Arterial Spin Labeling: Techniques, Clinical Applications, and Interpretation.

Arterial spin labeling (ASL) is an emerging noninvasive MRI technique for assessing cerebral perfusion. An important advantage of ASL perfusion is the lack of a requirement for an exogenous tracer. ASL uses magnetically labeled water protons from arterial blood as an endogenous diffusible tracer. For this reason, ASL is an attractive perfusion imaging modality for children and for patients with contraindications or adverse reactions to gadolinium, patients with renal failure, and those who need repeated follow-up imaging. Another advantage of ASL is the possibility of quantifying cerebral blood flow, which provides an opportunity for comparative analysis among multiple longitudinal studies, unlike other MR perfusion techniques, which are semiquantitative and yield relative perfusion parameters. Advances in MRI technology and pulse sequence design have translated ASL beyond the research arena to successful clinical implementation. However, ASL is still underused in routine clinical practice. Some disadvantages of ASL include a lower signal-to-noise ratio and a longer acquisition time than those with dynamic susceptibility contrast-enhanced MRI. Additional factors limiting the use of ASL include variations in existing techniques and pulse sequence design, the complexity of implementation and postprocessing, insufficient experience with and/or knowledge of the potential clinical applications, and the absence of interpretation guidelines. The authors review the technical and physiologic basis of ASL perfusion, as well as artifacts, pitfalls, and its current clinical applications. A practical approach for interpreting ASL findings is also suggested.

"Bunch of acai berries sign": a new radiological sign in patients with CNS involvement in Chagas disease.

Chagas disease is an infection caused by Trypanosoma cruzi, a parasite endemic in Latin America. Acute involvement of the CNS by Chagas has been considered rare, but presumed reactivation of chronic disease in immunosuppressed patients has been the subject of recent reports. Our objective is to describe the clinical and imaging characteristics of four patients with Chagas disease and CNS involvement, and the patients had to have available MRI and a diagnosis confirmed by biopsy. The imaging findings were similar, highlighting the presence of focal cerebral lesions with hypointensity on T2-WI, and these lesions assume a "bunch of acai berries appearance", a fruit involved in the transmission of T. cruzi. The post Gd T1-WI shows punctate enhancement. Knowledge of this pattern may be crucial to recognize this disease in immunocompromised patients from endemic areas.

Corticospinal tract involvement in spinocerebellar ataxia type 3: a diffusion tensor imaging study.

PURPOSE: The aim of this study was to evaluate the integrity of the corticospinal tracts (CST) in patients with SCA3 and age- and gender-matched healthy control subjects using diffusion tensor imaging (DTI). We also looked at the clinical correlates of such diffusivity abnormalities. METHODS: We assessed 2 cohorts from different Brazilian centers: cohort 1 (n = 29) scanned in a 1.5 T magnet and cohort 2 (n = 91) scanned in a 3.0 T magnet. We used Pearson's coefficients to assess the correlation of CST DTI parameters and ataxia severity (expressed by SARA scores). RESULTS: Two different results were obtained. Cohort 1 showed no significant between-group differences in DTI parameters. Cohort 2 showed significant between-group differences in the FA values in the bilateral precentral gyri (p < 0.001), bilateral superior corona radiata (p < 0.001), bilateral posterior limb of the internal capsule (p < 0.001), bilateral cerebral peduncle (p < 0.001), and bilateral basis pontis (p < 0.001). There was moderate correlation between CST diffusivity parameters and SARA scores in cohort 2 (Pearson correlation coefficient: 0.40-0.59). CONCLUSION: DTI particularly at 3 T is able to uncover and quantify CST damage in SCA3. Moreover, CST microstructural damage may contribute with ataxia severity in the disease.

Case 278: Mutation in ROBO3 Gene-Horizontal Gaze Palsy and Progressive Scoliosis.

HistoryA 13-year-old girl was born to consanguineous parents. She presented with mild intellectual impairment, convergent strabismus, horizontal gaze palsy, and bilateral abducens palsy. Vertical gaze was preserved, and no abnormalities suggesting facial paralysis were noted. In addition, she reported progressive back pain since she was 5 years old. Other symptoms were denied. No medications or related drugs had been administered thus far. The patient underwent brain MRI for further evaluation. Current and previous spine radiographs were also reviewed.

Case 278.

HistoryA 13-year-old girl was born to consanguineous parents. She presented with mild intellectual impairment, convergent strabismus, horizontal gaze palsy, and bilateral abducens palsy. Vertical gaze was preserved, and no abnormalities suggesting facial paralysis were noted. In addition, she reported progressive back pain since she was 5 years old. Other symptoms were denied. No medications or related drugs had been administered thus far.

