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First case history shows fungal meningitis can destroy brain fast

Exserohilum, a type of black mold, has killed most of the victims in an outbreak of fungal meningitis.
Exserohilum, a type of black mold, has killed most of the victims in an outbreak of fungal meningitis.Centers for Disease Control and Prevention

She had a history of pain, the 51-year-old woman who showed up at a Maryland emergency room with a headache so bad it made her face hurt. Within 10 days she was dead, one of the first victims of an outbreak of fungal meningitis that has killed at least 20 people and made nearly 260 sick.

The case is the first to be described in medical detail, and shows that doctors need to act quickly if someone shows up with symptoms after having been given an injection that may have been contaminated with fungus. 

She’d been treated for neck pain and a chronic condition called fibromyalgia that is defined by aches and pains all over the body and general weakness and fatigue. She had decided to try a new treatment, an injection in her neck of a steroid to help stop the pain there. It works in some patients – studies show it provides relief about half the time.

It should have been an in-and-out procedure. The steroid injections are considered very safe. The woman had no risk factors, Dr. Jennifer Lyons of the Howard Hughes Medical Institute and Johns Hopkins University in Baltimore reported. “She had not received injections previously, had no history of immune compromise or trauma, and was not taking any long-term medications,” they wrote in the report published in the Annals of Internal Medicine.

A week later, she developed the severe headache – so bad it drove her to the ER. Now, of course, doctors know that anyone who has had a steroid injection and shows up with symptoms like a severe headache should be checked for infection. But this woman was one of the first affected, before word got out.

Headaches are a common side-effect of any injection to the spinal cord. The ER staff did a CT scan to make sure she wasn’t having a stroke or perhaps suffering from a brain tumor and she was sent home.

But she was back the next day, suffering from double vision and nausea. She was dizzy and off-balance – all classic symptoms of meningitis. But she didn’t have a fever and her blood looked normal: no evidence of the immune system reaction seen when meningitis is caused by bacteria or viruses. An MRI didn’t show anything amiss.

All this time, the fungus must have been spreading through the patient’s brain and spine. (She hasn’t been identified in the report to protect her privacy and that of her family). State and federal investigators have found three different types of mold in samples of steroid taken from the New England Compounding Center in Massachusetts and from spinal fluid taken from some of the victims.

Fungal meningitis is rare, and most of the cases have been caused by a mold called Exhiloserum that had never been known to cause meningitis before. Doctors have reported that the mold seems to grow very slowly and cause very subtle symptoms at first.

The Centers for Disease Control and Prevention believes that quick treatment will help, and with the Food and Drug Administration has put out a call for doctors to proactively check with patients who have been treated with any product from NECC – especially injections.

In the case of the 51-year-old woman, the steroid injection mainlined mold right into the fluid that bathes the brain and the spine.

Her doctors didn’t suspect this, although they did an MRI and checked for viruses or bacteria. As she deteriorated, losing her ability to speak and breathe on her own, they dosed her with antivirals and antibiotics, as well as steroids. When they first checked her spinal fluid, it didn’t look that bad. Now doctors know that even small signs of inflammation might mean a patient is infected with these particular molds, and they should start immediate treatment with antifungals.

When she continued to worsen, mystified doctors transferred the patient to Johns Hopkins, where Lyons and a large team of colleagues took over.

There tested her for herpes, shingles, Epstein-Barr virus, West Nile and another virus called cytomegalovirus. They even checked for fungus – a type of yeast called cryptococcus and one called histoplasma that is found in animal droppings and that can cause an alarming type of pneumonia. Negative. It would not have occurred to them to test for a black mold more commonly known for infecting grains, and the tests probably would not have shown it, anyway.

By now the infection was showing up in her brain, and there was swelling at the place on her neck where she had been injected. By the ninth day she was in a coma and they started an antifungal drug, liposomal amphotericin B. But she was brain-dead the next day. Finally, tests revealed the Exserohilum black mold that had killed her.

An autopsy showed it had ravaged her brainstem and eaten into blood vessels and her spinal cord.

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