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Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease of childhood. Enthesitis-related arthritis (ERA) has been one of the most controversial subtypes of JIA with a higher risk of axial involvement. Our aim was to assess the frequency and spectrum of MRI findings of spine involvement in patients with JIA and determine if the axial involvement is always clinically symptomatic in patients with positive MRI findings. In this retrospective cross-sectional observational study we included known or suspected JIA patients who underwent spinal MRI examination between 2015 and 2017 and followed up in the Pediatric Rheumatology outpatient clinic. The demographic and clinical data were reviewed from the medical charts and electronic records. All patients were grouped as clinically symptomatic and asymptomatic for spinal involvement and MRI findings were re-evaluated for presence of inflammatory and erosive lesions. Of the 72 JIA patients, 57 (79.2%) were diagnosed with ERA, and 15 (20.8%) with non-ERA subtypes of JIA. Overall, 49 (68%) patients with JIA had positive spinal MRI findings (inflammatory and/or erosive lesions). Twenty-seven (47%) ERA patients were clinically symptomatic for spine involvement and among them, 19 (70.3%) had positive spinal MRI findings. Although 30 ERA (53%) patients were clinically asymptomatic, 23 of them (77%) had positive spinal MRI findings, as well. Eleven (73%) patients diagnosed with non-ERA JIA subtypes were clinically symptomatic for spine involvement at the time of MRI. Among them, four (36.3%) had inflammatory and/or erosive lesions on spine MRI. Four (26%) non-ERA patients were clinically asymptomatic for spine involvement, but three (75%) of them showed positive findings on spinal MRI. Inflammatory and/or erosive lesions of the thoracolumbar spine could exist in patients with JIA, regardless of the presence of symptoms. Not only because the significant proportion of ERA patients show asymptomatic axial involvement but also the presence of axial involvement in patients who were classified as non-ERA depending on current ILAR classification underlines the necessity of using MRI for accurate classification of patients with JIA.

OBJECTIVE The objective of this study was to assess the prevalence and spectrum of spinal dysraphism in a cohort of children with cloacal exstrophy (CEX) using MRI. METHODS Children with CEX presenting between 1999 and 2019 with baseline spinal MRI were included. The images were reviewed in consensus to assess the type of dysraphism. The dysraphisms were initially reviewed and described based on their descriptive anatomy, and then classified according to anomalies of gastrulation, primary neurulation, or secondary neurulation. RESULTS Thirty-four children were included. Thirty-three of these children had closed spinal dysraphism, and 1 had a normal spine. Of the 33 cases of closed spinal dysraphism, the conus and/or filum terminale were involved in all cases. The most common malformations were spinal lipoma (n = 20) and terminal myelocystocele (n = 11). The lipomas were heterogeneous: 4 dorsal, 9 transitional, 4 chaotic, and 3 terminal. A large subgroup (10/20, 50%) within the lipomas had an unusual morphology of noncontiguous double lipomas, the proximal fat related to the conus and the distal fat within the filum. These were difficult to characterize using existing classifications. In 2 cases, only a thickened filum was noted. The majority of these malformations were compatible with a disorder of secondary neurulation. CONCLUSIONS Complex spinal dysraphisms are consistently associated with CEX. The unusual dysraphism patterns found in this group of patients highlight the limitations of current embryological classifications. Given the propensity for neurological deterioration in this group of patients, spinal MRI should be routinely performed. The type and distribution of malformations seen have implications for the wider understanding of the pathogenesis and classification of lumbosacral lipomas.

Background Generally, asymptomatic epidermoid cysts (ECs) or ECs with minor symptoms controlled by medication are not treated further. Although epidermoid carcinomas are only sporadically reported, the possibility of malignant transformation of ECs is of concern. Case Description A 77-year-old man under observation for a left cerebellopontine angle tumor presented with no symptoms, including headache, dizziness, or other cranial nerve abnormalities typical of cerebellopontine angle tumors. The tumor manifested as an extra-axial mass in the left cerebellopontine angle and measured 24 × 16 mm (length × width) on magnetic resonance imaging (MRI). As he was asymptomatic, his primary physician did not pursue invasive treatment but ordered follow-up MRI every 2 years. The tumor decreased in size 2 years after the initial MRI, and no tumor fragmentation was observed on the patient’s spinal MRI. Ultimately, the tumor shrank to 19 × 11 mm over 22 years. Conclusions The reduction of an EC is extremely rare. We report a case of spontaneous regression of an EC and consider similar previous cases.

