Something seemed to be blocking the signals to his brain. what was that?
“You can’t see the ceiling, can you?” the man asked his 31-year-old wife. She grimaced, then shook her head. She lay in bed and gazed up at the shadows and familiar shapes cast by the wintry morning sun. But she couldn’t see them. It was as if a thick white fog lay between her and these daily changes. Squinting didn’t help. Opening her eyes as wide as she could didn’t do it either. All her life she had perfect vision. It was a secret source of pride. She had never even seen an ophthalmologist. But this morning changed everything.
She first noticed the cloudiness in his eyes six months earlier. She is a professional violinist and teacher. That summer, she took her students to Italy to learn about sacred music and art. As she gazed at the frescoes decorating the ceiling of a favorite cathedral, a shimmering shape with jagged, jagged edges appeared out of nowhere. The dots seemed to sparkle as the star-shaped image slowly grew larger. Within the shimmering outline, the colors blended together, like crystals in a kaleidoscope. It was beautiful and terrifying. She lowered her head, closed her eyes and rubbed her aching neck.
When she opened her eyes, the splintered star, with its twinkling edges, was still there, distorting everything beyond it. It grew so large that it was almost all she could see. Then slowly it started to fade; after almost half an hour, the world began to return to its familiar look and shape. There had been similar experiences, although less severe: occasionally, when she got up quickly after sitting or lying down, she felt an intense pressure inside her head, and when she let go, everything appeared briefly faded and pale before returning to normal hues. These spells only lasted a few seconds and have only happened a handful of times over the past few years. She wrote it because of fatigue or stress. After that day in Italy, these bursts of twinkling stars appeared weekly, then daily.
Stranger still, the straight lines developed odd bumps and bumps when she looked at them out of the corner of her eye. Doors, curbs, and table edges seemed to wobble, creating bulges and dips. When she looked at the object full foot, he straightened up obediently but resumed his aberration once again on the sidelines.
A few days after her morning white veil, the young woman went to an optometrist near Fort Lee, NJ, Dr. Paul Shahinian. If the star bursts worried the young woman, Shahinian’s reaction to her examination was terrifying. She needed to see a neuro-ophthalmologist, he told her – an eye and brain specialist – and she needed to see one soon. All information gathered by sight is transmitted to the brain through a thick cable of fibers at the back of the eye called the optic nerve, the doctor explained, and the nerve in his left eye was extremely swollen. As she sat in her office, Shahinian called the specialists himself. The first two offices he called got the same answer: She couldn’t get an appointment for months. He then contacted a neuro-ophthalmologist who was new to the area, Dr Kaushal Kulkarni, and, after explaining the emergency, arranged for the patient to be seen the following week.
Kulkarni listened to the patient describe her strange visual abnormalities. Although his vision was still 20/20, the intermittent starbursts and bent lines seen in his peripheral vision – a phenomenon known as metamorphopsia – suggested that something was wrong with the way the brain received and processed his visual information. Kulkarni shined a bright, narrow light at the young woman’s right eye. As expected, both students contracted. He shifted the light to the left and both pupils immediately dilated. Moving him to the right, his pupils contracted again; turning back to the left, they suddenly widened again. Obviously, the signal on the left was not passing. The swelling cut off the flow of information from the eye to the brain. However, this seemed to be a one-sided problem: the fact that the left pupil constricted when light shone into the right eye indicated that information from the brain continued to pass.
There are many causes of this type of optic nerve injury. Shahinian had thought that, given the patient’s age, it was likely multiple sclerosis, an autoimmune disease in which the immune system mistakenly attacks the nerve fibers that connect the brain to the body. Kulkarni agreed it was a possibility and ordered an MRI to look for evidence of this or other abnormalities. Was it a tumor? Or a stroke? Other autoimmune diseases also had to be taken into account. It could also be the result of an infection: Lyme disease could do that; like cat scratch fever, an infection caused by the bacteria Bartonella henselae; even syphilis, often called the great imitator because of its ability to manifest itself in so many ways, could cause this kind of injury.
He sent the patient to the lab for testing. The blood test results came quickly. It wasn’t Lyme or Bartonella or syphilis. None of the inflammatory markers suggestive of autoimmune disease were elevated. It was MRI that held the answer. Kulkarni didn’t see the bright white dots and dashes that would suggest MS. Instead, a large round object, a mass the size of a plum, dominated the middle part of the left side of his brain.
Kulkarni called the patient and told her the MRI was abnormal. She came back a few days later. He couldn’t tell her based on the photos what type of tumor she had. The most common would be a meningioma, a tumor of the tissue that lines the brain. An acoustic neuroma was rarer but also possible. It is a slow-growing tumor that starts in the tissue that connects the ear to the brain. She was a little young for that; these tumors usually appear in men and women over the age of 40, and they usually cause problems with hearing and balance rather than vision. Either way, the tumor was so big that it blocked the flow of cerebrospinal fluid to the brain, causing the nerve to swell. It should be removed.
Kulkarni knew the patient had no health insurance. He called the neurosurgeons he knew, trying to figure out how to get this woman the care she needed. The only answer seemed to be to go to the emergency department. Through a law called the Emergency Medical Treatment and Labor Act, all emergency departments are required to provide stabilizing treatment to any patient who presents, regardless of insurance status or ability to pay. The care provided is not free; patients are charged, but they must be treated.
She went to the emergency department at NewYork-Presbyterian Hospital/Columbia University Medical Center, and the mass was removed. The pathologist confirmed that the tumor was an acoustic neuroma. Reading about this type of growth, the patient noted that hearing loss is a common symptom. She had hearing loss in her left ear, but she hadn’t made a connection between this problem and that of her eyes. She thought the constant exposure to the sound of her violin might have caused a little damage. She told herself it was just the price to pay for doing what she loved. After the operation, his hearing did not change, but the strange starbursts disappeared completely. However, straight lines still tend to distort in his peripheral vision.
Even before her hair had grown enough to hide the scar, the bills started rolling in. The numbers were even scarier than the images of the tumor. The final accounting totaled approximately $650,000. She and her husband feared that they would have to declare bankruptcy. They had money — they were saving for a rainy day, but it was a tsunami. Salvation came, unexpectedly, when a friend of a friend asked if they had spoken to the hospital about financial assistance. It turned out that Columbia Presbyterian had a financial aid program. In fact, all nonprofit hospitals are required to provide financial assistance to those in need; it is mandated by the Affordable Care Act. They didn’t have to go bankrupt. They are grateful that the ordeal ended well. And, two years after the operation, they are beginning to replenish their savings, because you never know when it might still rain.
Lisa Sanders, MD, is a contributing editor at the magazine. His latest book is “Diagnosis: Solving the Most Baffling Medical Mysteries”. If you have a solved case to share, email her at [email protected]