Netting the butterfly – man develops hydrocephalus after skull fracture

Bruce H. Dobkin

THOMAS WRIGHT SAT STIFFLY AND SQUINTED AT a blank wall in my examining room, as if trying to visualize the scenes his wife was reconstructing for me. The incidents were fixed firmly in her memory, but for him they might not have existed at all.

It had all begun eight months earlier. Thinking back to that day, Mrs. Wright wondered if perhaps her husband had overdone his exercise: in the two years since retiring from his position as an executive with a supermarket chain, he had ridden a mountain bike for at least an hour every day. Could it have been fatigue that caused him to fall backward off a step stool while changing a light bulb in the garage? When she found him, he was unconscious, his head bathed in a pool of blood. She had pulled out a dish towel that was tucked into her apron, pressed it against his oozing scalp, and called the paramedics.

Doctors in the emergency room had sutured off the visible bleeders, but the real problem lay inside the skull. The jolt against the concrete floor had fractured that bony vault, leaving it cracked like the shell of a hard-boiled egg, with irregular lines and depressed islands. The trauma had torn blood vessels both on the surface and deep within his brain, bruising, shearing, and twisting his cerebral landscape with all the fury of a tornado. A CT scan revealed thin crescents of clotted blood between the skull and the surface of his brain’s frontal lobes, as well as a clot over his left temporal lobe. Blotches of blood filled two dime-size contusions deeper in his brain. As these bruises swelled, they pressed so dangerously against the area of the brain that controls breathing that his doctors decided to connect him to a mechanical ventilator.

He’d awakened the next day. “I called his name, and he opened his eyes and squeezed my hand,” Mrs. Wright recalled. “I was so hopeful, in spite of the cautious prognosis given by the doctors. You see, Tom had an accident when he was a teenager. A car sideswiped his bicycle. The head injury that time affected his speech for some months, but it didn’t keep him out of the war or out of college.” Her tone of voice and earnest expression made it clear that she wanted me to know her husband had been an accomplished person, someone who was still worth trying to help.

I asked my new patient if he remembered anything about the accident, the hospital stay, or the six weeks of rehabilitation therapy. He turned to me with a smile that puckered the crow’s-feet around his eyes. “Sometimes you don’t remember all the things,” he confided.

Next I asked him about the long scar running over the left side of his bald scalp. “Must have bumped the thing,” he said. He began picking at a callus below his wedding band.

His wife’s eyes reddened with tears. “Please,” she said, defending his lapse. “I know his memory and the way he stumbles using words isn’t good. I don’t expect that will come back any more than it has. But he was doing better a month ago. Why?”

“Well, first tell me about how he has changed,” I replied.

“It’s little things, you know. He used to dress himself when I put out his clothes; he’d ask, ‘What’s for dinner?’ when he was hungry–although, I’ll admit, sometimes it was lunchtime–and we’d walk half a mile in the park every morning. Now he hardly does anything.” She fiddled with the buttons on her red cardigan. It had an arrangement of purple, white, and yellow flowers on each side, with stitching loose enough to suggest that she had knit it herself. She looked up at me to deliver her real message. “If things get much worse, I won’t be able to manage him at home.”

“I understand,” I assured her. I began searching for clues, for some insight into her husband’s deterioration. “Do you think his walking has changed?” I asked. “Do his feet ever seem to get stuck?”

“No,” she answered, curious about what I might be getting at. “I mean, he just doesn’t seem to want to get around as much.”

“Has he had any trouble holding his urine or bowels?”

“He goes to the bathroom when he has to. I don’t believe he’s ever wet himself.”

With his barrel chest and muscular arms and calves, Thomas Wright looked more ready for a singles match on a tennis court than for a bed in a nursing home. As I began to examine him, however, I noticed that he was walking cautiously and ever so slightly scuffing the toes of his right foot on the ground as he swung that leg forward. When I tested the individual muscles, I found a trace of weakness in his right arm and leg, a residue of the trauma to the left side of his brain; because of that weakness, he had a mild limp.

