Lyme neuroborreliosis: An unusual case with extensive (peri)vasculitis of the middle cerebral artery.

Lyme disease is a tick-borne infection caused by Borrelia burgdorferi sensu latu. Neuroborreliosis is reported in approximately 10% of patients with Lyme disease. We report a patient with central nervous system (CNS) large vessel vasculitis, ischemic stroke, and tumefactive contrast-enhancing brain lesions, an unusual complication of neuroborreliosis. A 56-year-old man presented with headache and disorientation for 1 month. Magnetic resonance imaging revealed basal meningitis with rapidly progressing frontotemporoinsular edema and (peri)vasculitis. Transcranial ultrasound confirmed stenosed medial cerebral arteries. [18 F]GE-180 microglia positron emission tomography (PET) showed frontotemporoinsular signal more pronounced on the right. [18 F]FET amino acid PET demonstrated low tracer uptake, suggesting an inflammatory process. Cerebrospinal fluid (CSF) showed lymphomonocytosis (243/µl), intrathecal anti-Borrelia IgM (CSF/serum index = 15.65, normal < 1.5) and anti-Borrelia IgG (CSF/serum index = 6.5, normal < 1.5), and elevated CXCL13 (29.2 pg/ml, normal < 10 pg/ml). Main differential diagnoses of neurotuberculosis and perivascular CNS lymphoma were ruled out by biopsy and Quantiferon enzyme-linked immunosorbent assay. Ceftriaxone (28 days), cortisone, and nimodipine (3 months) led to full recovery. Neuroborreliosis is an important differential diagnosis in patients with CNS large vessel vasculitis and tumefactive contrast-enhancing brain lesions, mimicking perivascular CNS lymphoma or neurotuberculosis as main neuroradiological differential diagnoses. Vasculopathy and cerebrovascular events are rare in neuroborreliosis but should be considered, especially in endemic areas.

Detecting immunoglobulin G4-related intracranial arteriopathy with magnetic resonance vessel wall imaging: a preliminary experience in two cases.

BACKGROUND: Detecting immunoglobulin G4 (IgG4)-related intracranial arteriopathy, a rare neurovascular complication of IgG4-related disease, is challenging. While magnetic resonance (MR) vessel wall imaging (VWI) can visualize various neurovascular pathologies, its application to this arteriopathy has not been reported as of this writing. CASE PRESENTATION: A 74-year-old male and a 65-year-old female manifested multiple cranial nerve palsy and neck pain, respectively. Both cases exhibited multiorgan masses with markedly elevated serum IgG4 levels and were clinically diagnosed with IgG4-related disease. Three-dimensional T1-weighted black blood VWI with and without contrast agent identified intracranial vascular lesions characterized as nearly-circumferential mural thickening with homogeneous contrast enhancement in the internal carotid and vertebral arteries; some of the lesions had been unrecognized with screening MR angiography due to expansive remodeling. The former patient underwent corticosteroid therapy, and VWI after treatment revealed decreased mural thickening and enhancement. CONCLUSION: Further studies to elucidate characteristic findings of VWI might contribute to early detection of this treatable pathology.

Tocilizumab for severe refractory primary central nervous system vasculitis: A center experience.

BACKGROUND: Primary Central Nervous System Vasculitis (PCNSV) is responsible for 3%-5% of strokes before age 50. It presents with clinical, radiological, and pathological variability. Optimal management is unknown given the absence of randomized clinical trials. AIMS OF THE STUDY: Explore whether tocilizumab, an anti-interleukin-6 monoclonal antibody, is an effective treatment for refractory PCNSV. METHODS: Patients with PCNSV treated with tocilizumab in a single tertiary center were reviewed. RESULTS: Three patients were identified. In two of them, MRI-revealed ischemic lesions. The other presented with a subcortical hemispheric pseudotumoral lesion. Brain biopsy was inconclusive in two patients. Due to a significant number of relapses and clinical deterioration despite other immunosuppressive drugs, tocilizumab was initiated and induced a long remission period up to 44 months. Observed side effects were a fungic infection, neutropenia and thrombocytopenia (both transitory), and a pulmonary embolism in one of the cases. CONCLUSIONS: Tocilizumab might be a therapeutic option for PCNSV (Class IV evidence), given its efficacy and safety. We propose a novel pathway for diagnosis and therapeutics of PCNSV with the purpose of improving the diagnosis, monitoring, and prognosis of this heterogeneous disorder, setting the framework for future use of tocilizumab in this condition.

