310 results match your criteria Primary Angiitis of the CNS


X-Linked Lymphoproliferative Syndrome Presenting as Adult-Onset Multi-Infarct Dementia.

J Neuropathol Exp Neurol 2019 May;78(5):460-466

Department of Neurology, Mayo Clinic, Jacksonville, Florida.

Pathogenic hemizygous variants in the SH2D1A gene cause X-linked lymphoproliferative (XLP) syndrome, a rare primary immunodeficiency usually associated with fatal Epstein-Barr virus infection. Disease onset is typically in early childhood, and the average life expectancy of affected males is ∼11 years. We describe clinical, radiographic, neuropathologic, and genetic features of a 49-year-old man presenting with central nervous system vasculitis that was reminiscent of adult primary angiitis but which was unresponsive to treatment. Read More

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https://academic.oup.com/jnen/article/78/5/460/5445919
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http://dx.doi.org/10.1093/jnen/nlz018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6467195PMC
May 2019
3 Reads

Biopsy-proven primary angiitis of the central nervous system mimicking leukodystrophy: A case report and review of the literature.

J Clin Neurosci 2019 Mar 22. Epub 2019 Mar 22.

Department of Clinical Neurosciences, Fondazione IRCCS Istituto Neurologico "C. Besta", Via Celoria 11, 20133 Milan, Italy.

Primary Angiitis of the Central Nervous System (PACNS) is a rare form of idiopathic CNS vasculitis. Neuroimaging is often abnormal and characterized by multifocal brain lesions, but brain biopsy definitely confirms the diagnosis. We report the rare case of a 45-year-old female presenting with symptoms of intracranial hypertension and leukodystrophy-like neuroimaging findings. Read More

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http://dx.doi.org/10.1016/j.jocn.2019.03.021DOI Listing
March 2019
1 Read

Relapse rates and long-term outcome in primary angiitis of the central nervous system.

J Neurol 2019 Mar 23. Epub 2019 Mar 23.

Department of Neurology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.

Objective: To analyze the treatment response in patients with primary angiitis of the central nervous system (PACNS).

Methods: In a single-center retrospective observational study, we assessed relapses, remission, and long-term outcome by use of the modified Rankin Scale (mRS) under different immunotherapies. Eligible patients had CNS biopsy in favor of PACNS or neuroimaging compatible with PACNS after exclusion of an alternative diagnosis. Read More

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http://dx.doi.org/10.1007/s00415-019-09285-1DOI Listing

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

Neurol India 2019 Jan-Feb;67(1):105-112

Comprehensive Stroke Care Program, Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India.

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. Read More

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http://dx.doi.org/10.4103/0028-3886.253578DOI Listing
March 2019
14 Reads
1.084 Impact Factor

Anti-MOG encephalitis mimicking small vessel CNS vasculitis.

Neurol Neuroimmunol Neuroinflamm 2019 Mar 1;6(2):e538. Epub 2019 Feb 1.

Neurology Department (K.P., E.L.), University of Pennsylvania, Philadelphia; Rheumatology Department, Sant Joan de Deu Children's Hospital (E.I., J.A.), University of Barcelona, Spain; Department of Pathology (M.N.), University of Pennsylvania, Philadelphia; Pediatric Neuroimmunology Unit (V.G.-Á., T.A.), Sant Joan de Déu Children's Hospital, University of Barcelona, Spain; Department of Pathology (M.S.), Sant Joan de Déu Children's Hospital, University of Barcelona, Spain; and Neuroimmunology Program (A.S., T.A.), IDIBAPS-Hospital Clinic, University of Barcelona, Spain.

Objective: To report 2 patients with anti-myelin oligodendrocyte glycoprotein (MOG)-associated encephalitis who were initially misdiagnosed with small vessel primary CNS vasculitis.

Methods: Review of symptoms, MRI and neuropathologic features, and response to treatment. MOG antibodies were determined in serum and CSF using a cell-based assay. Read More

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http://dx.doi.org/10.1212/NXI.0000000000000538DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6384022PMC
March 2019
1 Read

Cognitive outcomes of childhood primary CNS vasculitis.

Neuropsychology 2019 Feb 14. Epub 2019 Feb 14.

Alberta Children's Hospital Research Institute, Department of Pediatrics, Cumming School of Medicine, University of Calgary.

Objective: To characterize the clinical cognitive phenotypes and severity of cognitive burden according to disease subtype in children with primary central nervous system vasculitis (cPACNS).

Method: This retrospective multicenter inflammatory brain disease database study examined the neuropsychological outcomes of 80 children (44 male; mean age = 7.89 years, SD = 4. Read More

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http://doi.apa.org/getdoi.cfm?doi=10.1037/neu0000513
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http://dx.doi.org/10.1037/neu0000513DOI Listing
February 2019
13 Reads

MOG antibody disease manifesting as progressive cognitive deterioration and behavioral changes with primary central nervous system vasculitis.

Mult Scler Relat Disord 2019 May 2;30:48-50. Epub 2019 Feb 2.

Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, 3-1-1, Maidashi, Higashi-ku, Fukuoka 812-8582, Japan. Electronic address:

We report a 60-year-old male with anti-myelin oligodendrocyte glycoprotein (MOG) antibody who developed progressive cognitive deterioration and behavioral changes, with no other focal signs, over 9 months. MRI showed numerous T2-hyperintense lesions with partial contrast enhancement in white and grey matter of cerebrum, cerebellum and spinal cord. A brain biopsy revealed perivascular inflammatory cell infiltration, disturbed vascular continuity and no demyelination, indicative of a lymphocytic pattern of primary CNS vasculitis (PCNSV). Read More

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http://dx.doi.org/10.1016/j.msard.2019.01.053DOI Listing

Atypical presentation of fulminant primary central nervous system angiitis.

J Neuroimmunol 2019 May 1;330:1-4. Epub 2019 Feb 1.

Department of Critical Care Medicine, University of Calgary, Calgary, AB T2N 1N4, Canada. Electronic address:

Background: Primary 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 Presentation: We 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. Read More

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http://dx.doi.org/10.1016/j.jneuroim.2019.01.019DOI Listing
May 2019
1 Read

RCVS score and diagnostic approach for reversible cerebral vasoconstriction syndrome.

Neurology 2019 Feb 11;92(7):e639-e647. Epub 2019 Jan 11.

From the Department of Neurology (E.A.R., A.B.S.), Massachusetts General Hospital, Boston; Department of Neurology (E.A.R., G.S.S.), Universidade Federal de São Paulo, Brazil; Hospital Israelita Albert Einstein (G.S.S.), São Paulo, Brazil; and Department of Neurology (M.A.T.), Hacettepe University, Ankara, Turkey.

Objective: To develop a method to distinguish reversible cerebral vasoconstriction syndrome (RCVS) from other large/medium-vessel intracranial arteriopathies.

Methods: We identified consecutive patients from our institutional databases admitted in 2013-2017 with newly diagnosed RCVS (n = 30) or non-RCVS arteriopathy (n = 80). Admission clinical and imaging features were compared. Read More

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http://dx.doi.org/10.1212/WNL.0000000000006917DOI Listing
February 2019
8 Reads

Anti-neutrophil cytoplasmic antibody associated central nervous system vasculitis with brain and spinal cord subarachnoid hemorrhage: A rare case report and review of the literature.

J Clin Neurosci 2019 Apr 26;62:253-255. Epub 2018 Dec 26.

Department of Neurosurgery, United States. Electronic address:

This case report describes a 48-year old female who presented with altered mental status, lower extremity weakness, low back pain and a recent history of subjective fevers and night sweats found to have posterior parieto-occipital and spinal subarachnoid hemorrhage on imaging. Further work-up revealed vasculitic changes in the intracranial vasculature and the external carotid artery on angiography. She also demonstrated positivity for perinuclear anti-neutrophil cytoplasmic (p-ANCA) antibodies overall consistent with ANCA associated central nervous system vasculitis (AAV). Read More

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http://dx.doi.org/10.1016/j.jocn.2018.12.001DOI Listing
April 2019
3 Reads

Health-related quality of life in children with inflammatory brain disease.

Pediatr Rheumatol Online J 2018 Nov 20;16(1):73. Epub 2018 Nov 20.

Rheumatology, Department of Pediatrics, Alberta Children's Hospital, 2888 Shaganappi Trail NW, Calgary, AB, T3B 6A8, Canada.

Objective: To quantify the impact of inflammatory brain diseases in the pediatric population on health-related quality of life, including the subdomains of physical, emotional, school and social functioning.

Methods: This was a multicenter, observational cohort study of children (< 18 years of age) diagnosed with inflammatory brain disease (IBrainD). Patients were included if they had completed at least one Health Related Quality of Life Questionnaire (HRQoL). Read More

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http://dx.doi.org/10.1186/s12969-018-0291-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6245877PMC
November 2018
20 Reads

High-resolution Intracranial Vessel Wall Imaging in Monitoring Treatment Response in Primary CNS Angiitis.

Neurologist 2018 Nov;23(6):188-190

Second Department of Neurology, School of Medicine, "Attikon" University Hospital, National and Kapodistrian University of Athens.

Introduction: High-resolution vessel wall imaging (HR-VWI) is emerging as a tool of notable utility in the diagnosis of intracranial vessel pathology. Its role in monitoring vessel wall disease response to treatment, however, is less well-established.

Case Report: We report the case of a 45-year-old man with left middle and anterior cerebral artery infarcts and an National Institute of Health Stroke Scale (NIHSS) score of 2. Read More

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http://dx.doi.org/10.1097/NRL.0000000000000198DOI Listing
November 2018
5 Reads

Intracranial Atherosclerosis Versus Primary Angiitis of the Central Nervous System: a Case Report.

Cureus 2018 Jul 23;10(7):e3031. Epub 2018 Jul 23.

Ross University School of Medicine, California Hospital Medical Center, Los Angeles, USA.

