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https://www.readbyqxmd.com/read/27570401/primary-cns-vasculitis-presenting-as-intraventricular-bleeding
#1
Sreeja Hareendranathan Pillai, Sapna Erat Sreedharan, Girish Menon, Santhosh Kannoth, Sylaja Pn
Primary angiitis of the central nervous system (PACNS) is a rare disorder affecting both medium- and small-sized vessels. Intracranial haemorrhages though less reported are in the form of parenchymal haemorrhage and subarachnoid haemorrhage. We report a case of PACNS with intraventricular haemorrhage due to aneurysms secondary to progression of vasculitis.
July 2016: Annals of Indian Academy of Neurology
https://www.readbyqxmd.com/read/27432677/other-noninfectious-inflammatory-disorders
#2
Álex Rovira, Cristina Auger, Antoni Rovira
Idiopathic inflammatory-demyelinating diseases (IIDDs) represent a broad spectrum of central nervous system (CNS) disorders, including monophasic, multiphasic, and progressive disorders that range from highly localized forms to multifocal or diffuse variants. In addition to the classic multiple sclerosis (MS) phenotypes, several MS variants have been described, which can be differentiated on the basis of severity, clinical course, and lesion distribution. Other forms of IIDD are now recognized as distinct entities and not MS variants, such as acute disseminated encephalomyelitis, and neuromyelitis optica spectrum disorders...
2016: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/27354225/diagnostic-yield-and-safety-of-brain-biopsy-for-suspected-primary-central-nervous-system-angiitis
#3
Jose Torres, Caitlin Loomis, Brett Cucchiara, Michelle Smith, Steven Messé
BACKGROUND AND PURPOSE: The utility and safety of brain biopsy for suspected primary angiitis of the central nervous system (PACNS) are uncertain. Factors predictive of a positive biopsy have not been well described. Our aim was to evaluate the diagnostic yield and safety of brain biopsy in suspected PACNS and determine whether any prebiopsy variables are associated with a positive biopsy. METHODS: This is a retrospective study of consecutive patients who underwent diagnostic brain biopsy for PACNS at a single institution...
August 2016: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/27123289/a-rare-case-of-tumor-mimicking-primary-angiitis-of-the-central-nervous-system
#4
L I Sun, Lijun Zhu, Teng Zhao, Dayan Wang, Dihui Ma, Rensheng Zhang, Shaokuan Fang
Primary angiitis of the central nervous system (PACNS) is a rare, but severe vascular disease. The present study reports the case of a 42-year-old male who developed PACNS. Magnetic resonance imaging (MRI) scans initially led to a misleading diagnosis of malignant glioma, and surgery was performed. The mass was resected, and a pathological examination confirmed a cerebral vasculitis. Single therapy with high doses of steroid did not improve the patient's condition, while a subsequent lesion appeared on the opposite side one year later...
May 2016: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/27112683/central-nervous-system-vasculitis-in-adults-and-children
#5
Marinka Twilt, Susanne M Benseler
Primary angiitis of the central nervous system (PACNS) is an inflammatory brain disease targeting the cerebral blood vessels, leading to a wide spectrum of signs and symptoms, including neurologic deficits, cognitive dysfunction, and psychiatric symptoms. The inflammation could be reversible if diagnosed and treated early. The diagnosis requires the careful consideration and rapid evaluation of systemic underlying conditions and disease mimics. The differential diagnosis is distinctly different for angiography-positive and -negative PACNS subtypes and differs depending on age, so there is childhood PACNS or adult PACNS...
2016: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/27043703/reversible-cerebral-vasoconstriction-syndromes-and-primary-angiitis-of-the-central-nervous-system-clinical-imaging-and-angiographic-comparison
#6
Aneesh B Singhal, Mehmet A Topcuoglu, Joshua W Fok, Oguzhan Kursun, Raul G Nogueira, Matthew P Frosch, Verne S Caviness
Reversible cerebral vasoconstriction syndromes (RCVS) and primary angiitis of the central nervous system (PACNS) are invariably considered in the differential diagnosis of new cerebral arteriopathies. However, prompt and accurate diagnosis remains challenging. Here we compared the features of 159 RCVS to 47 PACNS patients and developed criteria for prompt bedside diagnosis. Recurrent thunderclap headache (TCH), and single TCH combined with either normal neuroimaging, border zone infarcts, or vasogenic edema, have 100% positive predictive value for diagnosing RCVS or RCVS-spectrum disorders...
June 2016: Annals of Neurology
https://www.readbyqxmd.com/read/26951336/coexisting-vascular-lesions-in-reversible-cerebral-vasoconstriction-syndrome
#7
M Akif Topcuoglu, Oguzhan Kursun, Aneesh B Singhal
BACKGROUND: The pathophysiology of reversible cerebral vasoconstriction syndrome (RCVS) is not known. Published cases have documented coexisting cervical artery dissection and unruptured aneurysms, raising the possibility that ultrastructural vessel wall abnormalities underlie the development of vascular lesions as well as RCVS. METHODS: In this retrospective study we compared the frequency of neurovascular abnormalities in 158 consecutive RCVS patients, 44 patients with primary angiitis of the central nervous system (PACNS, positive controls), and 177 non-stroke patients with acute neurological symptoms (non-arteriopathy controls)...
