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Primary Central Nervous System Vasculitis

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Presentation

  • Compared with the 115 patients without intracranial hemorrhage, the 16 patients presenting with intracranial hemorrhage were more frequently women, less frequently had altered cognition, a persistent neurologic deficit, or stroke at presentation, less[ncbi.nlm.nih.gov]
  • We report the first case of a patient in whom spinal subdural hematomas were the first presenting signs of PCNSV. A 69-year-old woman presented with sudden-onset severe back pain and paraplegia following an acute hypertensive event.[ncbi.nlm.nih.gov]
  • We present a detailed history and clinical course of a patient with PCNSV along with a literature review. A 50-year-old Chinese female presented with a 6-month history of mild to moderate headache and sudden onset of visual loss.[ncbi.nlm.nih.gov]
  • Despite important pathophysiological and angiographic differences, PCNSV and MMD may present with similar clinical and auxiliary findings.[ncbi.nlm.nih.gov]
  • The condition rapidly deteriorated and the patient died 6 weeks after presentation. Rapidly progressive PCNSV represents the worst end of the clinical spectrum of PCNSV.[ncbi.nlm.nih.gov]
Sneezing
  • Interestingly, patients usually are able to identify a trigger for the exacerbations such as strenuous exercise, sexual activity, bathing, swimming, coughing, sneezing, defecating, urinating, laughing, or other valsalva triggering maneuvers.[clevelandclinicmeded.com]
Projectile Vomiting
  • vomiting, which partially relieved her symptoms, and diplopia of seven months’ duration.[jpgmonline.com]
Hemianopsia
  • Bitemporal hemianopsia accounts for the majority of visual symptoms, with impaired acuity and diplopia being relatively uncommon. Endocrine dysfunction has been reported in up to 80% of cases.[surgicalneurologyint.com]
Clumsiness
  • While his brain MRI was being planned, he became clumsy, inattentive and started taking extraordinarily long time to tailor-cut the cloth material. He lost his job because of his inability to properly cut and sew clothes.[ruralneuropractice.com]
  • On neurologic examination dysarthria, a left facial weakness, clumsiness in rapid alternating movements of the left hand and dysmetric finger-to-nose testing beside abnormalities in pain and temperature sensation on the left side were found.[bmcneurol.biomedcentral.com]
Frontal Headache
  • His past medical history revealed persistent frontal headache for the last 3 weeks. On admission the patient was nervous and slightly confused. Fever was not present, although the patient complained of flu-like symptoms 4 weeks before.[bmcneurol.biomedcentral.com]
Recurrent Headache
  • headaches, new neurological or systemic manifestations, persistent inflammation evidenced from blood and CSF analysis, and new CNS lesions shown by MRI or vascular imaging may reflect lack of disease control or complications of immunosuppressive therapy[touchneurology.com]
Cerebellar Sign
  • She also had bilateral cerebellar signs. The fundoscopy showed bilateral papilloedema. Her mini-mental status examination was normal.[jpgmonline.com]
Bitemporal Hemianopsia
  • Bitemporal hemianopsia accounts for the majority of visual symptoms, with impaired acuity and diplopia being relatively uncommon. Endocrine dysfunction has been reported in up to 80% of cases.[surgicalneurologyint.com]

Workup

  • This review discusses the state-of-the-art of current imaging techniques in the workup of PCNSV patients and highlights future prospects.[ncbi.nlm.nih.gov]
  • An intensive workup including MRI, conventional cerebral angiography and CSF studies is required to avoid misdiagnosis.[ncbi.nlm.nih.gov]
  • She was admitted while a broad infectious and inflammatory workup commenced.[ijcasereportsandimages.com]
  • Diagnostic Investigations The original diagnostic criteria for PACNS proposed by Calabrese and Mallek 28 in 1988 require that a patient has: • a history of a neurological deficit that remains unexplained after a vigorous diagnostic workup, including lumbar[touchneurology.com]
  • Findings usually reflect aseptic meningitis, with modest pleocytosis and elevated protein levels with a negative infectious workup.[clevelandclinicmeded.com]
Enlarged Sella
  • An unusual cause of hyperprolactinemia and enlarged sella turcica. JAMA. 1981. 246: 1811-2 36. Rivera JA. Lymphocytic hypophysitis: Disease spectrum and approach to diagnosis and therapy. Pituitary. 2006. 9: 35-45 37.[surgicalneurologyint.com]
Hyperprolactinemia
  • An unusual cause of hyperprolactinemia and enlarged sella turcica. JAMA. 1981. 246: 1811-2 36. Rivera JA. Lymphocytic hypophysitis: Disease spectrum and approach to diagnosis and therapy. Pituitary. 2006. 9: 35-45 37.[surgicalneurologyint.com]
Human Herpesvirus 8
  • Immunohistochemical stains for the varicella-zoster virus, Herpes 1 and 2, cytomegalovirus, adenovirus, human herpesvirus 8, EBV, toxoplasma, amyloid were all negative. Repeat testing of autoimmune and infectious etiologies remained negative.[surgicalneurologyint.com]
Ischemic Changes
  • Ischemic changes with diffusion restriction are seen in 50–75% of cases.[ 6 8 15 27 40 43 49 ] Contrast enhancing lesions resembling tumor are seen in 33% of cases.[ 6 15 22 27 40 46 ] Conventional digital subtraction angiography is the gold standard[surgicalneurologyint.com]
HLA-DR1
  • RESULTS: No statistically significant associations were found for HLA-B, HLA-DR1 and HLA-DQB1 variant. In the PCNSV group, only the HLA-A*69 variant was found more often than expected statistically.[ncbi.nlm.nih.gov]