Primary angiitis of the central nervous system (PACNS) is a rare disease with various clinical presentations. It is the preferred name for vasculitis that is confined to the central nervous system (CNS) and is often considered a diagnosis of exclusion in vascular or inflammatory CNS diseases. This case describes a 46-year-old right-handed female with a past medical history of hypertension (HTN), hyperlipidemia (HLD), diabetes mellitus type two (DM2), obesity, and hemorrhagic stroke who was transferred from an outside facility after a one-month hospitalization to be evaluated for CNS vasculitis. Read More

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http://dx.doi.org/10.7759/cureus.3031DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6150750PMC
July 2018
3 Reads

Primary angiitis of the CNS and reversible cerebral vasoconstriction syndrome: A comparative study.

Neurology 2018 Oct 19;91(16):e1468-e1478. Epub 2018 Sep 19.

From the Department of Internal Medicine (H.d.B., A.A.), Caen University Hospital; University of Caen-Normandie (H.d.B., E.T., A.A); Biostatistics and Clinical Research Unit (J.-J.P.), Caen University Hospital; Emergency Headache Centre (J.M., C.B.), Lariboisière Hospital, Assistance Publique des Hôpitaux de Paris; Department of Neurology (C.A., A.D.), Montpellier University Hospital; Department of Neuroradiology (G.B., O.N.), Sainte-Anne Hospital, Paris-Descartes University; INSERM UMR 894 (O.N.); Department of Vascular Neurology (M.Z.), Saint Joseph Hospital; Université Paris-Descartes (M.Z.), INSERM UMR S 919, Paris; Department of Neurology (E.T.), Caen University Hospital; University of Caen-Normandie (E.T.), INSERM U919; Department of Neurology (M.-G.B.), Lariboisière Hospital, Assistance Publique des Hôpitaux de Paris, France; and Division of Rheumatology (C.P.), Vasculitis Clinic, Mount Sinai Hospital, Toronto, Ontario, Canada.

Objectives: To further improve the distinction between primary angiitis of the CNS (PACNS) and reversible cerebral vasoconstriction syndrome (RCVS).

Methods: We compared 2 large French cohorts of patients with PACNS (n = 110, retrospectively and prospectively enrolled) and RCVS (n = 173, prospectively enrolled).

Results: Patients with RCVS were predominantly female ( < 0. Read More

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http://dx.doi.org/10.1212/WNL.0000000000006367DOI Listing
October 2018
9 Reads
8.290 Impact Factor

Diagnosis and Treatment of Primary Central Nervous System Angiitis.

Curr Treat Options Neurol 2018 Aug 4;20(9):38. Epub 2018 Aug 4.

Neurology, University of Iowa, Iowa City, IA, USA.

Purpose Of Review: 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. Read More

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http://dx.doi.org/10.1007/s11940-018-0522-5DOI Listing
August 2018
3 Reads

Amyloid-Beta-Related Angiitis with Distinctive Neuro-Ophthalmologic Features.

Neuroophthalmology 2018 Aug 19;42(4):237-241. Epub 2017 Sep 19.

Department of Neurology, Mayo Clinic, Phoenix, Arizona, USA.

Amyloid beta-related angiitis (ABRA) is a subtype of cerebral amyloid angiopathy-related inflammation, with distinctive pathology and prognosis compared with cerebral amyloid angiopathy (CAA). On a spectrum of increasing severity, ABRA is considered to be in-between the less aggressive inflammatory-CAA and the more severe primary central nervous system (CNS) angiitis. Whereas retinal pathological changes were described in subjects with primary or secondary CNS angiitis, and non-inflammatory CAA, bilateral posterior pole superficial and peripapillary retinal hemorrhages have not been reported as initial signs in patients with pathology-confirmed ABRA, accompanying neurological spells and characteristic neuroimaging findings. Read More

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http://dx.doi.org/10.1080/01658107.2017.1374982DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6056207PMC
August 2018
4 Reads

[Misleading cerebral arterial wall gadolinium-enhancement in malignant lymphoma].

Rinsho Shinkeigaku 2018 Jul 30;58(7):456-459. Epub 2018 Jun 30.

Department of Neurology, Tosei General Hospital.

A previously healthy, 77-year-old woman presented with gradual cognitive decline and acute gait imbalance. On admission, despite no obvious paralysis, she tilted to the right. Her Mini-Mental State Examination score was slightly low (23/30). Read More

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http://dx.doi.org/10.5692/clinicalneurol.cn-001173DOI Listing
July 2018
22 Reads

Immune cell profiling in the cerebrospinal fluid of patients with primary angiitis of the central nervous system reflects the heterogeneity of the disease.

J Neuroimmunol 2018 Aug 7;321:109-116. Epub 2018 Jun 7.

Clinic of Neurology with Institute of Translational Neurology, University Hospital Münster, University Münster, 48149 Münster, Germany. Electronic address:

Primary angiitis of the central nervous system (PACNS) is a rare and heterogeneous inflammatory disease of the CNS vasculature with poorly understood pathophysiology. Comprehensive immune-cell phenotyping revealed increased frequencies of leukocytes in the cerebrospinal fluid (CSF) of PACNS patients compared to patients with multiple sclerosis, ischemic stroke, and somatoform disorders (n = 18 per group). Changes in the intrathecal immune-cell profile were heterogeneous in PACNS. Read More

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http://dx.doi.org/10.1016/j.jneuroim.2018.06.004DOI Listing
August 2018
28 Reads

Cerebrospinal Fluid Concentrations of Neuronal Proteins Are Reduced in Primary Angiitis of the Central Nervous System.

