Radial contrast enhancement on brain magnetic resonance imaging could be diagnostic of primary angiitis of the central nervous system: a case report and review of the literature

Abstract

IntroductionPrimary angiitis of the central nervous system is a rare disease of unclear etiology. There is no single test diagnostic of primary angiitis of the central nervous system. We report an unusual pattern on brain magnetic resonance imaging that might be specific for primary angiitis of the central nervous system.Case presentationA 47-year-old Caucasian man developed progressive bilateral hand tremor, difficulty walking, cognitive slowing and headache. A physical examination showed bilateral hand tremor with dysmetria, hyperreflexia and abnormal gait. Magnetic resonance imaging of his brain showed bilateral, symmetrical, increased intensity on T2-weighted images concurrent with linear contrast enhancement in a radial distribution throughout his white matter, sparing subcortical regions in his centrum semiovale, corona radiata, basal ganglia and brainstem. Magnetic resonance spectroscopy demonstrated elevated choline and decreased N-acetyl aspartate. Except for elevated protein and lymphocytic pleocytosis, examination of his cerebrospinal fluid showed no abnormalities. Serological tests for rheumatologic, vasculitic, paraneoplastic, infectious and peroxisomal disorders were negative. A brain biopsy revealed primary angiitis of the central nervous system. Our patient was treated with steroids and intravenous cyclophosphamide, with improvement in signs and symptoms as well as changes on magnetic resonance imaging.ConclusionBilateral, symmetrical, increased intensity on T2-weighted images concurrent with linear contrast enhancement in a radial distribution throughout the white matter on magnetic resonance imaging of the brain should be recognized as a feature of primary angiitis of the central nervous system, and might avoid the need for a brain biopsy to diagnose primary angiitis of the central nervous system.

Glial fibrillary acidic protein immunoglobulin G as biomarker of autoimmune astrocytopathy: Analysis of 102 patients

“…A radiological hallmark of autoimmune GFAP astrocytopathy is the striking radial linear periventricular enhancement. A similar radiological pattern has been reported in patients with lymphomatoid granulomatosis, neurosarcoidosis, and CNS vasculitis, including those lacking cerebral infarcts diagnosed with “angiogram negative primary central nervous system vasculitis” . This “microvascular vasculitis” subtype is known to be steroid responsive, and has a better prognosis than vasculitis in general .…”

“…[7][8][9][10][11] This "microvascular vasculitis" subtype is known to be steroid responsive, and has a better prognosis than vasculitis in general. A similar radiological pattern has been reported in patients with lymphomatoid granulomatosis, neurosarcoidosis, and CNS vasculitis, including those lacking cerebral infarcts diagnosed with "angiogram negative primary central nervous system vasculitis".…”

“…A similar radiological pattern has been reported in patients with lymphomatoid granulomatosis, neurosarcoidosis, and CNS vasculitis, including those lacking cerebral infarcts diagnosed with "angiogram negative primary central nervous system vasculitis". [7][8][9][10][11] This "microvascular vasculitis" subtype is known to be steroid responsive, and has a better prognosis than vasculitis in general. 12 The authors speculate that some reported cases of microvascular vasculitis, particularly those accompanied by optic disk edema and radial periventricular enhancement, had autoimmune GFAP astrocytopathy.…”

MRI differential diagnosis of suspected multiple sclerosis

“…29e31 The pattern of enhancement is typically described as linear or in a radial fashion as opposed to ring-like configuration as seen in MS. 32 Areas of ischaemic or haemorrhagic infarct may also be seen.…”

Primary Angiitis of the Central Nervous System

“…This situation is not uncommon. In the Duke Box 1 MRI findings in histologically proven PACNS Normal 25,37 Progressive confluent white matter lesions [66][67][68][69][70][71][72][73][74] Cortical and subcortical T2 lesions [75][76][77] Multiple diffusion-positive lesions 78,79 Large intraparenchymal hematoma 80 Multiple microhemorrhages 66,72,81 Multiple small enhancing lesions 71,78,82 Large single and multiple enhancing mass lesions 26,50,[83][84][85][86] Enhancing small-vessel walls/small perivascular spaces 69,70,87,88 Leptomeningeal enhancement 14,77 Powers series, 5 of 30 biopsies were nondiagnostic. 11 In the Johns Hopkins series, 35 of 41 and in the University of Michigan series, 15 of 61 had a nondiagnostic biopsy.…”