Presentation Title

A Distinctive Case of Primary Angiitis of the Central Nervous System

Speaker Credentials

Medical Education

Format

Poster

Start Date

6-11-2020 10:00 AM

End Date

6-11-2020 10:15 AM

Abstract

Primary central nervous system angiitis (PACNS) is an exceedingly rare pathology with an incidence of just 2.4 cases per 1 million person years. The primary criteria for diagnosis of PACNS are idiopathic neurological deficits, inflammatory arteritis of the CNS as evidenced by angiographic imaging or histology and the absence of systemic autoimmune conditions or vasculitides. We present the case of a middle aged gentleman, previously in good health, who presented to our institution in florida, acutely encephalopathic after receiving terrible family news. Initial workup revealed diffuse white and gray matter hypodensities and widespread diffusion restriction along with Myelin Basic Protein >150 and CRP 29. CSF was unremarkable and cardiac workup was negative. Cerebral arteriography revealed bilateral middle cerebral artery narrowing consistent with CNS vasculitis and the patient was started on high dose steroids. Brain biopsies were done and patient began improving after 2 weeks of high dose intravenous steroids. While elevations in CRP may be evidence of vasculitis, elevations of myelin basic protein are not usually seen in patients with CNS vasculitis. Differentiating CNS vasculitis from multiple sclerosis and stroke can be daunting for the experienced clinician and the clinical, laboratory and neuroradiological distinctions warrant further discussion.

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Nov 6th, 10:00 AM Nov 6th, 10:15 AM

A Distinctive Case of Primary Angiitis of the Central Nervous System

Primary central nervous system angiitis (PACNS) is an exceedingly rare pathology with an incidence of just 2.4 cases per 1 million person years. The primary criteria for diagnosis of PACNS are idiopathic neurological deficits, inflammatory arteritis of the CNS as evidenced by angiographic imaging or histology and the absence of systemic autoimmune conditions or vasculitides. We present the case of a middle aged gentleman, previously in good health, who presented to our institution in florida, acutely encephalopathic after receiving terrible family news. Initial workup revealed diffuse white and gray matter hypodensities and widespread diffusion restriction along with Myelin Basic Protein >150 and CRP 29. CSF was unremarkable and cardiac workup was negative. Cerebral arteriography revealed bilateral middle cerebral artery narrowing consistent with CNS vasculitis and the patient was started on high dose steroids. Brain biopsies were done and patient began improving after 2 weeks of high dose intravenous steroids. While elevations in CRP may be evidence of vasculitis, elevations of myelin basic protein are not usually seen in patients with CNS vasculitis. Differentiating CNS vasculitis from multiple sclerosis and stroke can be daunting for the experienced clinician and the clinical, laboratory and neuroradiological distinctions warrant further discussion.