Primary Angiitis Of Central Nervous System Research Articles

Ruptured Saccular Aneurysm Caused by Necrotizing Arteritis of the Polyarteritis Nodosa Type in Primary Angiitis of Central Nervous System.

Primary angiitis of central nervous system (PACNS) is a rare idiopathic vasculitis that typically involves small arteries. An 18-year-old woman was operated on for resection of a ruptured aneurysm in a cerebral artery. Multiple aneurysms of cerebral arteries had been detected by neuroimaging examinations since the age of 12, and she had been administered drugs following a diagnosis of PACNS since the age of 15. The resected aneurysm was a ruptured saccular aneurysm occurring in a medium-sized artery. Histologically, necrotizing arteritis of the polyarteritis nodosa (PAN) type was noted in the aneurysmal wall. It consisted of an admixture of acute and healing stages. In the acute stage, fibrinoid necrosis in the intima and media and intense inflammatory cell infiltrate in the entire wall were present. The inflammatory cells mainly consisted of lymphocytes, including plasma cells, neutrophils, and macrophages. In the healing stage, disappearance of fibrinoid necrosis, fibrosis in the intima and media, and scarce inflammatory cells were noted. The acute stage was mainly present near the ruptured site. From these findings, the aneurysm was considered to have been caused by necrotizing arteritis of the PAN type. Although saccular aneurysmal formation in a medium-sized artery is rare in PACNS, an understanding of aneurysms produced by necrotizing arteritis of the PAN type offers useful information for the diagnosis and treatment of PACNS.

Inflammatory cerebral amyloid angiopathy with poor outcome – case report

The article presents a clinical case of a 62-year-old patient diagnosed with inflammatory amyloid angiopathy after extensive diagnostic imaging and based on the characteristic radiological picture. Inflammatory amyloid angiopathy is a rare condition with intermediate features between the classic form of amyloid angiopathy and primary central nervous system angiitis. Multiple characteristic features suggesting the diagnosis may be seen on brain magnetic resonance imaging. There are no laboratory markers of the disease. Knowledge of this disease is important as it allows for a quick diagnosis and initiation of appropriate treatment. The response to treatment varies, with full symptom resolution in some patients, and resistance to treatment in others, such as the patient described in this case report.

Primary Central Nervous System Angiitis Mimicking a Space-Occupying Lesion

Abstract Background and Purpose Intracranial space-occupying lesions are a sine qua non for neoplastic lesions; however, occasionally non-neoplastic lesions mimic neoplastic lesions, leading to diagnostic dilemmas. We report our experience with three patients who presented with a progressive hemispheric syndrome and the diagnostic considerations involved in the cases. Materials and Methods In this retrospective study, we included three patients with primary angiitis of central nervous system (PACNS) who underwent craniotomy and biopsy, suspecting it to be mass lesions. Demographic features, clinical features, radiological features, histopathology, treatment, and clinical outcomes were studied. Results Majority were males. The male:female ratio was 2:1. Lobar involvement was common. MR brain with contrast showed features of high-grade glioma. Despite hemispheric involvement, there was no mass effect. Perilesional edema was seen in all cases. All underwent craniotomy and biopsy; histopathology was consistent with PACNS. All patients were treated with corticosteroids and cyclophosphamide. Rituximab was used in addition to cyclophosphamide in one patient. At 2 years follow-up, two patients were in disease remission and one patient died due to disease progression. Conclusion PACNS has a protean clinical manifestation. A high index of suspicion is required in cases with atypical clinical presentations, radiological features, and normal angiograms. Early histological diagnosis and aggressive immunotherapy with high-dose corticosteroids combined with intravenous cyclophosphamide yields favorable outcomes.

Primary Angiitis of the Central Nervous System - Diagnosis and Management.