"When should primary angiitis of the central nervous system (PACNS) be suspected?": literature review and proposal of a preliminary screening algorithm.

BACKGROUND: Primary angiitis of the CNS (PACNS) is a process causing variously combined neurological disturbances. Its rarity and kaleidoscopic presentation make it difficult to diagnose and even to suspect. OBJECTIVE: (1) To provide an up-to-date review on PACNS and (2) to create a preliminary screening algorithm based on clinical and radiological first-level data, useful to suspect PACNS and guide further investigations. METHODS: Review of PUBMED case series on PACNS, published from 2002 to 2017, collection of frequencies of clinical and neuroimaging features and calculation of median values. Classification of features as "major" or "minor" if frequency was higher or lower than median value. Combination of features in sets of criteria represented by all possible combinations of major and minor clinical and neuroradiological features. Application of criteria to published PACNS case reports and selection of the ones best identifying patients with definite PACNS. RESULTS: We reviewed 24 case series. "Major" clinical features were headache, stroke, cognitive impairment, focal neurological deficits; "minor" were seizures, altered consciousness, psychiatric disorders. "Major" neuroradiological features were multiple parenchymal lesions, parenchymal/meningeal contrast enhancement, magnetic resonance angiography vessel abnormalities, vessel wall enhancement; "minor" were parenchymal/subarachnoid hemorrhage, single parenchymal lesion. The selected sets of criteria able to identify all PACNS patients were (1) one clinical (major/minor) + one major neuroradiological feature; and (2) Two clinical (≥ 1 major) + one minor neuroradiological feature. CONCLUSION: Our review provides a detailed clinical/neuroradiological picture of PACNS. The proposed algorithm should be regarded as a preliminary screening tool to move the first steps towards PACNS diagnosis that needs validation.

Primary angiitis of the central nervous system: Clinical profiles and outcomes of 45 patients.

OBJECTIVE: To describe the clinical profile, treatment response and predictors of outcome in patients with primary angiitis of the central nervous system (PACNS) from a single tertiary care center. METHODOLOGY: Retrospective analysis of consecutive patients diagnosed with PACNS from January 2000 to December 2015. Outcome was defined as poor when the 6-month modified Rankin scale (mRS) was ≥3. RESULTS: The median age of the 45 patients included in this study was 36 (range 19-70) years at disease onset and 31 (68.9%) were males. The initial presentation was ischemic stroke in 15 (33.3%), hemorrhagic stroke in 4 (8.9%), headache in 11 (24.4%), seizures in 8 (17.8%) and cognitive dysfunction in 5 (11.1%) patients. Diagnosis was confirmed by a four vessel cerebral digital subtraction angiogram (DSA), biopsy and by both biopsy and DSA in 26 (57.8%), 15 (33.3%) and 4 (8.9%) patients, respectively. All patients received glucocorticoids and 14 patients received in addition either cyclophosphamide or azathioprine as their first treatment. The median duration of follow-up was 33.1 (0.7-356) months. A poor 6-month outcome was observed in 12 (26.7%) patients. Relapse occurred in 25 (55.6%) patients and 7 (15.6%) died. Predictors of a poor outcome consisted of cognitive dysfunction at diagnosis (80% vs 20%; P = 0.014) and NIHSS ≥5 (62.5% vs 37.5%; P <.0005). None of the patients with a normal EEG had a poor outcome (P = 0.046). Predictors of relapse were a higher NIHSS at admission (P =.032) and a normal DSA (P = 0.002). CONCLUSION: In this cohort, severe deficits and cognitive symptoms at onset and an abnormal EEG were associated with a poor 6-month outcome.