Front Neurol 2018 5;9:407. Epub 2018 Jun 5.

Department of Neurology, University of Münster, Münster, Germany.

Primary angiitis of the central nervous system (PACNS) is a rare autoimmune vasculitis limited to the CNS often causing substantial disability. Understanding of this disease is impaired by the lack of available biomaterial. Here, we collected cerebrospinal fluid (CSF) from patients with PACNS and matched controls and performed unbiased proteomics profiling using ion mobility mass spectrometry to identify novel disease mechanisms and candidate biomarkers. Read More

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http://dx.doi.org/10.3389/fneur.2018.00407DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5996103PMC
June 2018
7 Reads

CNS Vasculitis: an Approach to Differential Diagnosis and Management.

Curr Rheumatol Rep 2018 May 30;20(7):37. Epub 2018 May 30.

Cleveland Clinic Center for Vasculitis Care and Research, 9500 Euclid Ave, A50, Cleveland, OH, 44195, USA.

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. Read More

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http://dx.doi.org/10.1007/s11926-018-0747-zDOI Listing
May 2018
9 Reads

The first case of bacillus Calmette-Guérin-induced small-vessel central nervous system vasculitis.

Clin Rheumatol 2018 Aug 9;37(8):2297-2302. Epub 2018 May 9.

Department of Rheumatology, Centre Intégré Universitaire de Santé et de Services Sociaux de l'Estrie, Centre Hospitalier Universitaire de Sherbrooke, 3001 12e Avenue Nord, Sherbrooke, QC, J1H 5N4, Canada.

To present an unrecognized vascular complication of bacillus Calmette-Guérin (BCG) therapy administered for superficial bladder carcinoma. We also review the potential mimickers for primary angiitis of the central nervous system (PACNS) as well as complications of intravesical BCG therapy. An 89-year-old Caucasian man with a history of relapsing high-grade bladder carcinoma treated with intravesical BCG presented with recurring episodes of right upper limb paresthesia with clumsiness and dysarthria. Read More

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http://link.springer.com/10.1007/s10067-018-4136-9
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http://dx.doi.org/10.1007/s10067-018-4136-9DOI Listing
August 2018
15 Reads
1.770 Impact Factor

Pearls & Oy-sters: The critical role of histopathology in diagnosing cancer-associated necrotizing CNS vasculitis.

Neurology 2018 Apr;90(17):808-811

From Wake Forest University School of Medicine (J.S.); and the Departments of Neurology (J.T., R.E.S., A.G.), Pathology (R.M.), Radiology (C.G.), and Rheumatology (R.W.), Wake Forest Baptist Medical Center, Winston-Salem, NC.

Objective: To highlight the importance of a broad differential and histopathologic confirmation in patients with newly diagnosed cancer with brain lesions atypical for CNS metastasis.

Methods: We report 2 cases of biopsy-proven CNS vasculitis in patients undergoing treatment for a newly diagnosed nonmetastatic cancer. Comprehensive medical record review was performed to identify the clinical presentation, representative neuroimaging, histopathologic features, and response to treatment. Read More

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http://dx.doi.org/10.1212/WNL.0000000000005350DOI Listing
April 2018
3 Reads

Primary lymphomatoid granulomatosis in the central nervous system: A report of three cases.

Neuropathology 2018 Apr 10. Epub 2018 Apr 10.

Department of Pathology, Seoul National University, College of Medicine, Seoul, Korea.

Lymphomatoid granulomatosis (LYG) is a rare lymphoproliferative disorder characterized by infiltration of Epstein-Barr virus (EBV)-positive large atypical B-cells in an angiocentric fashion in a mixed inflammatory background. The histologic spectrum of LYG ranges from reactive proliferation to diffuse large B-cell lymphoma according to the number of EBV+ B-cells. It is known that virtually all patients have pulmonary involvement, whereas primary LYG of the other organs has been rarely reported. Read More

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http://dx.doi.org/10.1111/neup.12467DOI Listing
April 2018
20 Reads

Tc-HMPAO brain SPECT in the monitoring of cerebral vasculitis therapy.

Rev Esp Med Nucl Imagen Mol 2018 Jul - Aug;37(4):211-217. Epub 2018 Mar 28.

Department of Radiological, Oncological and Anatomo-Pathological Sciences, "Sapienza" University of Rome, Roma, Italia.

Objective: The central nervous system (CNS) may be involved in a variety of inflammatory diseases of the blood vessels, generally known as vasculitis. The clinical diagnosis of such involvement in early stages is difficult, since a mild cognitive impairment can be the only symptom. It was hypothesized that brain-perfusion SPECT would be able to reveal CNS involvement and to monitor the course of the disease. Read More

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http://dx.doi.org/10.1016/j.remn.2017.10.009DOI Listing

Erratum: Spinal cord inflammation in children with small vessel primary cns vasculitis.