Primary angiitis of central nervous system (PACNS) is a rare idiopathic disorder affecting blood vessels of brain, spinal cord, and meninges, consequently leading to infarct and less frequently hemorrhage. CNS vasculitis can also occur as part of systemic vasculitis or secondary to autoimmune diseases or infections. The clinical manifestations of PACNS are non-specific and no single laboratory investigation or neuroimaging finding can reliably diagnose this condition. Histopathological evidence of transmural inflammation of blood vessels of CNS is the gold standard, but is generally pursued subsequent to conventional angiogram (CA) because of its invasive nature. The differentials of PACNS are exhaustive and include systemic vasculitis, secondary vasculitis, non-inflammatory intracranial vasculopathies, demyelination, and neoplasm. These alternative conditions can often be distinguished by history, examination, immunological testing, cerebrospinal fluid analysis, and neuroimaging. CA can detect vasculitic changes in the large to medium cerebral arteries but the specificity is low. Recent advancements in vessel wall imaging techniques have further enabled the distinction of various intracranial vasculopathies from CNS vasculitis. The disease has considerable morbidity and fatality unless timely treatment with immunosuppressive agents is initiated. Induction therapy with glucocorticoids and cyclophosphamide followed by azathioprine, mycophenolate mofetil, or methotrexate as maintenance therapy is the cornerstone of management. Biological agents such as rituximab and anti-tumour necrosis factor alpha inhibitors (infliximab and etanercept) may be used in refractory cases. This review discusses the approach to the diagnosis, determinants of outcome, and management.

High-Resolution Vessel Wall Imaging in Primary Angiitis of Central Nervous System.

Background:High-resolution vessel wall imaging (HRVWI) can aid in differentiating the various intracranial vasculopathies, but has been sparingly used in the diagnosis of primary angiitis of central nervous system (PACNS). This study is aimed to describe the vessel wall imaging characteristics of PACNS.Materials and Methods:Patients with confirmed diagnosis of PACNS according to the Calabrese and Mallek criteria who had abnormal HRVWI were included in this retrospective descriptive study. Magnetic resonance image of brain, conventional four-vessel cerebral digital subtraction angiogram, and HRVWI were read by a neuroradiologist. The vessel wall parameters assessed were T1W and T2W appearances, pattern of wall thickening and contrast enhancement, and remodeling index.Results:HRVWI done in 21 patients with PACNS yielded abnormality in 20 (95.2%) who were included in the analysis. The mean age at presentation was 42.55 ± 9.48 years and 14 (70%) were males. The median number of vessels involved were four (range 2–12). The commonest vessels affected were proximal middle cerebral artery (70%) and internal carotid artery (55%). Vessel wall thickening was concentric, eccentric, and absent in 12 (60%), 1 (5%), and 7 (35%) patients, respectively. Vessel wall enhancement was diffuse in 17 (85%), eccentric in 1 (5%), and absent in 2 (10%) patients. One patient had T2W hyperintense stenotic lesion. Remodeling index was negative in 11 (55%) patients.Conclusion:Distinctive vessel wall appearances were observed by HRVWI in PACNS, concentric vessel wall thickening and enhancement being more frequent. Hence, HRVWI can be considered as an additional noninvasive imaging modality in the diagnosis of PACNS.

Extended stereotactic brain biopsy in suspected primary central nervous system angiitis: good diagnostic accuracy and high safety

ObjectiveTo evaluate the diagnostic accuracy and safety of extended stereotactic brain biopsy (ESBB) in a single center cohort with suspected primary angiitis of the central nervous system (PACNS).MethodsA standardized stereotactic biopsy targeting MRI-positive lesions and collecting samples from the meninges and the cortex as well as from the white matter was performed in 23 patients with clinically suspected PACNS between 2010 and 2017. The relationship between biopsy yield and clinical characteristics, cerebrospinal fluid parameters, MR-imaging, time point of biopsy and exact localization of biopsy as well as number of tissue samples were examined.ResultsPACNS was confirmed in 7 of 23 patients (30.4%). Alternative diagnoses were identified in 7 patients (30%). A shorter time period between the onset or worsening of symptoms (p = 0.018) and ESBB significantly increased the diagnostic yield. We observed only minor and transient postoperative complications in 3 patients (13.0%). ESBB led to a direct change of the therapeutic regime in 13 of 23 patients (56.5%). Careful neuropathological analysis furthermore revealed that cortical samples were crucial in obtaining a diagnosis.ConclusionESBB is a safe approach with good feasibility, even in critically ill patients, and high diagnostic accuracy in patients with suspected PACNS changing future therapies in 13 of 23 patients (56.5%). Early biopsy after symptom onset/worsening is crucial and (sub)acute MRI-lesions should be targeted with a particular need for biopsy samples from the cortical layer.

CNS vasculopathies: Challenging mimickers of primary angiitis of the central nervous system.

