A case report: primary CNS angiitis

Holay, Madhuri; Game, Prasad D.; Kharkar, Sandip; Chudalwar, Piyush

doi:10.18203/2320-6012.ijrms20150280

Cited by 2 publications

(8 citation statements)

References 3 publications

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“…It is considered one of the vasculopathies that pose a real diagnostic challenge for physicians 8 . It is limited to the parenchyma of the brain and spinal cord, arteries, and meningeal veins that leads to inflammation and destruction of the vessels, especially the small-medium vessels 3 . This leads to narrowing or occlusion of these blood vessels 9 .…”

Section: Discussionmentioning

confidence: 99%

“…Subsequently, haemorrhagic and ischaemic events will occur in the adjacent tissues, which will cause diverse clinical presentations 6 . It constitutes about 1.2% from all the vasculitis that involve the CNS 3 . It was first described by Cravioto and Feigin in mid 1950s 8 .…”

Section: Discussionmentioning

confidence: 99%

“…The exact pathogenesis is still unknown. A Th1-mediated response is thought to cause the granulomatous nature of the vascular inflammatory lesions 3 . What triggers this immune mediated cascade and its propagation that leads to blood vessel destruction is still unknown 11 .…”

Section: Discussionmentioning

confidence: 99%

“…The annual incidence of this disease is reported to be 2.4 per 100 000 people. Most reported cases are from Europe and North America 3 . It can occur at any age, but it occurs predominantly in the fourth to sixth decades 2 .…”

Section: Discussionmentioning

confidence: 99%

“…It has an annual incidence rate of 2.4 per one million people. Men are more affected than women with an average age of presentation of 50 years 3 . Other than the heterogenous clinical presentations, accurate diagnosis of this condition poses some challenges as there are no laboratory tests or imaging modalities with sufficient sensitivity and specificity to confirm the diagnosis and exclude all mimickers of vasculitis.…”

Section: Introductionmentioning

confidence: 99%

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Primary central nervous system angiitis: a case report

M. M. Zeer,

N. A. Arda,

Abu Salim

et al. 2024

Annals of Medicine &Amp; Surgery

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Introduction: Primary central nervous system angiitis is a rare idiopathic vasculitis that is limited to the central nervous system. It has a wide range of clinical presentations that can mimic other vasculopathies. Case presentation: A 24-year-old female patient presents with various nonspecific neurological complaints in a progressive course. After a challenging diagnostic workup, she was diagnosed by tissue biopsy to have primary central nervous system angiitis. Discussion: Although primary central nervous system angiitis has been reported increasingly recently, its pathogenesis is still unknown and its diagnosis is still very challenging. No universal criteria have been adopted, and there is no laboratory test or imaging modality with sufficient sensitivity and specificity to confirm the diagnosis and exclude other mimickers. Conclusion: To prevent misdiagnosis, clinicians treating patients with suspected primary central nervous system angiitis should be aware of its differentials.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Primary central nervous system angiitis: a case report

M. M. Zeer,

N. A. Arda,

Abu Salim

et al. 2024

Annals of Medicine &Amp; Surgery

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Central Nervous System Systemic Lupus Erythematosus (CNS-SLE) Vasculitis Mimicking Lewy Body Dementia: A Case Report Emphasizing the Role of Imaging With an Analysis of 33 Comparable Cases From the Scientific Literature

Abraham

Neel

Bishay

et al. 2020

J Geriatr Psychiatry Neurol

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Introduction: Neuropsychiatric symptoms occur in 30% to 40% of patients living with systemic lupus erythematosus (SLE). Brain imaging may play a pivotal role in determining the etiology as it did for the case presented here. Methods: A new case of central nervous system (CNS) SLE is presented along with an analysis of 33 comparable cases from the scientific literature. Results: A 70-year-old female with subacute cutaneous lupus presented to a university-based geropsychiatry program after 1 year of benign visual hallucinations and several months of shuffling gait, recurrent falls, and forgetfulness. These symptoms were highly suggestive of Lewy body dementia; however, the patient’s history of basal ganglia infarct, cognitive testing demonstrating inattention and executive dysfunction, and follow-up brain imaging, which did not reveal acute findings, aligned with cerebral pathology previously attributed to vasculitis and supported the diagnosis of subcortical dementia due to SLE-CNS vasculitis. Oral prednisone 20 mg daily resolved her symptoms. Over the next 19 months, her prednisone was tapered completely and her symptoms did not return. A systematic literature search identified 33 comparable cases. Conclusion: An analysis of previously published cases suggests that extending the duration of the prednisone taper beyond 1 year may decrease the risk of later occurring neuropsychiatric symptoms in this patient population.

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A case report: primary CNS angiitis

Cited by 2 publications

References 3 publications

Primary central nervous system angiitis: a case report

Primary central nervous system angiitis: a case report

Central Nervous System Systemic Lupus Erythematosus (CNS-SLE) Vasculitis Mimicking Lewy Body Dementia: A Case Report Emphasizing the Role of Imaging With an Analysis of 33 Comparable Cases From the Scientific Literature

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