Background: Lateral meningocele is defined by the presence of protrusions of the arachnoid and the dura matter extending laterally through inter- or intravertebral foramina. It is an extremely rare condition; to the best of our knowledge, only a few cases are reported in the literature and most of them in childhood. Case presentation: Authors reported a case of a 48 years old lady who consulted for a lombo-sacral mass right-sided with a CSF fistula. The Spinal MRI objectified a meningocele lateralized in the right side associated with multiples malformations. The patient underwent surgery and the meningocele was closed after excision of the associated subcutaneous lipoma. The long term outcome was favourable and the follow up was assured by clinical examination monthly in the first year. Conclusion: Lateral meningocele is very rarely reported, it is usually associated with multiples malformations. Surgical treatment is a good option for treatment for avoiding complications. The prognostic depends on the preoperative status and the associated malformations.

OBJECTIVESolitary posterior fossa low-grade glial tumors (SPFLGT) in children are rarely associated with leptomeningeal dissemination (LMD). To date, there are no clear guidelines regarding the role of screening and surveillance spinal MRI (sMRI) in children with SPFLGT, at diagnosis or during follow-up periods. The current study reviews a cohort of children with SPFLGT, focusing on sMRI findings.METHODSIn this binational retrospective study, the authors analyzed 229 patients with SPFLGT treated and followed over 13 years. One hundred twelve children had at least 1 total sMRI screening or surveillance examination. One hundred seventeen had no sMRI, but did not present with clinical spinal signs or symptoms. Collected data included demographics, disease characteristics, radiology, pathology, and clinical follow-up data.RESULTSFor the 112 children with at least 1 sMRI, the mean duration from diagnosis to first sMRI was 11.73 ± 28.66 months (range 0–165 months). All sMRI scans were conducted as screening examinations, with no spinal-related symptoms. One patient was found to have a sacral intradural lesion concurrent to the brain tumor diagnosis. Over the course of 180 radiological and 533 clinical follow-up years for the 112 patients with sMRI, and 582 clinical follow-up years for the 117 patients with no sMRI, there were no additional cases with spinal tumor spread.CONCLUSIONSThe yield of screening sMRI in the absence of cranial metastasis, or spinal symptoms, is extremely low. Because preoperative sMRI is recommended for medulloblastomas and ependymomas, it may be logical to acquire. During the follow-up period the authors recommend limiting sMRI in patients without symptoms suggesting a spinal lesion, in patients without known cranial metastases, or recurrence or residual SPFLGT.

Lumbar spinal MRI has the advantage of providing a wealth of visible structures. The assessment of its signal intensities, however, can be challenging. This chapter supplies a systematic guide to assess lumbar spinal MRI scans. Furthermore, it gives an overview of signal intensities of diverse physiological and pathological entities.

Case summary A 10-year-old male neutered Russian Blue cat was presented with a 2-month history of progressive non-ambulatory paraparesis. Spinal MRI revealed a well-demarcated, compressive intradural extramedullary mass at the level of T1 vertebra. The mass had subtle hyperintensity on T2-weighted images, was isointense on T1-weighted images and had diffuse, marked enhancement following gadolinium administration. Neuroaxis MRI, including limited brain sequences, excluded other visible lesions. Thoracic and abdominal radiographs were unremarkable. The mass was resected via a dorsal C7–T2 laminectomy and durotomy. Histopathology revealed a neoplasm composed of columnar-to-polygonal cells forming bilayered palisading patterns with a few apical cilia. Three mitoses were noted in 10 high-power fields. This was consistent with an epithelial neoplasm and initially a metastatic adenocarcinoma was considered most likely. Full-body CT with contrast and including the brain found rhinitis but did not identify any additional neoplastic foci. Biopsies of the nasal cavity and fine-needle aspiration of the spleen and liver were unremarkable. On immunohistochemical evaluation, pan-cytokeratin and E-cadherin immunolabelling was observed; however, synaptophysin, thyroglobulin, chromogranin A and glial fibrillary acidic protein was not detected. This, along with the histological morphology and absence of a primary tumour, was compatible with an ectopic choroid plexus neoplasm. Follow-up performed at 3, 14 and 24 months postoperatively revealed neurological improvement without recurrence. Relevance and novel information We describe the presentation, histopathological and immunohistochemical features and outcome of a case of a rare ectopic choroid plexus neoplasm in the spinal cord of a cat.