March 7, 2016: Cephalalgia: An International Journal of Headache
https://www.readbyqxmd.com/read/26837371/primary-angiitis-of-the-central-nervous-system-avoiding-misdiagnosis-and-missed-diagnosis-of-a-rare-disease
#8
REVIEW
Shamik Bhattacharyya, Aaron L Berkowitz
Primary angiitis of the central nervous system (PACNS) is an extremely rare condition, defined as a vasculitis limited to the CNS with no identifiable cause. Its presentation is non-specific and includes headache, cognitive dysfunction and focal neurological signs. Laboratory studies, neuroimaging and angiography are neither sufficiently sensitive nor specific for diagnosis; a definitive diagnosis requires brain biopsy. As a result, PACNS is commonly misdiagnosed. Here, we review its clinical, laboratory and radiological features, and focus on avoiding common diagnostic pitfalls...
June 2016: Practical Neurology
https://www.readbyqxmd.com/read/26773697/frontal-lobe-dementia-syndrome-as-a-first-manifestation-of-primary-angiitis-of-the-central-nervous-system-pacns
#9
Marlene Bönstrup, Katja Ott, Markus Glatzel, Tim Magnus
This case presents a clinical course of a frontal lobe dysexecutive syndrome with dementia caused by a primary angiitis of the central nervous system (PACNS) of exclusively very small vessels. An isolated frontal lobe dementia syndrome as a primary manifestation of PACNS highlights the diverse clinical manifestations of the disease. The patient presented with a progressive cognitive decline with loss of memory, disinhibited behavior, inappropriate affect and frontal release signs. The diagnostic workup essentially revealed a lymphocytic pleocytosis in the cerebrospinal fluid and a generalized cortical atrophy without any vascular abnormalities...
February 2016: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/26167025/primary-angiitis-of-central-nervous-system-the-story-of-a-great-masquerader
#10
Bhupender Kumar Bajaj, Shweta Pandey, Bhargavi Ramanujam, Ankur Wadhwa
Primary angiitis of central nervous system (PACNS) is characterized by non-caseating granulomatous angiitis restricted to CNS. The condition often masquerades as migraine, stroke, epilepsy, dementia, demyelinating disorder and CNS infection. The protean manifestations frequently lead to misdiagnoses. We present a case of a young male from rural background that remained undiagnosed for years as the possibility of PACNS was not considered. He had history suggestive of migraine-like headaches followed by seizures...
July 2015: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/26119979/primary-angiitis-of-the-central-nervous-system-presenting-as-a-mass-lesion
#11
Calvin Gan, Julian Maingard, Lauren Giles, Pramit M Phal, K Meng Tan
We report a 51-year-old Asian man with primary angiitis of the central nervous system (PACNS) with atypical presentation as a mass lesion. PACNS is an uncommon condition causing inflammation and destruction of the blood vessels of the central nervous system. The aetiology is unclear and multiple mechanisms have been proposed. Its incidence is estimated at 2.4 per million per year, affecting patients of all ages (median 50 years) and more commonly Caucasian men. In Australia, 12 patients fulfilled the diagnostic criteria for PACNS between 1998 and 2009 at The Royal Melbourne Hospital, a university-affiliated tertiary referral centre...
September 2015: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/26044211/mass-forming-primary-angiitis-of-central-nervous-system-with-rosai-dorfmann-disease-like-massive-histiocytosis-with-emperipolesis
#12
Seong-Ik Kim, Soo Hee Kim, Hwa Jin Cho, Hannah Kim, Chun-Kee Chung, Seung Hong Choi, Sung-Hye Park
Primary angiitis of the central nervous system (PACNS) is a vasculitis restricted to the CNS without systemic involvement. We report a case of PACNS that was radiologically tumor-mimicking, and pathologically similar to the Rosai-Dorfmann disease. A 20-year-old woman presented with a focal facial motor seizure. Magnetic resonance image revealed heterogeneously enhanced well-demarcated solitary cerebral mass in the posterior frontal lobe. Histopathologically, the lesion showed lymphoplasmacytic vasculitis with massive parenchymal infiltration of large histiocytes with emperipolesis...
August 2015: Pathology International
https://www.readbyqxmd.com/read/25907920/primary-angiitis-of-the-central-nervous-system-diagnostic-criteria
#13
REVIEW
William J Powers
Primary angiitis of the central nervous system (PACNS) is a vasculitis of small arteries and veins of unknown cause restricted to the central nervous system (CNS), and controversy and disagreement exist over the means to establish the diagnosis. Cerebral arteriography, cerebrospinal fluid examination, and MRI singly or in combination do not have sufficiently demonstrated positive predictive value to establish the diagnosis. An alternative diagnosis is established at biopsy in 35% of cases. Histologic confirmation is required for the diagnosis of PACNS...