Authors:

Paediatr Child Health 2017 Nov 13;22(8):509. Epub 2017 Sep 13.

[This corrects the article DOI: 10.1093/pch/pxx086.079. Read More

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http://dx.doi.org/10.1093/pch/pxx130DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5804907PMC
November 2017
2 Reads

Primary central nervous system vasculitis associated with lymphoma.

Neurology 2018 Mar 2;90(10):e847-e855. Epub 2018 Feb 2.

From the Departments of Neurology (R.D.B.), Biomedical Statistics and Informatics (T.J.H.C.), Radiology (J.H.), Hematology (S.M.A.), Anatomic Pathology (C.G.), and Rheumatology (G.G.H.), Mayo Clinic, Rochester, MN; and Rheumatology Division (C.S.), Azienda USL-IRCCS di Reggio Emilia e Università di Modena e Reggio Emilia, Italy. Dr. Salvarani is currently a visiting clinician at the Department of Neurology, Mayo Clinic, Rochester, MN.

Objectives: To record the clinical findings, response to therapy, and course of patients with primary CNS vasculitis (PCNSV) associated with lymphoma.

Patients And Methods: We reviewed the histories of 936 patients with a diagnosis of any type of vasculitis and lymphoma who were seen at the Mayo Clinic over a 32-year period. Ten patients with both PCNSV and lymphoma were identified. Read More

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http://dx.doi.org/10.1212/WNL.0000000000005062DOI Listing
March 2018
3 Reads

The role of biopsies and autopsies in the diagnosis of cognitive impairment, with emphasis on small vessel diseases: A critical appraisal enriched by personal experience.

Authors:
Leila Chimelli

Dement Neuropsychol 2017 Oct-Dec;11(4):356-363

Instituto Estadual do Cérebro Paulo Niemeyer - Laboratory of Neuropathology and Universidade Federal do Rio de Janeiro - Pathology Department, Rio de Janeiro, RJ - Brazil.

Acquired and hereditary microangiopathies cause cerebral small vessel diseases (CSVD) that impair cognition. The most frequent is primary angiitis of the CNS (PACNS), whose diagnosis remains challenging, requiring a multidisciplinary approach. Secondary vasculitis, CADASIL, miscellaneous microangiopathies and lymphomas, also cause cognitive impairment. Read More

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http://www.scielo.br/scielo.php?script=sci_arttext&pid=S
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http://dx.doi.org/10.1590/1980-57642016dn11-040004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5769993PMC
January 2018
4 Reads

Central vein sign differentiates Multiple Sclerosis from central nervous system inflammatory vasculopathies.

Ann Neurol 2018 02 15;83(2):283-294. Epub 2018 Feb 15.

Department of Neuroscience, Drug and Child Health, University of Florence, Florence, Italy.

Objectives: In multiple sclerosis (MS), magnetic resonance imaging (MRI) is a sensitive tool for detecting white matter lesions, but its diagnostic specificity is still suboptimal; ambiguous cases are frequent in clinical practice. Detection of perivenular lesions in the brain (the "central vein sign") improves the pathological specificity of MS diagnosis, but comprehensive evaluation of this MRI biomarker in MS-mimicking inflammatory and/or autoimmune diseases, such as central nervous system (CNS) inflammatory vasculopathies, is lacking. In a multicenter study, we assessed the frequency of perivenular lesions in MS versus systemic autoimmune diseases with CNS involvement and primary angiitis of the CNS (PACNS). Read More

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http://dx.doi.org/10.1002/ana.25146DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5901412PMC
February 2018
52 Reads

Progressive Fatal Myelopathy Secondary to Isolated Spinal Cord Vasculitis.

Front Neurol 2017 19;8:705. Epub 2017 Dec 19.

Department of Neurology, University Hospital Galway, Galway, Ireland.

A 56-year-old woman with various neurological signs which developed over a 1-year period was admitted for evaluation. MRI showed a markedly abnormal signal in the cervical spine. Despite treatment with IV steroids, she developed a progressive myelopathy, became quadriplegic, and required intubation. Read More

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http://dx.doi.org/10.3389/fneur.2017.00705DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5742205PMC
December 2017
5 Reads

Child Neurology: Primary angiitis of the CNS.

Neurology 2017 Dec;89(23):e268-e271

From Partners Pediatric Multiple Sclerosis Center (G.K., T.C.), Massachusetts General Hospital; and Partners Multiple Sclerosis Center (T.C.), Brigham and Women's Hospital, Boston, MA.

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http://dx.doi.org/10.1212/WNL.0000000000004718DOI Listing
December 2017
6 Reads

New onset refractory status epilepticus due to primary angiitis of the central nervous system.

Epilepsy Behav Case Rep 2017 19;8:100-104. Epub 2017 Aug 19.

Department of Neurosciences, King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia.

