Ronald I. Jacobson, MD, of Maria Fareri Children's Hospital at Westchester Medical Center, on a Diagnosis of Pseudotumor Cerebri

Ronald I. Jacobson, MD, Maria Fareri Children’s Hospital at Westchester Medical Center

In December 2009, then 13-year-old Isabelle Bock should have been on stage playing a polichonelle and a mouse in “The Nutcracker” ballet. Instead, she was confined to a hospital bed. A complex web of symptoms threatened to leave her permanently blind.

The first hint of trouble popped up a year and a half earlier. Isabelle was having headaches. Her neuro-ophthalmologist, Scott Forman, MD, discovered “papilledema,” or swelling of the optic nerves in the back of her eyes, and began treating her. By November 2009, the swelling had subsided.

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But days after her follow-up appointment, Isabelle’s health took a nosedive. She lost her appetite, began vomiting, experienced facial weakness, and suffered with double vision. The optic-nerve swelling increased and her eye movements were limited, almost as if her eyes were stuck. She was admitted to Maria Fareri Children’s Hospital at Westchester Medical Center in Valhalla, where Ronald I. Jacobson, MD, is chief of pediatric neurology.

Dr. Jacobson ran some tests. When he tapped Isabelle’s limbs with a hammer: nothing. She had no deep tendon reflexes, and a spinal tap found that her intracranial pressure was “very, very elevated.” The diagnosis? Pseudotumor cerebri, a condition in which there is an overabundance of cerebrospinal fluid, a clear liquid that acts as a buffer, protecting the brain and spinal cord.

Isabelle was treated with medicine to reduce her spinal fluid production and, in turn, alleviate the pressure. High doses of immunoglobulins were administered by IV to turn off the autoimmune attack on her peripheral nerves and improve her nerve-muscle function. Five days after she was admitted to the hospital, she was released—alert, happy, and, Dr. Jacobson believed, generally improving.

But Isabelle’s headaches persisted and dark spots in her field of vision, called scotoma, grew larger. What was wrong? Without timely treatment, her doctors feared she would become permanently blind. The case was unfolding much like an episode of the TV medical drama series House. “I actually remember this like it was yesterday,” Dr. Jacobson says. “Dr Forman called me and said, ‘Oh my goodness, she’s so much worse. Can you see her immediately?”’

Isabelle was whisked to the emergency room and readmitted.

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On the phone with Dr. Jacobson, Dr. Forman wondered, could it be optic neuritis, a type of optic nerve inflammation associated with infection or autoimmune disorders. Or was it an exacerbation of her intracranial pressure? The doctors knew that they needed to act quickly to prevent blindness from setting in. “And, as I’m driving to the hospital, a light bulb goes off in my mind,” Dr. Jacobson recalls. “The ‘Aha!’ Moment was not to limit the treatment to one particular cause.”

Dr. Jacobson corralled a team of medical professionals, including Dr. Forman, a group of neurosurgeons, and an infectious disease doctor, to devise a plan. Isabelle first was given steroid medication. If it didn’t work, it would quickly rule out the diagnosis of optic neuritis. When, a day or two later, there was little meaningful improvement, Dr. Jacobson says he felt more comfortable in moving ahead with the next steps in the treatment plan. A catheter was placed in Isabelle’s lower back to drain off excess spinal fluid, lowering the pressure in her skull. When her vision slowly improved, pediatric neurosurgeon Avinash Mohan, MD, implanted a permanent shunt in her skull to funnel surplus fluid into her abdominal cavity.

So what was wrong with Isabelle? Quite a bit. She had, it turned out, Lyme disease, but, Dr. Jacobson says, “We do not know whether Lyme was the underlying trigger, but assuming it was, a possible linkage would be Lyme-induced pseudotumor, or Miller Fisher variant of Guillain-Barré syndrome causing pseudotumor. So we were sorting out all three or four over the short time frame.”

Despite some visual impairment, the now 15-year-old is healthy and grateful to have had such a caring medical team on her side. “They were just phenomenal doctors,” she says.

Isabelle’s father, Steven Bock, MD, a conventional and alternative medicine practitioner in Rhinebeck, New York, was impressed with Dr. Jacobson’s expertise and the way he connected the dots. “He liked to think aloud. He says, “I just really respected his thinking.”

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As for Dr. Jacobson, “I felt like I was laser-beam focused on figuring this out.”

Photo by Chris Ware

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