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Calabrese and cns vasculitis

E C Obusez, F Hui, R A Hajj-Ali, R Cerejo, L H Calabrese, T Hammad, S E Jones
BACKGROUND AND PURPOSE: High-resolution MR imaging is an emerging tool for evaluating intracranial artery disease. It has an advantage of defining vessel wall characteristics of intracranial vascular diseases. We investigated high-resolution MR imaging arterial wall characteristics of CNS vasculitis and reversible cerebral vasoconstriction syndrome to determine wall pattern changes during a follow-up period. MATERIALS AND METHODS: We retrospectively reviewed 3T-high-resolution MR imaging vessel wall studies performed on 26 patients with a confirmed diagnosis of CNS vasculitis and reversible cerebral vasoconstriction syndrome during a follow-up period...
August 2014: AJNR. American Journal of Neuroradiology
Rula A Hajj-Ali, Leonard H Calabrese
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...
February 2014: Journal of Autoimmunity
Leonard H Calabrese
No abstract text is available yet for this article.
April 2013: La Presse Médicale
Rula A Hajj-Ali, Leonard H Calabrese
Despite the multiple areas of progress that have been made in our understanding of primary angiitis of the central nervous system (PACNS ), many challenges exist when facing this diagnosis. This review will address the clinical presentation, diagnosis and differential diagnosis of PACNS, with emphasis on the recognized advancement in the field. Appreciation of different mimics especially the recognition of reversible cerebral vasoconstriction syndromes (RCVS) as a major mimic will be addressed. Finally, approach to treatment will be summarized...
February 2013: Autoimmunity Reviews
Rula A Hajj-Ali, Aneesh B Singhal, Susanne Benseler, Eamonn Molloy, Leonard H Calabrese
Meaningful progress in our understanding and clinical approach to primary angiitis of the CNS (PACNS) has been made in the past three decades. Increased recognition of PACNS and general advances in diagnosis of neurological disorders have led to an aggressive diagnostic approach and a proliferation of case reports providing enriched clinical and pathological descriptions. We have witnessed major advances not only in the diagnosis of PACNS but in the recognition of its mimics. Epidemiological, clinical, neuroradiagnostic, and laboratory findings have enhanced our diagnostic accuracy and recognition of PACNS mimics, however, many challenges to our understanding and management of the disease in children and adults remain...
June 2011: Lancet Neurology
Rula A Hajj-Ali, Leonard H Calabrese
PURPOSE OF REVIEW: In the past decade, primary and secondary central nervous system (CNS) vasculitides have been more commonly diagnosed and recognized than previously. With the increasing awareness of these disorders, it is crucial for the treating physician to differentiate between causes of CNS vasculitis and to recognize their marked clinical and pathophysiological heterogeneity. This review focuses on the major forms of primary CNS vasculitis, as well as secondary CNS vasculitis with emphasis on their clinical findings, diagnoses, and treatment...
January 2009: Current Opinion in Rheumatology
J Hernández-Rodríguez, E S Molloy, L H Calabrese, R A Prayson, C A Langford
In the diagnosis of primary central nervous system (CNS) vasculitis, it is crucial to rule out clinical, angiographic, and pathological mimics. We report a case of arteriovenous malformation (AVM) mimicking primary CNS vasculitis. A young male presented with intracerebral haemorrhage and no other clinical, laboratory, or angiographic features suggesting vasculitis. Cerebral biopsy showed perivascular inflammation and slight infiltration of the muscular layer of cerebral vessels by chronic inflammatory cells close to the haemorrhagic areas...
November 2008: Scandinavian Journal of Rheumatology
E S Molloy, A B Singhal, L H Calabrese
OBJECTIVE: To describe the occurrence of mass lesions (ML) in primary angiitis of the central nervous system (PACNS) and assess the utility of diagnostic testing and treatment. METHODS: We examined the case records of the Cleveland Clinic (CC), Massachusetts General Hospital (MGH), and the English language medical literature, for biopsy-proven PACNS cases presenting as a solitary ML. Relevant clinical variables were extracted and analysed with JMP software. RESULTS: We identified a total of 38 ML: eight of 202 (4...
December 2008: Annals of the Rheumatic Diseases
Leonard H Calabrese, Eamonn S Molloy, Aneesh B Singhal
No abstract text is available yet for this article.
November 2007: Annals of Neurology
Shaye I Moskowitz, Leonard H Calabrese, Robert J Weil
BACKGROUND: Primary central nervous system vasculitis has traditionally been described as an aggressive condition, with significant morbidity and mortality. A subgroup of patients has been identified who have a similar clinical presentation, but with a benign course. This syndrome of BACNS is successfully treated with low-dose steroids and calcium-channel blockers. Histologic confirmation, when performed, is normal. METHODS: Intracerebral hemorrhage is a rare presenting finding in the setting of BACNS...
