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Background: Primary angiitis of the central nervous system (PACNS) is a relapsing-remitting disease with a heterogeneous course. Case series have delineated the long-term disease course but not acute outcomes or their determinants. The national United States hospital burden of PACNS has not been quantified., Methods: Analysis of the United States Nationwide Readmissions Database (2016-2018) to characterize the frequency of PACNS hospitalizations, demographic features, inpatient mortality, and discharge outcomes., Results: During the 3-year study period, unweighted 1843 (weighted 3409) patients with PACNS were admitted to the 1078 Healthcare Cost and Utilization Project HCUP participating hospitals; with weighting, this value indicates that 1136 patients were admitted each year to US hospitals, representing yearly 0.01 cases per 100 000 national hospitalizations. The majority of patients were hospitalized in metropolitan teaching hospitals (81.6%). The median age at admission was 54.9 (IQR: 44.0-66.5) years and 59.4% were women. Neurologic manifestations included ischemic stroke in 38.2%, transient ischemic attack in 20.2%, seizure disorder in 22.8%, and intracranial hemorrhage in 13.0%. Overall, 60.0% of patients were discharged home, 35.0% discharged to a rehabilitation facility or nursing home and 5.0% died before discharge. Patient features independently associated with the discharge to another facility or death included older age (odds ratio [OR], 1.03 [95% CI, [1.03-1.04]]), male sex (OR, 1.22 [1.04-1.43]), intraparenchymal hemorrhage (OR, 1.41 [1.08-1.84]), ischemic stroke (OR, 2.79 [2.38-3.28]), and seizure disorder (OR, 1.57 [1.31-1.89])., Conclusion: Our study showed PACNS is still a rare inflammatory disorder of the blood vessels of the central nervous system suggesting an annual hospitalization of 5.1 cases per 1,000,000 person-years in the more diverse and contemporary US population. Overall, 4 in 10 had unfavorable discharge outcome, being unable to be discharged home, and 1 in 20 died before discharge., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Purpose of Review: To review understand the epidemiology, background, neuropharmacology, and histopathology of literature verified cases, and likely etiopathogenic mechanisms., Recent Findings: There are only a handful of histologically confirmed patients in the literature with cerebral vasculitis because of drug abuse., Summary: There is little justification for invasive laboratory investigation given the ready availability of highly accurate vascular neuroimaging techniques to dictate management, which usually rests upon avoidance of further exposure and minimizing the secondary neurotoxic effects of the abused substances and polypharmacy use.

Objective: To determine that children with arterial ischemic stroke (AIS) due to an identifiable arteriopathy are distinct from those without arteriopathy and that each arteriopathy subtype has unique and recognizable clinical features., Methods: We report a large, observational, multicenter cohort of children with AIS, age 1 month to 18 years, enrolled in the International Pediatric Stroke Study from 2003 to 2014. Clinical and demographic differences were compared by use of the Fisher exact test, with linear step-up permutation min- p adjustment for multiple comparisons. Exploratory analyses were conducted to evaluate differences between cases of AIS with and without arteriopathy and between arteriopathy subtypes., Results: Of 2,127 children with AIS, 725 (34%) had arteriopathy (median age 7.45 years). Arteriopathy subtypes included dissection (27%), moyamoya (24.5%), focal cerebral arteriopathy-inflammatory subtype (FCA-i; 15%), diffuse cerebral vasculitis (15%), and nonspecific arteriopathy (18.5%). Children with arteriopathic AIS were more likely to present between 6 and 9 years of age (odds ratio [OR] 1.93, p = 0.029) with headache (OR 1.55, p = 0.023), multiple infarctions (OR 2.05, p < 0.001), sickle cell anemia (OR 2.9, p = 0.007), and head/neck trauma (OR 1.93, p = 0.018). Antithrombotic use and stroke recurrence were higher in children with arteriopathy. Among arteriopathy subtypes, dissection was associated with male sex, older age, headache, and anticoagulant use; FCA-i was associated with hemiparesis and single infarcts; moyamoya was associated with seizures and recurrent strokes; and vasculitis was associated with bilateral infarctions., Conclusion: Specific clinical profiles are associated with cerebral arteriopathies in children with AIS. These observations may be helpful indicators in guiding early diagnosis and defining subgroups who may benefit most from future therapeutic trials., (© 2020 American Academy of Neurology.)

