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Hecheng Ren, Lin Ma, Mingsheng Yu, Ming Wei, Long Yin
No abstract text is available yet for this article.
November 2018: Neurology India
Elina Liu, Marinka Twilt, Pascal N Tyrrell, Anastasia Dropol, Shehla Sheikh, Mark Gorman, Susan Kim, David A Cabral, Rob Forsyth, Heather Van Mater, Suzanne Li, Adam M Huber, Elizabeth Stringer, Eyal Muscal, Dawn Wahezi, Mary Toth, Pavla Dolezalova, Katerina Kobrova, Goran Ristic, Susanne M Benseler
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)...
November 20, 2018: Pediatric Rheumatology Online Journal
Muhamad Alhaj Moustafa, Eric L Crowell, Sherif Elmahdy, Vera Malkovska, Ashvini K Reddy
Frosted branch angiitis (FBA) is a rare form of retinal vasculitis with typical perivascular edema taking the shape of frost on a tree branch. It was reported only twice as the initial presentation of Hodgkin lymphoma (HL). Here, we present the first case of paraneoplastic FBA as the initial sign of HL relapse in an elderly female.
October 2018: Clinical Case Reports
M Marrodan, J N Acosta, L Alessandro, V C Fernandez, E Carnero Contentti, N Arakaki, A A Kohler, M P Fiol, S F Ameriso, J Correale
INTRODUCTION: To assess clinical and/or imaging features useful to distinguish between Susac syndrome (SuS) and primary angiitis of central nervous system (PACNS). METHODS: Multicenter retrospective analysis of two cohorts of Argentine patients diagnosed with SuS and PACNS. RESULTS: 13 patients diagnosed with SuS (6 women and 7 men, mean age 35 ± 10 years) and 15 with PACNS (10 women and 5 men, mean age 44 ± 18 years) were analyzed...
September 26, 2018: Journal of the Neurological Sciences
Joshua A Ronen, Aileen Nguyen, Jerrica N Mueller, Hyonju Lee
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...
July 23, 2018: Curēus
Hubert de Boysson, Jean-Jacques Parienti, Jérôme Mawet, Caroline Arquizan, Grégoire Boulouis, Cécilia Burcin, Olivier Naggara, Mathieu Zuber, Emmanuel Touzé, Achille Aouba, Marie-Germaine Bousser, Christian Pagnoux, Anne Ducros
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 ( p < 0.0001), with migraines ( p < 0.0001), and were more often exposed to vasoactive substances ( p < 0...
October 16, 2018: Neurology
Zhihui Dong, Tianyue Pan, Yuan Fang, Zheng Wei, Shiyang Gu, Gang Fang, Yifan Liu, Yang Luo, Hao Liu, Tiejun Zhang, Meiyu Hu, Daqiao Guo, Xin Xu, Bin Chen, Junhao Jiang, Jue Yang, Zhenyu Shi, Ting Zhu, Yun Shi, Peng Liu, Weiguo Fu
BACKGROUND: Peripheral blood mononuclear cells (PBMNCs) and purified CD34+ cells (PCCs) are increasingly being used at treating no-option critical limb ischaemia (NO-CLI). We aimed to compare the efficacies and uncover the advantages associated with each treatment approach. METHODS: A randomised single-blinded non-inferiority trial (Number: NCT 02089828) was performed. NO-CLI patients were 1:1 randomised to the PBMNCs and PCCs groups, and compared in relation to safety and efficacy outcomes...
September 2018: EBioMedicine
Vincent Jachiet, Raphael Lhote, Pierre Rufat, Micheline Pha, Julien Haroche, Sophie Crozier, Corinne Dupel-Potier, Dimitri Psimaras, Zahir Amoura, Fleur Cohen Aubart
OBJECTIVES: Clinical involvement of the nervous system is uncommon during sarcoidosis. Cerebrovascular events are rarely reported during sarcoidosis and may be confused with primary angiitis of the central nervous system. The characteristics and outcomes of cerebrovascular events during sarcoidosis have not been well-evaluated. PATIENTS AND METHODS: We conducted a retrospective case series in our institution with an updated review of the literature from 1962 to 2017 to characterize the clinical presentation, imaging, histology and outcomes of stroke that were thought to be causally related to sarcoidosis...
October 2018: Journal of Neurology
Laura Chu, Marsha Eustace, Natalia Pittman
No abstract text is available yet for this article.
August 6, 2018: Canadian Journal of Neurological Sciences. le Journal Canadien des Sciences Neurologiques
Kaustubh Limaye, Edgar A Samaniego, Harold P Adams
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. It is important to recognize the different conditions that may mimic the clinical and radiologic presentation. We present a contemporary literature review that should update physicians who encounter this patient population...
