What is Bickerstaff’s Brainstem Encephalitis, a rare disorder reported in Pune, alongside rising Guillain-Barré syndrome cases?

What is BBE and its symptoms?

BBE is an acute onset, rapidly progressive, post-infectious demyelinating neurological illness, said Dr Sudhir Kumar, consultant neurologist, Apollo Hospitals, Hyderabad.

“Common symptoms include imbalance while walking (ataxia), ophthalmoplegia (double vision and other visual disturbances), drowsiness, and weakness of limbs,” said Dr Kumar.

Symptoms occur acutely and get worse over a few days, he added.

“Most patients report mild respiratory infections (such as flu) or a diarrheal illness 1-2 weeks before the onset of neurological symptoms. Although the exact pathogenesis remains unclear, it is presumably related to an immune reaction triggered by a previous infection from pathogens like Campylobacter jejuni, Mycoplasma pneumonia, or Haemophilus influenza,” said Dr Kumar.

Here’s what you must note (Photo: Getty Images/Thinkstock)

Similarity and difference between BBE and GBS

BBE has a few similarities to Guillain-Barre Syndrome (GBS). “Both the conditions are post-infectious immune-mediated acute neurological illnesses. The main difference is that GBS predominantly affects the peripheral nervous system, whereas BBE predominantly affects the central nervous system,” said Dr Kumar.

Diagnosis

Diagnosis of BBE can be confirmed by doing an MRI of the brain, which shows the involvement of the brainstem. “Lumbar puncture is done to rule out active infection. Cerebrospinal fluid (CSF) analysis is also similar to GBS – normal or mildly elevated cell counts, elevated protein, and normal sugar,” said Dr Kumar.

Serum antiganglioside antibodies (anti-GQ1b) are found in two-thirds of patients. However, the absence of these antibodies does not rule out the diagnosis, he stressed.

Treatment

Treatment of BBE is similar to GBS. Both intravenous immunoglobulins (IVIG) injections and plasma exchange are effective.

Timely diagnosis and management are the need of the hour to regain movement and coordination, said Dr Pankaj Agarwal, head of the department of neurology, Gleneagles Hospital Parel Mumbai.

“One should not panic as BBE has a good prognosis and is manageable with immunotherapy, plasma exchange, respiratory support if breathing is compromised, physical therapy, and medication for symptom management,” said Dr Agarwal.

Dr Agarwal noted that those with BBE need “the long-term follow-up to avoid any relapses”.

Precautions

Precautions to prevent BBE are similar to those of GBS. Use a face mask outdoors, especially in public or crowded places, take the annual flu vaccine, and consume hygienic food and water.

Stay vigilant and seek medical advice for neurological symptoms such as rapidly progressive leg or hand weakness and new-onset tingling or numbness in limbs, said Dr Agarwal.