Intracranial vessel wall lesions in patients with systematic lupus erythematosus.

BACKGROUND: Systemic lupus erythematosus (SLE) is related to vasculitis, which causes brain infarctions; however, the pathology of large cerebral vessels has not been fully established. PURPOSE: To demonstrate the prevalence of vessel wall lesions (VWLs) in SLE patients using 3D vessel wall imaging and to assess the relationship between VWLs and brain infarctions. STUDY TYPE: Retrospective. SUBJECTS: Sixty SLE patients and 50 healthy subjects (HS). FIELD STRENGTH/SEQUENCE: Each subject underwent 3T MRI, which included 3D FSE PDWI (CUBE). ASSESSMENT: For each of the 33 segments of the intracranial artery (internal carotid artery ∼ M3 segment of middle cerebral artery [MCA]), the VWLs were scored as either positive or negative, and the VWL score was calculated as the sum of the segments with VWLs. We also evaluated brain lesions on conventional MRI. STATISTICAL TESTS: We used logistic regression analyses to determine the clinical (serological test and cardiovascular risk factors) and imaging characteristics associated with infarctions in SLE patients. RESULTS: For the peripheral vessels such as MCA, VWLs were more common for SLE patients than for HS (43.3% versus 16.7% in M1 segment, 60.4% versus 16.7% in M2 segment, both P < 0.01). There were 21 infarctions in 13 patients (21.7%), and the median VWL score was larger in the patients with infarctions than in those without (13 versus 6, P < 0.01). Multivariate logistic regression analyses revealed a high VWL score ( ≥ 9) to be the only factor independently associated with the presence of infarctions (odds ratio: 10.1, 95% confidence interval: 1.01-101; P < 0.049). DATA CONCLUSION: We demonstrated a substantially high prevalence of VWLs among SLE patients, which were associated with brain infarctions. LEVEL OF EVIDENCE: 3 Technical Efficacy: Stage 2 J. Magn. Reson. Imaging 2018;47:1237-1246.

CNS Vasculitis: an Approach to Differential Diagnosis and Management.

PURPOSE OF REVIEW: The goal of this review is to provide an up-to-date approach to diagnosis and management of patients with central nervous system (CNS) vasculitis. RECENT FINDINGS: Challenges in diagnosis of CNS vasculitis still exist due to the broad differential diagnosis and generally nonspecific initial clinical manifestations. Differentiation between primary angiitis of the CNS (PACNS) and secondary causes is important in guiding management. Recent longitudinal cohort studies have improved our understanding of PACNS. Advances in neuroimaging and molecular testing have enhanced diagnostic decision-making. Therapy remains largely empiric, guided by observational data. Despite the limited use of targeted therapies, glucocorticoids and cyclophosphamide remain the mainstays of therapy in PACNS. Securing a diagnosis through a careful, team-based approach with emphasis on ruling out possible mimics is paramount in the management of patients with CNS vasculitis.

A Rare Complication of Sarcoidosis.

BACKGROUND: Cerebral vasculitis is a serious, but uncommon inflammatory condition of the blood vessel walls, with an annual incidence of 1-2 per million. A variety of disorders including encephalopathy, stroke, seizure, acute or subacute focal deficits should be considered as a differential diagnosis. CLINICAL CASE: A 56-year old male with a past history of pulmonary sarcoid presented with a unilateral facial numbness and loss of balance arising during sleep. His computed tomography scan of the brain was normal but brain magnetic resonance imaging with gadolinium demonstrated scattered infarcts in mixture of stroke topography not purely in keeping with embolism nor intrinsic small vessel disease. Further investigations including carotid ultrasound, transthoracic echo and 24-hour electrocardiogram were within normal limits. However, computed tomography angiography showed evidence of a widespread intracranial vasculopathy, as well as evidence of dissection of the left common carotid artery. His elevated calcium was consistence with a sarcoidosis relapse and cerebrospinal fluid analysis was in keeping with a central nervous system inflammatory process. Treatment was commenced with high dose steroids with additional pulsed intravenous cyclophosphamide together with antiplatelet therapy and a statin. CONCLUSIONS: This case illustrates the intracranial vasculopathy as a rare complication of sarcoidosis. Although sarcoid is well recognized to affect the central nervous system, it is unusual in the form of cerebral vasculitis.

