Abstract Details

Title Fulminant Neutrophilic Vasculitis of the Central Nervous System

Topic Cerebrovascular Disease and Interventional Neurology

Presentation(s) P11 - Poster Session 11 (8:00 AM-9:00 AM)

Poster/Presentation
Number 14-005

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.

Authors/Disclosures
Camille Barbour, MD (Camille Barbour, MD) PRESENTER	Dr. Barbour has nothing to disclose.
Parin Nanavati, MD (Parin Nanavati)	Dr. Nanavati has nothing to disclose.
Lisa Bianco, DO	Dr. Bianco has nothing to disclose.
William R. Meador, MD	Dr. Meador has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for ForensisGroup. The institution of Dr. Meador has received research support from PCORI. The institution of Dr. Meador has received research support from ROCHE/GENENTECH.
Neda Wick	Neda Wick has nothing to disclose.
Rati Chkheidze, MD	Dr. Chkheidze has nothing to disclose.
Michael J. Lyerly, MD, FAAN (University of Alabama At Birmingham)	Dr. Lyerly has nothing to disclose.

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