Background and Purpose- We aimed to analyze the long-term outcomes of patients with primary central nervous system vasculitis according to the different therapeutic strategies used to induce remission. Methods- We assessed the rate of prolonged remission (defined by the absence of relapse at ≥12 months after diagnosis) and the functional status at last follow-up in patients with primary central nervous system vasculitis included in the French cohort, who achieved a first remission according to the 3 main groups of treatments administered: glucocorticoids only (group 1); induction treatment with glucocorticoids and an immunosuppressant, but no maintenance (group 2); and combined treatment with glucocorticoids and an immunosuppressant for induction followed by maintenance therapy (group 3). Good functional status was defined as a modified Rankin Scale score ≤2 at the last follow-up. Results- Remission was achieved with the initial induction treatment in 106 (95%) of the 112. Prolonged remission without relapse was observed in 70 (66%) patients after 57 (12-198) months of follow-up. A good functional status at last follow-up (ie, modified Rankin Scale score ≤2) was observed in 63 (56%) patients. Overall mortality was 8%. The initial severity and the radiological presentations were comparable in the 3 treatment groups. More prolonged remissions ( P=0.003) and a better functional status at the last follow-up ( P=0.0004) were observed in group 3. In multivariate analysis, the use of maintenance therapy was associated with prolonged remission (odds ratio, 4.32 [1.67-12.19]; P=0.002) and better functional status (odds ratio, 8.09 [3.24-22.38]; P<0.0001). Conclusions- This study suggests that maintenance therapy with an immunosuppressant combined with glucocorticoids lead to the best long-term clinical and functional outcomes in patients with primary central nervous system vasculitis after having achieved remission with either glucocorticoids alone or in combination with another immunosuppressant.

Background and Purpose: Primary angiitis of the central nervous system remains challenging. To report an overview and pictorial review of brain magnetic resonance imaging findings in adult primary angiitis of the central nervous system and to determine the distribution of parenchymal, meningeal, and vascular lesions in a large multicentric cohort., Methods: Adult patients from the French COVAC cohort (Cohort of Patients With Primary Vasculitis of the Central Nervous System), with biopsy or angiographically proven primary angiitis of the central nervous system and brain magnetic resonance imaging available at the time of diagnosis were included. A systematic imaging review was performed blinded to clinical data., Results: Sixty patients met inclusion criteria. Mean age was 45 years (±12.9). Patients initially presented focal deficit(s) (83%), headaches (53%), cognitive disorder (40%), and seizures (38.3%). The most common magnetic resonance imaging finding observed in 42% of patients was multiterritorial, bilateral, distal acute stroke lesions after small to medium artery distribution, with a predominant carotid circulation distribution. Hemorrhagic infarctions and parenchymal hemorrhages were also frequently found in the cohort (55%). Acute convexity subarachnoid hemorrhage was found in 26% of patients and 42% demonstrated pre-eminent leptomeningeal enhancement, which is found to be significantly more prevalent in biopsy-proven patients (60% versus 28%; P =0.04). Seven patients had tumor-like presentations. Seventy-seven percent of magnetic resonance angiographic studies were abnormal, revealing proximal/distal stenoses in 57% and 61% of patients, respectively., Conclusions: Adult primary angiitis of the central nervous system is a heterogenous disease, with multiterritorial, distal, and bilateral acute stroke being the most common pattern of parenchymal lesions found on magnetic resonance imaging. Our findings suggest a higher than previously thought prevalence of hemorrhagic transformation and other hemorrhagic manifestations., (© 2017 American Heart Association, Inc.)

Granulomatous arteritis characterizes the pathology of giant cell arteritis, granulomatous aortitis, and intracerebral varicella zoster virus (VZV) vasculopathy. Because intracerebral VZV vasculopathy and giant cell arteritis are strongly associated with productive VZV infection in cerebral and temporal arteries, respectively, we evaluated human aortas for VZV antigen and VZV DNA. Using 3 different anti-VZV antibodies, we identified VZV antigen in 11 of 11 aortas with pathologically verified granulomatous arteritis, in 1 of 1 cases of nongranulomatous arteritis, and in 5 of 18 control aortas (28%) obtained at autopsy. The presence of VZV antigen in granulomatous aortitis was highly significant (P = .0001) as compared to control aortas, in which VZV antigen was never associated with pathology, indicating subclinical reactivation. VZV DNA was found in most aortas containing VZV antigen. The frequent clinical, radiological, and pathological aortic involvement in patients with giant cell arteritis correlates with the significant detection of VZV in granulomatous aortitis., (© The Author 2016. Published by Oxford University Press for the Infectious Diseases Society of America. All rights reserved. For permissions, e-mail journals.permissions@oup.com.)

