Medical Mystery: What caused fluid buildup in woman's brain?

Posted: February 02, 2014

What a headache!

Coworkers at the nail salon were worried about Xi. She had been a remarkably reliable worker for the last 20 years. Always on time, she seemed to enjoy her work. Meticulous and skillful, she trimmed cuticles, filed nails and applied polish to her clients' fingers and toes, taking pride in perfection.

Over the last month, however, Xi had started to show up late, sometimes with bedraggled hair and rumpled clothes. Her work was deteriorating, too. Regular customers were concerned, asking if she was ill or suffering.

At first, she said she was fine. But several times during the last week, she complained of a terrible headache. Finally, one morning, she did not come to work at all.

That day, Xi couldn't get out of bed. Her children, both in their 20s, found her in her room, confused, agitated, and mumbling incoherently.

By the time she reached the emergency room, she was totally disoriented, speaking gibberish and having trouble seeing clearly. Her family told the doctors that Xi, 48, had immigrated from Vietnam in 1986. She had always been in good health, they said, and took care of herself. She did not smoke or drink. Her greatest passion was cooking for her extended family who lived in a rowhouse in South Philadelphia.

Her physical exam was not very revealing. No fevers, no abnormal bumps or lumps, no worrisome sounds in her heart or lungs. Her doctors ordered a routine battery of studies, including a blood count, liver and kidney function tests, and a drug screen.

Every result was normal.

What was going on?

Since brain tumors often present with symptoms like hers, the doctors sent Xi for a CAT scan.

An hour later, the neuroradiologists called with their findings. While they had not seen any tumors, the scan showed that Xi had hydrocephalus, that is, excessive buildup of the tiny lakes that bathe the brain with cerebrospinal fluid. It appeared that she had some sort of mechanical obstruction to the channels that allow fluid to flow from the bottom of the brain.

The pressure that this excess fluid puts on the brain is extremely dangerous, so the neurosurgeons immediately took Xi to the operating room and decompressed the obstruction. Within 12 hours, the pressure in her brain was relieved, her headache disappeared and she became more lucid.

But what had caused the blockage? She was taken for an MRI, which revealed inflamed meninges at the base of the brain. The meninges are the thick layer of tissue that envelopes the brain and keeps the organ in a biologic cocoon, separated from the rest of the body. The next step was to look at the spinal fluid itself under the microscope and send it off to the lab for other chemical tests.

The fluid contained hundreds of white blood cells, only seen in inflammatory conditions such as infections, tumors, or autoimmune diseases. And the protein in the fluid was very high and the glucose level very low.

Infectious-disease doctors were asked to help figure out the mystery. There were not many suspects on the list of actors that could have produced hydrocephalus-inflamed basilar meninges and loads of white blood cells in the spinal fluid, along with high protein and a low sugar.

Cancers that spread tumors to the brain such as lymphoma and breast cancer could do this. So could Listeria, a bacterial infection seen in the elderly or those on steroids. The fungus Crytococcus was a possibility, but it is usually seen in patients on steroids or people with advanced, untreated HIV. The last possibility was tuberculosis of the meninges.


Solution:

Cultures from the microbiology lab came back negative for everything. Cytology studies looking for cancer found nothing. Likewise for tests of Xi's spinal fluid, which should have found signs of tuberculosis. Her chest X-ray was normal.

What infection could do all this, yet not show up in the spinal fluid?

And if she had TB, why was her chest X-ray normal?

Well, it happens. In cases of TB meningitis, the bug can be wily. So a TB skin test was done on her forearm. Two days later, a blotch one inch across appeared. Positive!

The doctors could not consider this a lockdown diagnosis, but it certainly seemed to be the most probable.

She was started on a standard combination of four anti-TB medications and steroids to reduce the inflammation in her brain, the result of the infection. The spinal fluid collected when she was first admitted had been saved because sometimes the bacteria can grow very slowly. Sure enough, four weeks later, tuberculosis appeared in the samples.

Within three months, Xi was back to near-normal, working at the nail salon.

Doctors speculated that Xi's TB had lain dormant for 20 years and spontaneously bloomed. This can happen. It was also possible that she had acquired the infection more recently. In the United States, 50 percent of tuberculosis cases are diagnosed in immigrants.

While this infection remains a chronic threat to our health, luckily there are excellent diagnostic and care options.


John Stern is an infectious-disease doctor at Pennsylvania Hospital.

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