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Diagnosing Guillain Barre Syndrome in Zika-affected Haiti

When neurologist Aaron Berkowitz, MD, PhD, arrived in Haiti in early January 2016, the Zika virus had already been identified in many countries in the Americas. Soon after Dr. Berkowitz’s arrival, the Centers for Disease Control and Prevention (CDC) would confirm cases of Zika virus infection in Haiti as well.

Dr. Berkowitz, who leads the Global Neurology Program at BWH, has made frequent trips to Haiti, where he and colleagues from Partners In Health have established the first neurology training program in the country. During his January visit, Dr. Berkowitz and neurology fellow Roosevelt Francois, MD (the first neurology trainee in Haiti), were asked to consult on an unusual case. A young man was unable to move any of the muscles of his face and noted painful tingling in his fingertips and toes. Despite the atypical presentation of symptoms, Dr. Berkowitz quickly recognized what it was: Guillain-Barré syndrome (GBS).

GBS is an umbrella term for a variety of neuropathies that occur after an infection triggers the immune system to attack the body’s nerves. Classic GBS symptoms include numbness, tingling, and weakness, which most commonly affect the arms and legs, but the most severe cases can affect the respiratory muscles, preventing people from breathing.

The patient whom Dr. Berkowitz examined in Haiti had a rarer variant of GBS.

“I attribute my ability to diagnose this case in Haiti to having trained under Allan Ropper, MD,” says Dr. Berkowitz, who considers Dr. Ropper, the Executive Vice Chair of Neurology at BWH, to be a clinical mentor. “In the 1990s, Dr. Ropper described this constellation of symptoms as one of the variants of GBS, known as facial diplegia with acral paresthesias.”

Aaron Berkowitz, MD, PhD, (left), and Roosevelt Francois, MD, (right), spotted symptoms of a rare variant of GBS in a patient with confirmed Zika virus infection in Haiti.

Following in the tradition of clinical mentorship, Dr. Berkowitz took the opportunity to teach Dr. Francois about GBS, noting that this particular variant was quite rare – Dr. Berkowitz had only seen it once before in his career.

Two days later, however, another patient presented at the hospital with the same symptoms.

“After evaluating the patient, Dr. Francois turned to me and said, ‘I know exactly what this is,’” recalls Dr. Berkowitz.

Both patients reported that they had previously experienced headaches and fever, two symptoms often associated with Zika infection. Any infection can potentially trigger GBS in patients, and given the ongoing outbreak of Zika, Dr. Berkowitz and others at the hospital began to suspect a connection. At the time, however, very little had been published in the scientific literature about Zika and GBS.

The first confirmed case of Zika infection in Haiti was reported the next week, and not long after, a blood sample from one of the patients Drs. Berkowitz and Francois saw in Haiti was sent to the CDC and tested positive for Zika infection. Given the patient’s GBS diagnosis, Dr. Berkowitz and his colleagues now consider this to be the index case for Zika-associated GBS in Haiti. Their paper on the case was published in the journal Neurology.

According to the World Health Organization, to date, a total of nine countries or territories have reported an increase in the incidence of GBS and/or laboratory confirmation of Zika virus infection among GBS cases.

Dr. Berkowitz notes that there are very few neurologists in many of the regions that have been most affected by Zika. In Haiti, he says, there is just one neurologist for a population of 10 million people. Given the variations of GBS and the non-traditional symptoms patients can sometimes present, diagnosing GBS will be challenging for clinicians who have not been trained in neurology.