Walsh & Hoyt: Acute Febrile Cerebrovasculitis

In 1986, Wenzel et al. reported the occurrence of fever, headache, altered mental status, and multifocal signs in five patients who also complained of abdominal pain. Neurologic disturbances included decorticate and decerebrate posturing, seizures, plantar responses that were bilaterally extensor, nuchal ridigity, somnolence, simultagnosia, and slurred speech. Neuro-ophthalmologic manifestations included papilledema in two patients, vertical nystagmus in one patient, and ocular bobbing in another. One patient had a trochlear nerve paresis associated with generally slowed saccadic eye movements, one had a unilateral abducens nerve paresis also associated with diffuse slowing of saccades, and one patient had bilateral abducens nerve pareses. A fourth patient reported diplopia but had no objective evidence of ocular motor dysfunction or strabismus, although he did have an incomplete homonymous hemianopsia. The fundi of two patients were abnormal and suggested microvascular disease. Ophthalmoscopy in one of these patients showed numerous cottonwool spots in the retinas, multiple branch retinal artery and vein occlusions, vitritis, and retinitis. Ophthalmoscopy in the second patient showed an area of nodular choroiditis in one eye. Laboratory findings included a normal-to-high white blood cell count and normal platelet counts. All five patients had increased intracranial pressure associated with CSF pleocytosis. CT scanning gave normal results in three of the five patients but showed diffuse and focal cerebral edema in the other two. All five patients had EEGs that showed diffuse slowing. Two of the patients died. Both brain biopsies and postmortem material showed cerebral vasculitis and perivasculitis that were worse in the venules and capillaries. The greatest number of lesions were present in the brainstem and thalami, but numerous lesions were present throughout the white and gray matter. Although serologic testing showed no evidence of antibodies to R. rickettsii, R. typhi, or R. prowazekii, the clinical manifestations and pathologic findings were thought to be most consistent with a rickettsial infection. Wenzel et al. called the condition acute febrile cerebrovasculitis.