Bickerstaff syndrome is a neurological disease that affects the muscles.
Definition: What is Bickerstaff Syndrome?
Bickerstaff syndrome or Bickerstaff brainstem encephalitis is a rare neurological disease characterized by paralysis of the eye muscles and ataxia (lack of coordination of movements). It is often triggered by previous infections and can be considered an atypical form of Guillain-Barré syndrome (GBS) because the two diseases share clinical features.
What causes Bickerstaff Syndrome?
It is usually caused by a viral or bacterial infection that triggers an abnormal reaction in the immune system, which then attacks the nervous system. Infections already associated with this syndrome include:
- herpes simplex virus
- cytomegalovirus,
- Epstein-Barr virus,
- 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 upper respiratory tract or gastrointestinal tract infections. Although the exact pathological mechanism is not fully understood, it is associated with the presence of the anganglioside antibody, anti-GQ1b” according to the Rare Diseases Portal (Orphanet).
What are the symptoms of Bickerstaff Syndrome?
Symptoms of Bickerstaff syndrome can vary from person to person in severity and combination. They may include:
- Paralysis of the eye muscles
- Muscle weakness in arms and legs
- Quick and exaggerated reflexes
- Drowsiness
- Abnormal response of the feet to stimulation
- Sensory disturbances (tingling, numbness)
- Ataxia or loss of coordination of movements
- Confusion
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 facial paralysis and mild weakness in all four limbs. He also reported tingling sensations in his hands and feet.
Diagnosis is based on clinical findings and observation of the symptoms described. The doctor takes a medical history from the patient. Blood tests such as anti-GQ1b IgG antibodies and cerebrospinal fluid tests may be performed to look for signs of inflammation or other abnormalities. An electroencephalogram assesses brain activity and can confirm the diagnosis.
What are the treatments for Bickerstaff Syndrome?
Treatment is based on the use of two main methods: immunotherapy with intravenous immunoglobulin (IVIg) and plasma exchange (or plasmapheresis). Plasma exchange involves removing the “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’s Brainstem Encephalitis, Orphanet, November 2023
- National Diagnostic and Treatment Protocol (PNDS) for 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
