Dr. João Ricardo de Almeida is part of a team in Brazil that's investigating the cases of microcephaly — brain damage in infants born to mothers who contracted Zika virus during their pregnancy. He's examined dozens of brain scans, and he says that the scans are "very scary to look at."

"You see very profound abnormalities," says the neuro-radiologist. "Usually it's striking."

And they're notably different than scans of other babies born with the birth defect.

That's one of the disturbing findings in a large-scale study of the babies born with microcephaly. A team of doctors — from a neuro-pediatrician to an ophthalmologist — have taken a good look at dozens of affected infants. They're conducting the study at Roberto Santos General Hospital in the city of Salvador in Bahia.

One goal is to establish whether the Zika virus is in fact the cause of the thousands of cases of microcephaly in babies born since the fall.

Dr. Adriana Mattos examines three-month-old Barbara Antonia, who has microcephaly. Her mother, Ana Claudia Teixera, caught the Zika virus when she was four months pregnant.

Dr. Adriana Mattos examines three-month-old Barbara Antonia, who has microcephaly. Her mother, Ana Claudia Teixera, caught the Zika virus when she was four months pregnant.

Lourdes Garcia-Navarro/NPR

"Of course the evidence is mounting but we need to prove," says Dr. Antonio Raimundo de Almeida, director of the hospital, which is in the city of Salvador in the state of Bahia. (He is a cousin of the other Dr. de Almeida.)

This week, 16 mothers and their microcephalic babies came to the hospital for a battery of tests.

"We do a full history, we do a blood test, everything," hospital director de Almeida says.

In the waiting room, the mothers cradle their infants, who all have the small cranium that is typical of microcephaly. Microcephaly itself is not a disease. It's a condition caused by the failure of a fetus's brain to develop in the mother's womb. There can be a number of causes, including toxoplasmosis, cytomegalovirus, syphilis, rubella and genetic abnormalities. So first, the researchers need to rule out these causes out.

In their research, the doctors have made some startling discoveries: There are some unique markers in the infants who have suspected cases of Zika-related microcephaly.

In one of the rooms, Dr. Adriana Mattos examines 3-month-old Barbara Antonia. Her mother, Ana Claudia Teixera, caught Zika when she was four months pregnant.

Dr. Mattos flips the child so she's lying on her chest. The doctor points out that in these Zika-related cases, the muscles in the upper body and neck are unusually stiff. And that's very different from cases of microcephaly caused by other infections.

Dr. João Ricardo de Almeida says the infants born to mothers who were infected in the first trimester seem to suffer the most brain damage. And that kind of damage also appears to be different than what you would see with microcephaly caused by other types of infections.

"Regarding Zika there seems to be some particular abnormalities that we do not see in [microcephaly cases caused by] toxoplasmosis or cytomegalovirus or rubella."

A normal brain has ridges like coral. The brains of these babies look "like a smooth rock," he says.

He says the degree of brain damage he is seeing will probably mean that rehabilitation will be very difficult.

"They are not going to be functional," he says of the babies he has examined. "They'll need care for the rest of their lives."

Dr. Albert Ko from Yale University has been collaborating with the study in Bahia. He says that while the cases of microcephaly are getting all the attention, the Zika virus could be having a wider range of effects on the development of a fetus.

"We are seeing cases in the hospital of children who have normal size heads but are having neurological lesions and eye lesions," he says. "And we are extremely concerned ... this might suggest that [the microcephaly cases] are just the tip of the iceberg."

In other words, even children who appear normal may suffer from a range of developmental delays. So the deeper the investigation goes into this outbreak, the more worrying it becomes.

Valdemar Geo contributed to this report.

Copyright 2016 NPR. To see more, visit NPR.

Transcript

STEVE INSKEEP, HOST:

This next story underlines how much we don't know about the Zika virus. That's the virus that's been linked to many birth defects - or so doctors think. NPR's Lourdes Garcia-Navarro traveled to the Brazilian city of Salvador, where researchers are trying to answer a question that seemed like it was already answered.

LOURDES GARCIA-NAVARRO, BYLINE: And that question is, is the Zika virus responsible for the rise in the cases of microcephaly that doctors are seeing across the country? So far, doctors suspect the link is there, but they're trying to prove it beyond a reasonable doubt. This week, director Antonio Raimundo de Almeida at the Roberto Santos General Hospital in Salvador, in the state of Bahia, gathered 16 mothers with their microcephalic babies for a battery of tests.

ANTONIO RAIMUNDO DE ALMEIDA: We do a full history. We do blood tests - everything. It's a full team

GARCIA-NAVARRO: In the waiting room, the mothers cradled their infants, who all have the telltale small cranium of microcephaly. To be clear, microcephaly is a symptom and not a disease. The condition happens when a fetus's brain doesn't develop in the mother's womb, so the skull doesn't expand to a normal size - hence the small head. And there are many things that can cause it, Dr. Almeida says.

ALMEIDA: Toxoplasmosis, cytomegalovirus, syphilis...

GARCIA-NAVARRO: ...As well as Rubella and genetic abnormalities. So first, they need to rule out that any of those have caused these cases of microcephaly. A team of six doctors - from a neuropediatrician to an ophthalmologist - have gotten a good look at large group of affected infants. And the doctors have made some startling discoveries. Suspected cases of Zika-related microcephaly have some unique markers.

In one of the rooms, Dr. Adriana Mattos examines 3-month-old Barbara Antonia, who has microcephaly.

ADRIANA MATTOS: (Speaking Portuguese).

GARCIA-NAVARRO: Dr. Mattos turns the child on her chest and points out that Barbara Antonia appears to be able to lift her head, and that she has unusual upper-body strength.

MATTOS: (Speaking Portuguese).

GARCIA-NAVARRO: But in fact, what she says is that in many of these Zika-related cases, the muscles in the upper body and neck are unusually stiff, which is very different than what we find in other infections that cause microcephaly, she says. Dr. Joao Ricardo de Almeida is a neuroradiologist, and he's been reviewing dozens of ultrasound brain scans of the microcephalic infants.

JOAO RICARDO DE ALMEIDA: You see, like, very profound abnormalities. Usually it's striking. It's really scary to look at, you know.

GARCIA-NAVARRO: He says mothers who were infected in the first trimester have infants who seem to suffer the most damage, and that kind of damage also appears to be different.

J. ALMEIDA: Regarding Zika, there seems to be some particular abnormalities that we do not see in, for example, toxoplasmosis or cytomegalovirus or rubella.

GARCIA-NAVARRO: If you look at a brain, which we've all seen, we have these ridges like coral, and these babies don't have that.

J. ALMEIDA: They don't. It's like a smooth rock.

GARCIA-NAVARRO: And he says the degree of damage that he's seeing will probably mean that rehabilitation will be very difficult.

J. ALMEIDA: They're not going to be functional. They're going to be having to, you know, be taken care of, like, for the rest of their lives.

GARCIA-NAVARRO: Dr. Albert Ko from Yale University has been collaborating with Bahia's study. He says while microcephaly is getting all the attention, the virus could be having a far wider range of effects on fetuses.

ALBERT KO: We are seeing cases in the hospital of children who have normal-sized heads but are having neurological lesions and eye lesions. And we're extremely concerned about that because that may suggest that these cases of microcephaly - those 4,000 cases - are just the tip of the iceberg.

GARCIA-NAVARRO: Children could appear normal, but may suffer a range of developmental delays, he says. In other words, scientists say, the deeper they investigate this outbreak, the more worrying it becomes. Lourdes Garcia-Navarro, NPR News, Salvador, Brazil. Transcript provided by NPR, Copyright NPR.

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