HIS COGNITIVE TROUBLES, ON THE other hand, were not at all mild. He could not name the month and guessed that the year was 1978. When I told him the date, he forgot it a minute or two later. He could spell cat forward but not in reverse. He immediately repeated the names of three items that I asked him to remember but could not recall any of them, even with some clues, a minute later. He did not know his own date of birth or the names of his three children. “Who can keep up?” he mused. I asked him to give me a list of ten animals, but all he could come up with was “zoo whatchamacallits.” He could tell me the names of only about half the common objects and parts of the body that I pointed to. The word thigh, for instance, eluded him. Mrs. Wright sighed sadly when instead of copying the box I had drawn he penciled only three oddly interested lines. When I asked him to connect, in alphabetical order, scattered circles containing letters from A to L, he knew to draw a line between A and B but appeared stumped by the next move.

In all, the examination took less than an hour, but it was all the time I needed to map the landscape of Mrs. Wright’s anguish. The cerebral tornado had left in its wake a man with the equivalent of a severe case of Alzheimer’s disease.

There should have been one major difference, however. The intellectual decline of people with Alzheimer’s gets inexorably steeper. Mr. Wright’s deterioration should have halted shortly after the accident.

I reviewed the three-inch pile of evaluations from speech and physical therapists and the reports made by his physicians. The only difference between what had been written three months earlier and what I had just seen was that back then he had been described as frustrated by his errors. Now he seemed resigned or unconcerned. I began to wonder whether the changes his wife had chronicled might simply reflect a darkening of his mood. Could Mr. Wright have become so depressed by his constant failures that he was withdrawing from a life he no longer understood?

Next I looked at the half-dozen radiology studies of his brain taken during the first days after the accident and then two and six months later. They showed that the clots had gradually disappeared, but patchy wedges of destroyed gray and white matter persisted. That was bad news: these tissue injuries made it impossible for Mr. Wright to take full advantage of his brain’s resources for language, memory, attention span, and judgment.

The scans did, however, raise the possibility of a sometimes treatable diagnosis. I noticed that his cerebral ventricles, the dark, butterfly-shaped, fluid-filled caverns in the center of the brain, had enlarged modestly over time. The ventricles make about 30 tablespoonfuls of clear spinal fluid a day; slow currents move the fluid through a narrow channel out to the surface, where it bathes and cushions the brain and spinal cord. Normally, about the same amount of fluid that’s created is absorbed by the surface of the brain. If it’s not removed as fast as it’s produced, the pool grows and the ventricles begin to enlarge, pushing aside the rather elastic tissue of the surrounding brain. The nerve fibers compressed by the expanding pool are those that play a role in walking, memory, and bladder control. The disease–the excess spinal fluid and the trouble that fluid cause–is called hydrocephalus, literally meaning water in the head.

Most often, when we think of hydrocephalus, we think of babies with large heads. Their hydrocephalus generally occurs when the outlet channels that direct the fluid from the ventricles to the brain’s surface fail to develop. But hydrocephalus can also be the result of tumors that compress or obstruct the fluid’s flow. Sometimes a head injury or bleeding damages the delicate, spongy tissues that cover the surface of the brain, so they cannot absorb the fluid. In any of these cases, the ventricles begin to expand and symptoms begin to appear–symptoms that could explain the kind of deterioration Thomas Wright was experiencing.

After I had told Mr. and Mrs. Wright about the condition, I explained that there is only one way to get the fluid that is produced and that which needs to be removed back into equilibrium. A neurosurgeon drills a tiny hole through the skull and passes a thin plastic tube into one of the butterfly wings. A one-way valve directs the excess fluid to the other end of this shunt, which is placed in the patient’s belly. The now-decompressed butterfly gradually shrinks back to its normal size.

“Would he get a little better or, I mean, could he get back to being himself?” Mrs. Wright asked.

I told her that if her husband indeed had hydrocephalus, the shunt should enable him to regain the ground he had lost in the last few months. But, I added, there was no test I could do to predict with certainty how he would respond. All too often after a serious head injury like his, the ventricles are enlarged only because the rest of the brain has withered around them. And that’s not hydrocephalus: the butterfly is simply spreading its wings to fill the vacuum. In such a case, merely shunting fluid away would have little effect on the permanent brain damage wrought by the injury.

