Imaging Spectrum of CNS Vasculitis

Razek, Ahmed Abdel Khalek Abdel; Álvarez, H.; Bagg, Stephen; Refaat, Sherif; Castillo, Maurício

doi:10.1148/rg.344135028

Cited by 200 publications

(87 citation statements)

References 73 publications

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“…Severe neurological symptoms are reported in approximately 29% of patients and include headache, psychosis, coma, convulsion, neuropathy, and stroke. Cerebral vasculitis has been demonstrated in 12–15% of CS/ACS patients [15, 16]. Conventional imaging may show areas of ischemic change or infarction, meningoencephalitis, cerebral venous sinus thrombosis, and cranial neuropathy.…”

Section: Discussionmentioning

confidence: 99%

Altered Brain Glucose Consumption in Cogan’s Syndrome

Mora

Ruffini

Ghetti

et al. 2016

Journal of Ophthalmology

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Purpose. Prospective, controlled cohort study to investigate possible alterations in brain glucose metabolism (CMRglc) in patients with Cogan's syndrome (CS). Patients and Methods. Functional mapping of the CMRglc was obtained by quantitative molecular imaging positron emission tomography, combined with computed tomography (FDG-PET/CT). The patients were divided into three clinical groups: typical CS; atypical CS (ACS); autoimmune inner ear disease (AIED). The unmatched control group (CG) consisted of subjects requiring FDG-PET/CT for an extracranial pathology. Statistical mapping searched areas of significant glucose hypometabolism in all the affected patients (DG) and in each clinical subgroup. The results were compared with those of the CG. Results. 44 patients were enrolled (DG) and assigned to the three study groups: 8 patients to the CS group; 21 patients to the ACS group; and 15 to the AIED group. Sixteen subjects formed the CG group. Areas of significant brain glucose hypometabolism were identified in all the study groups, with the largest number and extension in the DG and CS. Conclusions. This study revealed areas of significantly altered CMRglc in patients with CS (any subform) without neurologic complains and normal conventional neuroimaging. Our results suggest that FDG-PET/CT may represent a very useful tool for the global assessment of patients with Cogan's syndrome.

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Section: Discussionmentioning

confidence: 99%

Altered Brain Glucose Consumption in Cogan’s Syndrome

Mora

Ruffini

Ghetti

et al. 2016

Journal of Ophthalmology

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“…The two CNS vasculitis described with brain miliary enhancement at MRI are primary angiitis of the central nervous system (PACNS) and secondary CNS vasculitis due to chronic graft versus host disease [26,27].…”

Section: Primary and Secondary Cns Vasculitismentioning

confidence: 99%

“…PACNS, also known as granulomatous angiitis or isolated cerebral vasculitis, leads to a wide spectrum of clinical manifestations including diffuse or focal throbbing headaches, focal or generalized seizures, encephalopathy, brainstem events, stroke-like episodes, aphasia, myelopathy and diffuse neurological dysfunction [26]. Although vessels of any size may be involved, small-and medium-sized ones are most commonly targeted [28].…”

Section: Primary and Secondary Cns Vasculitismentioning

confidence: 99%

“…Although vessels of any size may be involved, small-and medium-sized ones are most commonly targeted [28]. Males are affected twice as often as females and the onset usually occurs after 40 years of age [26]. Leptomeningeal biopsy can provide a definitive diagnosis, but this procedure is not routinely performed also in light of the fact that a negative biopsy does not rule out the condition [26].…”

Section: Primary and Secondary Cns Vasculitismentioning

confidence: 99%

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Brain miliary enhancement

et al. 2020

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Purpose Miliary enhancement refers to the presence of multiple small, monomorphic, enhancing foci on T1-weighted postcontrast MRI images. In the absence of a clear clinical presentation, a broad differential diagnosis may result in invasive procedures and possibly brain biopsy for diagnostic purposes. Methods An extensive review of the literature is provided for diseases that may present with miliary enhancement on T1weighted brain MR images. Additional disease-specific findings, both clinical and radiological, are summarized and categorized by the presence or absence of perivascular space involvement. Results Miliary pattern of enhancement may be due to a variety of underlying causes, including inflammatory, infectious, nutritional or neoplastic processes. The recognition of disease spread along the perivascular spaces in addition to the detection or exclusion of disease-specific features on MRI images, such as leptomeningeal enhancement, presence of haemorrhagic lesions, spinal cord involvement and specific localisation or systemic involvement, allows to narrow the potential differential diagnoses. Conclusion A systematic approach to disease-specific findings from both clinical and radiological perspectives might facilitate diagnostic work-up, and recognition of disease spread along the perivascular spaces may help narrowing down differential diagnoses and may help to minimize the use of invasive diagnostic procedures.

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“…Amphetamine induced vasculitis, also known as reversible cerebral vasoconstriction syndrome, is commonly associated with convexity subarachnoid haemorrhage, focal intracerebral haemorrhage, cerebral infarction, and reversible cerebral oedema 2. The exact pathogenesis is unknown, but it has been identified in both first-time and long-standing users of amphetamines and is suspected to be related to vascular changes induced by sudden and drastic catecholamine release 3.…”

Section: Descriptionmentioning

confidence: 99%