Guillain-Barre Syndrome

Guillain-Barré Syndrome (GBS)

Definition: an acute, predominantly motor, inflammatory demyelinating polyneuropathy

Aetiology/ risk factors:

-occurs a few weeks (1-3) after an infection

e.g. Camylobacter jejuni (from undercooked chicken)

CMV, mycoplasma, herpes zoster, HIV, EBV

post-vaccinations

-may occur with lymphoma or Hodgkin’s disease

-infective trigger causes antibodies which react with self-antigen on myelin or neurones

-no cause found in 40% (idiopathic)

Epidemiology: incidence = 1-2/100,000/yr (UK)

Affects all age groups

Symptoms:

-may have preceding diarrhoea and vomiting

-progressive symmetrical ascending muscle weakness starts a few weeks after an infection

proximal muscles are more affected e.g. trunk, respiratory, cranial nerves (esp. facial)

Cranial nerve involvement: dysphagia, dysarthria, facial weakness

May advance quickly, affecting all limbs at once, and can lead to paralysis.

-pain common (e.g. back, limbs)

-may also have ascending paraesthesia (pins and needles) or sensory features may be absent

Cranial nerve involvement: dysphagia, dysarthria, facial weakness

Signs:

Motor: hypotonia, flaccid paralysis, arreflexia (typically ascending)

Sensory: impairment of sensation in multiple modalities (typically ascending)

Cranial nerve palsies (less frequently): facial nerve weakness, abnormality of external ocular movements, signs of bulbar palsy

⇒Miller-Fisher variant (rare) = ophthalmoplegia + ataxia + arreflexia

Autonomic dysfunction: sweating, increased pulse, BP changes, arrhythmias

Type II respiratory failure- CO2 flap, bounding pulse, drowsiness

Image result for co2 flap

CO2- flapping tremor, their hands will flap up and down

 

Investigations:

Blood: FBC, ESR, glucose (diabetic neuropathy?), LFT (alcoholic neuropathy?), B12 (deficiency?), electrophoresis, ANA/ANCA (vasculitis?)- to exclude other causes

Anti-ganglioside antibodies- positive in Miller-Fisher variant and 25% of GBS

C.jejuni serology- may consider

Nerve conduction studies: slow conduction, or conduction block but can be normal in early phase of disease

Lumbar puncture: CSF shows raised protein (e.g. >5.5g/L), normal white count, glucose normal

Spirometry, oxygen saturation, ABG- assess for ventilatory weakness, respiratory failure

ECG- arrhythmias may develop

Management:

Acute:

-IV Immunoglobulins (0.4g/kg/24h) for 5 days

Plasma exchange (plasmapheresis) also good

   May reduce duration or severity of disease.

Supportive:

-monitor vital capacity and ECG

-DVT prophylaxis

-Regular physiotherapy with care of pressure areas

-Dysphagia may necessitate nasogastric feeding

-Transfer to ITU if there is respiratory involvement (in severe cases)

-Do 4 hourly forced vital capacity (FVC)

-Ventilate sooner than later

Complications:

-paralysis

-respiratory involvement, respiratory failure

-cardiac arrhythmias

-sepsis e.g. aspiration pneumonia, UTI

-pulmonary embolus

-incomplete recovery

-relapse

-mortality = 10%

Prognosis: progressive phase of up to 4 weeks, followed by recovery

~ 85% make a complete or near-complete recovery (usually within 3-6M)

-10% are unable to walk alone at one year, and have residual neurological disability

-those that are completely paralysed can still make a complete recovery

References: Cheese & Onion, Rapid Medicine
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