So, I explained, before recommending the surgery doctors want to be as sure as they can that the patient is indeed suffering from hydrocephalus. And what gives them the most certainty in that diagnosis is seeing a particular triad of symptoms: memory loss, incontinence, and a peculiar problem with walking called an apraxia, in which the person’s feet seem to stick to the ground as he tries to take a step. Mr. Wright’s case wasn’t nearly so clear. His ventricles were not as large as I had seen in other patients who responded to a shunt, his memory loss could well be due solely to the brain trauma he’d sustained at the time of his fall, his walking was not typical of apraxia, and he was not incontinent. In other words, I told the couple, pursuing a shunt would mean betting on a long shot that carried a 10 percent risk of a potentially life-threatening complication–like the introduction of bacteria that cause meningitis, a disease in which the membranes surrounding the brain and spinal cord become inflamed.

HAVING GIVEN THEM ALL THE HARD facts I could, I told them it might be best simply to hold off on a decision for a while, to see if the typical triad of symptoms evolved. Mrs. Wright immediately agreed: she said she wasn’t ready to try something that might hurt him. Before they left, I gave Mr. Wright a prescription for an antidepressant, to lift his mood and be sure that it wasn’t affecting his concentration, memory, and drive.

Three weeks later I got a call from Mrs. Wright. She said that her husband was much worse. He could hardly walk, and he was wetting himself. Although these new symptoms did indeed complete the triad, I wanted to make sure that they were not the result of the antidepressant medication. Even the low dose Mr. Wright was taking could spark these sorts of side effects in his traumatized brain. So I asked Mrs. Wright to stop giving him the pills and to watch for the symptoms to begin clearing. When she pushed him and his new wheelchair into my office a week later, I knew discontinuing the medicine hadn’t done the trick.

“I can’t keep him at home this way,” she said. “He’s like a baby and getting worse every day.”

As for Mr. Wright, he seemed to have no idea what was happening. “What can I help you with?” I asked him.

“Oh, just visiting,” he answered.

“What kind of work do I do?” I tugged at the lapels of my white coat.

“Good question,” he smiled.

I helped him stand up and told him to walk through the doorway. His body leaned forward slightly, but his feet stayed glued to the ground. I gave him a gentle nudge while holding on to his belt at the small of his back; he took two steps like an ox whose feet were stuck in deep mud. Then he stopped, and urine ran down onto his shoes. He had the triad, all right. But did he have the disease?

“What about that operation?” Mrs. Wright asked sadly. It was her only hope, she explained, and I agreed. If she were to place him in a nursing home, this helpless man would most likely die from bedsores and infections.

I admitted him to the hospital and ordered yet another set of MRI images. When I looked at them and compared them with the older studies, the butterfly’s wings were at best only slightly larger. So I called one of my neurosurgical colleagues and described Mr. Wright’s case. “Sounds like there’s nothing much left to lose,” he said.

The surgery went smoothly. Three days later Mr. Wright took a few steps with less of the inertia that had held him back in my office the week before. He was still quite unsteady, however, and just as confused. To buy his wife some time before she had to become a full-time caretaker again, I arranged for ten days of inpatient rehabilitation therapy.

A WEEK AFTER THOMAS WRIGHT’S release from the hospital, he opened the door of my exam room, motioned his wife to walk in, and strode in behind her. According to the smiling Mrs. Wright, he now dressed and fed himself, walked four blocks a day with her, and was logging half an hour each day on his stationary bicycle. When I examined him I found that, remarkably, his right-side weakness had improved and his walking was only slightly wobbly. Even more amazing, he could more easily retrieve information stored in his memory. He recalled the words red, cabbage, and Oldsmobile a few minutes after I said them, although I did have to remind him that one was a color, one a vegetable, and one a car.

There was one intriguing change–Mr. Wright had developed some odd rituals. For example, Mrs. Wright told me how, that morning, he had torn all the sheets off a roll of toilet paper and neatly stacked them on the sink. I asked him about the toilet paper business.

“Oh, I get onto it and it goes,” he replied, perhaps not so cryptically. It seemed that once something captured his attention, his still-damaged frontal lobes–responsible for a number of behaviors, including sifting out what’s important from what isn’t–wouldn’t let him see past it.

But although many words and memories still eluded him, the shunt had restored more of his cognition than I would have predicted. In retrospect, I realized that the hydrocephalus must have been working insidiously against him from the beginning, as he tried to recover from the acute effects of the brain trauma.

“What’s that bump along your head?” I asked, as my finger traced the subcutaneous route of the shunt and its soft valve.

“Oh, operated on to a degree, I suppose,” he answered.

COPYRIGHT 1993 Discover

COPYRIGHT 2004 Gale Group

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