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Cerebral amyloid angiopathy (CAA) is a progressive neurodegenerative small vessel disease that is associated with intracranial hemorrhage and cognitive impairment in the elderly. The clinical and radiographic presentations have many overlapping features with vascular cognitive impairment, hemorrhagic stroke, and Alzheimer disease (AD). Amyloid-β-related angiitis (ABRA) is a form of primary CNS vasculitis linked to CAA, with the development of spontaneous autoimmune inflammation against amyloid in the vessel wall with resultant vasculitis. The diagnosis of ABRA and CAA is important. ABRA is often fatal if untreated and requires prompt immunosuppression. Important medical therapies such as anticoagulation and antiamyloid agents for AD are contraindicated in CAA. Here, we present a biopsy-proved case of ABRA with underlying occult CAA. Initial 1.5T and 3T MR imaging did not suggest CAA per the Boston Criteria 2.0. ABRA was not included in the differential diagnosis due to the lack of any CAA-related findings on conventional MR imaging. However, a follow-up 7T MR imaging revealed extensive cortical/subcortical cerebral microbleeds, cortical superficial siderosis, and intragyral hemorrhage in extensive detail throughout the supratentorial brain regions, which radiologically supported the diagnosis of ABRA in the setting of CAA. This case suggests an increased utility of high-field MR imaging to detect occult hemorrhagic neuroimaging findings with the potential to both diagnose more patients with CAA and diagnose them earlier., (© 2024 by American Journal of Neuroradiology.)

Primary central nervous system vasculitis (PCNSV) is a poorly understood neuroinflammatory disease of the CNS affecting the intracranial vasculature. Although PCNSV classically manifests as a multifocal beaded narrowing of the intracranial vessels, some patients may not have angiographic abnormalities. A rare subset of patients with PCNSV present with masslike brain lesions mimicking a neoplasm. In this article, we retrospectively review 10 biopsy-confirmed cases of tumefactive PCNSV (t-PCNSV). All cases of t-PCNSV in our series that underwent CTA or MRA were found to have normal large and medium-sized vessels. T-PCNSV had a variable MR imaging appearance with most cases showing cortical/subcortical enhancing masslike lesion (70%), often with microhemorrhages (80%). Diffusion restriction was absent in all lesions. In summary, normal vascular imaging does not exclude the diagnosis of t-PCNSV. Advanced imaging techniques including MR perfusion and MR spectroscopy failed to demonstrate specific findings for t-PCNSV but assisted in excluding neoplasm in the differential diagnosis. Biopsy remains mandatory for definitive diagnosis., (© 2020 by American Journal of Neuroradiology.)

Coronavirus disease 2019 (COVID-19) is a viral infection caused by the Severe Acute Respiratory Syndrome coronavirus 2 (SARS-CoV-2), which spreads rapidly from person to person and manifests in most symptomatic patients as a respiratory illness, similar to prior SARS viruses. Neurologic manifestations of COVID-19 are uncommon; those so far reported include encephalopathy, stroke from large-vessel occlusion, and polyneuropathy. We report a unique neurologic complication of COVID-19 in a patient who had extensive cerebral small-vessel ischemic lesions resembling cerebral vasculitis in a characteristic combined imaging pattern of ischemia, hemorrhage, and punctuate postcontrast enhancement. Also, a characteristic lower extremity skin rash was present in our patient. Our observation lends support to the increasingly suspected mechanism of "endotheliitis" associated with this novel coronavirus., (© 2020 by American Journal of Neuroradiology.)

Central nervous system vasculitides are elusive diseases that are challenging to diagnose because brain biopsies have high false-negative rates. We sought to test the ability of contrast-enhanced, high-resolution 3D vessel wall MR imaging to identify vascular inflammation and direct open biopsies of intracranial target vessels and adjacent brain parenchyma. Eight of 9 specimens revealed vascular inflammation. We conclude that vessel wall MR imaging can identify inflamed intracranial vessels, enabling precise localization of biopsy targets., (© 2018 by American Journal of Neuroradiology.)

Background and Purpose: 3D-TOF-MRA and DSA are 2 available tools to demonstrate neurovascular involvement in primary central nervous system vasculitis. We aimed to compare the diagnostic concordance of vessel imaging using 3D-TOF-MRA and DSA in patients with primary central nervous system vasculitis., Materials and Methods: We retrospectively identified all patients included in the French primary central nervous system vasculitis cohort of 85 patients who underwent, at baseline, both intracranial 3D-TOF-MRA and DSA in an interval of no more than 2 weeks and before treatment initiation. Two neuroradiologists independently reviewed all 3D-TOF-MRA and DSA imaging. Brain vasculature was divided into 25 arterial segments. Concordance between 3D-TOF-MRA and DSA for the identification of arterial stenosis was assessed by the Cohen κ Index., Results: Thirty-one patients met the inclusion criteria, including 20 imaged with a 1.5T MR unit and 11 with a 3T MR unit. Among the 25 patients (81%) with abnormal DSA findings, 24 demonstrated abnormal 3D-TOF-MRA findings, whereas all 6 remaining patients with normal DSA findings had normal 3D-TOF-MRA findings. In the per-segment analysis, concordance between 1.5T 3D-TOF-MRA and DSA was 0.82 (95% CI, 0.75-0.93), and between 3T 3D-TOF-MRA and DSA, it was 0.87 (95% CI, 0.78-0.91)., Conclusions: 3D-TOF-MRA shows a high concordance with DSA in diagnostic performance when analyzing brain vasculature in patients with primary central nervous system vasculitis. In patients with negative 3T 3D-TOF-MRA findings, the added diagnostic value of DSA is limited., (© 2017 by American Journal of Neuroradiology.)

