain Barre syndrome on nerve conduction studies?
Decreased Motor Nerve Conduction Velocity
Prolonged Distal Motor Latency
Increased F Wave Latency
What is the most appropriate management step in suspected cases of Guillain Barre syndrome?
Secondary Care Admission
When do we need to supportively manage Guillain Barre syndrome?
In severe cases, where respiratory failure develops
How do we supportively manage Guillain Barre syndrome?
Intubation
Ventilation
ITU Admission
What are the three pharmacological management options for Guillain Barre syndrome?
IV Immunoglobulins
Plasma Exchange
VTE Prophylaxis
How long can the recovery period last in Guillain Barre syndrome?
Months to years
What percentage of patients fully recover from Guillain Barre syndrome?
80%
What percentage of patients are left with neurological disability from Guillain Barre syndrome?
15%
What percentage of patients die from Guillain Barre syndrome?
5%
What is the most common cause of death in Guillain Barre syndrome?
Pulmonary embolism
What is Miller Fisher syndrome?
It refers to a variant of Guillain Barre syndrome, in which anti-ganglioside Q1b antibodies are formed
What are the four clinical features associated with Miller Fisher syndrome?
Ophthalmoplegia
Areflexia
Ataxia
Descending Muscle Weakness (Arms - Legs)
What muscles are affected first in Miller Fisher syndrome?
Eye muscles