Neuromyelitis Optica Spectrum Disorders: Spectrum of MR Imaging Findings and Their Differential Diagnosis.

Neuromyelitis optica (NMO) is an autoimmune demyelinating disorder for which the aquaporin-4 (AQP4) water channels are the major target antigens. Advances in the understanding of NMO have clarified several points of its pathogenesis, clinical manifestations, and imaging patterns. A major advance was the discovery of the AQP4 antibody, which is highly specific for this disorder. Descriptions of new clinical and radiologic features in seropositive patients have expanded the spectrum of NMO, and the term NMO spectrum disorder (NMOSD) has been adopted. NMOSD is now included in a widening list of differential diagnoses. Acknowledgment of NMOSD imaging patterns and their mimicry of disorders has been crucial in supporting early NMOSD diagnosis, especially for unusual clinical manifestations of this demyelinating disease. This pictorial review summarizes the wide imaging spectrum of NMOSD and its differential diagnosis, as well as its historical evolution, pathophysiology, and clinical manifestations. ©RSNA, 2018.

Thrombus Features in Hyperacute Ischemic Stroke: A Perspective on Using Length and Density Evaluation.

BACKGROUND: More insights in the etiopathogenesis of thrombi could be helpful in the treatment of patients with acute ischemic stroke (AIS). One of the most confident and early imaging findings of stroke includes arterial hyperdensity. The purpose of this study was to determine whether thrombi's density and length would be useful for predicting their origin. METHODS: We evaluated 68 consecutive patients with AIS to correlate the presence of thrombi and their imaging features with the stroke subtype. RESULTS: After excluding patients with small-artery occlusion mechanism and undetermined and other causes, the stroke etiologic subtypes were large-artery atherosclerosis (LAA) in 59.0% of the patients, cardioembolism in 31.0%, and cervical artery dissection (CAD) in 10.0%. CAD more often caused thrombi with the longest length and highest attenuation, while thrombi that originated from the LAA had the smallest length and lowest attenuation. The mean Hounsfield unit (HU) values of all thrombi (with and without hyperdensity) on noncontrast computed tomography were 62.4 (50.0-70.0) in CAD, 53.8 (42.0-65.0) in cardioembolism, and 48.6 (27.0-65.0) in LAA. The length measurements were 28.5 mm (12.0-52.0) in CAD, 13.7 mm (5.0-31.0) in cardioembolism, and 10.8 mm (3.0-25.0) in the LAA subtype. The minimum cutoff value of 60 HU and a length greater than 20 mm were able to discriminate the CAD thrombi with an accuracy of 86.8% and 92.6%, respectively. CONCLUSION: Our study findings show how important thrombus analysis is in patients with AIS. Thrombus analysis can allow early suspicion of CAD before dedicated imaging of the cervical arteries is performed.

The active extravasation of contrast (spot sign) depicted on multidetector computed tomography angiography might predict structural vascular etiology and mortality in secondary intracranial hemorrhage.

OBJECTIVE: Intracerebral hemorrhage (ICH) occurs in 10% to 15% of all strokes and is accompanied by high rates of mortality, disability, and neurological sequelae. Our aim was to assess the presence and prognostic implications of the active extravasation of contrast within the hemorrhage (spot sign) in a series of patients with secondary ICH. METHODS: We analysed 59 subjects who arrived at a tertiary hospital with secondary ICH and a brain parenchyma hemorrhage greater than 2.0 cm in any axis. RESULTS: Spot sign was observed in 11 subjects, including 8 patients with saccular aneurysm, 1 with arteriovenous malformation, 1 with coagulation disorder and 1 with venous sinus thrombosis. A 37.5% mortality rate was documented in the spot sign-negative group, whereas the presence of this imaging finding was followed by an 81.8% in-hospital mortality rate. CONCLUSIONS: Spot sign was correlated with vascular etiology and was a predictor of mortality in our series of patients.

Magnetic resonance imaging of anterior temporal lobe cysts in children: discriminating special imaging features in a particular group of diseases.

