section name header

Information

Editors

EmilYlikallio

Guillain-Barré Syndrome (Polyradiculitis)

Essentials

  • Guillain-Barré syndrome (polyradiculitis, polyneuritis) denotes an inflammation of unknown origin of nerve roots that affects mainly the motor nerves and has an ascending course.
  • Remember the possibility of polyneuritis (polyradiculitis) in a patient presenting with limb weakness or numbness.
  • The patient is treated in a hospital where mechanical ventilator treatment is available.
  • See also the Orphanet article ORPHA2103 http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=834.

Epidemiology and aetiology

  • The incidence in Western countries is 0.9-1.9/100 000 inhabitants/year worldwide.
  • Presents in 3 clinical forms: acute (95% of cases), chronic and relapsing.
  • The aetiology is unknown. A preceding infection (campylobacter, cytomegalovirus, Epstein-Barr virus, mycoplasma, zika virus, etc.), vaccination or other immunomanipulation may be identifiable in up to 70% of cases.

Symptoms

  • Ascending, symmetrical symptoms of pins and needles, numbness and muscle weakness starting in the lower limbs and moving upwards.
  • Muscle weakness dominates the clinical picture.
  • About 25-30% of the patients develop respiratory muscle weakness which requires mechanical support for respiration. Respiratory paralysis may develop in hours.
  • Cranial nerve palsy and symptoms of autonomic nervous system (arrhythmia, fluctuations of blood pressure, orthostatic symptoms etc.) may occur.
  • Typically no disturbance of bladder or bowel function
  • Often associated with shoulder and/or back pain

Diagnosis

  • The tendon reflexes are weak or absent (may be normal at the initial phase).
  • Sensory disturbances are only minor.
  • Symmetric muscle weakness
  • Usually no leucocytes in CSF. Sometimes leucocytes may be present, up to 50/mm3 .
  • Increased protein concentration of CSF, even up to 6 000-7 000 mg/l (sometimes a rise in protein content is not seen until during the second week of illness).
  • Spinal MRI may show nerve root enhancement.
  • ENMG findings may confirm the diagnosis but no earlier than three weeks after the onset of illness.
  • In the acute phase, blood tests are beneficial primarily in exclusion diagnostics.

Differential diagnosis

  • In the typical clinical picture, the condition can be recognized on clinical grounds. Otherwise, muscle weakness has several options for differential diagnosis (see Muscle Weakness and Paralysis Symptom).
  • Infections such as acute poliomyelitis or neuroborreliosis (asymmetrical, leucocytosis in CSF)
  • Acute myelitis (upper motor neurone lesion, bladder paralysis)
  • Polyneuropathy associated with diphtheria
  • Botulism
  • Myasthenia gravis Myasthenia Gravis
  • Acute polyneuropathy (e.g. critical illness polyneuropathy, CIP) Polyneuropathies or muscle disease (e.g. necrotizing autoimmune myopathy, NAM)
  • Cauda equina syndrome (saddle anaesthesia as well as disturbance of bladder, bowel and/or sexual functions)

Treatment

Prognosis

  • The progressive phase lasts about 2-4 weeks, the stable phase about 2 weeks, and recovery takes about 6 weeks.
  • The majority of patients recover completely in a year.
  • The condition recurs in fewer than 5% of the patients.
  • In rare cases, chronic inflammatory demyelinating polyneuropathy begins like acute polyradiculitis. Symptom progression over 8 weeks after the onset of the condition may be suggestive of this.
  • Mortality is 3-7%.

Related Keywords

ATC Code:

J06BA02

Primary/Secondary Keywords