Objective:

To present an atypical case of a 75-year-old male who developed fulminant idiopathic, hemorrhagic vasculitis.

Background:

Primary central nervous system vasculitis (PCNSV) is a rare condition characterized by inflammation of cerebral vessels, without associated systemic disease. Patients typically present with a subacute clinical course with varied symptoms of headaches, seizures, cognitive changes, and focal neurological deficits.

Design/Methods:

N/A

Results:

A 75-year-old male presented after being found unresponsive but regained consciousness with an initially non-focal neurologic exam. Initial computed tomography (CT) and angiography of the head was negative. He became acutely unresponsive within minutes of initial CT and subsequent magnetic resonance imaging (MRI) of the brain revealed a subacute pontine hemorrhagic infarct. Neurologic exam demonstrated trace brainstem reflexes, fixed 5 mm pupils, and absent cough, gag and corneal reflexes. Oculocephalic response was preserved and he generated spontaneous breaths. Follow-up MRI showed extensive periventricular restricted diffusion with confluent FLAIR signal abnormality and faint patchy enhancement, affecting the deep brain structures, entirety of the brainstem and visualized upper cervical cord, as well as patchy involvement of the cerebrum and cerebellum. Despite aggressive efforts, he lost all brainstem reflexes within 24 hours of onset and expired five days from the initial presentation. Pathologic examination of the brain revealed global edema (1630 grams), uncal and tonsillar herniation, and a dorsal pontine hematoma. Histopathologically, severe neutrophilic vasculitis with fibrinoid necrosis was found involving the cerebrum, brainstem, and cerebellum. No evidence of bacterial, fungal, viral, or parasitic infection was identified. There was no evidence of neoplasia. No similar vascular lesions were identified in any other organ system evaluated at autopsy.

Conclusions:

We present a case of rapidly progressive CNS inflammation originating in the brainstem. Although an atypical time course for vasculitis, the pathologic findings are most consistent with idiopathic neutrophilic vasculitis although hemorrhagic leukoencephalitis remains a consideration.