Page 158 - The Vasculitides Volumes 2

134 Patricia K. Coyle

Diagnosis is based on culture and stain. Treatment includes administration of doxycycline,
oxytetracycline, or cephalosporin.

Other Bacterial Agents

Mycoplasma
Mycoplasmas are very small bacteria that have a plasma membrane boundary, but lack a

cell wall. The nervous system is a major extrapulmonary target, and neurologic disease can
occur after primary atypical pneumonia or de novo [36]. Mycoplasma encephalitis reflects
direct brain invasion or an immune-mediated syndrome. There is evidence for vascular injury
and microthrombi, with endothelial cell infection [36, 37]. Stroke occurs in both children and
adults [38]. Diagnosis is based on serology and PCR when positive. Therapy involves a
course of macrolide antibiotics, although neurologic involvement may be post-infectious and
immune-mediated. Anticoagulation can be considered for thrombotic disease [38].

Bartonella
Bartonella are facultative gram negative intracellular bacteria that cause human and

zoonotic disease. Bartonella henselae causes cat scratch disease. Several Bartonella species
are associated with neuroretinitis, a retinal vasculitis. Immunocompromised hosts are
vulnerable to more severe infections. Bartonella species can produce cutaneous and systemic
vasoproliferative lesions [39]. Bartonella henselae is known to invade and colonize vascular
endothelial cells, among others [40]. Among a broad neurologic spectrum, there are rare cases
of ischemic stroke and cerebral arteritis. Diagnosis is based on serology and PCR. Therapy
involves doxycycline, or azithromycin.

Trophyrema Whippelii
Tropheryma (T.) whippelii, a member of gram positive Actinobacteria family, is the

etiologic agent of Whipple disease is the soil-borne gram positive bacillus. CNS involvement,
which occurs in up to 43% of cases, may be the initial presentation of infection in 5% of cases
[41]. CNS involvement occurs in the setting of active Whipple disease, as well as in those
relapsing previously treated disease, and isolated CNS involvement [42]. Stroke presentation
while very rare has been described [43] and should be in the differential diagnosis of CNS
vasculitis. Stroke also occurs with leptomeningeal arterial fibrosis and thrombosis, or
associated with endocarditis [41, 44]. Clues to diagnosis are extraneural disease including
weight loss, fever, polyarthritis, diarrhea, and uveitis. Oculomasticatory myorhythmia and
supranuclear gaze palsy are characteristic neurologic features. The diagnosis can be
confirmed with PCR and tissue biopsy that reveals macrophages that stain positive for
glycogen with the Periodic Acid Schiff (PAS) assay, or the identification of the causative
organism by immunoreactive antigen. Effective treatment includes administration third
generation cephalosporins followed by long-term, on average two years of trimethoprim-
suflamethoxazole (TMP-SMZ) and doxycycline.

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