Neurosyphilis, or chronic heavy metal poisoning: Karen Blixen's lifelong disease.
BACKGROUND: Since the 1490s, the treatment of syphilis has consisted of heavy metals--first mercurial and later arsenic and bismuth preparations. Tabes dorsalis, as described by Duchenne in the 1850s, is made up of various characteristic neurologic symptoms. "Gastric crises," sudden stabbing pains followed by vomiting and diarrhea, was originally included by Duchenne, but later, syphilologists disputed its relevance to syphilis. Poisoning by heavy metals, including mercury, may produce similar pain reactions and tabes-like neurologic symptoms. METHODS: According to an earlier published pathography, the Danish author Karen Blixen (1885-1962), also known under the pseudonym Isak Dinesen, suffered from a lifelong disease described as tabes dorsalis. She got syphilis in 1914 and took mercury pills for a year, after which she experienced a severe mercurial intoxication. The Wassermann reaction (WR) in peripheral blood was positive only once, in 1915, before treatment with arsphenamine (Salvarsan), which she received during hospitalization in Copenhagen in 1915 to 1916. Her spinal fluid was examined several times from 1915 to 1956. Apart from an increased number of cells in 1915, the fluid remained unremarkable and the WR was always negative. RESULTS AND CONCLUSIONS: It was postulated that her illness, ending with a cachectic state, was the result of heavy metal poisoning from the various treatments and not a monosymptomatic tabes dorsalis with negative serology.