A rare neurological disease, Bickerstaff syndrome is characterized by muscle weakness and ocular paralysis.
Definition: what is Bickerstaff Syndrome?
Bickerstaff syndrome or Bickerstaff brainstem encephalitis is a rare neurological disease which is characterized by a paralysis of eye muscles and ataxia (lack of coordination of movements). It is often triggered by previous infections and can be considered a atypical form of Guillain-Barré syndrome (GBS) because the two diseases share clinical characteristics.
What causes Bickerstaff Syndrome?
It is usually caused by a viral or bacterial infection which triggers an abnormal reaction in the immune system which then begins to attack the nervous system. Among the infections already associated with this syndrome:
- herpes simplex virus
- the Epstein-Barr virus,
- the varicella zoster virus
- the measles virus
- Salmonella typhi (typhoid fever bacteria)
- Mycoplasma pneumoniae (bacteria responsible for respiratory tract infections)
- Campylobacter jejuni (bacteria that can cause GBS)
“It has been reported to occur following infections of the upper respiratory tract or gastrointestinal tract. Although the exact disease mechanism is not fully understood, it is associated with the presence of the anganglioside antibody, anti-GQ1b” according to the Rare Disease Portal (Orphanet).
What are the symptoms of Bickerstaff Syndrome?
Symptoms of Bickerstaff syndrome can vary from person to person in severity and combination. This could be:
- Paralysis for the eye muscles
- Muscular weakness in the arms and legs
- Vivid and exaggerated reflexes
- Abnormal foot response to stimulation
- Sensory disorders (tingling, numbness)
- Ataxia or loss of coordination movements
In a study published in the Journal of neurology, neurosurgery and psychiatry (BMJ), a 17-year-old developed fever and diarrhea, followed by unsteady gait, dizziness and vomiting. The patient was drowsy and had a facial paralysis and mild weakness in all four limbs. He also reported tingling sensations in the hands and feet.
The diagnosis is based on the clinical results and observation of symptoms described. The doctor collects the patient’s medical history. Blood tests such as testing for anti-GQ1b IgG antibodies and cerebrospinal fluid tests may be performed to look for signs of inflammation or other abnormalities. Electroencephalogram assesses brain activity and can confirm the diagnosis.
What are the treatments for Bickerstaff Syndrome?
The treatment is based on the use of two main methods: immunotherapy with intravenous immunoglobulin (IVIg) and plasma exchange (or plasmapheresis). Plasma exchange involves removing “diseased” plasma and replacing it with healthy plasma or a replacement solution. This technique aims to rid the plasma of toxic substances such as abnormal antibodies linked to the disease. The medical profession can prescribe pain medication and in the event of serious complications, hospitalization may be necessary.
How does the disease progress?
The evolution is generally positive with “complete remission of symptoms within 6 months in more than half of patients with appropriate treatment” indicates Orphanet. Some patients may have after-effects in rarer cases.
- Bickerstaff brainstem encephalitis, Orphanet, November 2023
- National Diagnostic and Care Protocol (PNDS) Guillain-Barré Syndrome, HAS, September 2021
- Bickerstaff brainstem encephalitis associated with cytomegalovirus infection, BMJ, May 2000
- Fisher syndrome or Bickerstaff brainstem encephalitis? Anti-GQ1b IgG antibody syndrome involving both the peripheral and central nervous systems, BMJ, 2012