Primary angiitis of the central nervous system: serial MRI of brain and spinal cord

Abstract

MRI findings in primary angiitis of the central nervous system (PACNS) are highly variable, ranging from normal to diffusely abnormal. We describe brain and spinal cord abnormalities in patients with PACNS and changes over time, to provide criteria which could be useful for differential diagnosis. We reviewed six patients, with a final diagnosis of PACNS, who underwent serial contrast-enhanced brain and spinal MRI. Follow-up ranged from 12 to 60 months. Brain MRI showed multiple small abnormalities in all patients, giving high signal on T2-weighted images, focal or diffuse, mainly in deep and subcortical white matter; four patients had both supra- and infratentorial lesions. On the initial MRI, in five patients, almost 90% of the abnormal foci showed contrast enhancement. Virchow-Robin perivascular spaces were enlarged and simultaneously enhancing in four patients. Three patients also had spinal cord abnormalities, in the cervical and thoracic segments in two, and exclusively cervical segment in one. Two patients had brain biopsy-proven PACNS; in the remainder, the diagnosis of PACNS was presumptive, considering similarities in clinical and MRI features and MRI follow-up. On MRI, after steroid and immunosuppressive therapy, a significant decrease in the number and size of the abnormalities, enhancing and nonenhancing and of enhancing perivascular spaces was observed. Simultaneous enhancement of brain and spinal cord lesions and of perivascular spaces, at the onset of the disease, which resolves during follow-up, can therefore suggest PACNS.

Evaluation of Hyperintense Vessels on FLAIR MRI for the Diagnosis of Multiple Intracerebral Arterial Stenoses

“…5,6 The diagnosis of intracerebral arterial stenosis can be clinically challenging because the initial presentation is highly variable, with focal to diffuse neurological manifestations and acute to chronic evolution. [5][6][7] Digital subtraction angiography (DSA) remains the keystone diagnostic technique for delineating proximal or distal segmental stenoses of intracranial arteries. Yet, in practice, MRI precedes angiographic studies in the evaluation of these patients.…”

Leptomeningeal Enhancement As A Sole Magnetic Resonance Imaging Finding of Secondary Central Nervous System Vasculitis: A Case Report

“…In cerebral vasculitis, leptomeningeal enhancement can be detected together with other findings such as multiple infarcts of different ages in various vascular territories, parenchymal lesions, and petechial hemorrhages, nonspecific areas of increased signal intensity on FLAIR or T2- weighted image. Direct signs of vessel wall changes like thickened wall, intramural contrast may also be detected by DSA, MRA and MRI [5,6]. Occasionally, enhancement may be marked and extend into the adjacent leptomeningeal tissue referred as perivascular enhancement.…”

“…Contrast-enhanced magnetic resonance imaging (MRI), magnetic resonance angiography (MRA) and digital subtraction angiography (DSA) are the modality of choice used to detect and monitor cerebral involvement. The main findings consist of multiple subcortical infarcts, parenchymal and leptomeningeal enhancement, petechial hemorrhages on MRI and multifocal caliber changes of the vessels on MRA _____________________________________________________________________________________________________________________________ and DSA [5,6]. Leptomeningeal enhancement is a rare finding of cerebral vasculitis.…”

Pseudotumoral presentation of primary central nervous system vasculitis

“…Diff use white matter lesions and microangiopathy are commonly observed 2,6,9 . Despite a positive biopsy, DSA can be normal if small brain vessels are aff ected 4,9 . On the other hand, a negative biopsy cannot exclude vasculitis 4 .…”