Angiitis of the central nervous system (CNS) is difficult to diagnose but potentially fatal. When stroke occurs in a younger individual or is associated with multiple infarcts on imaging, clinicians must decide how far to pursue a possible diagnosis of vasculitis. The aim of this study is to establish the prevalence of primary and secondary cerebral angiitis among patients presenting with stroke. Hospital attendances over a 10year period were surveyed by searching for diagnostic codes and key words specific for cerebral vasculitis/angiitis. Case notes were reviewed by the authors using two sets of criteria for angiitis of the CNS. Thirty-two patients were initially considered likely to have cerebral angiitis by treating physicians. Thirteen had been admitted to hospital with stroke. During this period, there were 7475 admissions for ischaemic and haemorrhagic stroke. Six patients had a final diagnosis of vasculitic stroke but only one had definite CNS angiitis with a first presentation as ischaemic stroke (0.02%). Most patients who did have cerebral vasculitis developed multifocal or subacute neurological deficits, or already had an immunological disorder known to be associated with secondary CNS angiitis. Of 19 patients given an alternative final diagnosis, the most common were atherosclerotic/embolic cerebrovascular disease (n=9) and reversible cerebral vasoconstriction syndrome (n=7). Stroke is rarely the first manifestation of cerebral vasculitis. Our findings suggest that routine screening for angiitis in stroke patients may not be warranted., (Copyright © 2015 Elsevier Ltd. All rights reserved.)

Autonomic central nervous system (CNS) angiitis was classified with primary angiitis of the CNS (PACNS) or CNS angiitis secondary to systemic vasculitis. Mean onset age of PACNS was about 50 years. Typical symptoms were a chronic progressive headache and encephalopathy. Cerebrospinal fluid analysis was compatible with aseptic meningitis. Findings from imaging were varied and included arterial stenosis. Exclusion diagnosis of PACNS mimics and a brain biopsy were required for a definite diagnosis. Some collagen diseases could cause secondary CNS vasculitis. Patients with secondary CNS vasculitis were required more extensive immunosuppression therapy.

Objectives: To study the incidence and prevalence of primary systemic vasculitides (PSV) in the Costa del Sol region (southern Spain) and to compare the major epidemiological studies in PSV with the results obtained in our area., Methods: Retrospective study including permanent residents ≥14 years (or older) diagnosed with PSV at the Hospital Costa del Sol (Marbella, Spain) between 1994 and 2010. Epidemiological data were collected and the annual incidence rate during the study period and the prevalence in 2010 were calculated per million population, except for GCA, which was estimated per 100,000 population >50 years., Results: Seventy-four adult patients were diagnosed with PSV, representing an annual incidence of 15.8 (95%CI 12.2-19.4) patients/million population. These diagnoses included 29 (39.1%) giant cell arteritis (GCA), 5 (6.7%) Takayasu's arteritis (TKA), 3 (4%) poly-arteritis nodosa (PAN), 29 (39.1%) antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) [10 (13.5%) granulomatosis with polyangiitis (GPA) (Wegener), 16 (21.6%) microscopic polyangiitis (MPA) and 3 (4%) eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss)], 7 (9.4%) IgA vasculitis (Henoch-Schönlein) (IgAV) and one (1.3%) cryobulinaemic vasculitis (CV). The annual incidence and 2010 prevalence for each of the PSV, respectively, were: GCA: 2.2/12.2; TKA: 1.1/10.5; PAN: 0.6/2.6; AAV: 6.2/44.8 (GPA: 2.1/15.8; MPA: 3.4/23.8; EGPA: 0.6/5.3); IgAV: 1.5/7.9; and CV: 0.2/0., Conclusions: The first epidemiological study of PSV in southern Spain corroborates their infrequency, with GCA and AAV as the PSV most often diagnosed. In southern Spain, the incidence and prevalence of PSV are lower than in northern Spain and in countries in the Northern Hemisphere.

