Arterial Spin Labeling Applications in Pediatric and Adult Neurologic Disorders.

Arterial spin labeling (ASL) is a powerful noncontrast magnetic resonance imaging (MRI) technique that enables quantitative evaluation of brain perfusion. To optimize the clinical and research utilization of ASL, radiologists and physicists must understand the technical considerations and age-related variations in normal and disease states. We discuss advanced applications of ASL across the lifespan, with example cases from children and adults covering a wide variety of pathologies. Through literature review and illustrated clinical cases, we highlight the subtleties as well as pitfalls of ASL interpretation. First, we review basic physical principles, techniques, and artifacts. This is followed by a discussion of normal perfusion variants based on age and physiology. The three major categories of perfusion abnormalities-hypoperfusion, hyperperfusion, and mixed patterns-are covered with an emphasis on clinical interpretation and relationship to the disease process. Major etiologies of hypoperfusion include large artery, small artery, and venous disease; other vascular conditions; global hypoxic-ischemic injury; and neurodegeneration. Hyperperfusion is characteristic of vascular malformations and tumors. Mixed perfusion patterns can be seen with epilepsy, migraine, trauma, infection/inflammation, and toxic-metabolic encephalopathy. LEVEL OF EVIDENCE: 4 TECHNICAL EFFICACY STAGE: 3.

Clinical and Radiologic Features, Pathology, and Treatment of Baló Concentric Sclerosis.

OBJECTIVE: To describe clinical, radiologic, and pathologic features of Baló concentric sclerosis (BCS) and assess overlap between BCS and other CNS inflammatory demyelinating diseases. METHODS: Retrospective review of BCS cases from US and Australian tertiary care centers. RESULTS: We identified 40 BCS cases with 38 available MRIs. Solitary MRI lesions were present in 26% (10/38). We saw >1 active concurrent BCS lesion in 45% (17/38). A third (13/38) had multiple sclerosis-suggestive lesions on the index MRI, of which 10 fulfilled Barkhof criteria. In patients with serial MRI performed within 1 month of the index MRI, lesions expanded radially with sequentially increased numbers of T2 hyperintense rings 52% (14/27). Initially nonenhancing or centrally enhancing lesions subsequently developed single or multiple enhancing rings (41%; 9/22) and incomplete enhancing rings (14%; 3/22). Discordance between rings as they appear on apparent diffusion coefficient, diffusion-weighted imaging, and gadolinium-enhanced imaging was observed in 67% (22/33). Aquaporin-4 immunoglobulin G (n = 26) and myelin oligodendrocyte glycoprotein immunoglobulin G (n = 21) were negative in all patients with serum available. Clinical response to steroid treatment was seen in 46% (13/28). A monophasic clinical course was present in 56% (18/32) at last follow-up (median 27.5 months; range 3-100 months). The initial attack was fatal in 10% (4/40). Median time from symptom onset to death was 23 days (range 19-49 days). All 17 patients with pathology available demonstrated typical findings of multiple sclerosis. Patients with active demyelinating lesions all demonstrated oligodendrocytopathy (pattern III). CONCLUSIONS: BCS may be a distinct subtype of multiple sclerosis characterized by pattern III immunopathology.

Diagnostic Yield of Lateral Decubitus Digital Subtraction Myelogram Stratified by Brain MRI Findings.

OBJECTIVE: To assess the diagnostic yield of lateral decubitus digital subtraction myelography (LDDSM) and stratify LDDSM diagnostic yield by the Bern spontaneous intracranial hypotension (SIH) score of preprocedure brain MRI. METHODS: This retrospective diagnostic study included consecutive adult patients investigated for SIH who underwent LDDSM. Patients without preprocedure brain and spine MRI and patients with extradural fluid collection on spine MRI (type 1 leak) were excluded. LDDSM images and brain MRIs were assessed by 2 independent blinded readers; a third reader adjudicated any discrepancies. Diagnostic yield of LDDSM was assessed, both overall and stratified by Bern SIH scoring. RESULTS: Of the 62 patients included in this study, 33 (53.2%) had a CSF leak identified on LDDSM. Right-sided leaks were more common (70.6%), and the most commonly identified levels of leaks were at T6, T7, and T10. No leak was found in any of the 9 patients with Bern SIH score of 2 or less. Of the 11 patients with Bern SIH score of 3-4, 5 (45.5%) had a CSF leak identified; of the 42 patients with Bern SIH score of 5 or higher, 28 (66.7%) had a CSF leak identified. CONCLUSIONS: LDDSM has a high diagnostic yield for finding the exact location of spinal CSF leak, and the diagnostic yield increases with higher Bern SIH score. No leaks were found in patients with Bern SIH score of 2 or less, suggesting that foregoing invasive testing such as LDDSM in these patients may be appropriate unless accompanied by high clinical suspicion. CLASSIFICATION OF EVIDENCE: This study provides Class II evidence that for patients with suspected SIH, higher Bern SIH scores are associated with a greater likelihood of LDDSM-identified CSF leaks.