Primary angiitis of the central nervous system (CNS) is an inflammatory vasculopathy affecting the brain and spinal cord. It is a difficult diagnosis to make because of its insidious nonspecific course and its multiple mimics. This review identifies and discusses some noninfectious mimickers of primary CNS angiitis, including: reversible cerebral vasoconstriction syndrome, Sneddon's Syndrome, amyloid-beta-related angiopathy, Susac Syndrome, and neurosarcoidosis. Each condition will be reviewed in terms of epidemiology, pathology, clinical presentation, diagnostic approach, and treatment. Distinguishing these mimics from the primary angiitis of the CNS is important for proper treatment and prognosis.

CLIPPERS and its mimics: evaluation of new criteria for the diagnosis of CLIPPERS

ObjectiveTo evaluate the accuracy of the recently proposed diagnostic criteria for chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).MethodsWe enrolled 42 patients with hindbrain punctate and/or linear...

Atypical presentation of fulminant primary central nervous system angiitis

BackgroundPrimary Angiitis of the Central Nervous System (PACNS) is a rare cause of CNS vasculitis that should be included as part complete differential diagnosis, especially in cases with suggestive imaging findings and an absence of secondary causes for CNS vasculitis. Case presentationWe describe a case of a 47-year-old previously healthy Caucasian male presenting with rapid progression of encephalopathy and fevers. Extensive infectious, autoimmune, and imaging workups were unrevealing. A diagnosis of PACNS was made posthumously on histopathology. ConclusionsPACNS is a challenging diagnosis owing to frequent discrepancies between radiologic and histopathologic findings. Tissue biopsy is key to diagnosing PACNS.

Primary angiitis of the central nervous system - An Indian experience.

Primary angiitis of the central nervous system - An Indian experience.

Clinical and imaging features distinguishing Susac syndrome from primary angiitis of the central nervous system

Introduction: To assess clinical and/or imaging features useful to distinguish between Susac syndrome (SuS) and primary angiitis of central nervous system (PACNS). Methods: Multicenter retrospective analysis of two cohorts of Argentine patients diagnosed with SuS and PACNS. Results: 13 patients diagnosed with SuS (6 women and 7 men, mean age 35 ± 10 years) and 15 with PACNS (10 women and 5 men, mean age 44 ± 18 years) were analyzed. Cognitive impairment (11 out of 13 patients vs. 5 out of 15, p = .006), ataxia (7 out of 13 vs. 2 out of 15, p = .042) and auditory disturbances (7 out of 13 vs. 0 out of 15, p = .003) were more frequent in SuS patients; whereas seizures were more frequent in PACNS patients (8 out of 15 vs. 1 out of 13, p = .035). On MRI, corpus callosum (CC) involvement was observed more often in SuS, with abnormalities in CC genu, in 13 out of 13 SuS patients vs. only 2 out of 15 PACNS patients (p < .001); in CC body these were present in 13 out of 13 SuS patients vs. 1 out of 15 PACNS patients, (p < .001); and in CC splenium in 12 out of 13 Sus patients vs. 1 of 15 PACNS, p < .001). Cortical lesions were more frequent in PACNS patients (10 out of 15 vs. 3 out of 13 SuS patients, p = .02), as were hemorrhages (5 out of 15 vs. 0 out of 13 SuS, p = .04) and multiple basal ganglia infarcts (7 out of 15 vs. 1 out of 13 Sus, p = .037). Conclusion: Specific clinical and/or MRI findings may help distinguish SuS from PACNS with potential therapeutic implications.

Diagnosis and Treatment of Primary Central Nervous System Angiitis

Primary central nervous system angiitis (PCNSA) is a rare disease. Varying clinical pictures coupled with lack of sensitive and specific diagnostic tests lead to challenges in management of these patients. This unfortunately may lead to both under- or over-diagnosis and unnecessary treatment. It is important to recognize the different conditions that may mimic the clinical and radiologic presentation. We present a contemporary literature review that should update physicians who encounter this patient population. Recent additions to the literature in form of case reports and single-center series show that digital subtraction angiography was the most widely used test to diagnose PCNSA. It is also important to note that brain biopsy is still considered "gold standard" and should be pursued as it not only has information that establishes the diagnosis but also may help in ruling out the diagnosis from mimics. In around 39% of suspected cases, an alternate diagnosis was eventually confirmed highlighting the importance of a comprehensive work-up. For treatment, almost all the literature supports the use of glucocorticoids as the initial treatment and if the patient has a relapse or develops steroid toxicity then the second most commonly used agent was cyclophosphamide. There are increasing reports of benefits with other agents such as methotrexate, azathioprine, mycophenolate, infliximab, and etanercept. The diagnosis and management of PCNSA continues to be a challenge. Formation of prospective patient registries with continued immunologic research for novel targets and immunomodulatory modalities may hold promise for future care of these patients.