Primary Angiitis of the central nervous system is a rare and poorly understood variant of vasculitis. We narrate a case of a 46-year-old male who presented with new onset refractory status epilepticus mimicking autoimmune encephalitis. In this case we are reporting clues that could be useful for diagnosis and extensive literature review on the topic. Read More

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http://dx.doi.org/10.1016/j.ebcr.2017.07.005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5645167PMC
August 2017
2 Reads

Central Nervous System Disease in Antineutrophil Cytoplasmic Antibodies-Associated Vasculitis.

Authors:
Jonathan Graf

Rheum Dis Clin North Am 2017 11;43(4):573-578

Division of Rheumatology, University of California, Zuckerberg San Francisco General Hospital, San Francisco, 1001 Potrero Avenue, Box 0868, San Francisco, CA 94143, USA. Electronic address:

Central nervous system (CNS) disease is an uncommon but significant complication of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) and affects 3 primary areas of the CNS: the pituitary, the pachymeninges, and the CNS vasculature. Pituitary disease in uncommon, but hormonal deficiencies can be long lasting even in the face of excellent disease response. Chronic hypertrophic pachymeninigitis occurs in anti-proteinase 3-positive patients with systemic GPA and in anti-myeloperoxidase-positive patients with a milder and more limited form of the disease. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S0889857X173005
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http://dx.doi.org/10.1016/j.rdc.2017.06.006DOI Listing
November 2017
31 Reads

Primary Angiitis of the Central Nervous System.

Rheum Dis Clin North Am 2017 11;43(4):503-518

Division of Rheumatology, Russell/Engleman Rheumatology Research Center, University of California, San Francisco, 513 Parnassus Avenue, Medical Sciences S865, Box 0500, San Francisco, CA 94143-0500, USA. Electronic address:

Primary angiitis of the central nervous system (PACNS) is rare but often included in the differential diagnosis for unusual or unexplained neurologic symptoms owing to its array of clinical manifestations. PACNS is not a single entity, but rather an umbrella term for distinct subtypes of disease affecting different parts of the CNS, different sized vessels, and with different histologic and pathologic findings. Although brain biopsy is considered the gold standard test, it is neither highly sensitive nor specific. Read More

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http://dx.doi.org/10.1016/j.rdc.2017.06.001DOI Listing
November 2017
8 Reads

Successful treatment of recurrent primary CNS vasculitis with etanercept.

J Neurol Sci 2017 10 1;381:200-202. Epub 2017 Sep 1.

Department of Neurology, Omori Red Cross Hospital, Japan.

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http://dx.doi.org/10.1016/j.jns.2017.08.3257DOI Listing
October 2017
5 Reads

Neuroradiologic Characteristics of Primary Angiitis of the Central Nervous System According to the Affected Vessel Size.

Clin Neuroradiol 2019 Mar 5;29(1):37-44. Epub 2017 Sep 5.

Department of Diagnostic and Interventional Neuroradiology, University Medical Center Hamburg-Eppendorf, Martinistrasse 52, 20246, Hamburg, Germany.

Introduction: Magnetic resonance imaging (MRI) has an important impact in diagnosing primary angiitis of the central nervous system (PACNS). However, neuroradiologic findings may vary immensely, making an easy and definite diagnosis challenging.

Methods: In this retrospective, single center study, we analyzed neuroradiologic findings of patients with PACNS diagnosed at our hospital between 2009 and 2014. Read More

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http://dx.doi.org/10.1007/s00062-017-0622-8DOI Listing
March 2019
11 Reads

Association of primary central nervous system vasculitis with the presence of specific human leucocyte antigen gene variant.

Clin Neurol Neurosurg 2017 Sep 23;160:137-141. Epub 2017 Jun 23.

Department of Neurology, Alfried-Krupp-von Bohlen und Halbach Hospital, Alfried-Krupp-Straße 21, 45130 Essen, Germany.

Objectives: The etiology and genetic susceptibility of primary central nervous system vasculitis (PCNSV) are still unclear.

Patients And Methods: We analyzed the DNA of 25 Caucasian patients with PCNSV for human leucocyte antigen genes HLA-A, HLA-B, HLA-DRB1, and HLA-DQB1, respectively. HLA-frequencies of the 25 patients with PCNSV were compared with HLA-frequencies of matched Caucasian controls. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S03038467173017
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http://dx.doi.org/10.1016/j.clineuro.2017.06.009DOI Listing
September 2017
12 Reads

Subtypes of primary angiitis of the CNS identified by MRI patterns reflect the size of affected vessels.

J Neurol Neurosurg Psychiatry 2017 09 13;88(9):749-755. Epub 2017 Jul 13.

Department of Neurology, University Hospital Hamburg-Eppendorf, Germany.

Objective: To describe patterns of diagnostic findings, and identify subgroups of primary angiitis of the central nervous system (PACNS).

Methods: We retrospectively analysed 31 patients with PACNS. Cases were selected by predetermined diagnostic criteria and stratified into biopsy-proven and imaging-based PACNS. Read More

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http://jnnp.bmj.com/lookup/doi/10.1136/jnnp-2017-315691
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http://dx.doi.org/10.1136/jnnp-2017-315691DOI Listing
September 2017
14 Reads

Diagnostic test results in primary CNS vasculitis: A systematic review of published cases.