Case report - Introduction This is the case of an adolescent referred to rheumatology following 5 years of back pain. After years of trying a number of treatments without much success, the cause was found to be a previously undiagnosed urological pathology. The case highlights awareness of non-rheumatological causes and incidental findings which can redirect a patient towards more appropriate treatment and reduce the potential for long-term adverse health issues and anxiety. Case report - Case description B was referred age 16 to rheumatology with a 5-year history of lower back pain. She had previously seen paediatricians with symptoms initially attributed to constipation due to intermittent straining and hard stool. However, constipation remedies had not relieved the pain which progressed gradually to a more persistent dull ache with impact on daily activities. Various analgesics (including paracetamol and non-steroidal anti-inflammatories), exercises and acupuncture had not helped. There was no history of recurrent urinary tract infections or symptom correlation with fluid intake, menstruation or bowel habit. No inflammatory features or connective tissue disease symptoms were noted and family history was unremarkable Clinical examination was normal apart from mild tenderness in the lumbar region. Rheumatoid factor was borderline positive (15 iu/mL) with the rest of blood tests normal including renal function, inflammatory markers (CRP, ESR), anti CCP and ANA. She had minimal microscopic haematuria without proteinuria. MRI spine in 2015 was normal. In view of her young age and symptoms affecting daily activities, STIR sequence spinal MRI was requested. This excluded any new or old inflammatory changes but incidentally identified a dilated left pelvi-calyceal system. Renal ultrasound confirmed a grossly hydronephrotic left kidney with hydroureter and minimal renal tissue suggesting longstanding obstruction. No calculi were seen. The patient was referred to urologists. Further investigations (including MRI abdomen) confirmed similar findings and a distal ureteric stricture. A MAG 3 renogram showed a normal right kidney but only 12% functioning of the left kidney. Urologists have advised surgery (removal of left kidney and ureter) which may relieve symptoms or a conservative non-surgical approach (continue analgesia, physiotherapy and monitoring). The patient and her family are relieved to have a possible cause identified and are considering the surgical option due to ongoing flank discomfort. Case report - Discussion This was an interesting finding of hydroureter and hydronephrosis causing longstanding back pain presenting to rheumatologists. Until completion of the spondyloarthropathy protocol MRI (STIR images), aetiology had been unclear. Hydronephrosis and hydroureter has no specific age or racial predilection. Signs and symptoms may depend on whether obstruction is acute/chronic. Chronic cases may be asymptomatic or present as a dull discomfort (like this case). Some cases may only present in adulthood with pain precipitated by fluid intake. Blood tests may show impaired kidney function. Post-mortem studies suggest 50% of people have at least one renal abnormality (e.g., renal cysts, duplex ureters) with autopsy series incidence of hydronephrosis reported as 3.1%. Causes include anatomical abnormalities such as vesico-ureteric reflux, urethral strictures (usually present in childhood), calculi, benign prostatic hyperplasia, or intrapelvic neoplasms, pregnancy and infections (e.g., TB). Sudden onset unilateral renomegaly was reported in one case of primary Sjogren’s with lymphocytic interstitial nephritis and positive Sjogren’s autoantibodies. Our patient has no clinical or serological evidence of connective tissue disease. Minor pelvi-calyceal distension can occur as a normal finding in well-hydrated patients and pregnancy. However, significant hydronephrosis requires assessment to determine cause as it may affect long term renal function. Imaging via computed tomography, ultrasound and urograms can help guide further management. In this case the preceding cause and duration of pathology is unknown. Sterile, giant hydronephrosis treatment options include observation and ureteric stent or nephrostomy in patients unfit for surgery. Nephrectomy is advised for pain and recurrent infection in a non-functioning kidney. Complications may include bowel perforation, vascular injury and urine leakage. Both open and minimally invasive procedures have good reported outcomes. The COVID-19 pandemic and exams have affected timing of any elective procedures and the patient understands surgery may or may not offer complete symptom resolution. Case report - Key learning points

Spinal cord abscess is a rare entity, particularly in the pediatric population. Spinal cord abscesses can be located in extradural, subdural and intradural (intramedullary or extramedullary) regions of the cord. Among these locations, intramedullary is extremely uncommon. There have been few case reports of intramedullary spinal cord abscess since its first description in 1830. We describe a 2 year-old boy with a history of neonatal meningitis due to E.coli who presented with refusal to walk and was subsequently found to have intramedullary spinal cord abscesses at multiple levels. Culture of the abscesses again revealed E.coli. The patient was noted to have a pit located just superiorly to his sacral spine. Imaging revealed the presence of a dorsal dermal sinus tract. It is important to evaluate anatomical abnormalities, especially in the setting of serious bacterial infections, such as meningitis, as they have the potential to serve as a reservoir for infection.