Arterial Wall Imaging in Pediatric Stroke.

Arteriopathy is common in childhood arterial ischemic stroke (AIS) and predicts stroke recurrence. Currently available vascular imaging techniques mainly image the arterial lumen rather than the vessel wall and have a limited ability to differentiate among common arteriopathies. We aimed to investigate the value of a magnetic resonance imaging-based technique, namely noninvasive arterial wall imaging (AWI), for distinguishing among arteriopathy subtypes in a consecutive cohort of children presenting with AIS. Children with confirmed AIS and magnetic resonance angiography underwent 3-Tesla AWI including T1-weighted 2-dimensional fluid-attenuated inversion recovery fast spin echo sequences pre- and post-gadolinium contrast. AWI characteristics, including wall enhancement, wall thickening, and luminal stenosis, were documented for all. Twenty-six children with AIS had AWI. Of these, 9 (35%) had AWI enhancement. AWI enhancement was associated with anterior circulation magnetic resonance angiography abnormality and cortical infarction in 8 of 9 (89%) children and normal magnetic resonance angiography with posterior circulation subcortical infarction in 1 (1 of 9; 11%) child. AWI enhancement was not seen in 17 (65%), 10 (59%) of whom had an abnormal magnetic resonance angiography. Distinct patterns of pre- and postcontrast signal abnormality were demonstrated in the vessel wall in the region of interest in children with transient cerebral arteriopathy, arterial dissection, primary central nervous system angiitis, dissecting aneurysm, and cardioembolic stroke. AWI is a noninvasive, high-resolution magnetic resonance AWI technique, which can be successfully used in children presenting with AIS. Patterns of AWI enhancement are recognizable and associated with specific AIS pathogeneses. Further studies are required to assess the additional diagnostic utility of AWI over routine vascular imaging techniques, in childhood AIS.

Abstract TP204: Large-Artery Infarction in Primary Angiitis of the Central Nervous System

Introduction: Primary angiitis of central nervous system (PACNS) typically manifests with accumulating small-sized ischemic strokes and white-matter changes. Large-artery infarcts (LA-PACNS) have been observed, however it is unclear whether LA-PACNS is a unique subtype. Methods: We compared the clinical/imaging phenotype, predictors, treatment response and prognosis of LA-PACNS and small-vessel PACNS (SV-PACNS) from a cohort of 51 cases meeting published criteria for PACNS (Ann Neurol. 2016;79:882-94) who were managed at our hospital from 1993-2015. Results: Overall mean age 51±15 years; 72% men. Six patients (12%, 95% CI: 3%-21%; mean age 55±13 years; 67% men) had LA-PACNS; 4 (67%) presented with discrete stroke syndromes versus 49% in SV-PACNS. The frequency of headache, encephalopathy, seizure and focal deficits were not significantly different however LA-PACNS had more hypertension. CSF, performed in all patients, showed inflammatory changes in 63% SV-PACNS versus 33% LA-PACNS (p=0.4). Brain biopsy was performed in 5 LA-PACNS and 32 SV-PACNS. In LA-PACNS, one had granulomatous angiitis (GACNS), one had amyloid-beta related angiitis (ABRA), and 3 had negative results. In SV-PACNS, 18 (56%) showed abnormal results including lymphocytic/chronic (12), GACNS (5), ABRA (1). There were no significant differences in the frequency of angiographic abnormalities (67% vs 53%), arteriopathy severity score (10±10 vs 8±13), or arterial segment involved. There were no significant differences in the degree of white matter change, microbleeds, or contrast enhancement on MRI however LA-PACNS had more FLAIR dot sign indicating slow flow and collateralization in leptomeningeal arteries (40% vs 5%, p=0.05). Except for two patients with SV-PACNS, all were treated with steroids; 23 SV-PACNS and all LA-PACNS also received cyclophosphamide. The interval from onset to diagnosis was shorter in LA-PACNS (42 vs 110 days) and follow-up duration was similar (5.4±7.5 years vs. 5.9±6.3 years). Mortality (33% LA-PACNS vs 16% SV-PACNS) and full remission (50% vs 62%) were not significantly different. Conclusions: In our relatively large cohort of PACNS, LA-PACNS and SV-PACNS showed similar clinical, imaging, angiographic features and outcome.

