Saturday, December 29, 2012

A Circus of Pain -


It was a cool fall day, but the sun seemed extremely bright as the young man helped guide nine circus elephants to their new pens. Even though the man was wearing sunglasses, the morning sun reflecting off the metal equipment felt like a knife cutting into his right eye. His head throbbed behind the eye, and an occasional tear rolled down his cheek. When the animals were finally secured, he returned to his trailer. ''O.K., I do need a doctor,'' he said to his girlfriend. His hand was cupped over the side of his face. ''Right now.''


Ken Orvidas

It was the worst headache of his life, the 25-year-old patient told the doctor in the emergency room of Highland Hospital in Rochester. It started five days earlier when the circus was in Connecticut. At first it wasn't a big deal. He would take a couple of aspirin, and it would disappear. But when the medicine wore off, the headache was still there. In fact, each time it seemed just a little worse. That morning, when he got out of bed, the pain was unbearable. He took aspirin, Advil, Tylenol. Nothing put a dent in it.The pain was sharp and on the right. It felt as if someone were slamming a door inside his head. He'd had the occasional headache but never something like this.

He didn't smoke, rarely drank and took no medications. He had no recent head trauma, though he was head-butted by a zebra a few years ago. That hurt — it broke his glasses — but not this much. His mother had migraines, and perhaps that's what this was. Maybe, the doctor said, though a week was a long time for a migraine.


For doctors, a description of a headache as the worst is a red flag. We worry about headaches described as the first (for someone who doesn't have headaches) or the worst (for someone who does) or those that are ''cursed'' by the presence of other symptoms like weakness or confusion. He didn't have other symptoms, but the doctor was concerned because he called it the worst.


The doctor ordered a painkiller and blood tests to look for signs of infection or inflammation. She also ordered a CT scan of the head to look for a tumor or evidence of blood. The blood tests were normal. The CT was not.

Within the brain, there are compartments where spinal fluid is made. The fluid then circulates around the brain and spinal cord and is reabsorbed. Two of these compartments, known as the lateral ventricles, are usually mirror images of each other. But in this patient, the ventricle on the right, where his headache was located, was much larger than the one on the left. That suggested there might be a blockage in the circulation of the spinal fluid on the right side, which was causing pressure to build.That could certainly cause a headache — and permanent damage if not addressed quickly.


A slide from the CT scan of the patient's head.
Even before the E.R. doctor saw the CT scan, she called neurology for help in figuring out this patient's terrible headache. The neurology resident examined the patient and his CT scan, but it wasn't clear to him how the pieces fit together. If the asymmetry were caused by an obstruction, the patient should have symptoms associated with increased brain pressure — like nausea — but he didn't. The resident knew that he didn't have enough data to make a diagnosis. Watching the patient over time would give him more. If there was a blockage in his brain, he should begin to feel nauseated and weak. If he didn't, it was very unlikely that the asymmetry reflected a blockage. The patient was admitted to the hospital, where nurses were to examine him every four hours to look for any change.


Overnight the headache became worse, despite the use of several powerful painkillers. By morning the patient was exhausted from the pain and nearly incoherent from the narcotics. He never, however, developed symptoms of increased pressure in his brain.The neurologist speculated that this was a migraine and recommended he go home and follow up as an outpatient.

The neurosurgeons weren't so sure there wasn't an obstruction.The patient's worsening pain was worrisome. They recommended an M.R.I. If there was a change in the size of the ventricle, when compared with the CT, they could drill a small hole into his skull and relieve the pressure.


Dr. Bilal Ahmed, the internist taking over the patient's care that morning, first heard about the new patient from his team of residents outside the patient's door. They told him that he was a young circus worker who had been hit in the head by a zebra, had an abnormal CT and was probably going to surgery later in the day.

As they stood there, a nurse hurried out of the patient's room. ''He's got a rash,'' she told the doctors. The team went into the room, and Dr. Ahmed glanced at the patient now hidden beneath a pile of blankets. He introduced himself to the patient's girlfriend. As she started to speak, Dr. Ahmed held a finger to his lips. ''Don't say anything,'' he told her. ''I want to see for myself.''

''May I look?'' he asked the young man. A matted head of dark curls slowly emerged from beneath the mound of blankets. The patient sat up slowly, blinking in the dim light. His right eyelid was swollen and drooped drunkenly over the pupil so that only the lower ridge of the greenish brown iris was visible. The right side of his forehead was red, as if he had a sunburn on that half of his face. And there was a sprinkling of bumps over his eye and forehead.

Was this zoster? Dr. Ahmed wondered out loud. He touched the reddened skin around the lesions.The young man winced.That part of his forehead had been intensely sensitive ever since this headache started.


Herpes zoster — or shingles — is the re-emergence of the herpes virus that causes chickenpox. The word ''shingles'' comes from the Latin ''cingulum,'' which means ''belt'' or ''girdle''; the rash of herpes zoster often appears in a band, usually on the trunk or chest. When a chickenpox infection resolves, the virus takes refuge in branches of the nerves just outside the spinal cord, where it usually resides for decades. Sometimes the virus re-emerges, but the reasons are unclear. Most of these outbreaks are painful but not dangerous — except when the virus emerges in the nerves near the eyes.


Dr. Ahmed called the neurosurgeon. Was there a link between this patient's shingles and the asymmetric ventricles? No, he was told. If this guy has shingles — and it sounded as if he did — then the asymmetry was probably something he was born with.The M.R.I., done later that day, confirmed that there was no obstruction. In the meantime, the patient was started on an antiviral medication. Despite the treatment, his vision began to blur. The bumps on his face, which led to the diagnosis, had spread to his eye as well. Two years later, his vision is still impaired on that side.


In this case, as in so many, time is a powerful and frequently undervalued diagnostic tool. The rash appeared days after the symptoms began; that is common in zoster. But without the telltale rash, there was only the pain and the abnormal CT, and that led his doctors to worry that his pain was the result of pressure building up in his brain. A truism in medicine is that when we hear hoof beats we should think of ordinary horses as the cause rather than the rare zebra. In this case, time revealed that what looked likely to be zebra — an obstruction on the right side of the brain — was actually the everyday horse of herpes zoster.