Granulomatous Angiitis of the Central Nervous System Associated with Hodgkin's Lymphoma: Case Report and Literature Review.

Granulomatous angiitis of the central nervous system (GACNS) is a rare cerebrovascular disorder. It usually presents with multifocal neurologic symptoms symptoms including stroke, encephalopathy, and headache. A limited number of case reports describe neurological deficits resulting from GACNS as the manifesting symptoms of Hodgkin's lymphoma (HL). We describe the case of a patient with neurological symptoms from GACNS that led to the diagnosis of HL, as well as a literature review focusing on the association between GACNS and HL.

Relapsing polychondritis complicated by cognitive dysfunction: two distinct clinical phenotypes?

Relapsing polychondritis (RPC) is a rare, immune-mediated condition affecting approximately 3.5 per million population per year. Neurological involvement in RPC is still rarer and is presumed to be the result of a vasculitic process, although this is seldom confirmed in the literature. We present two cases of RPC complicated by cognitive dysfunction with contrasting clinical trajectories. Our findings suggest that there are two clinical phenotypes of cognitive dysfunction in RPC. The first is a fulminant, multisystem presentation with sub-acute cognitive decline mimicking central nervous system vasculitis, and we provide histopathological evidence of this process occurring. The other is an insidious cognitive decline without associated constitutional or systemic symptoms.

Autoimmune-Mediated Psychosis: A Case of Susac Syndrome in a Drug User.

OBJECTIVE: Susac syndrome, a rare disorder, is thought to be mediated by autoantibodies. One of the potential targets of these autoantibodies could be an antigen in the microvessels of the brain, the retina, and the inner ear leading to central nervous system (CNS) alterations, visual disturbances, and hearing deficits. Our aim is to expand clinicians' diagnostic options when facing psychosis due to medical conditions. METHODS: A case report was conducted for this study. RESULTS: This paper reports the case of a young male drug user who presented with psychosis, confusion and CNS vasculitis. First deemed to be drug-induced CNS vasculitis, it was finally diagnosed as Susac syndrome. CONCLUSIONS: Although an infrequent entity, Susac syndrome should remain an option in the differential diagnosis of several neurological and psychiatric presentations.

[Clinical aspects and diagnostics of cerebral vasculitis]. / Klinik und Diagnostik zerebraler Vaskulitiden.

CLINICAL/METHODICAL ISSUE: Vasculitis is a rare cause of diseases of the central nervous system (CNS). Vasculitis can be divided into primary and secondary forms, of which the vast majority can be manifested in various organ systems, including the CNS. Isolated vasculitis of the CNS is limited to the CNS and clinical neurological symptoms as with the other forms of vasculitis, are headaches, encephalopathy, focal deficits and seizures. A criterion of isolated CNS vasculitis is the clinical and laboratory diagnostic exclusion of other forms of vasculitis and the involvement of other organ systems. STANDARD RADIOLOGICAL METHODS: Multiple leaps in the caliber of intracranial arteries in cerebral angiography and multiple, small contrast medium-enhanced lesions in magnetic resonance imaging (MRI) of the brain are typical findings, which, however, can also be found in other forms of vasculitis. PERFORMANCE: The only way of proving meningitis is by a biopsy of the brain meninges and parenchyma. It is necessary to make as accurate a diagnosis as possible, especially in the context of therapeutic options of immunosuppression with steroids and cyclophosphamide. ACHIEVEMENTS: Cerebral vasculitis is a rare entity but it is an important diagnosis to consider when the appropriate clinical symptoms are present. Thorough laboratory diagnostics and subsequent brain biopsy are necessary to confirm the diagnosis in order to then be able to initiate a specific treatment.