Poster 170: Preservation of American Sign Language Ability in Patient With Primary Central Nervous System (CNS) Angiitis and Intra-Parenchymal Hemorrhage (IPH) Causing Verbal Apraxia and Aphasia: A Case Report

Poster 170: Preservation of American Sign Language Ability in Patient With Primary Central Nervous System (CNS) Angiitis and Intra-Parenchymal Hemorrhage (IPH) Causing Verbal Apraxia and Aphasia: A Case Report

Role of magnetic resonance imaging in the diagnosis of primary central nervous system angiitis.

The present study reported on the use of magnetic resonance imaging (MRI) in the diagnosis of primary angiitis of the central nervous system (PACNS). A total of 19 consecutive patients with a clinical diagnosis of PACNS confirmed by clinical follow-up were enrolled in the present study. All patients underwent unenhanced and enhanced MRI prior to and after steroids or steroids plus immunosuppressive therapy. At baseline, all patients showed lesions on MRI in the grey and white matter. Lesions presented as slightly hypointense on T1-weighted images (T1WI), slightly hyperintense on T2WI, hyperintense on fluid-attenuated inversion recovery, iso- or slightly hyperintense on diffusion-weighted images (DWI) and hyperintense on apparent diffusion coefficient (ADC) mapping. After contrast injection, the lesions showed patchy, cord-like or goral enhancement. Seven cases had unilateral lesions and the other 12 cases had bilateral lesions. On all sequences, indistinct margins characterised most of the lesions, and certain lesions were oedematous. Treatment with steroids or steroids plus immunosuppressive agents resulted in improvement or disappearance of symptoms, and seventeen patients had evidently improved according to MRI. In conclusion, PACNS has unique characteristics on MRI; DWI, ADC mapping and enhanced images are of great importance for the diagnosis and clinical management of early-stage PACNS.

Abstract TP111: Primary Angiitis of Central Nervous System . Clinical Profile and Outcome of Forty Seven Patients

Introduction: Primary angiitis of central nervous system (PACNS) is a rare and serious disorder of uncertain cause, with significant morbidity and mortality. Objective: To determine the clinical profile, response to treatment and outcome in patients with PACNS. Methodology: This is retrospective study of 47 patients from January 2000 to Dec 2015 with either angiographically or biopsy proven PACNS. Outcome at 6 months was considered poor, if modified rankin scale was &amp;gt 2. Relapse was defined as occurrence of new neurological deficits after clinical improvement, progression of existing disability or radiological worsening. Results: The median age at diagnosis was 36 years (IQR 17) and 33 (70.2%) were males. Twenty-nine patients (61.7%) had angiography positive, 14 patients (38.3%) had biopsy confirmed and 4 patients (8.5%) had both angiography and biopsy positive PACNS .Stroke was the initial presentation in 33 patients (70.2%) followed by rapidly progressive dementia in 11 patients (23.4%) and myelopathy in 3 patients (6.4%). After a median follow-up of 2.8 years (range 0.5-15) , poor 6 month outcome was observed in 12 (25.5%), relapse in 24 (51.1%) and mortality in 3 (6.4%) patients. The median delay from onset of symptoms to start of immunosuppressive medication was 3.5 months (0.5- 63).More number of patients in the delayed treatment group had relapse compared to the early treatment group (59.1% vs 52.2%, p= 0.76). Among the 34 patients who received steroids as the initial treatment, 61.8% had relapse, whereas in patients on steroid with cyclophosphamide, only 38.5% had relapse (p=0.176). Of the 25 patients with abnormal CSF, 16 (64%) had relapse (p=0.209) and of the 18 patients with abnormal meningocortical biopsy, 15 patients (83.3%) had relapse. The NIHSS at admission &amp;gt 5 (p=0.046), and presence of severe small vessel ischemic changes in MRI (p= 0.04) were predictors of relapse. Conclusion: PACNS is a rare disease with delayed diagnosis. Severity of neurological deficit at presentation and presence of severe small vessel ischemic changes in MRI were predictors of future relapse.