Sickle cell anemia (SCA)-associated cerebral vasculopathy and moyamoya is a unique entity reflecting the abnormal interactions between sickled red blood cells (RBCs) and the cerebral arterial endothelium. Endothelial injury, coagulation activation, and the inflammatory response generated by sickled RBCs are implicated in the development of cerebral vasculopathy, but the pathophysiology remains incompletely understood. SCA-specific screening and treatment guidelines have successfully reduced the incidence of overt strokes in this high-risk population. However, despite aggressive hematological management, many children with cerebral vasculopathy due to SCA have progressive vasculopathy and recurrent strokes; therefore, more effective therapies, such as revascularization surgery and curative hematopoietic stem cell transplant, are urgently needed., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Objectives: Polyarteritis nodosa (PAN) is a rare vasculitis in childhood and poor information is known about its long-term outcome. Our aim was to describe the clinical features, at onset and during the disease course, of childhood-onset PAN and identify a potential correlation with persistent organ damage and worse outcome in a cohort of paediatric patients with a confirmed diagnosis of PAN., Methods: A retrospective collection of demographic and clinical data of 52 Caucasian children diagnosed with PAN, fulfilling the EULAR/PRES diagnostic criteria, recruited from eight paediatric rheumatologic centres and one transition unit, was performed. A statistical correlation was made between clinical involvement at onset or during the overall disease course and patients' final outcome., Results: Data from 52 patients (31 males, 21 females) were collected: their mean age at onset was 7.9 years (median 6.3) and mean follow-up period was 6.2 years (median 5.4). At the last follow-up visit, 27 patients (51.9%) were off therapy in clinical remission, 17 (32.7%) were in clinical remission while on medication, and 6 (11.6%) had a persistent or relapsing disease course. Two patients (3.8%) deceased because of severe cerebral involvement. Cranial nerve palsy during the disease course was significantly correlated with a worse prognosis (p=0.011). The presence of nephrogenic hypertension at onset and seizures during the disease course were significantly associated with the development of irreversible organ damage (p= 0.040 and 0.011, respectively)., Conclusions: Childhood PAN is a severe disease with substantial risk of long-term morbidities. In our cohort of patients the worst outcome was significantly correlated with renal and neurological involvement.

Objective: To describe characteristics and outcomes of a multicenter cohort of patients diagnosed as having primary angiitis of the central nervous system (PACNS)., Methods: In 2010, we initiated a cohort study of adults diagnosed as having PACNS ≤15 years ago and with followup of >6 months (unless they died earlier of biopsy-proven PACNS). Its first analysis was planned at 2 years. Multidisciplinary investigators verified that appropriate investigations were done and excluded patients with possible alternative diagnoses. We analyzed patient demographics and symptoms, laboratory, radiographic, and histologic findings, and treatments. Studied outcomes included treatment response(s), relapse, death, and disability., Results: We included 52 patients (30 males; median age at diagnosis 43.5 years [range 18-79 years]) in whom PACNS was diagnosed between 1996 and 2012. Nineteen (61%) of 31 patients who had undergone brain biopsy had histologic vasculitis (biopsy-proven PACNS), while the other 12 patients had normal or noncontributive biopsy samples. An additional 21 patients had signs suggestive of PACNS on conventional cerebral angiography. All but 1 patient received corticosteroids, and 44 patients received cyclophosphamide (CYC). After a median followup of 35 months (range 2-148 months) postdiagnosis (1 patient with biopsy-proven PACNS died 2 months after diagnosis), 32 patients responded to treatment with improved modified Rankin scale scores, 4 patients (8%) did not respond, 14 patients (27%) had relapse of their disease at least once, and 3 patients (6%) died (1 patient after a relapse). Relapse was more common in patients with than in those without meningeal gadolinium enhancements on magnetic resonance imaging (MRI) (8 of 10 [80%] versus 6 of 32 [19%]; P = 0.001) and more common in patients with than in those without seizures at diagnosis (8 of 17 [47%] versus 6 of 35 [17%]; P = 0.04)., Conclusion: In this cohort of patients with PACNS, most patients received corticosteroids and CYC, and mortality was low. Patients with seizures at diagnosis or meningeal enhancements on MRI may be prone to relapse and require a different treatment strategy., (Copyright © 2014 by the American College of Rheumatology.)