The frequency of longitudinally extensive transverse myelitis in MS: A population-based study.

BACKGROUND: Determining the frequency of longitudinally-extensive transverse myelitis (LETM: T2-lesion ≥3 vertebral segments) in multiple sclerosis (MS) is essential to assess its utility in differentiating from aquaporin-4-IgG (AQP4-IgG) positive neuromyelitis optica spectrum disorder (NMOSD) and myelin-oligodendrocyte-glycoprotein-IgG (MOG-IgG) myelitis. We sought to determine the frequency of LETM in MS during a myelitis attack. METHODS: We identified Olmsted County (MN, USA) residents on 12/31/2011 with inflammatory demyelinating disease. Inclusion criteria were: 1) Clinical myelitis episode accompanied by a new spinal magnetic resonance imaging (MRI) lesion (≤6 weeks from onset); 2) MS diagnosis by 2010 McDonald criteria; 3) Seronegative for AQP4-IgG and MOG-IgG. MRI characteristics were determined. RESULTS: Sixty-seven patients (median age at myelitis: 41 years [range, 16-65]; 76% females) with 92 myelitis attacks accompanied by a new MRI spinal cord lesion were identified. The frequency of LETM was 0%. The median T2-hyperintense lesion length in vertebral segments was 1.0 (range, 0.5-2.5) and 82/92 (89%) were peripheral in location on axial sequences; 58% had associated gadolinium enhancement. Two patients (2% of attacks) had multiple short lesions resembling LETM on sagittal images but axial sequences confirmed multiple non-contiguous short lesions. CONCLUSION: LETM is rare in adult MS myelitis and its presence should prompt evaluation for AQP4-IgG, MOG-IgG or other etiologies. Careful scrutiny of axial images is important as coalescence of multiple short lesions may lead to the artifactual appearance of an LETM.

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

OBJECTIVE: A novel autoimmune central nervous system (CNS) disorder with glial fibrillary acidic protein (GFAP)-IgG as biomarker was recently characterized. Here, 102 patients with GFAP-IgG positivity are described. METHODS: The 102 included patients had: (1) serum, cerebrospinal fluid (CSF), or both that yielded a characteristic astrocytic pattern of mouse tissue immunostaining; (2) confirmation of IgG reactive with specific GFAP isoforms (α, ɛ, or κ) by cell-based assays; and (3) clinical data available. Control specimens (n = 865) were evaluated by tissue (n = 542) and cell-based (n = 323) assays. RESULTS: Median symptom onset age was 44 years (range = 8-103), and 54% were women. The predominant phenotype (83 patients; 81%) was inflammation of meninges, brain, spinal cord, or all 3 (meningoencephalomyelitis). Among patients, highest specificity for those phenotypes was observed for CSF testing (94%), and highest sensitivity was for the GFAPα isoform (100%). Rare GFAP-IgG positivity was encountered in serum controls by tissue-based assay (0.5%) or cell-based assay (1.5%), and in CSF controls by cell-based assay (0.9%). Among patients, striking perivascular radial enhancement was found on brain magnetic resonance imaging in 53%. Although cases frequently mimicked vasculitis, angiography was uniformly negative, and spinal imaging frequently demonstrated longitudinally extensive myelitic lesions. Diverse neoplasms encountered were found prospectively in 22%. Ovarian teratoma was most common and was predicted best when both N-methyl-D-aspartate receptor-IgG and aquaporin-4-IgG coexisted (71%). Six patients with prolonged follow-up had brisk corticosteroid response, but required additional immunosuppression to overcome steroid dependency. INTERPRETATION: GFAPα-IgG, when detected in CSF, is highly specific for an immunotherapy-responsive autoimmune CNS disorder, sometimes with paraneoplastic cause. Ann Neurol 2017;81:298-309.

