The treatment of all of these forms of neurosyphilis consists of the administration of penicillin G, given intravenously in a dosage of 18 to 24 million units daily (3 to 4 million units q4h) for 10 to 14 days. The CDC recommends procaine penicillin and probenecid, and ceftriaxone as an alternative to penicillin if there is an allergy to either drug. Penicillin is so much preferred that even these patients are ideally desensitized to the drug. The Jarisch-Herxheimer reaction, which occurs after the first dose of penicillin and is a matter of concern in the treatment of primary syphilis, is of little consequence in neurosyphilis; it usually consists of no more than a mild temperature elevation and systemic leukocytosis.
In patients who have neurosyphilis and are co-infected with HIV, longer treatment and surveillance for relapse, which is more common in this group than patients without HIV, has been recommended An alternative to penicillin in these patients is doxycycline orally for 30 days.
The effects of treatment on certain symptoms of neurosyphilis, especially of tabetic neurosyphilis, are unpredictable and often little influenced by treatment with penicillin; they require symptomatic measures. Lightning pains may respond to gabapentin or carbamazepine. Analgesics may be helpful, but opiates should generally be avoided. Neuropathic (Charcot) joints require bracing or fusion. Atropine and phenothiazine derivatives are said to be useful in the treatment of visceral crises.
In all forms of neurosyphilis, the patient should be reexamined every 3 to 6 months after treatment and the CSF should be retested after a 6-month interval. If after 6 months the patient is free of symptoms and the CSF abnormalities have been reversed (disappearance of cells as well as reduction in protein, gamma globulin, and serology titers), no further treatment is indicated. Followup should include clinical examinations at approximately 12 months and another lumbar puncture. If a pleocytosis remains, these procedures should be repeated every 6 months. In the opinion of most experts, a persistent weakly positive serologic (VDRL) test after the cells and protein levels have returned to normal does not constitute an indication for additional treatment. Such a CSF formula ensures that the disease is quiescent or arrested. Others are not convinced of the reliability of this concept and prefer to give more penicillin. If at the end of 6 months there are still an increased number of cells and an elevated protein in the fluid, another full course of penicillin should be given. Clinical relapse is almost invariably attended by recurrence of cells and increase in protein levels. Rapid clinical progression in the face of a negative CSF suggests the presence of a nonsyphilitic disease of the brain or cord.
Until comparatively recently, the nonvenereal spirochetes were of little interest to neurologists of the Western world. Yaws, pinta, and endemic syphilis rarely, if ever, affected the nervous system. Leptospirosis was essentially an acute liver disease with only one variant causing nonicteric lymphocytic meningitis; tickand louse-borne relapsing fevers were medical curiosities that did not involve neurologists. However, in the late 1970s, a multisystem disease with prominent neurologic features was recognized in the eastern United States (it had been known in Northern Europe). It was named after the town of Lyme, Connecticut, where a cluster of cases was first recognized in 1975. An early skin manifestation of the disease had previously been described in Western Europe and referred to as erythema chronicum migrans. In 1982, Burgdorfer and colleagues identified the causative spirochetal agent, Borrelia burgdorferi. Later manifestations of the disease—taking the form of acute radicular pain followed by chronic lymphocytic meningitis and frequently accompanied by peripheral and cranial neuropathies—had long been known in Europe as the