Routine spinal MRI can be used for bone marrow lesions detection. The most useful standard pulse sequence is T1- WI, which helps both in local and diffuse bone marrow diseases. Additional new pulse sequences, including chemical shift imaging and diffusion weighted imaging, can be used as solving-problem techniques.

Approximately 25% of patients with anorectal malformation have tethered cord. The traditional way of determining conus medullaris level on magnetic resonance imaging (MRI) relies on counting vertebrae, which may be challenging due to vertebral numeric variation, segmentation anomalies, as well as transitional vertebral body anatomy. The purpose of this study was to utilize more reliable anatomic landmarks (foramen magnum, conus termination, and the upper limit of the iliac crest) to establish a consistent ratio that may differentiate patients with normal and low-lying conus.A single institution database search identified two groups of patients: 255 with normal and 85 with abnormal spinal MRI, who underwent tethered cord release. The conus medullaris ratio was calculated in both groups. The ratio was obtained by dividing the distance between the conus level and the iliac crest by the distance between the foramen magnum and the conus level (IRB # 16-2330).The mean ratio was significantly higher in the non-tethered group compared to the tethered patients (0.184 [sd 0.03] versus 0.118 [sd 0.09]; P.0001). The ratio proved to be a good discriminator between normal and abnormal patients, with area under the curve (AUC) equal to 0.749, meaning that at random, there is a 75% chance that the tethered cord patient will have a lower ratio than the non-tethered cord patient."The Conus Medullaris Ratio" is a good predictor of low-lying conus level on MRI and offers an easy alternative to counting vertebral body levels, particularly in patients with variant or abnormal vertebral body anatomy.II, Study of Diagnostic Test.

Objectives To study the presence of high signal intensity of the intervertebral disc, syndesmophytes, vertebral corner bridging and transdiscal ankylosis on spinal T1-weighted MR images in spondyloarthritis (SpA). Methods A retrospective case-control study of whole spine MRI examinations, obtained in 100 patients with axial SpA and in 100 control patients, was performed. All disco-vertebral units (DVUs) were analyzed on T1-weighted MR images for normal or high signal intensity of the intervertebral disc, presence of syndesmophytes, vertebral corner bridging or transdiscal ankylosis and correlated with final diagnosis. Sensitivity, specificity, and positive and negative likelihood ratios were calculated. Results In this study group, intradiscal high signal intensity, vertebral corner bridging and transdiscal ankylosis on T1-weighted MR images of the spine were all highly specific (specificity: 100%) for diagnosis of axial SpA. However, these signs all had low sensitivity (vertebral corner bridging: 15.0%; intradiscal high signal intensity on T1-weighted MR images: 12.0%; transdiscal ankylosis: 8.0%). Syndesmophytes on spinal MRI were observed in 25 patients but had a more limited diagnostic value (sensitivity: 16.0%, specificity: 91.0%). Conclusions When present in a patient with inflammatory back pain, intradiscal high signal intensity on T1-weighted MR images could be a specific and reliable sign of the presence of axial SpA. Vertebral corner bridging and transdiscal ankylosis also show potential as specific and reliable signs of axial SpA. In contrast, syndesmophytes on MRI do not show potential as a specific or reliable sign of axial SpA.

A 59-year-old man presented with a 9-month history of progressive weakness and numbness in his lower extremities (grade 4/5). Spinal MRI demonstrated extensive edema in the spinal cord and prominent intramedullary enhancement. Selective angiography revealed a right L1 spinal dural arteriovenous fistula (SDAVF) with intradural double draining veins (figure 1). The fistula was treated with surgery successfully. The patient's symptoms gradually improved after surgery. Postoperative MRI showed significant resolution of the edema and enhancement (figure 2). SDAVF with intradural double draining veins is extremely rare.1,2 This case clearly showed a rare draining pattern of intradural double draining veins in SDAVF.

Pyogenic subdural spinal collections are rare but an important pathology to recognise and manage appropriately. We report the case of a 56-year-old female who developed a posterior subdural spinal collection associated with local discitis. There was no direct communication between the infected disc and subdural space, and the collection was located posteriorly within the subdural space which makes this case all the more unusual. We discuss the need for spinal subdural collections to be considered as a differential in patients with back pain and lower limb neurology (especially when there is a known spinal infective focus), the importance of careful interpretation of imaging, and the pathophysiological mechanisms and organisms known to cause spinal subdural collections.

