Primary angiitis of the central nervous system – A challenging diagnosis

Paul, Shabeer Ahmad; Roy, Devlina; Mondal, Gobinda; Bhattacharyya, Ramesh; Ghosh, K.; Das, Sarbajit; Krishna, Hema; Patra, Chandrakanta; Kiran, Jyoti; Benito‐León, Julián

doi:10.1016/j.jneuroim.2022.577844

Cited by 9 publications

(9 citation statements)

References 103 publications

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“…It is a multifactorial inflammatory process that primarily affects the arterioles and small-medium-sized CNS vessels, most likely located in the meninges and the cortex. The venous system is rarely affected [ 1 ].…”

Section: Clinical Discussionmentioning

confidence: 99%

“…Primary Angiitis of the Central Nervous System (PACNS) is a rare form of vasculitis with an annual incidence rate of 2.4/1000000 [ 1 ]. PACNS is limited to the brain and the spinal cord.…”

Section: Introductionmentioning

confidence: 99%

“…PACNS is limited to the brain and the spinal cord. The pathological picture portrays inflammation and destruction of the central nervous system (CNS) vessels without evidence of vasculitis outside this level, making it a distinct clinical entity [ 1 ]. The leptomeningeal and parenchymal blood vessels are the main affected vessels in the disease process [ 2 , 3 ].…”

Section: Introductionmentioning

confidence: 99%

“…However, the immune system appears to play a central role in the disease process [ 4 ]. Due to the lack of specific universal diagnostic criteria and imaging findings, the diagnosis remains a challenging one [ 1 , 2 ]. Presentation varies from a simple headache to focal weakness, encephalopathy and stroke-like presentations [ 2 ].…”

Section: Introductionmentioning

confidence: 99%

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Right arm weakness and mouth deviation as a presentation of Primary Angiitis of the Central Nervous System treated with rituximab: A case-report

Sarhan

Al-Jasim

Alaraj

et al. 2022

Annals of Medicine &Amp; Surgery

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Section: Clinical Discussionmentioning

confidence: 99%

“…Primary Angiitis of the Central Nervous System (PACNS) is a rare form of vasculitis with an annual incidence rate of 2.4/1000000 [ 1 ]. PACNS is limited to the brain and the spinal cord.…”

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Right arm weakness and mouth deviation as a presentation of Primary Angiitis of the Central Nervous System treated with rituximab: A case-report

Sarhan

Al-Jasim

Alaraj

et al. 2022

Annals of Medicine &Amp; Surgery

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“…При ПВЦНС наблюдается поражение сосудов любого калибра головного, редко спинного мозга и их оболочек, без вовлечения в патологический процесс сосудов других органов и систем. В научной медицинской литературе аббревиатура ПВЦНС также используется для обозначения следующих заболеваний: первичный ангиит центральной нервной системы (ЦНС), изолированный васкулит ЦНС (ангиит) [12,13,[16][17][18]. Несмотря на то, что в последнее десятилетие внимание к данной нозологической форме возросло, она по-прежнему остается редким и малоизученным заболеванием.…”

Section: Introductionunclassified

Modern Concepts on the Clinic and Diagnosis of Primary Vasculitis of the Central Nervous System

Фомина

Yakovlev

Utkina

2023

Arhivʺ vnutrennej mediciny

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Primary vasculitis of the central nervous system (PACNS) is a rare form of unknown cause vasculitis that affects the vessels of the brain, spinal cord and meninges without systemic damage. It was found that the average age of the onset of the disease was 50 years. Clinical manifestations depend on the caliber of the affected vessels. The most common initial symptoms are headache and vascular cognitive impairment associated with small vessel involvement. The development of stroke and focal symptoms is interrelated with vascular cognitive impairment and manifests as the lesion of the middle/large cerebral arteries. PACNS is difficult to diagnose, since symptoms of vasculitis outside the central nervous system are rare, serologic markers of inflammation are normal. The analysis of cerebrospinal fluid is usually abnormal due to a moderate nonspecific increase in the level of total protein or the number of leukocytes. Deviations from the norm (cerebral infarction, sometimes tumor-like lesions) are detected according to the data of magnetic resonance imaging of the brain in 97 % of patients with PACNS. Angiography has low sensitivity and low specificity, since it allows to verify vasculitis of only middle and large cerebral arteries, revealing segmental narrowing. To detect inflammation of the blood vessels, as well as to exclude other diseases, it is necessary to perform a biopsy of the substance and the soft membranes of the brain.

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Cortical Microhemorrhage Presentation of Small Vessel Primary Angiitis of the Central Nervous System

Guo,

Zhang,

Dong

et al. 2024

Annals of Neurology

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ObjectivePrimary angiitis of the central nervous system (PACNS) is a rare vasculitis restricted to the brain, spinal cord, and leptomeninges. This study aimed to describe the imaging characteristics of patients with small vessel PACNS (SV‐PACNS) using 7 T magnetic resonance imaging (MRI).MethodsThis ongoing prospective observational cohort study included patients who met the Calabrese and Mallek criteria and underwent 7 T MRI scan. The MRI protocol includes T1‐weighted magnetization‐prepared rapid gradient echo imaging, T2 star weighted imaging, and susceptibility‐weighted imaging. Two experienced readers independently reviewed the neuroimages. Clinical data were extracted from the electronic patient records. The findings were then applied to a cohort of patients with large vessel central nervous system (CNS) vasculitis.ResultsWe included 21 patients with SV‐PACNS from December 2021 to November 2023. Of these, 12 (57.14%) had cerebral cortical microhemorrhages with atrophy. The pattern with microhemorrhages was described in detail based on the gradient echo sequence, leading to the identification of what we have termed the “coral‐like sign.” The onset age of patients with coral‐like sign (33.83 ± 9.93 years) appeared younger than that of patients without coral‐like sign (42.11 ± 14.18 years) (P = 0.131). Furthermore, the cerebral lesions in patients with cortical microhemorrhagic SV‐PACNS showed greater propensity toward bilateral lesions (P = 0.03). The coral‐like sign was not observed in patients with large vessel CNS vasculitis.InterpretationThe key characteristics of the coral‐like sign represent cerebral cortical diffuse microhemorrhages with atrophy, which may be an important MRI pattern of SV‐PACNS. ANN NEUROL 2024

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Primary angiitis of the central nervous system – A challenging diagnosis

Cited by 9 publications

References 103 publications

Right arm weakness and mouth deviation as a presentation of Primary Angiitis of the Central Nervous System treated with rituximab: A case-report

Right arm weakness and mouth deviation as a presentation of Primary Angiitis of the Central Nervous System treated with rituximab: A case-report

Modern Concepts on the Clinic and Diagnosis of Primary Vasculitis of the Central Nervous System

Cortical Microhemorrhage Presentation of Small Vessel Primary Angiitis of the Central Nervous System

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