The Guillain-Barre syndrome (GBS) or Landry-Guillain-Barre-Strohl syndrome, also known as post-infectious polyneuropathy or acute idiopathic polyneuritis, is an acute acquired, frequently severe, monophasic autoimmune illness of the peripheral nervous system (PNS). GBS manifests itself with the clinical picture characterised by gait disturbance, pain, weakness, rapidly ascending symmetric flaccid muscle paralysis, areflexia with distal predominance (involving lower motor neuron), sensory disturbance, variable autonomic involvement, and increased cerebrospinal fluid protein without pleocytosis.

Diagnostic criteria
Assessment of current diagnostic criteria for Guillain-Barre syndrome

Required features

• Progressive weakness in both arms and legs
• Areflexia (or hyporeflexia).

Features supportive of diagnosis

• Progression of symptoms over days to 4 weeks
• Relative symmetry
• Mild sensory signs or symptoms
• Cranial nerve involvement, especially bilateral facial weakness
• Recovery beginning 2 to 4 weeks after progression ceases
• Autonomic dysfunction
• Absence of fever at onset
• Typical CSF (albuminocytologic dissociation)
• EMG/nerve conduction studies (characteristic signs of a demyelinating process in the peripheral nerves)

Features casting doubt on the diagnosis

• Asymmetrical weakness
• Persistent bladder and bowel dysfunction
• Bladder or bowel dysfunction at onset
• >50 mononuclear leukocytes/mm³ or presence of polymorphonuclear leukocytes in CSF
• Distinct sensory level.

Features that rule out the diagnosis

• Hexacarbon abuse
• Abnormal porphyrin metabolism
• Recent diphtheria infection
• Lead intoxication
• Other similar conditions: poliomyelitis, botulism, hysterical paralysis, toxic neuropathy.

References:

1. Asbury AK, Cornblath DR. Assessment of current diagnostic criteria for Guillain-Barré syndrome. Ann Neurol. 1990;27 Suppl:S21-4. [Medline]
2. Hughes RA, Swan AV, van Doorn PA. Intravenous immunoglobulin for Guillain-Barré syndrome. Cochrane Database Syst Rev. 2010;(6):CD002063. [Medline]

Created Jul 28, 2011.