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AIDS patients as well as neuroretinitis in both immunocompromised and immune-competent patients Demonstration of elevated complement- xing titers and detection of the organism by PCR or by silver staining from an excised lymph node are diagnostic A single high antibody titer is inadequate for this purpose Treatment Erythromycin, chloramphenicol, or doxycycline seem to eradicate the infection; macrolide antibiotics have been recommended in recalcitrant cases Most patients recover completely, but one of our patients and a few reported by others have died Anthrax This rare form of meningoencephalitis is included here because of the current interest in Bacillus anthracis as a weapon of warfare Lanska was able to collect from the literature 70 patients with meningeal infection, most of whom were encephalopathic He has estimated that fewer than 5 percent of infected individuals will acquire a meningoencephalitis; in the 2001 US outbreak, only 1 of 11 cases with anthrax pneumonitis developed this complication Re ecting the main site of natural infection, the majority of cases originated in cutaneous anthrax In addition to a typically fulminating course after a prodrome of one or several days, the exceptional feature was a hemorrhagic and in ammatory spinal uid formula Subarachnoid hemorrhage was prominent in autopsy material, re ecting presumably necrosis of the vessel wall as a toxic effect of B anthracis Although natural isolates are sensitive to penicillin, bioengineered strains are resistant; therefore combined treatment with rifampin, vancomycin, and either cipro oxacin or doxycycline has been recommended initially The bene t of speci c antitoxin is uncertain once meningoencephalitis has occurred Brucellosis This worldwide disease of domesticated livestock is frequently transmitted to humans in areas where the infection is enzootic In the United States, it is distinctly rare, with 200 cases or less being reported annually since 1980 During the 1950s it was a fashionable explanation for chronic fatigue In the Middle East, infection with Brucella is still frequent, attributable to the ingestion of raw milk In Saudi Arabia, for example, al Deeb and coworkers reported on a series of 400 cases of brucellosis, of which 13 presented with brain involvement (acute meningoencephalitis, papilledema and increased intracranial pressure, and meningovascular manifestations) The CSF showed a lymphocytic pleocytosis and increased protein content Blood and CSF antibody titers to the organism were greater than 1/640 and 1/128, respectively Prolonged treatment with rifampin, cotrimoxazole, and doxycycline suppressed the infection Whipple Disease This is a rare but often discussed disorder, predominantly of middle-aged men Weight loss, fever, anemia, steatorrhea, abdominal pain and distention, arthralgias, lymphadenopathy, and hyperpigmentation are the usual systemic manifestations Less often, infection is associated with a number of neurologic syndromes It is now known to be caused by a gram-positive bacillus, Tropheryma whippelii, which resides predominantly in the gut Biopsy of the jejunal mucosa, which discloses macrophages lled with the periodic acid Schiff (PAS)-positive organisms, is diagnostic PAS-positive histiocytes have also been identi ed in the CSF as well as in periventricular regions, in the hypothalamic and tuberal nuclei, or diffusely scattered in the brain The neurologic manifestations most often take the form of a slowly progressive memory loss or dementia of subacute or early chronic evolution Supranuclear ophthalmoplegia, ataxia, seizures,.

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myoclonus, nystagmus, and a highly characteristic oculomasticatory movement described as myorhythmia (which looks more to us like rhythmic myoclonus) have been noted less often than the dementing syndrome The rhythmic myoclonus or spasm occurs in synchronous bursts involving several adjacent regions, mainly the eyes, jaw, and face Almost always the myorhythmias are accompanied by a supranuclear vertical gaze paresis that sometimes affects horizontal eye movements as well Presumably, the neurologic complications are the result of in ltration of the brain by the organism, but this has not been satisfactorily established Approximately half of the patients have a mild pleocytosis and some of these have PAS-positive material in the CSF A variety of brain imaging abnormalities have been recorded, none characteristic, but either enhancing focal lesions or a normal scan may be found The diagnosis is made mainly from PAS staining of an intestinal (jejunal) biopsy, as already mentioned, supplemented by PCR testing of the bowel tissue or biopsy material from brain or lymph node In cases of subacute progressive limb and gait ataxia occurring in middle-aged or older men in whom no cause is uncovered by less invasive means, it is justi able to perform these tests (see Chap 5) Rarely, the neurologic symptoms may occur in the absence of gastrointestinal disease (Adams et al) In the extensive review of 84 cases by Louis and colleagues, 71 percent had cognitive changes, half with psychiatric features; 31 percent had myoclonus; 18 percent, ataxia; and 20 percent had the oculomasticatory and skeletal myorhythmias that are virtually pathognomonic of Whipple disease (Schwartz et al) Treatment A course of penicillin and streptomycin followed by trimethoprim-sulfamethoxazole or ceftriaxone and continued for 1 year is one of the currently recommended treatment regimens An alternative approach is 2 weeks of ceftriaxone followed by treatment with trimethoprim-sulfamethoxazole or a tetracycline for a year Antibiotic-resistant cases and instances of relapse after antibiotic treatment are known The review by Anderson may be consulted for details Acute Toxic Encephalopathy Lyon and colleagues described acute encephalopathy of obscure origin in children, a febrile and sometimes fatal illness that could not be ascribed to direct infection of the nervous system During the height of a systemic bacterial or sometimes viral infection, the child sinks into coma, seizures are infrequent, the neck is supple, and the spinal uid shows no changes or only a few cells This is undoubtedly an illness of diverse causes, among them uid overload and electrolyte imbalance, Reye syndrome (page 969), and, possibly most common, postinfectious encephalitis Nonetheless, cases continue to be reported, such as those of Thi and colleagues, which can only be classi ed as a noninfectious bacterial encephalopathy or encephalitis A relationship to the septic encephalopathy of adults, which has been emphasized by the group from London, Ontario, is possible but unproved The term acute toxic encephalopathy still has some utility in these cases, but a careful search for better-characterized causes of febrile coma must be undertaken.