INTRODUCTION: We hypothesised that disorders with anterior temporal lobe (ATL) cysts might exhibit common peculiarities and distinguishable imaging features that could be useful for diagnosis. We reviewed a series of patients for neuroimaging contributions to specific diagnoses. METHODS: A literature search was conducted, and institutional imaging files were reviewed to identify MR examinations with ATL cysts in children. Patients were divided according to head size, calcifications, white matter and cortical abnormalities. Unsupervised hierarchical clustering of patients on the basis of their MR and CT items was performed. RESULTS: We identified 23 patients in our database in whom MR revealed ATL cysts. Our series included five patients with congenital muscular dystrophy (05/23=21.7 %), six with megalencephalic leukoencephalopathy with subcortical cysts (06/23=26.1 %), three with non-megalencephalic leukoencephalopathy with subcortical cysts (03/23=13.1 %), seven with congenital cytomegalovirus disease (07/23=30.4 %) and two with Aicardi-Goutières syndrome (02/23=8.7 %). After analysis, 11 clusters resulted in the highest discriminative indices. Thereafter, patients' clusters were linked to their underlying diseases. The features that best discriminated between clusters included brainstem abnormalities, cerebral calcifications and some peculiar grey and white matter abnormalities. A flow chart was drafted to guide the radiologist in these diagnoses. CONCLUSIONS: The authors encourage the combined interpretation of these features in the herein proposed approach that confidently predicted the final diagnosis in this particular group of disorders associated with ATL cysts.

Trio-based whole exome sequencing in patients with ectopic posterior pituitary.

Introduction: Ectopic posterior pituitary (EPP) is a rare congenital abnormality, sometimes associated with other midline defects, such as pituitary stalk interruption syndrome (PSIS), in which thin or absent pituitary stalk and anterior pituitary hypoplasia are combined to EPP. Most cases are sporadic, with few reports of familial cases, and many congenital hypopituitarism (CH) cases remain unsolved. Objective: To search for candidate genes associated with this condition, we performed trio-based whole-exome sequencing (WES) on patients with EPP, including two familial cases. Methods: This study included subjects with EPP and PSIS diagnosed by a simple MRI protocol (FAST1.2). We performed two distinct analyses in the trio-based WES. We looked for previously described genes associated with pituitary development. Next, we investigated the whole exome for variants inherited in a pattern consistent with a monogenic etiology. Results: Ten families were evaluated; eight were composed of a child with EPP and healthy parents, one has two affected siblings, and one family has a son and mother with EPP. When analyzing the previously described candidate variants associated with pituitary development, we found variants in GLI2 and FGFR1 in three families. We also found six other variants of interest in three patients: KMT2A, GALR3, RTN4R, SEMA3A, NIPBL, and DSCAML1. Conclusion: The analysis allowed us to find previously reported and not reported GLI2 variants, all inherited from healthy parents, which reinforces the incomplete penetrance pattern of GLI2 variants in the development of EPP and draws attention to possible future functional studies of those variants that have a recurrent expression in CH. We also found novel FGFR1 and SEMA3A variants that suggest an oligogenic mechanism in PSIS and EPP, as seen in patients with hypogonadotropic hypogonadism. We report the first case of a patient with Wiedemann-Steiner syndrome and PSIS, suggesting that the KMT2A gene may be related to pituitary development. Furthermore, the trios' analysis allowed us to find five other variants of interest. Future investigations may clarify the roles of these variants in the etiology of EPP and PSIS.

Imaging Aspects of Toxic and Metabolic Myelopathies.

Metabolic and toxic myelopathies usually occurs due to several different causes. Metabolic myelopathy usually occurs due to deficiency of a nutrient, such as vitamin B12. Toxic myelopathy occurs secondary to the exposure to an external toxic agent. Although they may have a difficult diagnosis, determination of the specific cause of myelopathy is of utmost importance, because many causes are amenable to treatment. Although they have many clinical, electrophysiologic, and neuropathologic similarities, imaging may aid in the suspicion of toxic or metabolic myelopathy. The aim of this article, is to review the imaging features of the main toxic and metabolic myelopathies.

Vascular Myelopathies.

There are many vascular disorders that can affect the spinal cord, and their prevalence and etiology are highly influenced by age, sex, and risk factors. This article reviews the embryology and anatomy of the spinal cord, as well as several vascular conditions, describing their clinical and imaging presentation, emphasizing the different imaging modalities' contributions to increasing specificity and better defining the most appropriate therapy strategy for improving the patient's prognosis.

Intracranial vessel wall magnetic resonance imaging features of infectious vasculitis.