Diagnosis and classification of central nervous system vasculitis.

Central nervous system vasculitis is one of the foremost diagnostic challenges in rheumatology. It results in inflammation and destruction of the vasculature within the CNS. When vasculitis is confined to brain, meninges or spinal cord, it is referred to as primary angiitis of the CNS. Secondary CNS vasculitis occurs in the setting of a systemic vasculitis, auto-inflammatory or infectious disease. Prompt and accurate diagnosis of CNS vasculitis is essential to prevent irreversible brain damage, and to secure precise treatment decisions. Progressive debilitating and unexplained neurological deficits, associated with abnormal cerebrospinal fluid is the typical picture of the disease. Biopsy of the brain remains the gold standard diagnostic test. The differential diagnosis of CNS vasculitis is highly diverse with a broad array of mimics at the clinical, radiographic and angiographic levels.

COVID-19 and the brain.

Entering the third year into the pandemic, overwhelming evidence demonstrates that Coronavirus disease 2019 (COVID-19) infection is a systemic illness, often with involvement of the central nervous system. Multiple mechanisms may underlie the development of neurologic manifestations of illness, including hypoxia, systemic illness, hypercoagulability, endothelial dysfunction, general critical illness, inflammatory response, and neurotropism of the severe acute respiratory syndrome coronavirus 2 (SARS-Co-V2) virus. COVID-19 infection is associated with neurologic involvement in all stages; acute infection, subacute/post-infection, and growing evidence also suggests during a chronic phase, the post-acute sequalae of COVID-19 (PASC). With over 20,000 published articles on COVID and the brain at the time of writing, it is virtually impossible to present an unbiased comprehensive review of how SARS-Co-V2 impacts the nervous system. In this review, we will present an overview of common neurologic manifestations, in particular focusing on the cerebrovascular complications, and proposed pathophysiology.

Atypical Neurological Manifestation in Childhood Microscopic Polyangiitis: A Case Report and Review of Literature.

Microscopic polyangiitis (MPA), a systemic necrotizing vasculitis of small vessels, is primarily associated with necrotizing and crescentic glomerulonephritis and pulmonary capillaritis. Neurological involvement, particularly of the central nervous system (CNS) is scarcely observed. The diversity of CNS symptoms could puzzle the diagnosis causing delays in treatment and potentially having a considerable effect on patient's quality of life or even death. The aim of this case report is to highlight the unusual manifestation of MPA in order to raise awareness of this orphaned disease among pediatricians or even pediatric rheumatologists and neurologists. Case Report: Herein we report the case of a 13-year-old Thai girl diagnosed with MPA presented with rapidly progressive glomerulonephritis (RPGN). Renal biopsy was performed demonstrated crescentic glomerulonephritis with negative immunofluorescence and positive titer of myeloperoxidase (MPO) antibody. Pulse methylprednisolone (MP) and cyclophosphamide (CYC) as well as plasmapheresis were initiated. Despite treatment with prednisolone (45 mg/day) and monthly CYC for two doses, she experienced a brief generalized tonic-clonic seizure during the follow-up period. The potential differential diagnosis of new-onset neurological manifestation contains infection owing to the immunocompromised status of the patient and CNS vasculitis as a result of the disease itself. Lumbar puncture was performed, and cerebrospinal fluid analysis demonstrated pleocytosis with negative infectious panel. Contrast magnetic resonance imaging studies of the brain showed multifocal patchy T2/FLAIR-hyperintense lesions in the cerebral as well as cerebellum regions, and irregular narrowing along the V4 segment of the right vertebral artery was demonstrated in magnetic resonance angiography. In the presence of CNS vasculitis, pulse MP and CYC were provided. The symptom of nervous system has progressively improved. Conclusion: In our case, MPA revealed RPGN with neurological manifestation. Despite the fact that it is scarcely reported, CNS vasculitis is one of the organ-threatening symptoms. To improve patient morbidity and mortality, multidisciplinary care teams with prompt diagnosis and treatment are highly recommended.

