Vasculitis in the central nervous system

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

doi:10.1002/art.2

Cited by 31 publications

(57 citation statements)

References 56 publications

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“…Therefore, a careful medical evaluation must be performed to confirm the diagnosis of PCNSV and to exclude other conditions associated with CNS vasculitis. In contrast to the study by Calabrese et al, in which lymphoproliferative disorders were observed in 30% of the cases with pathologically documented CNS vasculitis presenting with intracranial hemorrhage (2), none of our cases had evidence of leukemia or lymphoma.…”

Section: Discussioncontrasting

confidence: 99%

“…The frequency of intracranial hemorrhage in our cohort of patients with PCNSV is similar to the 11% frequency reported by Calabrese et al in a series of patients drawn from the English‐language literature or the Cleveland Clinic experience (2). Although the results are nearly the same, the populations of patients were different.…”

Section: Discussionsupporting

confidence: 89%

“…Primary central nervous system vasculitis (PCNSV) is a rare and poorly understood form of vasculitis that is limited to the brain and spinal cord (1, 2). It is heterogeneous in its presenting characteristics and therapeutic requirements (3–9).…”

mentioning

confidence: 99%

“…It is heterogeneous in its presenting characteristics and therapeutic requirements (3–9). Intracranial hemorrhage was documented in 11% of the cases in an analysis conducted by Calabrese et al of 168 patients drawn from the English‐language literature or Cleveland Clinic experience (2). Intracerebral hemorrhage was more common than subarachnoid hemorrhage.…”

mentioning

confidence: 99%

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Primary central nervous system vasculitis presenting with intracranial hemorrhage

Salvarani¹,

Brown²,

Calamia³

et al. 2011

Arthritis & Rheumatism

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Objective. To describe a subset of cases in a large retrospectively identified cohort of patients with primary central nervous system vasculitis (PCNSV) who present with intracranial hemorrhage.Methods. The study consisted of a cohort of 131 consecutive patients with PCNSV who were seen at the Mayo Clinic over a 25-year period from 1983 to 2007. The diagnosis of PCNSV was based on findings of brain or spinal cord biopsy, cerebral angiography, or both. Intracranial hemorrhage at presentation was defined as the presence of intracerebral or subarachnoid hemorrhage on computed tomography or magnetic resonance imaging (MRI) of the brain within 3 months of the date of PCNSV diagnosis. The clinical, laboratory, radiologic, and pathologic findings, therapy, and outcomes in patients presenting with intracranial hemorrhage were compared with those without intracranial hemorrhage.Results. Sixteen patients (12.2%) had evidence of intracranial hemorrhage at or near the time of diagnosis. Twelve patients had intracerebral hemorrhage, and 4 had subarachnoid hemorrhage. Twelve patients were diagnosed by findings on angiography and 4 by findings on CNS biopsy. Compared with the 115 patients without intracranial hemorrhage, the 16 patients presenting with intracranial hemorrhage were more frequently women, less frequently had altered cognition, a persistent neurologic deficit, or stroke at presentation, less frequently had MRI evidence of cerebral infarctions, and less frequently needed therapy at last followup.

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Section: Discussioncontrasting

confidence: 99%

Section: Discussionsupporting

confidence: 89%

mentioning

confidence: 99%

mentioning

confidence: 99%

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Primary central nervous system vasculitis presenting with intracranial hemorrhage

Salvarani¹,

Brown²,

Calamia³

et al. 2011

Arthritis & Rheumatism

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“…CNS vasculitis has been reported with Hodgkin's and non‐Hodgkin's lymphoma and angioimmunolymphoproliferative lesions (21). The lymphoproliferative disease can present outside the CNS or be restricted to the CNS.…”

Section: Differential Diagnosismentioning

confidence: 99%

A 60‐year‐old woman with headache, confusion, and hallucinations

Makol

Parisi

Petty

et al. 2011

Arthritis Care & Research

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History of the present illnessA 60-year-old woman, healthy and highly functioning at baseline, was in her usual state of health when she developed new-onset headache in a bilateral temporoparietal distribution. This was gradual in onset, associated with nausea and vomiting, and without photophobia or phonophobia. She was noted to be disoriented to time, place, and person by her family. She also rapidly developed auditory hallucinations, which prompted admission to an outside hospital. She was afebrile and hemodynamically stable. Kernig's and Brudzinski's signs were negative. Neurologic examination was nonfocal. A noncontrast computed tomography (CT) scan of the brain showed hypodensities with mild mass effect involving both posterior temporal lobes and left frontal subcortical white matter. Magnetic resonance imaging (MRI) of the brain (Figure 1) showed signal changes in the same locations with smaller areas of signal abnormality and localized mass effect involving the subcortical white matter of the frontal lobes and superior cerebellar vermis. Gradient-echo imaging demonstrated multiple tiny punctate areas of hemosiderin deposition in the brain bilaterally without focal intracranial hemorrhage or ischemia. She was empirically treated with acyclovir, ceftriaxone, and vancomycin for suspected meningoencephalitis. Cerebrospinal fluid (CSF) analysis showed 3 white blood cells (WBCs)/high-power field (hpf), 15 red blood cells (RBCs)/hpf, protein 16 mg/dl, and glucose 48 mg/dl. Herpes simplex virus (HSV) polymerase chain reaction (PCR) was negative. Electroencephalogram (EEG) showed right temporal and parietal sharp waves but no epileptogenic activity. Blood, CSF, and urine cultures returned negative, after which antibiotics were discontinued. She was treated symptomatically and headaches improved, but did not resolve completely. She was dismissed home and returned to work 2 weeks later.Four days later, she relapsed with severe headaches and disorientation. She was hospitalized and a repeat MRI (Figure 1) showed areas of leptomeningeal enhancement and multiple white matter lesions with T2 hyperintensity, patchy enhancement, and restricted diffusion. Repeat lumbar puncture showed 1 WBC/hpf, 8 RBCs/hpf, glucose 52 mg/dl, and protein 65 mg/dl. CSF studies were negative or normal for angiotensin-converting enzyme level, syphilis, Whipple's disease, Lyme, HSV, West Nile virus, and enterovirus PCR. Serologic studies for Western, Eastern, and California equine viral encephalitis were negative, as were CSF bacterial, mycobacterial, and fungal cultures. The erythrocyte sedimentation rate was 26 mm/hour and the C-reactive protein level was 1 mg/liter. Laboratory studies were normal or negative, including a complete blood cell count, liver and kidney functions, antinuclear antibody (ANA), extractable nuclear antigen (ENA) panel, antineutrophil cytoplasmic antibodies, lupus anticoagulant, and antiphospholipid antibodies. Chest/abdomen/pelvis CT and mammogram were also normal. The patient subsequently developed auditory hallucin...

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