Vasculitis is a complication of several infectious diseases affecting the central nervous system, which may result in ischemic and/or hemorrhagic stroke, transient ischemic attack, and aneurysm formation. The infectious agent may directly infect the endothelium, causing vasculitis, or indirectly affect the vessel wall through an immunological mechanism. The clinical manifestations of these complications usually overlap with those of non-infectious vascular diseases, making diagnosis challenging. Intracranial vessel wall magnetic resonance imaging (VWI) enables the evaluation of the vessel wall and the diseases that affect it, providing diagnostic data beyond luminal changes and enabling the identification of inflammatory changes in cerebral vasculitis. This technique demonstrates concentric vessel wall thickening and gadolinium enhancement, associated or not with adjacent brain parenchymal enhancement, in patients with vasculitis of any origin. It permits the detection of early alterations, even before a stenosis occurs. In this article, we review the intracranial vessel wall imaging features of infectious vasculitis of bacterial, viral, and fungal etiologies.

Midline brain-in-brain malformation associated with bilateral perirolandic cortical abnormalities: an image review of this rare disorder.

A midline brain-in-brain malformation was recently reported, but appropriate classification of this malformation remains uncertain. We describe a child with a complex brain malformation that was not restricted to the midline structures, enlarging the neuroanatomical spectrum of this pseudotumoral midline dysplasia associated with corpus callosum dysgenesis, azygos anterior cerebral artery, absent septum pellucidum and bilateral perirolandic cortical abnormalities. The spectrum of fusion of the cerebral hemispheres and associated brain hemispherical abnormalities is not completely understood. Our data are in line with previous arguments that this malformation could be an additional variant in the spectrum of holoprosencephaly.

Forging Connections in Latin America to Advance AI in Radiology.

The 1° Encontro Latino-Americano de IA em Saúde (1st Latin American Meeting on AI in Health) was held during the 2022 Jornada Paulista de Radiologia, the annual radiology meeting in the state of São Paulo. The event was created to foster discussion among Latin American countries about the complexity, challenges, and opportunities in developing and using artificial intelligence (AI) in those countries. Technological improvements in AI have created high expectations in health care. AI is recognized increasingly as a game changer in clinical radiology. To counter the fear that AI would "take over" radiology, the program included activities to educate radiologists. The development of AI in Latin America is in its early days, and although there are some pioneer cases, many regions still lack world-class technological infrastructure and resources. Legislation, regulation, and public policies in data privacy and protection, digital health, and AI are recent advances in many countries. The meeting program was developed with a broad scope, with expertise from different countries, backgrounds, and specialties, with the objective of encompassing all levels of complexity (from basic concepts to advanced techniques), perspectives (clinical, technical, ethical, and business), and specialties (both informatics and data science experts and the usual radiology clinical groups). It was an opportunity to connect with peers from other countries and share lessons learned about AI in health care in different countries and contexts. Keywords: Informatics, Use of AI in Education, Impact of AI on Education, Social Implications © RSNA, 2022.

Extra-pituitary midline structural abnormalities associated with ectopic posterior pituitary detected on a new rapid MRI protocol (FAST1.2).

Objective: Test if the MRI FAST1.2 protocol can detect extra-pituitary midline structural brain abnormalities in patients with ectopic posterior pituitary (EPP), and highlighting their radiological-laboratory correlations. Subjects and methods: Cross-sectional study of patients with EPP and control group. All individuals were submitted to FAST1.2, which combines the FAST1 protocol developed by our group with 3D T2DRIVE imaging. Results: We evaluated 36 individuals with EPP and 78 as control group. Pituitary stalk (PS) was identified in 7/36 patients in EPP group by FAST1, and in 24/36 patients in FAST1.2 (p < 0.001). FAST1 failed to detect PS in one individual in the control group, while the FAST1.2 defined the PS in all individuals. In EPP group, eleven had interhypothalamic adhesion (IHA), three septo-optic dysplasia, and one cerebellar malformation. We didn't observe higher frequency of panhypopituitarism or developmental delay in patients with IHA. In control group, three had pars intermedia cysts, one hydrocephalus, and one hypothalamic hamartoma. Conclusion: FAST1.2 allows confident recognition of midline structural abnormalities, including the pituitary stalk and IHA, thereby making MRI acquisition faster and with no need for contrast administration. IHA could be associated with defects in neuronal migration, as occur in patients with EPP, with no clinical significance.

Adding T2-Weighted Images to FAST1 Protocol to Evaluate the Anatomy of the Hypothalamic-Pituitary Region.