Diffusion tensor imaging analysis of rheumatoid arthritis patients with neuropsychiatric features to determine the alteration of white matter integrity due to vascular events.

INTRODUCTION: The pathophysiology of neuropsychiatric manifestations in rheumatoid arthritis is not well known. The magnetic resonance imaging of the brain in rheumatoid arthritis demonstrates non-specific findings, and in the majority of cases, magnetic resonance imaging fails to detect an abnormality, even in rheumatoid arthritis patients with neuropsychiatric manifestations. Therefore, we aimed to assess microstructural integrity changes of white matter in patients with rheumatoid arthritis by using different diffusion tensor imaging parameters. METHODS: Eighteen rheumatoid arthritis patients (10 with neuropsychiatric symptoms and 8 without any neuropsychiatric symptoms) and 14 controls were included. The volume of the T2 hyperintense lesions was assessed. Different diffusion tensor imaging parameters such as fractional anisotropy, apparent diffusion coefficient, trace, axial diffusivity, and radial diffusivity were obtained from six different regions of white matter. Inter group significant difference was determined by one-way analysis of variance followed by Tukey's post hoc test. The accuracy of diffusion tensor imaging matrices was evaluated from the receiver operating characteristic curve. RESULTS: No significant difference in lesions' volume was detected between rheumatoid arthritis patients with or without neuropsychiatric symptoms. There was an increased apparent diffusion coefficient and radial diffusivity (p < 0.05) as well as decreased fractional anisotropy and axial diffusivity (p < 0.5) in rheumatoid arthritis patients with neuropsychiatric symptoms compared to controls. Moreover, the apparent diffusion coefficient (p < .05) was increased in both positive and negative MRI of patients with neuropsychiatric features compared to the control group. The sensitivity and specificity of the apparent diffusion coefficient parameters was 73% and 72%, respectively. CONCLUSIONS: The various anisotropic metrics were altered in rheumatoid arthritis patients with neuropsychiatric symptoms by using diffusion tensor imaging analysis, representing that central nervous system vasculitis leads to tissue hypoxia resulting in vasogenic edema. This may lead to axonal and myelin degeneration of white matter fibers and neuronal cell disruption. Key Points • Our study confirms that neurovascular events are not uncommon in RA patients with NP features. Diffusion tensor imaging (DTI) is superior to conventional MRI scan for RA patients with NP features because it distinguishes between gray and white matter structures. • RA patients with NP features are more likely to have microstructural changes detected by DTI than by DWI, and it can provide comprehensive anatomical layouts describing regional disparities in neurodegeneration. • DTI's quantitative association of NP symptoms in a large patient cohort is an important study scope.

Inpatient Management of Encephalopathy.

Unexplained encephalopathy is a common occurrence in tertiary care centers and neurologic disorders should be considered after ruling out the infectious, toxic and metabolic etiologies. Neuroimaging combined with a thorough history and examination is often helpful in ruling out stroke and fulminant demyelinating encephalopathies. Autoimmune encephalopathy should be suspected in any patient with unexplained acute or subacute onset encephalopathy or rapidly progressing dementia. Anti-N-methyl-D-aspartate receptor (NMDA-R) encephalitis is the most studied form and Hashimoto encephalitis is the most controversial form of autoimmune encephalopathies. Obtaining a combined serum and Cerebrospinal fluid (CSF) autoantibody testing will increase the diagnostic yield of autoimmune and paraneoplastic encephalitis. When diagnosing NMDA receptor antibodies CSF is always more sensitive than serum and in contrast, voltage-gated potassium channel (VGKC) complex antibodies are more readily detectable in serum than in CSF. Neural-specific antibody tests frequently result after several weeks and treatment should be administered without a significant delay to prevent brain damage. Autoimmune encephalitis is often treatment responsive when immunotherapy (glucocorticoids, intravenous immune globulin, plasma exchange) is used in various combinations. The absence of inflammatory markers and autoantibodies in the serum or CSF may not rule out the possibility of paraneoplastic encephalopathies.

