The call came after I had drifted off to sleep, late on a Thursday night. "Hi, Mark, it's Pete Parsons in the ER. I have an eight-month-old here I'd like you to take a look at. He has a fever and the mom tells me that he's been real fussy all day. What puzzles me is that he has a funny kind of strabismus, and the mom is worried. She says his eyes didn't look like that before."
Suddenly I was wide awake. A seasoned emergency room physician who handles heart attacks and major trauma with calm competence can get very nervous when faced with a sick infant. I expect that reaction. But sometimes there are red flags that tell me I need to rush to the hospital immediately. This was a red flag: The baby had strabismus, or crossed eyes. Normally, that's not an emergency, but the mother said the boy's eyes hadn't been crossed before. And she said he had fever and fussiness. That combination of symptoms could mean trouble.
When I arrived at the emergency room, the nurse directed me to the curtained-off enclosure where little Jesse Rivera lay on a gurney. He was awake, but he wasn't moving much. And he wasn't looking at his mother, who was leaning anxiously over him, stroking his small hand. "Uh-oh, this could be a sick kid," I thought. To a pediatrician, "sick" means "I'd better do something, or this baby might die."
Jesse's cry wasn't the loud, vigorous sound of a baby who is frightened or angry or in pain. His cries came in short, repetitive bursts, with an unusually high pitch. I hadn't heard cries like that in years, but it's a sound you never forget. This was a sick baby.
When I looked at Jesse's eyes, I could see that they were crossed. I could also see that this was not typical strabismus, a condition in which one eye points slightly inward or outward while the child looks straight ahead. I caught Jesse's attention with one of the brightly colored stickers I use to test infants' vision. When the sticker was directly in front of him, his eyes were straight. But when I moved it to either side, his eyes quickly became crossed. I saw the problem: Although both of Jesse's eyes could move toward the center, neither could move to the side (toward his temples). When he tried to look to one side, the eye that appeared crossed actually moved correctly while the other one remained stuck looking straight ahead.
Something was interfering with the signaling in some of the nerves that controlled Jesse's eyes. Twelve pairs of cranial nerves run from the brain stem to various areas of the head and neck. Working together, three of these nerve pairs—the third (oculomotor), fourth (trochlear), and sixth (abducens)—control the muscles that allow your eyes to move together in tiny, precise jumps across this page, leap quickly up to watch the bird on the windowsill, and return just as quickly to the page.
I suspected that Jesse's problem lay in the sixth pair of nerves. These nerves run around the outside of the brain stem, a location that makes them vulnerable to compression when pressure within the skull increases. Bleeding, tumor, trauma, or infections are all conditions that can increase intracranial pressure. The additional pressure sometimes pinches the sixth nerve—on one or both sides—thus paralyzing the muscle that controls the eye's lateral movement.
Since 1987 the introduction of vaccines targeting two common causes of bacterial meningitis has lowered the incidence of infection in American infants and children. Streptococcus pneumoniae causes more than 3,000 cases of meningitis in the United States each year.
I was fairly certain that Jesse's malfunctioning nerves were the result of meningitis, an infection of the meninges, which are the membranes that surround the brain and spinal cord. He had a number of classic signs of the disease: fever, listlessness, the sixth-nerve palsy, and that high-pitched, irritable, meningitic cry, also probably due to increased intracranial pressure. Jesse needed immediate treatment if he was to avoid the worst complications of meningitis: seizures, coma, and death.
In most cases, meningitis occurs when infecting organisms—viruses or bacteria—circulate through the bloodstream and lodge in the membranes that cover the brain and spinal cord. Jesse might have picked up the virus from a playmate (in whom it may have caused only a mild cold). Or the cause might be a bacterium, one that typically lives harmlessly in the nose or throat until it suddenly gains access to the bloodstream during a minor illness. The wayward organisms then manage to find an inviting home in the cerebrospinal fluid, the watery layer that protects and cushions the brain and spinal cord.
With a virus, the infection is usually relatively mild and self-limited, though serious aftereffects—such as hearing loss—can occur. A bacterial infection, on the other hand, generally causes progressive inflammation of the meninges, swelling of the brain, and, if untreated, death or serious permanent brain damage. This can happen in a hurry. I didn't know how much time Jesse had before his brain would sustain irreversible damage. His crossed eyes told me he was already showing signs of brain swelling. I knew that I was dealing with minutes or hours, not days.
Usually I make the diagnosis of meningitis by taking a sample of cerebrospinal fluid and sending it to the lab to see if white blood cells or bacteria are present. The lab will also culture the sample to see if bacteria or viruses are growing. I collect the fluid for the sample by performing a lumbar puncture, also known as a spinal tap. I slide a needle between the vertebrae into the spinal canal and remove about a half-teaspoonful of fluid. The needle enters the canal well below where the spinal cord itself ends and the procedure is generally very safe. In Jesse's case, however, removing cerebrospinal fluid from the spinal canal was potentially catastrophic. The spinal tap could allow the increased pressure inside his skull to push the lower part of his brain a few millimeters down through the base of the skull, which might completely stop his breathing.
Instead, I made what is called a clinical diagnosis of meningitis, meaning one based on my history and physical examination without the benefit of laboratory tests. I immediately gave Jesse high intravenous doses of the antibiotics cefotaxime and vancomycin to target all the bacteria that were most likely to be causing his infection. I also added a powerful steroid, dexamethasone, to reduce the inflammation of the meninges and try to prevent further damage to his brain. I called the children's hospital, and they sent an intensive care team to pick him up in an ambulance.
Fortunately, Jesse responded really well to the treatment. A spinal tap, done carefully in the intensive care unit once he was stabilized, showed that his meningitis was caused by the bacterium Streptococcus pneumoniae, also called pneumococcus. This organism frequently causes pneumonia in children and adults, hence its name. (A less virulent form of this bacterium commonly causes ear infections in children.) He was placed on intravenous penicillin and gradually regained his normal level of activity and alertness, though he remained in the intensive care unit for a couple of days.
Several months after his illness, Jesse's mother brought him into the office for a checkup. Amazingly, he showed no signs of any long-term complications. His hearing and eyesight were absolutely normal. He toddled around my office, babbling away, joyfully unaware of that night when his life teetered so very close to the edge. I looked at his parents and we all smiled.
The Meningitis Research Foundation offers information about the disease and updates about current research to combat it: www.meningitis.org.