INTRODUCTION: Ectopic posterior pituitary (EPP) is a malformation of the hypothalamic-pituitary region presented as a spectrum from isolated growth hormone deficiency (GHD) to multiple pituitary hormone deficiencies (MPHDs). Our goal was to establish whether the FAST1.2 protocol, which combines the FAST1 protocol with 3D-T2 DRIVE images, could identify the pituitary stalk (PS) and the regional anatomy more accurately. METHODS: A retrospective study of 36 individuals with EPP and hypopituitarism and a control group of 78 individuals with eutopic posterior pituitary was conducted. All individuals were submitted to FAST1.2. The position and size of the pituitary lobes were described, and the presence/absence of the PS was confirmed. RESULTS: FAST1 identified the PS in 19% of individuals with EPP, while FAST1.2 identified the PS in 67% (p < 0.001). In the FAST1.2 protocol, the PS was visible in all control individuals. All EPP patients with isolated GHD had visible PS in FAST1.2, while only 58.6% of MPHD cases had visible PS. The size of the anterior lobe and the anteroposterior length of the posterior pituitary were smaller in the EPP group versus controls (p < 0.001). We noticed a reduced anterior pituitary lobe in both diameters in MPHD patients (p < 0.05). Six patients acquired new pituitary hormone deficiencies not recognized at the time of MRI; in this group, only 1 patient had a PS not visible in FAST1.2. DISCUSSION/CONCLUSION: The FAST1.2 protocol could prevent the misdiagnosis of idiopathic GHD in patients with short stature and could also be important in the progression to MPHD. The PS could be considered a predictor of hypopituitarism, but its use as an isolated indicator for the progression to MPHD is not recommended. Our results reinforce the use of the size of the anterior lobe as a predictor of hypopituitarism and a possible predictor of the degree of pituitary insufficiency. The FAST1.2 protocol could be used as an alternative to gadolinium administration, as a cheaper and faster method, while eliminating the potential risks associated with the administration of contrast media.

A rare association of tension pneumocephalus and a large frontoethmoidal osteoma: imaging features and surgical treatment.

Osteomas are the most common benign paranasal sinus tumors and are often found in the frontal and ethmoidal sinus. Intracranial complications such as pneumocephalus have occasionally been reported in association with osteomas. We describe a 33-year-old man with a large frontoethmoidal osteoma complicated by tension pneumocephalus and neurological disturbances, and we discuss the clinical and imaging resolutions after surgical management. We emphasize the imaging features and neuroendoscopic approach to avoid life-threatening conditions.

Imaging contribution for the diagnosis of carotidynia.

Carotidynia is a condition characterized by tenderness of the carotid artery and ipsilateral neck pain, eventually aggravated by head movements, swallowing, coughing and sneezing. We report a 52-year-old male presenting cervical pain associated with localized tenderness and reversible structural changes of the common carotid wall, emphasizing the applicability of the diagnostic imaging methods for the presumptive diagnosis of carotidynia in the appropriate clinical context.

Multiphasic disseminated encephalomyelitis associated with herpes virus infection in a patient with TLR3 deficiency.

We report a case of a 14-year-old girl that presented headache, amaurosis, drowsiness, fever, vomiting and diffuse reduction of muscle strength. She had been diagnosed with ADEM one year before and had a previous diagnosis of Toll-Like 3 receptor deficiency. Cerebrospinal fluid analysis revealed pleocytosis (28/mm3, 12/mm3 red blood cells, 70% lymphocytes cells, 2% monocytes cells, 28% neutrophils), normal total protein (38 pg/mL) and normal glucose level (53/mm3). Studies for CSF oligoclonal bands and serum anti-MOG were negative but polymerase chain reaction (PCR) testing was positive for herpes virus 1. In the first ADEM episode, PCR for herpes virus was also positive. Magnetic resonance imaging (MRI) of the brain revealed disseminated hyperintense lesions on T2-weighted and FLAIR images in the white matter of frontal, parietal and temporal lobes, corresponding to extensive asymmetric areas of demyelination that produced mass effect and gadolinium enhancement. Electroencephalography demonstrated irregular diffuse and generalized slow-wave activity with predominance in frontal region. The diagnosis of multiphasic disseminated encephalomyelitis (MDEM) triggered by herpes simplex virus was made. Herpes virus is a neurotropic virus that can cause a wide variety of neurological infection-triggered autoimmune disorders and that is particularly damaging to the central nervous system in situations of impaired immune system. TLR3 is expressed in astrocytes and dendritic cells of the central nervous system and is essential for natural immunity to herpes simplex. TLR3-deficient patients have already been described with herpes simplex encephalitis. TLR3 deficiency may predispose and explain autoimmune and demyelinating manifestations induced by herpes virus. The association of multiphasic disseminated encephalomyelitis triggered by herpes virus in a patient with TLR3 deficiency has not been previously reported in the literature.