I was on-call for consults at our local hospital several years ago, and was called late on a Saturday night in October to see a patient with vision changes admitted for a possible stroke. When I arrived at the room, I encountered a warm, bright and engaging 75-year-old woman who had retired a few years back from work in the pathology lab of that hospital. Her two adult daughters were present at her bedside and were concerned but positive since mom was in good spirits with no other symptoms besides her visual changes. In fact, one of the daughters, “Pam,” is a nurse on whom I had performed LASIK on a couple years earlier, so it turned out to be like seeing a friend.

PATIENT SYMPTOMS

The patient described a “scintillating scotoma” in her right upper quadrant visual field, very much like a migraine, but it had persisted for about 48 hours. She had a right upper quadrant field cut on confrontation. Her bedside dilated eye exam was significant only for mild nuclear cataracts.

Her CT and MRI scans of the brain performed on admission were completely normal, and she was on a heart monitor and had already been evaluated by neurology. The neuro exam was totally normal except for the field cut. The neurologist ruled out a stroke, but I suggested that they repeat imaging with special attention to the left parietal and occipital lobes. The scans were repeated with high resolution and IV contrast, but again came back completely normal. She was discharged with instructions to follow up with me in the office the following week.

A GRIM DIAGNOSIS

In the office, her fundus exam was again normal, but the visual field test now showed a complete right hemianopic defect, and she was very symptomatic. Clearly, she was losing vision rapidly without an explanation. I called the eminent neuro-ophthalmologist at our local university hospital, and over the phone he made the likely but unfortunate diagnosis: the fatal condition Creutzfeldt-Jakob Disease, with initial visual symptoms (Heidenhain variant CJD). He saw her within a couple days in consultation at my request, and a PET scan plus cerebrospinal fluid analysis confirmed his suspicions. I stayed in close touch with the family in the following weeks, even though no treatment was possible.

This was just before Thanksgiving. The patient had to move into her daughter’s home since she had lost almost all her vision. Within a few weeks, she had rapid neurological decline and unfortunately passed away before the end of the year.

WARMLY REMEMBERED

Pam was very grateful for my help and support and was thankful she could spend those final weeks close to her mother. This was an inspiring story of a warm, loving family making the best of an untreatable and fatal disease. Later I made a contribution in her memory to the hospital fund drive, for which the family was very grateful. When Pam comes to the office for her yearly eye exam, we always exchange fond memories of her mother.

I learned first-hand that even when unable to save a patient’s vision or life, we can still make a difference. OM