With IRB approval, a total of 132 sagittal views of T1-weighted (T1W) sequence in the spinal MRI scans was collected from 67 patients at our institution and used in this study. We developed a similarity-consistency abnormality detection model (SCAD) for classification of MRI patterns that are associated with low and high risk of multiple myeloma (MM) disease. Our SCAD model consisted of five CNN structures: a generator, an encoder, and three discriminators. The generator was used to capture the distribution of training samples by mapping the given distributions to the distribution of training samples. The encoder mapped the distribution of training samples back to the given distribution to speed up the inference time. The three discriminators were designed to force the generator and the encoder to meet a cycleconsistency constraint. The five components are trained together following the min-max game in GAN. The MRI patterns of each vertebra (normal, focal, variegated and diffused) were provided by an experienced radiologist as reference standard. We trained our SCAD model using the vertebras with the normal pattern, and deployed the trained SCAD model to the three non-normal patterns. The results showed that, our SCAD model achieved a test AUC of 0.71, 0.79 and 0.88 in identifying the focal, variegated and diffused patterns from the normal pattern, respectively.

Purpose We sought to identify correlations between working diagnosis, surgeon indication for obtaining spinal MRI and positive MRI findings in paediatric patients presenting with spinal disorders or complaints. Methods Surgeons recorded their primary indication for ordering a spinal MRI in 385 consecutive patients. We compared radiologist-reported positive MRI findings with surgeon response, indication, working diagnosis and patient demographics. Results The most common surgeon-stated indications were pain (70) and coronal curve characteristics (63). Radiologists reported 137 (36%) normal and 248 (64%) abnormal MRIs. In total, 58% of abnormal reports (145) did not elicit a therapeutic or investigative response, which we characterized as ‘clinically inconsequential’. In all, 42 of 268 (16%) presumed idiopathic scoliosis patients had intradural pathology noted on MRI. Younger age (10.3 years versus 12.0 years) was the only significant demographic difference between patients with or without intradural pathology. Surgeon indication ‘curve magnitude at presentation’ was associated with intradural abnormality identification. However, average Cobb angles between patients with or without an intradural abnormality was not significantly different (39° versus 37°, respectively). Back pain without neurological signs or symptoms was a negative predictor of intradural pathology. Conclusion Radiologists reported a high frequency of abnormalities on MRI (64%), but 58% of those were deemed clinically inconsequential. Patients with MRI abnormalities were two years’ younger than those with a normal or inconsequential MRI. ‘Curve magnitude at presentation’ in presumed idiopathic scoliosis patients was the only predictor of intrathecal pathology. ‘Pain’ was the only indication significantly associated with clinically inconsequential findings on MRI. Level of evidence III

Emergent spinal MRI is recommended for patients with back pain and red flags for infection. However, many of these studies are negative due to low prevalence of spinal infections. Our purpose was to assess if C-reactive protein (CRP) can be used to guide effective utilization of emergent MRI for spinal infections. 316/960 (33%) MRIs performed for infection by the emergency department over 75-month period had CRP levels obtained at presentation, after excluding patients receiving antibiotic or had spinal surgery in 100 mg/L as highly elevated. CRP was normal in 95/316 (30%) and abnormal in 221/316 (70%) patients. MRI was positive in 43/316 (13.6%) patients, all of whom had abnormal CRP. CRP (p

Spinal subdural hematoma (SSDH) was rarely reported in association with COVID-19. A fifty-five-year-old febrile male was presented with sudden onset of areflexic paraparesis, urinary retention, loss of all sensations below the twelve spinal thoracic segment, and severe back pain. COVID-19 was confirmed by positive RT-PCR and spinal MRI showed SSDH.

Highlights • Neuro sweet disease (NSD) rarely exhibits spinal cord involvement. • We experienced a case of NSD with longitudinally extensive transverse myelitis. • The shape of gadolinium enhanced lesion of spinal MRI was characteristic. • Above mentioned finding might be useful for diagnosis of neuro sweet disease.

