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EmilYlikallio

Guillain-Barré Syndrome (Polyradiculitis)

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.

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.

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

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.

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)

The patient is transferred to hospital with facilities for respirator treatment and, if needed, an intensive care unit.
No glucocorticoids are given in the acute forms Corticosteroids for Guillain-Barré Syndrome.
I.v. massive-dose immunoglobulin therapy Intravenous Immunoglobulin for Guillain-Barré Syndrome or plasmapheresis Plasma Exchange for Guillain-Barré Syndrome in severe cases (walking ability severely impaired, threat of respiratory muscle paralysis) that should be carried out as soon as possible (within the first 2-4 weeks from onset of the disease).
Early intubation of the patient, if there are signs of respiratory failure - PEF monitoring is essential.
The pulse and blood pressure should be monitored for possible autonomic nervous system disorder.
Physiotherapy, when the progression has stopped
Thrombosis prophylaxis for the severely paralyzed.

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%.