Treatment

  • Six patients responded to RTX with clinical and MRI improvement with no reported flares after RTX treatment. Our data support a potential role for RTX treatment in selected patients with PCNSV.[ncbi.nlm.nih.gov]
  • This may facilitate earlier treatment and may prevent irreversible or even lethal outcomes.[ncbi.nlm.nih.gov]
  • CEC significantly decreased with immunosuppressive treatment.[ncbi.nlm.nih.gov]
  • Finally, alternative treatments are needed for the few patients who are refractory to conventional treatments, as mentioned above.[em-consulte.com]
  • OBJECTIVE: To describe the treatment and outcomes of patients with primary central nervous system (CNS) vasculitis.[ncbi.nlm.nih.gov]

Prognosis

  • It has also been recognized that PCNSV is a heterogeneous disorder encompassing clinical subsets that differ in terms of prognosis and therapy.[ncbi.nlm.nih.gov]
  • The involvement of small cortical/leptomeningeal vessels was associated with a more benign course, whereas the involvement of larger/proximal cerebral vessels was related to a less favorable prognosis and identified cases that should be treated more aggressively[moh-it.pure.elsevier.com]
  • The outcome of PCNSV is frequently fatal, and early diagnosis and treatment could result in a more favorable prognosis [ 7 , 8 , 9 ].[synapse.koreamed.org]
  • Given that the prognosis of PACNS may be poor in the absence of specific therapy, early diagnosis and treatment is essential. Diagnosis is often delayed due to the extremely heterogeneous clinical presentations and non-specific MRI features ( 4 ).[spandidos-publications.com]

Etiology

  • Although intracranial vasculitis has been associated with certain systemic inflammatory disorders and lymphomas, the etiology of spinal cord vasculitis is unclear.[ncbi.nlm.nih.gov]
  • OBJECTIVES: The etiology and genetic susceptibility of primary central nervous system vasculitis (PCNSV) are still unclear.[ncbi.nlm.nih.gov]
  • Etiology and clinical manifestation is heterogeneous and optimal treatment not yet established. Methods: Retrospective case series.[thieme-connect.com]
  • It is a complex and often severe disease with multifaceted clinical and pathological appearances, suggesting multiple disease subtypes and the potential existence of multiple etiologic pathways.[ncbi.nlm.nih.gov]
  • Back to Top Pathophysiology Primary angiitis of the CNS is a heterogeneous disease with different clinical subsets of unknown etiology and pathogenesis.[clevelandclinicmeded.com]

Epidemiology

  • Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis. 2007; 66(2):222-227. Molloy ES, Langford CA. Vasculitis mimics.[anfamed.edu.uy]
  • Current Concept and Epidemiology of Systemic Vasculitides. Allergol Int. 2014 Oct 25;[Epub ahead of print]. 5. Kallenberg CGM. The diagnosis and classification of microscopic polyangiitis. J Autoimmun. 2014;48-49:90–3. 6.[revistas.fucsalud.edu.co]
  • Epidemiology PACNS is a rare disorder. The incidence has been estimated at 2.4 per 1,000,000 person-years. Most biopsy proven cases in the literature demonstrate a slight male predominance.[clinicaladvisor.com]
  • The early observation suggested the possibility of a spectrum of progression starting with LYH and progressing to GH.[ 25 ] In contrast, other authors cited key epidemiological factors of LYH that were not found in GH including female bias, association[surgicalneurologyint.com]
  • Knowledge regarding the epidemiology, pathogenesis, diagnosis and management continues to evolve. Optimal management remains uncertain.[ijcasereportsandimages.com]
Sex distribution
Age distribution

Pathophysiology

  • Despite important pathophysiological and angiographic differences, PCNSV and MMD may present with similar clinical and auxiliary findings.[ncbi.nlm.nih.gov]
  • Back to Top Pathophysiology Primary angiitis of the CNS is a heterogeneous disease with different clinical subsets of unknown etiology and pathogenesis.[clevelandclinicmeded.com]
  • Abnormality in the control of brain blood vessel pressure is believed to be the critical element in the pathophysiology of RCVS. The alteration in vascular tone may be spontaneous or evoked by various exogenous or endogenous factors.[vasculitisfoundation.org]
  • Pathophysiology The pathogenesis of PACNS and its predilection for central nervous system (CNS) vessels remains poorly understood.[touchneurology.com]
  • A considerable gap still exists, however, particularly with respect to its pathophysiological mechanisms.[medlink.com]

Prevention

  • This may facilitate earlier treatment and may prevent irreversible or even lethal outcomes.[ncbi.nlm.nih.gov]
  • This may facilitate earlier treatment and may prevent irreversible or even lethal outcomes. a Unit of Rheumatology, Arcispedale S.[journals.lww.com]
  • Early recognition is important because treatment with corticosteroids with or without cytotoxic drugs can often prevent serious outcomes.[ard.bmj.com]
  • Salgado has received personal compensation in an editorial capacity from Belvoir Media Group as Consulting Editor of A Special Report published by the editors of Heart Advisor Cleveland Clinic STROKE: Advances in Prevention and Treatment of Cerebrovascular[neurology.org]
  • ., cotrimoxazole to prevent pneumocystosis, calcium and vitamin D supplementation, prescription of mesna combined with cyclophosphamide infusions to prevent hemorrhagic cystitis).[em-consulte.com]

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