Drake KW, Adam RD. Neurology 2009; 73:1780-1786
Most patients present with headache only (77%) while 23 % had nuchal rigidity, 39 % had mental status changes, and one third focal signs especially gait disturbance or ataxia, may be due to hydrocephalus. Risk factors are HIV/chronic steroids but not diabetes. Also, liver failure, hem/lymph malignancies, and ESRD. Increased risk for males (2:1), Hispanic, black and Asian patients in endemic areas (black patients have 6:1 risk). CSF had mononuclear pleocytosis, 69 % had abnormally low glucose, occasionally high protein or eosinophils. CSF antibody/culture often negative on presentation (50 %), but in those patients, serum antibody test is usually positive. Also CSF cultures or brain biopsy occasionally used for diagnosis. Imaging may show basilar meningitis or hydrocephalus and vasculitic infarcts. Many patients had antecedent illnesses, including respiratory, that may or may not have been diagnosed as coccidio or occasionally osteomyelitis, lymphadenitis, skin lesions, and soft tissue masses. Treatment is with azoles, esp. fluconazole which has supplanted amphotericin and others. Relapse can occur years or even decades later if azole therapy is stopped. Shunts are frequently needed for treatment of hydrocephalus. Prognosis is now good for those compliant with therapy.
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