May 2007: Surgical Neurology
Susanne M Benseler
Central nervous system (CNS) vasculitis is an increasingly recognized, often devastating inflammatory brain disease of children and adults. In primary or isolated CNS vasculitis/angiitis of childhood (cPACNS), the vascular inflammation is limited to the brain and spinal cord. Secondary CNS vasculitis occurs in a variety of conditions including infections, collagen vascular diseases, systemic vasculidities, and malignancies. Mimics of CNS vasculitis in children include dissections and noninflammatory vasculopathies...
December 2006: Current Rheumatology Reports
J O Susac, L H Calabrese, E Baylin, R A Prayson, N E Medeiros, R P Hull, J P Tucker
A 39-year-old woman presented with multiple branch retinal artery occlusions almost three years before developing a mass lesion containing calcium in the left frontal lobe. Brain biopsy revealed a small vessel vasculitis and ischemic necrosis of brain with dystrophic calcification. We believe this to be the first case of primary CNS vasculitis with branch retinal artery occlusions and brain calcification.
2004: Clinical and Experimental Rheumatology
L H Calabrese
No abstract text is available yet for this article.
November 2003: Clinical and Experimental Rheumatology
L H Calabrese
A case of acute neurologic deficit accompanied by a cerebral angiogram consistent with CNS vasculitis is presented. The differential diagnosis and diagnostic decision process generated in this type of evaluation is illustrated.
November 2003: Clinical and Experimental Rheumatology
Rula A Hajj-Ali, Anthony Furlan, Alex Abou-Chebel, Leonard H Calabrese
OBJECTIVE: Benign angiopathy of the central nervous system (BACNS) is a subset of primary angiitis of the central nervous system (PACNS) with favorable outcome, not requiring prolonged immunosuppression. We retrospectively studied the clinical characteristics and prospectively assessed long-term outcome in a cohort of BACNS patients. METHODS: Patients meeting the clinical description for BACNS, evaluated and treated by a single investigator, were included. Data on demographics, signs and symptoms, laboratory studies, neuroimaging, brain biopsy, treatment, and complications were recorded...
December 15, 2002: Arthritis and Rheumatism
Rula A Hajj-Ali, Shekhar Ghamande, Leonard H Calabrese, Alejandro C Arroliga
Intensivists are sometimes faced with unexplained neurologic defects in ICU patients. A subacute presentation over weeks or months characterized by headache and mental status change with focal deficits in the absence of evidence of secondary vasculitis or other diseases mentioned in the differential diagnosis should arouse suspicion of PACNS. Delay in diagnosis of this rare condition may lead to additional morbidity and prolong ICU stay. There is also a risk of permanent cognitive dysfunction with untreated PACNS...
October 2002: Critical Care Clinics
Leonard H Calabrese
No abstract text is available yet for this article.
2002: Cleveland Clinic Journal of Medicine
L H Calabrese, G F Duna
Vasculitis resulting from drug use includes a wide variety of clinical and pathologic conditions that are, in general, empirically defined and poorly understood. Further complicating our grasp of these disorders are ambiguous terms such as hypersensitivity vasculitis, allergic vasculitis, leukocytoclastic vasculitis, serum sickness, and others, which are often used interchangeably without clear definition. The clinical picture varies widely from self-limiting to progressive and even fatal illness. These syndromes have now been reported in association with newer classes of therapeutic agents including biologic response modifiers...
January 1996: Current Opinion in Rheumatology
L H Calabrese
Angiitis of the CNS remains a poorly understood form of vascular inflammatory disease. Primary angiitis of the CNS or PACNS, a disease once considered extremely rare, recently has been reported more frequently. Traditional views of PACNS held it to be a relentless and uniformly fatal disease if untreated, but it is now viewed as more heterogeneous, with some relatively benign subsets. Its pathophysiology is poorly understood. Secondary vasculitis of the CNS can occur with a variety of other conditions and diseases; each requires a different diagnostic and therapeutic approach...
November 1995: Rheumatic Diseases Clinics of North America
L H Calabrese, L A Gragg, A J Furlan
OBJECTIVE: Primary angiitis of the central nervous system (PACNS) has been considered a rare and highly fatal disorder when untreated. In recent years the disease has been increasingly diagnosed by cerebral angiography, often without histologic confirmation. We have questioned whether cases of PACNS diagnosed on the basis of angiography alone are equivalent to histologically confirmed cases. METHODS: All cases of PACNS reported from 2 sources, including all cases reported in the English medical literature through January of 1990 as well as from the case records of the Cleveland Clinic Foundation were reviewed...
December 1993: Journal of Rheumatology
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