Guillain-Barre Syndrome

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Guillain-Barré Syndrome

Presentation

Symptoms

  • peak age 50-74 (smaller peak between 15 & 35)

  • acute/subacute monophasic illness

    • evolves over few days or 2-4 weeks

  • predominantly motor weakness

    • typically ascending

  • distribution

    • proximal or distal

    • may be generalised leading to need for ventilation

    • facial weakness common

  • sensory

    • distal tingling paraesthesia

  • autonomic disturbance

    • atonic bladder

    • cardiac dysrythmias

    • hypertension

    • postural hypertension

    • distal anhidrosis

Signs

  • papilloedema in small percentage

  • reflexes

    • tendon reflexes depressed

  • only peripheral neuropathy to have effects so far up thorax

Investigations

  • CSF

    • increased protein

  • Nerve conduction studies

    • conduction velocity reduced

Aetiology / Pathology

  • preceding infection 2-3 weeks before onset

  • inflammatory infiltrate leading to myelin breakdown and stripping by macrophages

  • occasionally get axonal degeneration

  • Involves cell-mediated immunity and antibodies

  • association with Hodgkins lymphoma

Differential Diagnoses

  • Lyme disease

    • prominent facial paralysis

    • rdicular pain

    • cellular CSF

  • Porphyric neuropathy

    • neuropsychiatric symptoms prominent

    • abdominal pain

    • purely motor

    • preservation of ankle jerks with loss of knee jerks

  • Diphtheric neuropathy

    • descending polyneuropathy

    • starts as bulbar palsy

Treatment

  • Intensive supportive care

  • NOT steroids (increased risk of relapse)

  • Plasma exchange

  • IV immunoglobulin (0.4g/kg)

 

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