Vasculitis in the central nervous system

Calabrese, Leonard H.; Duna, George F.; Lie, J.T.

doi:10.1002/art.1780400702

Cited by 32 publications

(30 citation statements)

References 58 publications

(47 reference statements)

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“…PACNS is a disease which primarily affects the leptomeningeal and cortical arteries or veins as granulomatous or lymphocytic vasculitis. 4 Therefore, PACNS was deemed even less likely in this case.…”

Section: Discussionmentioning

confidence: 88%

Idiopathic Carotid and Coronary Vasospasm: A New Syndrome?

Yoshimoto¹,

Matsuo²,

Umemoto³

et al. 2009

Journal of Neuroimaging

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Section: Discussionmentioning

confidence: 88%

Idiopathic Carotid and Coronary Vasospasm: A New Syndrome?

Yoshimoto¹,

Matsuo²,

Umemoto³

et al. 2009

Journal of Neuroimaging

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“…3,4 One of our patients presented with features suggestive of chronic meningitis and was treated for tuberculous meningitis elsewhere, similar to the presentation described earlier. 11 The PACNS-like neurological syndrome has also been reported in association with conditions like herpes zoster, amyloid angiopathy, demyelination, HIV infection and Hodgkin's disease. 1,[6][7][8][9]12 The laboratory work-up for systemic vasculitic disease activity is normal, 4 as was seen in our patients.…”

Section: Discussionmentioning

confidence: 99%

Primary angiitis of the central nervous system: MRI features and clinical presentation

Singh

John²,

Joseph

et al. 2003

Australasian Radiology

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We report the MRI features in a series of four patients with primary angiitis of the central nervous system (PACNS). Based on MRI features, clinical presentation, paraclinical investigations and laboratory tests, including cerebrospinal fluid (CSF) analysis, various differentials were considered. In two patients with MRI findings of cortical, subcortical and deep white matter lesions, lack of mass effect, focal areas of bleed and heterogeneous parenchymal, leptomeningeal or pial enhancement and a normal magnetic resonance angiography, a differential diagnosis of primary angiitis of the CNS was also considered. In all patients, an open brain biopsy was advised to establish a definitive diagnosis of PACNS. Here, we briefly discuss the MRI features, correlation with clinical presentation and paraclinical parameters for the diagnosis of this entity. We also briefly review the literature.

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“…Primary central nervous system vasculitis (PCNSV) is an uncommon condition in which vascular inflammatory lesions are limited to the brain and spinal cord. PCNSV has been generally considered a progressive CNS disorder that has a fatal course unless treated vigorously with glucocorticoids and immunosuppressive drugs (1)(2)(3). However, Calabrese et al (4) described a subset of patients who had a diagnosis based on angiography alone and who appeared to have a more benign course.…”

mentioning

confidence: 99%

Primary central nervous system vasculitis with prominent leptomeningeal enhancement: A subset with a benign outcome

Salvarani¹,

Brown²,

Calamia³

et al. 2008

Arthritis & Rheumatism

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Objective. Primary central nervous system vasculitis (PCNSV) is an uncommon condition that affects the brain and spinal cord. This study was undertaken to evaluate the clinical features and outcomes among patients with PCNSV who presented with prominent gadolinium meningeal enhancement on magnetic resonance imaging (MRI).Methods. Through retrospective review using the Mayo Clinic medical records linkage system, we identified 101 consecutive patients with PCNSV based on brain biopsy or conventional angiography (or both) between January 1, 1983, and December 31, 2003. We evaluated data on demographics, clinical findings, laboratory studies, imaging, biopsy of brain or spinal cord (or both), treatment, and neurologic outcome.Results. MRIs showed prominent leptomeningeal enhancement in 8 of 101 patients with PCNSV. In 6 of those 8, cerebral angiography or magnetic resonance angiography results were normal, but biopsy of the brain or spinal cord showed vasculitis in all 8. Granulomatous vascular inflammation was found in 6 specimens and was associated in 4 cases with vascular deposits of ␤-amyloid peptide. All 8 patients had a prompt response to therapy, with resolution of the MRI meningeal enhancement. Although 3 of the 8 patients had relapses during followup, the overall outcome was favorable. Patients with meningeal enhancement, compared with patients without enhancement, more commonly had substantial abnormalities of cerebrospinal fluid (100% versus 58%; P ‫؍‬ 0.02) and amyloid angiopathy (50% versus 12%; P ‫؍‬ 0.03).Conclusion. Prominent gadolinium leptomeningeal enhancement on MRI may point to a distinct subtype of PCNSV with small leptomeningeal artery vasculitis and rapid response to therapy.

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Vasculitis in the central nervous system

Cited by 32 publications

References 58 publications

Idiopathic Carotid and Coronary Vasospasm: A New Syndrome?

Idiopathic Carotid and Coronary Vasospasm: A New Syndrome?

Primary angiitis of the central nervous system: MRI features and clinical presentation

Primary central nervous system vasculitis with prominent leptomeningeal enhancement: A subset with a benign outcome

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