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Mounting Evidence Implicates Zika Virus In Microcephaly And Guillian-Barre Cases

Firm Proof Awaits Results From Case-Control And Cohort Studies

The lack of understanding about Zika virus, and especially the lack of certainty about the cause of the reported increases in microcephaly and Guillian-Barre Syndrome cases in Brazil and other parts of the Americas, have prompted the World Health Organization to declare a global public health emergency.

According to WHO’s Bruce Aylward quoted in the Washington Post, “This is classic ‘building your boat while you sail it’. People said that about Ebola and that was trying to get a bigger sail on the boat. Here we’re still stitching the sail, and we’re not quite sure what kind of sails you really need.”  

Also quoted was David Heymann, London School of Tropical Medicine and Hygiene epidemiologist, who contrasted Zika with Ebola saying the Ebola epidemic was an emergency because of what was known about the disease, whereas Zika is an emergency because of what is unknown.

Burden of Disease

Zika Virus Infections:

The Brazilian Ministry of Health (MOH) has estimated that as few as half a million or as many as 1.5 million infections have occurred in that country. Columbia has reported more than 31,000 suspected cases and Cape Verde more than 7,000. Overall, some experts estimate 3-4 million cases will occur over the next year.

According to WHO, the geographic range of the virus has increased significantly in 2015-16 especially in the Americas with 28 countries or territories now affected.

Cases of Microcephaly:

Brazil has reported approximately 5,280 cases of microcephaly or central nervous system abnormalities as of February, including 108 deaths.

Cases of Guillain-Barre syndrome:

Five countries in the Americas have reported increases in Guillain–Barre syndrome in association with the Zika virus outbreak. These cases in adults have been reported in much lower numbers than the microcephaly cases. The largest number is from Venezuela with 252 cases.

Proving Causality

While it is established that Zika virus is transmitted by the aedes species of mosquito, and that prevention of Zika is theoretically possible with effective mosquito control and/or effective protection against mosquito bites, the cause of the microcephaly and GBS cases is less firmly established.

To truly pin down the role of Zika virus in microcephaly, a collaborative case-control study between CDC, Brazil, and possibly other scientists will be carried “to look in more depth at different factors that the mothers were exposed to, and to look at laboratory tests from the mothers and babies, and really get a better idea of what proportion, if any, of these birth defects can be specifically linked with the Zika virus,” according to CDC’s Anne Schuchat.

Smoking Gun

A critical ingredient will be hammering out a good case definition of microcephaly since right now there are reports of over-reporting and misdiagnosis among the approximately 5,000 officially reported cases. The study has begun as of mid-February despite the strong conviction among Brazilian health authorities that Zika is the cause of the microcephaly cases. The circumstantial evidence or “smoking gun” is very strong and the Minister of Health has been quoted saying “We have no doubt that the epidemic of microcephaly that we are seeing in Brazil is caused by the Zika virus outbreak.”  

Favoring Causality

Arguments in favor of a causal link between Zika and microcephaly are:

1. There appears to be a significant increase in microcephaly cases since 2015 in association with an increase in Zika cases in Brazil. Initial estimates are that the country averaged only about 163 microcephaly cases per year prior to 2015 but has reported approximately 5,000 in the last few months. In the northeastern state of Pernambuco, microcephaly cases surged last year when only 9 cases are found in a typical year.

2. Zika was actually detected in 41 babies among 462 confirmed cases of microcephaly in Brazil. A total of at least 508 microcephaly cases have been confirmed to date.

3. A review of birth data from the Zika outbreak in French Polynesia in 2013-
14 indicated that the number of congenital abnormalities was increased there as well from 0-2 annually to 18 in 2014-15, including 9 microcephaly cases. Four of the mothers later tested showed evidence of having had Zika.

4. A baby born in Hawaii in December had a serologically confirmed Zika infection. The mother had experienced symptoms compatible with Zika in the second trimester.

5. The fetus of a baby in Slovenia had the virus isolated after pregnancy termination.

Favoring Other Etiologies

Arguments against a causal link:

1. Other viruses besides Zika such as dengue and chikungunya are circulating. Also, other known non-viral causes of microcephaly have not been ruled out.

2. Cases of microcephaly outside of

Brazil have not been reported. Reasons given to explain this unexpected finding if Zika is a true cause include the possibility that other affected populations have not been large enough  to produce the explosive spread possible in a large non-immune population such as Brazil. This assumes the incidence rate among pregnant women is low, and that previous outbreaks could not create a detectable or highly visible “cluster” of microcephaly cases. Or there may be other co-factors unique to Brazil that have helped make the outbreak of neurological outcomes possible and apparent.

Expected Future Wave of Cases

Another reason given by CDC Director Tom Frieden is that “we’re not surprised not to see cases of microcephaly in other countries because of the time frame between infection and delivery.” This assumes Brazil was one of the earliest affected areas and that a wave of cases will occur as time passes in other parts of the Americas.

There is a suspicion that these numbers may include over-reporting and misdiagnosis so that the correct number is still in doubt. Another recent report on the WHO data portal suggests there has been significant under-reporting of cases prior to 2015. Their best estimate of the background rate is some 10 times higher than official estimates or 2,725 cases annually with a minimum incidence of 92 per 100,000. This would make the current outbreak less extraordinary than initially believed.

 


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