Background and Purpose: The differential diagnosis of acute cervical pain includes nonvascular and vascular causes such as carotid dissection, carotid occlusion, or vasculitis. However, some patients present with unclassified vascular and perivascular changes on imaging previously reported as carotidynia. The aim of our study was to improve the description of this as yet unclassified clinico-radiologic entity., Materials and Methods: From January 2009 through April 2016, 47 patients from 10 centers presenting with acute neck pain or tenderness and at least 1 cervical image showing unclassified carotid abnormalities were included. We conducted a systematic, retrospective study of their medical charts and diagnostic and follow-up imaging. Two neuroradiologists independently analyzed the blinded image datasets., Results: The median patient age was 48 years. All patients presented with acute neck pain, and 8 presented with transient neurologic symptoms. Imaging showed an eccentric pericarotidian infiltration in all patients. An intimal soft plaque was noted in 16 patients, and a mild luminal narrowing was noted in 16 patients. Interreader reproducibility was excellent. All patients had complete pain resolution within a median of 13 days. At 3-month follow-up, imaging showed complete disappearance of vascular abnormalities in 8 patients, and a marked decrease in all others., Conclusions: Our study improved the description of an unclassified, clinico-radiologic entity, which could be described by the proposed acronym: TransIent Perivascular Inflammation of the Carotid artery (TIPIC) syndrome., (© 2017 by American Journal of Neuroradiology.)

Background and Purpose: Takayasu arteritis is a rare, large-vessel vasculitis that presents with symptoms related to end-organ ischemia. While the extracranial neurovascular manifestations of Takayasu arteritis are well-established, little is known regarding the intracranial manifestations. In this study, we characterize the intracranial and cervical neurovascular radiologic findings in patients with Takayasu arteritis., Materials and Methods: Patients with Takayasu arteritis who presented to our institution between 2001 and 2016 with intracranial and/or cervical vascular imaging were included in this study. Images were evaluated for the presence of vascular abnormalities, including intracranial or extracranial stenosis, vessel-wall thickening, dissection, subclavian steal, aneurysms, infarcts, and hemorrhages. Descriptive analyses are reported., Results: Seventy-nine patients with Takayasu arteritis met the criteria for inclusion in this study. The most common presenting neurologic symptoms were headache (32.9%) and dizziness (15.2%). Intracranial and extracranial vascular imaging was performed in 84.8% and 89.9% of patients, respectively. Among patients with intracranial vascular imaging, 3 (3.9%) had intracranial aneurysms, 3 (3.9%) had acute large-vessel occlusion, 6 (7.6%) had intracranial vasculitis, and 1 (1.3%) had reversible cerebrovascular constriction syndrome. Among patients with cervical vascular imaging, 42 (53.1%) had some degree of narrowing of the common carotid artery and 18 (22.8%) had narrowing of the ICAs. Seventeen patients (23.6%) had subclavian steal., Conclusions: Intracranial vascular abnormalities in patients with Takayasu arteritis presenting with neurologic symptoms are not rare, with cerebral vasculitis seen in 7.8% of patients, and stroke secondary to large-vessel occlusion, in 3.9% of patients. Cervical vascular manifestations of Takayasu arteritis were present in most patients in our study., (© 2017 by American Journal of Neuroradiology.)

Brucellosis is a zoonotic disease characterized by multisystem involvement. Nervous system involvement is rare, with a reported incidence of 3%-13%. Brucellosis can also be manifested in the form of vasculopathy. Cerebral vasculopathy due to brucellosis is a very rare entity, with only a few cases reported in the literature. We present a patient with neurobrucellosis who had involvement of cerebral vasculature demonstrated by angiography.

Cerebral vasculitis presenting with intracranial hemorrhage is a rare but known entity. We discuss here the case of a 61-year-old woman presenting with subarachnoid hemorrhage. Cerebral angiography showed vasculitic changes involving the small and medium-sized vessels. She also had a concomitant herpes zoster rash on her back. A 3-month follow-up angiogram revealed partial resolution of the vasculitic changes.