The CSF formula in a number of other chronic or acutely recurring meningitides corresponds to that of aseptic meningitis. These include (1) the Vogt-Koyanagi-Harada syndrome, which is characterized by various combinations of iridocyclitis, depigmentation of a thick swath of hair (poliosis circumscripta) and of the skin, vitiligo, loss of eyelashes, dysacusis, and deafness (the pathologic basis of the syndrome is not known). (2) Mollaret recurrent meningitis, many instances of which have been associated with HSV-1 (Steel) and others (perhaps most) with HSV-2 infection (Cohen et al). The syndrome is characterized by episodes of acute meningitis with severe headache and sometimes low-grade fever, lasting for about 2 weeks, and recurring over a period of several months or years. In a few patients of ours, in whom no virus could be identified in the CSF, antiviral therapy met with some success, although corticosteroids seemed to reduce the severity of acute episodes. A proportion of these cases follow bouts of genital herpes and individual cases have been reported with EBV, herpes-6 in children, and other viruses. A special syndrome that has been associated with HSV-2 is that of aseptic meningitis and bladder failure and vaginal or vulvar pain after a bout of genital herpes (Elsberg syndrome as reviewed by Ellie and colleagues). (3) In some patients, the recurrent attacks are associated with encephalopathy and headache; this is probably identical to the illness called “pseudomigraine with temporary neurological symptoms” by Gomez-Aranda et al and described earlier by Bartleson et al. The entity, also known as HaNDL syndrome (“headache neurologic deficit and lymphocytic pleocytosis”) is allied more closely with the headache syndromes as discussed and cited in Chap. 9. (4) Allergic or hypersensitivity meningitis, in the past occurring in the course of serum sickness and now more commonly of autoimmune diseases such as lupus erythematosus, and in relation to certain medications such as nonsteroidal anti-inflammatory drugs and intravenously administered immunoglobulin. (5) Behçet disease, which is an important acute, recurrent inflammatory CNS disease, particularly in individuals of Middle Eastern origin. It is essentially a diffuse inflammatory disease of small blood vessels that has several other characteristic features such as oral and genital ulcers and is more appropriately considered with the vasculitides in Chap. 33.
Other Causes of Aseptic, Chronic, and