May 2015: Neurologic Clinics
https://www.readbyqxmd.com/read/25858343/reversible-cerebral-vasoconstriction-syndrome
#14
Kenneth R L Bernard, Morris Rivera
BACKGROUND: Reversible cerebral vasoconstriction syndrome (RCVS) is an underappreciated and poorly understood cause of thunderclap headache (TCH). Although self-limited in the majority of patients, incidence is increasing, with presentations overlapping considerably with life-threatening conditions, such as aneurysmal subarachnoid hemorrhage and stroke. In addition, radiographic findings seen in RCVS are also present in primary angiitis of the central nervous system (PACNS). Misdiagnosis of RCVS might subject patients to unnecessary invasive testing and immunosuppressive therapy...
July 2015: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/25856111/diagnostic-value-and-safety-of-brain-biopsy-in-patients-with-cryptogenic-neurological-disease-a-systematic-review-and-meta-analysis-of-831-cases
#15
REVIEW
Harrison Xiao Bai, Yingjie Zou, Ashley M Lee, Eric Lancaster, Li Yang
BACKGROUND: The role of brain biopsy in patients with cryptogenic neurological disease is uncertain. OBJECTIVE: To determine the risks and benefits of diagnostic brain biopsy for nonneoplastic indications in immunocompetent patients. METHODS: Appropriate studies were identified by searching electronic databases. RESULTS: We screened 3645 abstracts and included 20 studies with a total of 831 patients. Indications for biopsy were: (1a) severe neurological disease of unknown etiology in adults (n = 7) and (1b) in children (n = 2); (2) suspected primary angiitis of the central nervous system (PACNS) (n = 3); (3) chronic meningitis of unknown cause (n = 3); (4) atypical dementia (n = 4); and (5) nonneoplastic disease (n = 1)...
August 2015: Neurosurgery
https://www.readbyqxmd.com/read/25846440/-angiitis-of-the-autonomic-central-nervous-system
#16
Kazuhide Ochi
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...
March 2015: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/25818085/psychosis-in-primary-angiitis-of-the-central-nervous-system-involving-bilateral-thalami-a-case-report
#17
Sangha Kim, Doh Kwan Kim
OBJECTIVE: To report a case of primary angiitis of the central nervous system (PACNS), a rare inflammatory disease restricted to the central nervous system (CNS), with unusual clinical presentation mimicking schizophrenia. METHOD: Case report. RESULTS: A 45-year-old male presented with alteration of consciousness and confusion. Brain magnetic resonance imaging (MRI) scan showed a mass-like enhancing lesion involving bilateral thalami, and biopsy revealed findings compatible with PACNS...
May 2015: General Hospital Psychiatry
https://www.readbyqxmd.com/read/25409482/primary-angiitis-of-the-central-nervous-system-presenting-as-unilateral-optic-neuritis
#18
Neal M Rao, Pradeep S Prasad, Charles C Flippen, Aaron S Wagner, Catherine M Yim, Noriko Salamon, Harry V Vinters
A middle-aged woman who experienced recurrent episodes of unilateral vision loss and eye pain. On presentation, magnetic resonance imaging (MRI) demonstrated left optic nerve enhancement with patchy hyperintensities in the white matter of both frontal lobes and ill-defined enhancement in a lenticulostriate distribution. Ophthalmologic examination revealed left optic disc edema with a macular scar consistent with neuroretinitis. Her subsequent clinical course was notable for 2 episodes of painful vision loss, without associated neurologic symptoms, which resolved with intravenous and oral steroids...
December 2014: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
https://www.readbyqxmd.com/read/25399939/primary-angiitis-of-the-central-nervous-system-in-adults-and-children
#19
REVIEW
Alicia Rodriguez-Pla, Paul A Monach
Primary angiitis of the central nervous system (PACNS) is a rare disease, although it is increasingly recognized both in adults and children. Little is known about pathogenesis, but efforts at classification into subtypes are being made, and the distinction of PACNS from reversible cerebral vasoconstriction syndrome has been a major advance. The prognosis for improvement, or at least stabilization, of neurologic function is good with prompt and aggressive treatment, but the diagnosis continues to be challenging...
2015: Rheumatic Diseases Clinics of North America
https://www.readbyqxmd.com/read/25369436/cns-vasculitis
#20
REVIEW
Seby John, Rula A Hajj-Ali
Central nervous system vasculitis (CNSV) is a complex disease that poses considerable diagnostic and therapeutic challenges. It is divided into primary angiitis of the CNS (PACNS), or secondary angiitis of the CNS when associated with systemic conditions. Clinical presentation can be extremely variable and there is no classic presentation. In addition, there is no single diagnostic test and the sensitivity/specificity of all currently available tests is suboptimal. As such, an exhaustive approach with thorough historical data, physical examination, and corroborating investigations should be performed to exclude or confirm a diagnosis of CNSV...
September 2014: Seminars in Neurology
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