Neurol Clin Pract 2017 Jun;7(3):256-265

Altnagelvin Hospital (FM, GM, MOM), Derry; National Drug Treatment Centre (PM), Dublin, Ireland; and University of Glasgow (KWM), Scotland.

Background: Primary CNS vasculitis (PCNSV) can be diagnosed using cerebral angiography or histopathology combined with clinical features. The original diagnostic criteria, which weigh each test equally, have not been validated. Limited sensitivity and specificity for biopsy and angiography are recognized. Read More

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http://dx.doi.org/10.1212/CPJ.0000000000000359DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6081966PMC
June 2017
17 Reads

Primary CNS vasculitis masquerading as glioblastoma: A case report and review.

Asian J Neurosurg 2017 Jan-Mar;12(1):69-71

Department of Pathology, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad, Telangana, India.

Isolated angitis of the central nervous system (IACNS)/primary angitis of central nervous system vasculitis (PACNS) is an uncommon vascular disease, sparingly presenting as an isolated inflammatory lesion on magnetic resonance imaging (MRI). The disease usually manifests as a long-drawn and progressive ischemic event. Delay in diagnosis due to focal nature of the lesion also contributes to the poor prognosis as the dismal natural history and immunosuppressive therapy. Read More

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http://dx.doi.org/10.4103/1793-5482.145187DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5379810PMC
April 2017
1 Read

Childhood primary large vessel CNS vasculitis: single-centre experience and review of the literature.

Clin Exp Rheumatol 2017 Mar-Apr;35 Suppl 103(1):213-220. Epub 2017 Mar 28.

Klinik- und Poliklinik für Kinder- und Jugendmedizin, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Germany.

Ischaemic brain injuries are rare conditions in the paediatric age group. Main causes include non-arteriosclerotic arteriopathies, which in childhood usually result from primary vasculitis of large or small vessels and lead to impaired perfusion and subsequent ischaemic brain lesions. In accordance with the nomenclature of systemic forms, CNS vasculitis is subdivided into groups, based on the size of affected vessels: angiography-positive primary angiitis of medium-sized and large vessels (pPACNS), and angiography-negative angiitis of small vessels (svPACNS). Read More

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July 2017
2 Reads

Maintenance therapy is associated with better long-term outcomes in adult patients with primary angiitis of the central nervous system.

Rheumatology (Oxford) 2017 10;56(10):1684-1693

Vasculitis Clinic, Division of Rheumatology, Mount Sinai Hospital, Toronto.

Objective: We aimed to analyse the effect of maintenance therapy after induction on the outcomes of adult patients with primary angiitis of the CNS (PACNS).

Methods: We analysed long-term outcomes (relapse, survival and functional status) of patients enrolled in the French multicentre PACNS cohort who achieved remission after induction treatment and with ⩾12 months' follow-up, according to whether or not they received maintenance therapy. Good outcome was defined as relapse-free survival and good functional status (modified Rankin scale ⩽ 2) at last follow-up. Read More

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http://dx.doi.org/10.1093/rheumatology/kex047DOI Listing
October 2017
32 Reads

Seizure syndrome as a first manifestation of solitary tumor-like mass lesion of PACNS: Two case reports.

Medicine (Baltimore) 2017 Mar;96(9):e6018

aDepartment of Neurology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University bDepartment of Neurology, The Fifth People's Hospital of Shanghai, Fudan University cDepartment of Neurology, Changhai Hospital, Second Military Medical University dDepartment of Pathology, Changhai Hospital, Second Military Medical University eDepartment of Radiology, Changhai Hospital, Second Military Medical University, Shanghai, China.

Rationale: Primary angiitis of the central nervous system (PACNS) is an inflammatory disease involving cerebrovascular and parenchymal, and solitary tumor-like mass lesion of PACNS (TLML-PACNS) is frequently misdiagnosed as neoplastic or other inflammatory diseases. However, seizure syndrome as a first manifestation of TLML-PACNS has rarely reported before.

Patient Concerns: Here, we report 2 cases of seizure syndrome, which was the first sign that presented prior to the diagnosis of TLML-PACNS by brain biopsy. Read More

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http://Insights.ovid.com/crossref?an=00005792-201703030-0001
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http://dx.doi.org/10.1097/MD.0000000000006018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5340432PMC
March 2017
5 Reads

Hemorrhagic primary CNS angiitis and vasoconstrictive drug exposure.

Neurol Clin Pract 2017 Feb;7(1):26-34

Massachusetts General Hospital and Harvard Medical School (MAT, MPF, ABS), Boston; Neurology Department (MAT), Hacettepe University Hospitals, Ankara, Turkey; Safar Center for Resuscitation (RMJ), University of Pittsburgh Medical Center, PA; and Government Medical College (JG), Kottayam, India.

