Primary angiitis of the central nervous system: Emerging variants

Manchester Royal Infirmary, Oxford Road, Manchester M13 9WL, UK.
QJM: monthly journal of the Association of Physicians (Impact Factor: 2.5). 10/2005; 98(9):643-54. DOI: 10.1093/qjmed/hci098
Source: PubMed


Primary angiitis of the central nervous system (PACNS), a serious disease, has not featured prominently in the spectrum of multi-organ disease seen in vasculitis clinics.
To evaluate the presentation, natural history and features of PACNS variants and compare to those of systemic vasculitides.
Retrospective analysis.
Patients (n=105) presented during 1988-2003 to a tertiary regional vasculitis clinic receiving unselected disease types. Data were collected from a clinical database, patient and laboratory records.
The frequency of PACNS presentation rose over the study period, compared with most of the other vasculitides. When PACNS was divided into small- and middle-sized vessel disease (SVD/MVD), their clinical courses differed substantially. SVD PACNS was responsive to immunosuppressive drugs, but relapsed during prolonged periods in all patients on maintenance immunosuppressives, or after withdrawal of treatment, causing recurrent, severe and irreversible CNS injury. MVD PACNS had isolated episodes at presentation, with a paucity of relapses during prolonged follow-up.
Similarities between SVD PACNS and microscopic polyarteritis suggest the former may represent a limited form of the latter. MVD PACNS has a distinctly more benign relapse pattern than its multisystem counterpart polyarteritis nodosa. Acute-phase serology was useful in designating inflammatory processes at presentation of patients presenting with encephalopathy caused by SVD only, but were unhelpful in defining relapses in this form of PACNS, the definition of which in all cases rested on clinical assessment and MR scanning. Direct cerebral angiography was not diagnostic in any case of SVD PACNS; positive brain biopsy is diagnostically unequivocal, but the total clinical syndrome with imaging may establish a diagnosis with highest probability. In MVD PACNS, angiography with MR scan proved diagnostic. We suggest an algorithm for a rational, minimally invasive approach to investigation. In PACNS, SVD and MVD are important variants, and decisions about therapy should incorporate these distinctions.

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    • "Reversible cerebral vasoconstriction syndrome (RCVS) has been proposed as a unifying term for a variety of similar syndromes [1] [2] including Call-Fleming syndrome [3], benign angiopathy of the central nervous system [4], postpartum cerebral angiopathy Abbreviations: RCVS, reversible cerebral vasoconstriction syndrome; CT, computerized tomography; CTA, computerized tomographic angiography; MRI, magnetic resonance imaging; MRA, magnetic resonance angiography; DSA, digital subtraction angiography; SAH, subarachnoïd hemorrhage; AVM, arteriovenous malformation ; CSF, cerebrospinal fluid; HLH, homonymous lateral hemianopia; mRS, modified Rankin score. * Corresponding author. "

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    • "An average annual incidence rate of 2.4 cases per 1,000,000 person-years was found by a report from US [1]. Histopathology usually reveals granulomatous inflammation affecting arterioles and small arteries of the parenchyma and/or leptomeninges [2]. Non-specific clinical manifestations and various imaging findings often lead to an incorrect or delayed diagnosis and treatment [3], particularly for an extremely rare form of tumor-like lesion. "
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    ABSTRACT: Isolated angiitis of the central nervous system (IACNS) is a rare but severe vascular disease, which could present like an isolated inflammatory lesion on magnetic resonance imaging (MRI). To date, only a few such cases with tumor-like IACNS have been reported. A 35-year-old woman presented with headache and left-sided weakness. MRI scans initially mislead us to a diagnosis of glioblastoma (GBM). Surgery was performed. The mass was sub-totally resected. Pathological examination confirmed a cerebral vasculitis. Radiological features, such as disproportionate mass effect, striped hemorrhage and abnormal enhancement of adjacent vessels, could be helpful to distinguish a tumor-like IACNS from a GBM. Single therapy with high doses of steroid did not improve the patient's condition. Combined therapy with prednisolone and cyclophosphamide showed great benefit to the patient. No relapse occurred during the period of 18 months follow-up. Although a tumor-like IACNS has no established imaging features, a diagnosis of tumor-like IACNS should be suspected when MRI shows inappropriate presentations of a tumor. Greater awareness of this potential manifestation of IACNS may facilitate more prompt diagnosis and treatment.
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    • "Based on the findings of 105 patients with suspected PACNS, a differentiation in small-and medium-vessel disease was suggested [MacLaren et al. 2005]. In this study, mediumvessel PACNS was compared with systemic PAN, had a benign course with isolated episodes and only rare relapses, and DSA with MRI was considered diagnostic. "
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    ABSTRACT: Vasculitides are characterized by inflammation and necrosis of the blood vessel wall. Large vessels including the aorta are affected in giant-cell arteritis, medium-size arteries in classic polyarteritis nodosa. The small-vessel vasculitides are separated in those with antineutrophil cytoplasm antibodies (ANCA) and those without. The primary angiitis of the central nervous system (PACNS) is a rare disorder affecting both medium- and small-sized vessels. Major symptoms of cerebral vasculitis are stroke, headache and encephalopathy. Diagnosis is based on laboratory and imaging findings. When cerebral affection occurs in systemic vasculitis an acute inflammatory response with raised erythrocyte sedimentation rate and increased values of C-reactive protein is present. In many cerebral vasculitides including PACNS, CSF studies reveal inflammatory findings. Magnetic resonance imaging, including ADC maps, diffusion and gradient echo sequences, is the investigation of choice to detect and monitor cerebral involvement. Certain MRI techniques and 18-fluorodeoxyglucose positron emission tomography allow the visualization of vessel wall inflammation when the lumen is still unaffected on angiography. The treatment recommendations for cerebral angitis are derived from protocols for systemic vasculitides. In general, a combination of steroids and pulse cyclophosphamide (CYC) is recommended for induction treatment. An alternative option is the use of the anti- CD20 antibody rituximab. Methotrexate, azathioprine and mycophenolate mofetil are recommended as alternatives to CYC once remission is achieved.
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