Tuesday, August 4, 2009

Rising from the ashes

Another well known person, another death … another opportunity for neurological education.


Frank McCourt, best known as the Pulitzer Prize winning author of Angela’s Ashes, died late last month at the age of 78. According to news reports, he had metastatic melanoma and was “gravely ill” with meningitis.

What was his immediate cause of death – melanoma or meningitis and what was the time course?

Are these two reports by those close to him in agreement, and do they help us in our diagnosis?

1. He was 78, gravely ill with meningitis and recently was treated for melanoma, the deadliest form of skin cancer and the cause of his death, said his publisher, Scribner.

2. We had this big dinner party in Roxbury (Conn.) last month, and he was there," said author Gay Talese, a longtime friend. "I made him a vodka martini, and he didn't look at all like he was going to disappear from the Earth in a month. He was very jovial, as usual."


We know that he had metastatic melanoma, which spreads to the brain 40 - 60% of the time. On autopsy 70 – 90% of people who died of melanoma have intracranial metastases. It is also the most common cause of bleeding (hemorrhage) in the brain from a metastatic cancer. Melanoma may metastasize to the meninges (covering of the brain); other causes of leptomeningeal metastases are cancers of the lung, breast, and GI tract.


So why does the second quote make it sound like he was doing well only a month before? Other reports have stated that he had 2 weeks of meningitis. What type of meningitis was this?

The possible types of meningitis he had were:

1. Carcinomatous meningitis -- cancer spreads to the leptomeninges (the covering of the brain). This causes an inflammatory reaction in the meninges, hence the “–itis.” Although most people think of infectious meningitis, this type is from irritation/inflammation and not infection. Sometimes leptomeningeal carcinomatosis is treated by radiation and intrathecal chemotherapy (chemotherapy into the sac surrounding the brain and spinal cord).

2. Infectious meningitis – bacterial, viral and fungal infections. He may have been immunosuppressed for several reasons: the cancer itself, chemotherapy, steroids to reduce the swelling/edema caused by the cancer. He may have had an opportunistic infection because of his immunosuppression or he may have had reactivation of a previous infection (e.g. syphilis).

3. Aseptic meningitis – a rare cause is ibuprofen (and other analgesic) use.

4. Chemical meningitis – from procedures that may irritate the meninges (e.g. putting chemotherapy, dye or anesthesia into the thecal sac).

5. Granulomatous/vasculitis – there is no reason to think that he had a separate condition, such as sarcoidosis, Wegener granulomatosis, Behcet disease, vasculitis or Vogt-Kayanagi-Harada disease.

6. Unknown -- in a third of people, no cause is found.


So, can we reconcile him dying from melanoma or dying from meningitis?

Yes.


The melanoma was probably ultimately responsible for the meningitis (either through leptomeningeal involvement or from infection due to immunosuppression). If he died from the meningitis, then melanoma is the underlying or proximate cause of death and meningitis is the immediate or dependent cause (or mode) of death.

Alternatively, the carcinomatous meningitis could have been a sign that the melanoma was metastatic to many other parts of his body – and then the immediate cause of death will remain a mystery.


Sound familiar?




- Dr. Daniel Kantor, MD BSE
Medical Director
Neurologique

info@neurologique.org
http://www.neurologique.org/

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