Objective: To determine the association between brain white matter (WM) damage and persistent fatigue among patients with granulomatosis with polyangiitis (GPA)., Methods: A case-control MRI study was conducted. Both cases, defined as GPA patients with chronic fatigue, and controls, defined as GPA patients without fatigue, underwent MRI brain scanning. Standard T1, T2 and FLAIR images were acquired and Scheltens white matter hyperintensity scores (SWMHS) reported in order to quantify macroscopic damage. In addition, diffusion tensor imaging (DTI) data were analysed using track-based spatial statistics to measure structural integrity and thus microscopic damage., Results: No statistically significant differences in macroscopic damage were observed between cases (n = 14) and controls (n = 14) (median SWMHS 6, interquartile range 3-7 vs median SWMHS 3.5, interquartile range 3-8; P = 0.52). Compared with controls, there were no regions of WM where cases recorded reduced structural integrity; however, significantly greater structural integrity was registered in the regions of the fornix and cingulum nerve bundles of cases (P < 0.05, corrected for multiple comparisons)., Conclusion: This study found no evidence to suggest that GPA-related fatigue is associated with either macroscopic or microscopic damage of the WM. On the contrary, regions of significantly greater structural integrity were observed among fatigued cases, which may reflect sustained activation secondary to chronic fatigue exposure.

Background: Primary angiitis of the central nervous system is a rare condition, which is often difficult to diagnose and is associated with significant morbidity and mortality. There is no standardised treatment protocol or randomised clinical trial evidence to guide management., Aim: To describe the clinical features, diagnostic modalities, treatment and outcomes in an Australian hospital population-based series of primary cerebral angiitis., Methods: Data were collected via retrospective medical record review of patients with primary angiitis of the central nervous system for the period 1 July 1998 to 30 June 2009, using previously published diagnostic criteria. Eligible patient records were identified in two ways; from routinely collected hospital episode data, coded using the ICD-10-AM coding standard and by review of cerebral biopsy data., Results: Ten of 12 included patients had a positive cerebral biopsy, with two patients diagnosed by angiography. Mean age at diagnosis was 47.2 years (range 18-73 years), with a female predominance of 5:1. Headache was the most common symptom experienced. Seventy per cent of the biopsy specimens showed a lymphocytic vasculitis. All patients received treatment with either steroids alone or a combination of steroids and cyclophosphamide, the latter treatment being initiated for those with a higher modified Rankin score of disability. Nine (75%) responded to treatment. There was one in-hospital death, and two patients had no documented response to treatment., Conclusion: This study presents the first Australian case series data of primary cerebral angiitis. Better understanding of management and outcomes of this rare condition would be gained through multicentre studies., (© 2012 The Authors; Internal Medicine Journal © 2012 Royal Australasian College of Physicians.)

Neuroborreliosis affects the nervous system after systemic infection with the spirochete Borrelia burgdorferi. Previously, cerebral vasculitis has been regarded as an extremely rare complication of neuroborreliosis. The data on the long-term outcome in patients with cerebral vasculitis due to neuroborreliosis are limited. The objective of this study was to perform a longitudinal analysis of cases of neuroborreliosis-associated cerebral vasculitis. We recruited all patients (n = 11) diagnosed with neuroborreliosis-associated in three neurological departments in an East German region. Inclusion criteria were sudden neurological deficits, magnetic resonance (MR) imaging findings that conform to cerebral ischemia or brain infarction, intrathecal synthesis of borrelia-specific antibodies, and non-atherosclerotic pathology of brain supplying arteries. Vasculitic changes were detected by digital subtraction angiography, MR angiography and/or transcranial Doppler ultrasound. Outcomes were measured by the modified Rankin scale (mRS) and EuroQoL Index. Cerebral vasculitis is a rare complication of Lyme disease (0.3% of all cases in the endemic area). Ten out of 11 patients diagnosed with neuroborreliosis-associated vasculitis cerebral vasculitis using clinical, radiological and immunological criteria developed ischemic stroke or transient ischemic attacks (TIA), 7 patients had recurrent stroke. Vasculitic alterations could be demonstrated in 8 patients that all except one developed ischemic lesions. The median mRS was 3 (range 0-4) at admission and 2 (range 0-6) at discharge. The posterior circulation was affected in 8 of 11 patients; thrombosis of the basilar artery was detected in 2 patients, one died in the acute stage. Neuroborreliosis can cause recurrent stroke or TIA on the basis of cerebral vasculitis. Lumbar puncture is needed for detection of this potentially life-threatening condition. Early recognition and adequate therapy would possibly improve outcome.