August 4, 2018: Current Treatment Options in Neurology
Oana M Dumitrascu, Erin M Okazaki, Steven H Cobb, Matthew A Zarka, Stephen A De Souza, Gyanendra Kumar, Cumara B O'Carroll
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...
August 2018: Neuro-ophthalmology
Carolin Beuker, Antje Schmidt, Daniel Strunk, Peter B Sporns, Heinz Wiendl, Sven G Meuth, Jens Minnerup
Primary angiitis of the central nervous system (PACNS) represents a rare inflammatory disease affecting the brain and spinal cord. Stroke, encephalopathy, headache and seizures are major clinical manifestations. The diagnosis of PACNS is based on the combination of clinical presentation, imaging findings (magnetic resonance imaging and angiography), brain biopsy, and laboratory and cerebral spinal fluid (CSF) values. PACNS can either be confirmed by magnetic resonance angiography (MRA)/conventional angiography or tissue biopsy showing the presence of typical histopathological patterns...
2018: Therapeutic Advances in Neurological Disorders
Hiroyasu Inoue, Keita Sakurai, Yuuya Kanou, Kentaro Yamada, Hiroyuki Yuasa
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). Gadolinium-enhanced, high-resolution T1 -weighted MRI showed abnormal arterial wall enhancement at the bilateral middle cerebral and right internal carotid arteries. The combination of arterial and parenchymal enhancement limited to the central nervous system (CNS), normal laboratory data including soluble interleukin-2 receptor, and random skin and bone-marrow biopsies was suggested of primary angiitis of the CNS (PACNS)...
July 27, 2018: Rinshō Shinkeigaku, Clinical Neurology
Daniel Strunk, Andreas Schulte-Mecklenbeck, Kristin S Golombeck, Gerd Meyer Zu Hörste, Nico Melzer, Carolin Beuker, Antje Schmidt, Heinz Wiendl, Sven G Meuth, Catharina C Gross, Jens Minnerup
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. While proportions of T-cell subsets remained unaltered, some PACNS patients showed a shift toward NK- or B cells...
August 15, 2018: Journal of Neuroimmunology
V G Madanagopalan, C K Nagesha, Girish Velis, Santosh Devulapally, S Balamurugan
A 54-year-old male sustained ocular trauma with a projectile. Examination of the right eye revealed an intraocular foreign body (IOFB) adjacent to the optic nerve head, vitritis, vitreous hemorrhage, and translucent perivascular sheathing of the retinal vessels in all quadrants suggesting frosted branch angiitis (FBA). The patient underwent vitrectomy with removal of the IOFB and silicone oil tamponade under steroid cover. With continued use of systemic and topical steroids after surgery, complete resolution of FBA and improvement in vision were noted in a week...
July 2018: Indian Journal of Ophthalmology
Kunal Bhatia, Laura Qi, Adnan I Qureshi
No abstract text is available yet for this article.
June 2018: Journal of Vascular and Interventional Neurology
Tillmann Ruland, Jolien Wolbert, Michael G Gottschalk, Simone König, Andreas Schulte-Mecklenbeck, Jens Minnerup, Sven G Meuth, Catharina C Groß, Heinz Wiendl, Gerd Meyer Zu Hörste
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. We identified 14 candidate proteins, including amyloid-beta A4 protein (APP), with reduced abundance in the CSF of PACNS patients and validated APP by Enzyme-linked Immunosorbent Assay (ELISA) in an extended cohort of patients with PACNS...
2018: Frontiers in Neurology
Shreeya Patel, Laura Ross, Shereen Oon, Mandana Nikpour
Primary angiitis of the central nervous system (PACNS) is a rare autoimmune vasculitis affecting the brain and spinal cord. Treatment with biological agents has revolutionised the treatment of many rheumatic conditions but there is scant literature regarding the use of biological agents in PACNS. We present three cases of PACNS treated with rituximab, including two cases of relapsed disease, and a literature review suggesting a role for rituximab in this condition.
June 2018: Internal Medicine Journal
Kevin Byram, Rula A Hajj-Ali, Leonard Calabrese
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...
May 30, 2018: Current Rheumatology Reports
Mari Yoshida
Vascular dementia involves several mechanism of pathogenesis. Cerebral small vessel diseases play a central role in vascular dementia, including sporadic cerebral small vessel diseases, cerebral autosomal-dominant or autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL or CARASIL), cerebral amyloid angiopathy, and amyloid β-related angiitis. Although these diseases have different pathomechanisms, chronic white matter hypoperfusion contributes to development of neuronal dysfunction as a common pathway in vascular dementia...
May 2018: Brain and Nerve, Shinkei Kenkyū No Shinpo
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