Superficial temporal vein: route to embolization of cavernous dural arteriovenous fistula.

Superficial temporal vein catheterization was used to embolize a dural arteriovenous malformation of the cavernous sinus in a 44-year-old woman. The pertinent venous anatomy is discussed. This route may provide crucial access to the cavernous sinus when conventional approaches such as the Inferior petrosal sinus catheterization are difficult.

Dural arteriovenous fistula: diagnosis, treatment, and outcomes.

OBJECTIVES: The objective of this study is to determine the sensitivity of computed tomography angiography (CTA) and magnetic resonance angiography (MRA) compared to the gold standard, conventional carotid angiography, in the diagnosis of a dural arteriovenous fistula (DAVF). Further objectives include identifying outcomes of treatment and complications as a function of pretreatment radiologic classification. STUDY DESIGN: This is a retrospective review of adults diagnosed with a DAVF between 1990 and 2006. METHODS: Data collected included age, gender, presenting clinical symptoms, diagnostic imaging modalities (conventional angiography, CTA, and/or MRA), interventions (self-compression of artery, embolization, craniotomy, or a combination of these), results (symptoms resolved, improved, or same), and complications (embolic or surgical). All DAVFs were classified according to the Cognard classification. RESULTS: There were a total of 46 patients with 51 fistulas, of whom 42 patients (46 fistulas) had complete follow-up data. Using conventional angiography as the gold standard, CTA had a sensitivity of 15.4%, whereas MRA had a sensitivity of 50%. Embolization (either single or multiple treatments) was the most common treatment, being done in 36/46 (78%) fistulas. Presenting symptoms were resolved or improved in 45/46 fistulas (97.8%) regardless of treatment or Cognard classification. There were complications in 12/46 (26%) patients, most of which were transient and resolved without permanent sequelae. CONCLUSIONS: A DAVF is a vascular lesion that is best diagnosed with conventional angiography, but can often be found with MRA. Treatment with endovascular embolization is effective and has few significant complications.

Unique anomalous origin of the left anterior cerebral artery.

We present the case of an anomalous origin of the left anterior cerebral artery (ACA) from the supraclinoid segment of the right internal carotid artery. Because of improved imaging quality, anomalies of the ACA-anterior communicating artery (AComA) complex are increasingly recognized on transaxial images. Although most of these anomalies are incidental findings of little clinical significance, some ACA-AComA complex anomalies are clinically significant. Recognition of these anomalies may be instrumental in developing a differential diagnosis or for improved surgical planning.

Cerebral vasculitis in graft-versus-host disease: a case report.

We describe a case of cerebral vasculitis seen on angiography in a patient with a history of non-Hodgkin lymphoma and graft-versus-host disease (GVHD) 2 years after match-related bone-marrow transplant. The vasculitis was ascribed to GVHD as other causes were excluded by laboratory tests. Other recent reports of GVHD-related vasculitis have been published, but none with angiographic changes.

Fluoroscopy fade for embolization of vein of Galen malformation.

A fluoroscopy fade technique in the embolization of a vein of Galen malformation in a 10-week-old infant is discussed herein. The availability of the fluoroscopy fade function eliminated the necessity of road mapping during the procedure and permitted minimal usage of contrast material and minimal radiation exposure for the patient. Techniques used during the embolization and the benefits of the fluoroscopy fade feature are also described.

Recognition and importance of an infraoptic anterior cerebral artery: case report.

Although variations of the anterior cerebral artery (ACA)-anterior communicating artery complex are commonly identified on imaging studies, an infraoptic course of the ACA is exceedingly rare. What is believed to be the first case of an infraoptic course of the ACA discovered with MR angiography and further characterized with conventional angiography is presented. The high prevalence of associated aneurysms and the implications for surgical planning make preoperative recognition of this anomaly critical.