MRI and clinicopathological correlation of childhood primary central nervous system angiitis

To review the long-term clinical and imaging follow-up of eight children with histopathologically proven childhood primary central nervous system angiitis (cPACNS). Eight children (5-17 years, five female and three male patients) with biopsy-proven cPACNS vasculitis were reviewed retrospectively. All children were followed at the Children's Hospital of Eastern Ontario. Magnetic resonance imaging (MRI) images of the brain parenchyma and vessel pattern of the arteries of the circle of Willis were reviewed at baseline and follow-up(s). All MRI images were correlated with clinical and histopathological findings. Seven (87.5%) children demonstrated parenchymal abnormalities: five of the seven (71.4%) presented with non-haemorrhagic lesions, one (14.3%) presented with a parenchymal bleed, and one (14.3%) showed diffuse oedema. One child remained negative for brain parenchymal abnormality at baseline and follow-up. Of the seven children with a parenchymal abnormality, parenchymal enhancement was seen in two (28.5%) and leptomeningeal enhancement was seen in one child. Only two children had positive diffusion-weighted imaging (DWI) restriction at baseline. Five of the seven children (71.4%) showed no magnetic resonance angiography (MRA) abnormalities at baseline. In one, MRA detected complete absence of flow. Follow-up MRA (24-74 months) demonstrated that five of the seven (71.4%) children had progressive paucity of the peripheral vascularity. Two children (28.5%) with shorter-term follow-up (7 and 13 months) did not demonstrate MRA changes. Long-term follow-up MRA demonstrated progressive paucity of peripheral vessels, which could be secondary to the inflammation affecting the peripheral vasculature causing reduction in vessel lumen size beyond the resolution of the maximum intensity projection (MIP) reconstruction.

Diagnostic Yield and Safety of Brain Biopsy for Suspected Primary Central Nervous System Angiitis.

The utility and safety of brain biopsy for suspected primary angiitis of the central nervous system (PACNS) are uncertain. Factors predictive of a positive biopsy have not been well described. Our aim was to evaluate the diagnostic yield and safety of brain biopsy in suspected PACNS and determine whether any prebiopsy variables are associated with a positive biopsy. This is a retrospective study of consecutive patients who underwent diagnostic brain biopsy for PACNS at a single institution. The relationship between biopsy yield and patient demographics, surgical technique, laboratory testing, neuroimaging, biopsy characteristics, and prebiopsy immunosuppressive therapy were examined. PACNS was confirmed in 9 of 79 patients (11%). Biopsy identified alternative diagnoses in 24 patients (30%), with cerebral amyloid angiopathy (8 patients), encephalitis (5 patients), demyelination (3 patients), and CNS lymphoma (3 patients) most commonly found. There was no correlation between a positive biopsy and cerebrospinal fluid results, neuroimaging, surgical technique, biopsy characteristics, or preoperative immunosuppressive therapy. Smaller biopsies (P=0.02) and closed procedures (P=0.013) were less likely to yield a diagnosis. Postoperative complications occurred in 13 patients (16%), 3 (4%) of which were serious. Brain biopsy leads to pathological confirmation of vasculitis in a minority of suspected PACNS cases but alternative diagnoses are often identified. Importantly, rare but meaningful complications may occur.

Central nervous system angiitis: a series of 31 patients

Central nervous system (CNS) angiitis is a rare inflammatory disorder. To date, clinical data are lacking and treatment remains a matter of debate. The aim of this study is to analyse the main characteristics, response to therapy and outcome of 32 patients with CNS angiitis. Single-centre retrospective study in a tertiary centre was made. Diagnosis of CNS angiitis was made by cerebral angiography and/or magnetic resonance angiography and/or CNS biopsy. The main features and outcomes of primary and secondary CNS angiitis were compared and predictive factors of a favourable outcome were searched. Thirty-one patients (median age 45 Q1-Q3 37-54) sex ratio F/M 2.1) were included. Main clinical features were hemiparesis (35.5%) and headache (29%). The median CSF protein level was 0.64(0.52-0.81) g/L and was superior to 1g/L in six cases. CNS magnetic resonance (MR) imaging findings were most frequently ischemic (96.8%), bilateral (83.9%), multiple (87.1%) and supratentorial (96.8%). The MR angiography was abnormal in all cases. Among the 31 patients in the study, 19 (61.3%) were diagnosed with primary CNS angiitis. Systemic lupus erythematosus (n = 6) and vasculitis (n = 4) were the most frequent aetiologies of secondary CNS angiitis. No difference was evidenced between primary and secondary CNS angiitis. Steroids were administered in 79.2% of treated patients and combined with immunosuppressants in 79.2% of cases. Eight cases of CNS angiitis relapse were noted. CNS angiitis remains a severe illness. Treatment often associated steroids and immunosuppressants, and diagnosis delay is significantly associated with a poorer prognosis.