Diagnostic Criteria for Guillain–Barré Syndrome (GBS)

Guillain–Barré syndrome (GBS) is a rare, but potentially fatal, immune-mediated disease of the peripheral nerves and nerve roots that is usually triggered by infections.

Patients with GBS typically present with weakness and sensory signs in the legs that progress to the arms and cranial muscles, although the clinical presentation of the disease is heterogeneous and several distinct clinical variants exist. Diagnosis of GBS is based on the patient history and neurological, electrophysiological and cerebrospinal fluid (CSF) examinations. Other diseases that have a similar clinical picture to GBS must be ruled out. Electrophysiological studies provide evidence of PNS dysfunction and can distinguish between the subtypes of GBS: acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN) and acute motor sensory axonal neuropathy (AMSAN). Disease progression can be rapid, and most patients with GBS reach their maximum disability within 2 weeks.

Diagnostic criteria for Guillain–Barré syndrome
This box lists the diagnostic criteria for Guillain–Barré syndrome (GBS) developed by the National Institute of Neurological Disorders and Stroke (NINDS) and subsequently modified in a review paper. We have added some features that cast doubt on the diagnosis, which were not mentioned in the original criteria, and have made some adaptations to improve readability. These criteria are not applicable to some of the specific variants of GBS.

Features required for diagnosis

  • Progressive bilateral weakness of arms and legs (initially only legs may be involved)a
  • Absent or decreased tendon reflexes in affected limbs (at some point in clinical course)a

Features that strongly support diagnosis

  • Progressive phase lasts from days to 4 weeks (usually <2 weeks)
  • Relative symmetry of symptoms and signs
  • Relatively mild sensory symptoms and signs (absent in pure motor variant)a
  • Cranial nerve involvement, especially bilateral facial palsya
  • Autonomic dysfunction
  • Muscular or radicular back or limb painb
  • Increased protein level in cerebrospinal fluid (CSF); normal protein levels do not rule out the diagnosisb
  • Electrodiagnostic features of motor or sensorimotor neuropathy (normal electrophysiology in the early stages does not rule out the diagnosis)b

Features that cast doubt on diagnosis

  • Increased numbers of mononuclear or polymorphonuclear cells in CSF (>50 × µl)
  • Marked, persistent asymmetry of weakness
  • Bladder or bowel dysfunction at onset or persistent during disease courseb
  • Severe respiratory dysfunction with limited limb weakness at onsetb
  • Sensory signs with limited weakness at onseta
  • Fever at onset
  • Nadir <24 hb
  • Sharp sensory level indicating spinal cord injurya
  • Hyper-reflexia or clonusb
  • Extensor plantar responsesb
  • Abdominal painb
  • Slow progression with limited weakness without respiratory involvement
  • Continued progression for >4 weeks after start of symptomsb
  • Alteration of consciousness (except in Bickerstaff brainstem encephalitis)b

aStatements in NINDS criteria that were adapted by authors to improve readability. bAdditional features which were not included in the NINDS. Note: for clarity, they have omitted ‘Features that rule out the diagnosis’ from the original NINDS criteria for this adapted version.

 

References:

  1. Leonhard SE, Mandarakas MR, Gondim FAA, Bateman K, Ferreira MLB, Cornblath DR, van Doorn PA, Dourado ME, Hughes RAC, Islam B, Kusunoki S, Pardo CA, Reisin R, Sejvar JJ, Shahrizaila N, Soares C, Umapathi T, Wang Y, Yiu EM, Willison HJ, Jacobs BC. Diagnosis and management of Guillain-Barré syndrome in ten steps. Nat Rev Neurol. 2019 Nov;15(11):671-683. [Medline]
  2. Walling AD, Dickson G. Guillain-Barré syndrome. Am Fam Physician. 2013 Feb 1;87(3):191-7. [Medline]

 

Created Oct 21, 2020.