Primary Angiitis of the Central Nervous System: An Uncommon Cause of Stroke in the Young.

Primary angiitis of the central nervous system (PACNS) usually presents with symptoms of headache, cognitive impairment, or stroke with a mean age of onset at 50 years. Inflammation of the cerebral vessels can cause narrowing, occlusion, or thrombosis resulting in tissue ischemia and necrosis of the involved vessel territory. Findings can be seen on Magnetic Resonance Imaging (MRI) scans of the brain and serological markers of inflammation are typically within normal limits. The nonspecificity of PACNS presents a challenge for accurate diagnosis and must be differentiated from secondary vasculitis and Reversible Cerebral Vasoconstriction Syndrome (RCVS). Cerebral angiography, even though having low sensitivity and specificity, could sometimes be the only diagnostic tool available.

Lyme neuroborreliosis-associated cerebrovascular events in the Finnish endemic area.

OBJECTIVES: Neuroborreliosis is a rare cause of cerebral vasculitis and stroke. The incidence of Lyme borreliosis in Finland has been increasing in the last 20 years, so we expect that Lyme neuroborreliosis-associated vasculitis can be a more common cause of stroke in the future. MATERIALS & METHODS: We have retrospectively identified all adult patients (>16 years old) diagnosed with borreliosis (A69.2 Lyme borreliosis), transient ischemic attack (TIA, G45), and ischemic stroke (I63) at Helsinki University Hospital during 1.1.2014-31.10.2019 at our neurological emergency department. Medical data and follow-up data were retrospectively collected from medical records. Neuroborreliosis was diagnosed according to the European Federation of Neurological Societies guidelines. RESULTS: We have identified 10 cases of neuroborreliosis-associated stroke or TIA and/or vasculitis. Vasculitis as a manifestation of borreliosis was diagnosed in six patients of 1454 (0.4%) and stroke or TIA in nine (0.6%) of all borreliosis patients at Helsinki University Hospital. Clinical outcomes for all our patients were good with a modified Rankin scale (mRS) 0-2. CONCLUSIONS: Lyme neuroborreliosis-associated vasculopathy and cerebrovascular events still remain rare but should be considered especially in Lyme borreliosis endemic areas. Prognosis is good with appropriate antibiotic treatment, but additional immunosupressive treatment is sometimes needed.

Primary Central Nervous System Vasculitis as an Unusual Cause of Intracerebral Hemorrhage: A Case Report.

A 64-year-old male with a history of transverse myelitis presented to the hospital with a decreased level of consciousness of one day's duration. CT of the head revealed intracranial hemorrhage measuring 2 x 1.2 cm in the right temporal lobe and multiple small hemorrhages in the left hemisphere, suggestive of vasculitis. Initial vasculitis workup was negative for antinuclear antibody (ANA), complement component 3 (C3), and antineutrophil cytoplasmic antibodies: P-ANCA, C-ANCA. Syphilis, hepatitis B and C, West Nile virus antibody [immunoglobulin G (IgG) and immunoglobulin M (IgM)], herpes simplex virus (HSV) polymerase chain reaction (PCR), and HIV 1 and 2 were also negative. In view of the CT scan findings suggestive of vasculitis and the vague presentation of primary central nervous system vasculitis (PCNSV), a brain biopsy was performed. It revealed angiocentric granulomatous inflammation with focal vessel disruption and associated parenchymal hemorrhage, consistent with a diagnosis of granulomatous vasculitis. The patient received levetiracetam, multiple high doses of steroids, and six cycles of cyclophosphamide for a six-month duration. After induction, he has remained in remission without any maintenance therapy until now (eight years post-presentation).