Background: Tuberculous cerebral vasculitis is a complication of tuberculous meningitis. This study was undertaken to determine the epidemiological characteristics, context, diagnostic means and outcomes under treatment of tuberculous cerebral vasculitides., Methods: All consecutive patients diagnosed with tuberculous cerebral vasculitis were identified from the databases of three Internal Medicine, one Neurology and one Infectious Disease Departments in three suburban Parisian hospitals., Results: We describe 10 cases: five men and five women (median age 33.5 [range: 27-55] years). Two were infected with the human immunodeficiency virus. Nine patients had tuberculous meningitis, eight with extraneurological involvement. The following manifestations led to the diagnosis: motor deficit, acute confusional state, headaches, involvement, coma and/or seizures. The cerebral vasculitis revealed tuberculosis in three patients, but tuberculosis was already known when vasculitis was diagnosed for the seven others. The cerebral computed-tomography scan showed cerebral infarctions in five patients, hydrocephalus and tuberculomas in four, while magnetic resonance imaging detected infarctions and leptomeningitis in nine patients, pachymeningitis in one, hydrocephalus and tuberculomas in seven. Therapy combined antituberculous agents with oral corticosteroids for all patients, preceded by a methylprednisolone pulse for five patients. Outcome was favorable for nine patients., Conclusion: We described the non-negligible frequency of tuberculous cerebral vasculitides, their clinical manifestations and their potential severity, and the diagnostic and monitoring contributions of magnetic resonance imaging and magnetic resonance angiography., (Copyright © 2011 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.)

Background: Past exposure to human cytomegalovirus has been suggested to participate in the pathogenetic events associated with atherosclerotic lesion establishment and progression. However, whether ongoing human cytomegalovirus infection is related to plaque instability, and subsequent acute cerebral ischemia, is relatively unknown. The purpose of this study was to evaluate the potential relationships between active human cytomegalovirus infection and ischemic stroke, especially in regard to metabolism and inflammation., Methods: Ninety-nine acute ischemic stroke patients, associated with large artery atherosclerosis, were divided into two groups based on the presence or absence of human cytomegalovirus immunoglobulin M (IgM) (human cytomegalovirus-IgM-positive/human cytomegalovirus-IgM-negative = 33:66). Baseline clinical characteristics, inflammatory factors, and biochemical assessments were compared in both groups. Then, all patients and human cytomegalovirus-IgM-positive patients were divided into quartiles according to their high-sensitivity C-reactive protein levels, respectively, and risk factors were compared. Finally, correlations between inflammatory factors (high-sensitivity C-reactive protein and white blood cell count) and other atherosclerosis risk factors in both human cytomegalovirus-IgM-positive and -negative subjects were evaluated., Results: An association between human cytomegalovirus-IgM seropositivity and atherogenic modification of metabolism and inflammatory status were not found in this study. Both age and white blood cell count increased across quartiles of high-sensitivity C-reactive protein in all subjects (P = 0.001), while age and low-density lipoprotein cholesterol increased across quartiles of high-sensitivity C-reactive protein in the human cytomegalovirus-IgM-positive group (P = 0.02 and 0.007, respectively). Multivariate linear regression analysis showed that high-sensitivity C-reactive protein was associated with age in human cytomegalovirus-IgM-positive group (P = 0.002), while no other factor was associated with white blood cell count in these subjects., Conclusion: Our study provided no evidence for the direct implication of active systemic human cytomegalovirus infection, represented by human cytomegalovirus-IgM positivity, in the pathogenesis of acute ischemic strokes, particularly those involving plaque instability and metabolic disorders.

