His wife was the first to notice. They were walking to a restaurant in downtown Huntington, N.Y., on Long Island. She was trailing a little behind, chatting with her sister. When she glanced up to watch this man she’d been married to for 50-some years, she was shocked to see an old man. At 72, he was hunched over, creeping along, barely lifting his feet off the ground. When had he gotten so old?
“Honey, stop shuffling!” she called out to him, a hint of irritation in her voice. “I’m not shuffling,” he replied indignantly. He denied it for weeks, but finally acknowledged that his gait really was different. Somehow this thing he did every day had become harder. His legs felt weak, his balance a little iffy.
That was seven years ago. Since then, they’d seen many doctors. His internist sent him to a neurologist. He had an M.R.I. to look at his brain and an M.R.A. to look at the blood vessels within it. Lots of blood tests. A specialized scan seeking evidence of Parkinson’s disease, which didn’t find it.
The M.R.I. report showed some spinal stenosis — where the bones crowd in on the spinal cord — in his neck and in his lower back. An orthopedic surgeon said there was nothing on the M.R.I. that would account for the way he was walking and nothing bad enough to require surgery. He saw more neurologists. More orthopedic surgeons. Surely someone could explain the strange new way he walked. He had more M.R.I.s. He had EMGs to evaluate his muscles and nerves. None offered any explanation.
He went back to the orthopedic surgeon. His neck didn’t hurt, but he wondered: Couldn’t the stenotic narrowing there affect his legs? It could, the surgeon agreed. He didn’t see any evidence that there was significant compression of the spinal cord, but he agreed to operate anyway. After the surgery to widen the stenotic bones and a month of rigorous physical therapy, the man was still shuffling.
Interventions and Dead Ends
He saw yet another neurologist. This one ordered a second scan looking for Parkinson’s. That one showed a small defect in the substantia nigra, the twin commas deep in the back of the brain where dopamine is stored. That finding suggested he did have Parkinson’s after all. But the defect seemed too small, the Parkinson’s too early-stage, to explain his very altered gait. Still, it was worth a try. He started the man on a medication for Parkinson’s called Sinemet. When there was no improvement after a month, he stopped the medication. Yet another neurologist disagreed: He was certain the man had Parkinson’s and restarted the medication. But when the shuffle didn’t abate, even at the highest dose, he too conceded that it probably wasn’t because of Parkinson’s disease.
The patient was frustrated. As he moved from doctor to doctor, his walk and balance continued to deteriorate. The Covid pandemic kept him out of specialists’ offices for nearly two years. Still, his wife continued her search for a doctor who could help. Finally, she turned to Hospital for Special Surgery. She looked up the neurologists on staff and started calling. The third doctor she tried agreed to see her husband and then referred him to an H.S.S. neurologist named Alexander Shtilbans.
Shtilbans, a specialist in movement disorders, listened as the patient, assisted by his wife, described what he felt. His legs were strangely heavy — leaden and awkward, as if they were stuck to the ground. He could walk for only a few minutes. After that, he felt as if there were a rope around his middle keeping him from moving forward. Shtilbans watched as the patient walked up and down the hallway. The man’s steps were short, with one foot barely ahead of the other, yet his stance was strangely wide. His left arm swung normally by his side, but his right arm hardly moved when he walked. On examination, that arm and leg were stiff and a little hard for the doctor to move, even when totally relaxed.
An Answer in the Pictures
The doctor reviewed imaging of the patient’s head and spine as the couple sat waiting nervously. Finally he spoke. Two things were quite clear even from this first look, the doctor said. Based on the Parkinson’s study and his exam, he did have a little Parkinson’s. That’s why his right arm and leg were stiff. But that wasn’t what was making his walk so abnormal.
He pointed to one of the M.R.I.s that showed the patient’s brain. Cerebrospinal fluid is made in spaces in the brain known as ventricles, he explained. And if you compare two of the images done several years apart, he said, you can see that the ventricles in the later image are larger than the ones in the earlier image. The patient couldn’t see a difference, but the doctor continued. That change suggests there is increased pressure in the brain, the result of having too much fluid in the ventricles and the surrounding brain tissue. That excess exerts extra pressure on the delicate tissues of the brain, which can injure them. Despite this physiology, the disorder is called normal pressure hydrocephalus, because when measured in a spinal tap, the pressure is often normal. But in fact there are waves of high pressure that occur over time. Only in the last quarter-century have doctors been able to measure the pressure in a way that reveals that fluctuation.
We make just over half a fluid ounce of spinal fluid an hour — about 17 ounces in a day. At any given moment we have around five ounces of cerebrospinal fluid in circulation. The fluid travels through the brain and spinal column before being absorbed by the veins at the top of the brain. Think of water pouring through a sink: What drains from the basin has to move as fast as what comes from the faucet. If it doesn’t, the sink overflows onto the floor. So it is with the spinal fluid in the brain, except that there is no floor, no place for the fluid to overflow. As we age, the cycle of making and absorbing spinal fluid can become less efficient. When the drain doesn’t quite keep up with the faucet, the fluid — which is not compressible — puts pressure on the brain tissue, causing dysfunction and ultimately permanent injury. It isn’t known exactly what causes the mismatch. The creation of the spinal fluid seems to be constant. What usually changes is the brain’s ability to absorb the stuff.
The symptoms of normal pressure hydrocephalus stem from this increased fluid. The disorder is characterized by a trio of symptoms. Most commonly, patients have gait changes. They might also develop urinary incontinence or dementia. If caught and treated early, the symptoms may be reversible. This is a common disorder in the very old, affecting more than one in 20 adults over the age of 80. It’s often missed because the three symptoms can be caused by other diseases also common in the elderly. Treatment involves placing a tube into the brain, which allows the excess fluid causing the pressure to drain into the abdomen.
Shtilbans referred the patient to Dr. Michael Kaplitt, a neurosurgeon at Weill Cornell Medical Center. Because N.P.H. is hard to diagnose, Kaplitt wanted to be certain that this was what the patient had, and that the treatment would be helpful. He ordered one more test. The patient was admitted to the hospital, where a tube was inserted at the bottom of his spinal canal, and a small amount of fluid was drained every hour. After 24 hours, the patient was instructed to walk down the hall. He was amazed. The heaviness in his legs was gone. He hadn’t felt this good walking in years. It was all he could do to keep himself from running.
Three weeks later, the patient had the surgery to place a permanent shunt. Again the difference was immediate and profound. Before the surgery, he could barely complete a single lap around the pool in his yard. Now he is walking two or three miles a day and working with a trainer to regain the strength he lost. He’s not quite as fast as he used to be, but as he reminded me, he is 79 years old. And to his wife’s great relief, and his own, when he walks, he no longer looks or feels like an old man.
Lisa Sanders, M.D., is a contributing writer for the magazine. Her latest book is “Diagnosis: Solving the Most Baffling Medical Mysteries.” If you have a solved case to share, write to her at [email protected].
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