Background: Primary angiitis of the CNS (PACNS) typically manifests with accumulating neurologic deficits from ischemic strokes. Intracerebral hemorrhage (ICH) is an uncommon complication. There is limited knowledge about the risk factors and features of hemorrhagic PACNS. Read More

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http://dx.doi.org/10.1212/CPJ.0000000000000324DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5310206PMC
February 2017
11 Reads

Characteristics of hypertrophic pachymeningitis in patients with granulomatosis with polyangiitis.

J Neurol 2017 Apr 20;264(4):724-732. Epub 2017 Feb 20.

Department of Neurology, Neuroscience Center, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-Ro, Gangnam-gu, Seoul, 135-710, Korea.

Hypertrophic pachymeningitis (HP) is an important neurologic complication of granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis). The aim of this study is to investigate the clinical features, radiological findings, and diagnostic pitfalls of GPA-related HP. A retrospective chart review was performed to screen patients diagnosed with GPA at Samsung Medical Center between 1997 and 2016. Read More

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http://dx.doi.org/10.1007/s00415-017-8416-0DOI Listing
April 2017
28 Reads

[Headaches in autoimmune diseases].

Wiad Lek 2016;69(6):773-777

Uniwersytet Medyczny w Lublinie, Polska.

Vasculitides in autoimmune diseases are an important cause of secondary headaches. The article discusses the incidence of headache in primary and secondary vasculitides of the central nervous system. The symptoms of primary CNS vasculitis are presented. Read More

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July 2017
6 Reads

Central nervous system vasculitis in adults: An update.

Autoimmun Rev 2017 Feb 11;16(2):123-131. Epub 2017 Jan 11.

Rheumatology Division, Universidade Estadual de Campinas, Campinas, São Paulo, Brazil. Electronic address:

Primary central nervous system vasculitis (PCNSV) is a challenging diagnosis due to broad clinical manifestations and variable specificity and sensitivity of laboratory and imaging diagnostic tools. Differential diagnosis includes reversible cerebral vasoconstriction syndrome (RCVS), secondary vasculitis of the CNS and other noninflammatory vasculopathies. Brain biopsy is essential for definitive diagnosis and to exclude mimickers. Read More

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http://dx.doi.org/10.1016/j.autrev.2016.12.001DOI Listing
February 2017
10 Reads

Pathogenesis of developmental anomalies of the central nervous system induced by congenital cytomegalovirus infection.

Pathol Int 2017 Feb 10;67(2):72-82. Epub 2017 Jan 10.

Department of Regenerative & Infectious Pathology, Hamamatsu University School of Medicine, Hamamatsu, Japan.

In humans, the herpes virus family member cytomegalovirus (CMV) is the most prevalent mediator of intrauterine infection-induced congenital defect. Central nervous system (CNS) dysfunction is a distinguishing symptom of CMV infection, and characterized by ventriculoencephalitis and microglial nodular encephalitis. Reports on the initial distribution of CMV particles and its receptors on the blood brain barrier (BBB) are rare. Read More

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http://dx.doi.org/10.1111/pin.12502DOI Listing
February 2017
5 Reads

Adult primary angiitis of the central nervous system: isolated small-vessel vasculitis represents distinct disease pattern.

Rheumatology (Oxford) 2017 03;56(3):439-444

Vasculitis Clinic, Division of Rheumatology, Mount Sinai Hospital, Toronto, Ontario, Canada.

Objectives: We aimed to identify whether presentations and outcomes in adult patients with isolated small-vessel primary angiitis of the CNS (PACNS) would differ from other patients with large/medium-vessel involvement.

Methods: In the French PACNS cohort, we compared the characteristics, treatments and outcomes of patients with isolated small-vessel disease (normal CT, MR and/or conventional angiograms, brain biopsy positive for vasculitis) with other patients who had large/medium-vessel involvement (vessel abnormalities on CT, MR or conventional angiograms). A good functional outcome was defined as a modified Rankin scale ⩽2 at last follow-up, regardless of the occurrence of relapse. Read More

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http://dx.doi.org/10.1093/rheumatology/kew434DOI Listing
March 2017
21 Reads

[Clinical aspects and diagnostics of cerebral vasculitis].

Radiologe 2016 Oct;56(10):890-897

Klinik für Diagnostische und Interventionelle Neuroradiologie, Universitätsklinikum des Saarlandes, 66421, Homburg/Saar, Deutschland.

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. Read More

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http://dx.doi.org/10.1007/s00117-016-0171-1DOI Listing
October 2016
1 Read

[Reversible cerebral vasoconstriction syndrome: A rare pediatric cause of thunderclap headaches].

Arch Pediatr 2016 Dec 14;23(12):1254-1259. Epub 2016 Sep 14.

Service de neuropédiatrie, hôpital des enfants, 330, avenue de Grande-Bretagne, 31300 Toulouse, France.

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by thunderclap headaches with diffuse segmental constriction of cerebral arteries that resolves spontaneously within 3 months. We report on a case of a 13-year-old boy presenting with acute severe headaches, triggered by physical exertion. His past medical history was uneventful. Read More

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http://dx.doi.org/10.1016/j.arcped.2016.07.012DOI Listing
December 2016
3 Reads