Background: Primary CNS vasculitis (PCNSV) is an uncommon disease in which lesions are limited to the brain and spinal cord. Our objective was to evaluate the frequency, clinical features, and outcome of spinal cord involvement in PCNSV., Methods: We retrospectively identified 101 consecutive patients with PCNSV. Spinal cord involvement was documented for five. Clinical findings, laboratory studies, and outcomes of patients with spinal cord involvement were assessed and compared with those without spinal cord manifestations., Results: Spinal cord symptoms developed before cerebral symptoms in one patient, concurrently in two, and after cerebral symptoms in two. CNS biopsy specimens showed necrotizing vasculitis in three patients and granulomatous vasculitis in two. MRI of the spinal cord showed enhanced thoracic lesions in all five. Cerebral angiograms from four patients had normal findings. One patient had a fatal clinical course. The other four had relapses during follow-up but responded well to therapy and had favorable overall outcomes. At the last follow-up (median, 19 months after diagnosis), the four patients had recovered with slight or moderate residual disability. No significant differences in clinical and laboratory features were observed when comparing patients with or without spinal cord involvement. Cerebral angiograms with evidence of vasculitis were significantly more frequent for patients without spinal cord involvement (p = 0.002)., Conclusion: Spinal cord involvement was documented in 5% of patients with primary CNS vasculitis. The thoracic cord was the predominantly affected site. Other than myelopathy, clinical characteristics were similar to those of the patients without spinal cord involvement.

Although ulcerative colitis and Crohn's disease have traditionally been considered to be inflammatory diseases limited to the gastrointestinal tract, it has been shown that both pathologies are frequently accompanied by various extraintestinal disorders. There is an increasing evidence that they may also manifest in the nervous system, including the peripheral and the central parts. Although some of these neurological complications have been known for a long time, such as cerebrovascular disease, vasculitis and autoinmune processes including neuropathies and cerebral demyelination, others have been recently described. With the exception of some of this complications such as the thromboembolism, evidence for a casual relationship relies merely on single case reports or case series. In this article, we try to review the existing evidence on neurological manifestations of both variants of inflammatory bowel disease.

We have completed a survey of European neurological practice concerning cerebral vasculitis. Twenty-nine respondents from 15 countries provided information concerning the diagnosis and management. The results confirmed the anticipated low frequency of the disease, but also illustrated the power of any putative collaborative effort. Interestingly, there was a wide variation in clinical practice, in particular concerning the perceived importance of cerebral angiography as a diagnostic test and the very common use of steroids as first-line treatment, rather than more potent immunosuppressive agents. This variation is probably to be explained at least, in part, by the absence of any firm evidence base to inform clinical practice. A European collaborative effort--in which there has emerged considerable interest--offers a realistic opportunity to generate sound clinical evidence and thence scientifically robust practical guidelines.

Objective: To register all newly diagnosed patients with primary systemic vasculitis (PSV) in two large regions in north and south Germany., Methods: Between 1 January 1998 and 31 December 1999, all newly diagnosed cases of PSV, as defined by the Chapel Hill Consensus Conference 1992, were identified in two large mixed rural/urban regions in north and south Germany with a combined population of 4,880,543, for a population-based prospective study. The following sources were used: (i) all departments of every hospital, including their out-patient clinics; (ii) all departments of pathology; and (iii) all reference immunological laboratories serving the catchment area. All cases were re-evaluated by the authors., Results: Over the whole period, 473 individuals were registered as having a new PSV. The incidence rates for all PSV were 54 cases per 1,000,000 inhabitants in the north and 48 in the south in 1998, and 48 and 41 respectively in 1999. People 50 yr and older had a three- to five-fold higher risk of getting PSVs than those under 50 yr. The incidences of antineutrophil cytoplasmic antibody (ANCA)-associated PSVs [Wegener's granulomatosis (WG), microscopic polyangiitis (MPA) and Churg-Strauss syndrome (CSS)] remained stable in both regions, at about 9.5 per 1,000,000 annually. The incidence of WG was two to three times greater than those of MPA and CSS. There was no difference in incidence rates between north and south Germany., Conclusion: First results from a population-based vasculitis register serving nearly 5,000,000 inhabitants in north and south Germany revealed no regional differences in the incidence of all PSVs between north and south. The incidence rates of ANCA-associated PSVs, such as WG and MPA, were lower than those in the UK and Norway but higher than that in Spain.