It has been described that patients with more complex anorectal malformations (ARM), lower sacral ratios and spinal anomalies have poorer rates of fecal and urinary continence. While the ARM subtype has been shown to be an independent predictor of fecal continence, it is not well understood how each of these anatomic factors impact urinary continence. The purpose of this study was to identify anatomic factors associated with urinary continence in children born with ARM. We performed a retrospective review of a large prospectively collected database of children with ARM. Inclusion criteria included diagnosis of ARM, age4 years, available lateral sacral ratio measurement and presence of spinal MRI. Any child with incomplete or absent continence data was excluded. Continence was defined as voiding per urethra volitionally, dry between voids and ≤1 urinary accident per week. Bivariable tests of association and log-binomial regression models were used to examine association between anatomic factors and urinary continence. A total of 434 patients were included in the study. 57.8% (n = 251) were male. Median age was 8.4 years (IQR 6.0-12.3). With regards to severity of ARM, 20.3% (n = 88) were complex, 23.3% (n = 101) were moderate and 56.5% (n = 245) were simple. Lateral sacral ratio included 11.1% (n = 48) that were0.4, 36.2% (n = 157) 0.4-0.7 and 52.8% (n = 229) 0.7. Spine status was found to be myelomeningocele in 4.4% (n = 19), low conus or tethered cord in 34.8% (n = 151) and normal or fatty filum in 60.8% (n = 264). Overall 62.2% were continent. ARM severity, lateral sacral ratio and spine status were each independent predictors of urinary continence on univariate and multivariable analysis. We conclude that in children born with ARM, the severity of ARM, lateral sacral ratio and spine status each independently predict urinary continence. These results allow us to better understand these complex patients and their ability to develop urinary continence. This is crucial in enabling proper patient and family counseling and thus, setting appropriate expectations.

Objectives Several brain and spinal magnetic resonance imaging signs have been described in spontaneous intracranial hypotension. Their correlations are not fully studied. This study aimed to explore potential mechanisms underlying cerebral neuroimaging findings and to examine associations among spinal and brain magnetic resonance imaging signs. Methods We conducted a retrospective review of magnetic resonance myelography and brain magnetic resonance imaging records of patients with spontaneous intracranial hypotension. Categorical principal component analysis was employed to cluster brain neuroimaging findings. Spearman correlation was employed to analyze associations among different brain neuroimaging findings and between brain and spinal neuroimaging findings. Results In patients with spontaneous intracranial hypotension (n = 148), categorical principal component analysis of brain neuroimaging signs revealed two clusters: Cerebral venous dilation and brain descent. Among all brain magnetic resonance imaging signs examined, only midbrain-pons angle associated with anterior epidural cerebrospinal fluid collection length (surrogate spinal cerebrospinal fluid leak severity) (n = 148, Spearman’s ρ = −0.38, p Conclusion This study indicates that there are two factors behind the brain neuroimaging findings in spontaneous intracranial hypotension: Cerebral venous dilation and brain descent. Certain brain neuroimaging signs correlate with spinal cerebrospinal fluid leakage severity, depending on different circumstances.

PURPOSE/OBJECTIVE(S) Spinal cord (SC) delineation is critical in spine SBRT. While underestimating cord can lead to myelitis, overestimating SC may compromise PTV coverage, resulting in higher rates of tumor recurrence. CT myelogram as part of CT simulation is a radiological means of better visualizing the SC and minimizing MRI fusion error. The aim of this study is to compare SC contours based on CT simulation with myelogram and with fused MRI. MATERIALS/METHODS Eight patients with 9 spinal metastases treated with spine SBRT were evaluated. All patients had spinal MRI and myelogram performed prior to CT simulation. The spinal MRI was fused and the SC volume was contoured from the superior to the inferior endplate of the target vertebral body volume based on the (1) CT myelogram images and (2) fused T2 MRI images. Comparing MRI to myelogram-defined SC, centroid deviations (deviations in the center point of the cord) were assessed at every slice through the VB target volume, and maximum deviations of the cord were assessed; all are reported with descriptive statistics. The SC cc volume based on CT myelogram and MRI were compared using paired t-test. RESULTS Between MRI- versus myelogram- defined SC volumes, average deviations of the centroid point cord contours was 0.06cm, ranging from 0.01-0.1cm; average deviation of the outmost aspect of the contours were on average between 0.07-0.11cm. Finally, the volume of the cord was significantly smaller when defined on MRI as compared to myelogram - 2.778cc (SD=0.98) vs. 3.44cc (SD=1.43), p=0.03 - respectively There was only one case that had a SC volume based on T2 MRI that was bigger than that based on myelogram (3.5cc vs. 2.9cc). CONCLUSIONS These data suggest average deviations on MRI of approximately 0.1cm, largely undercontouring the SC volume based on MRI as compared to CT simulation with myelogram.

The aim of this study is to clarify the details of distribution patterns of spinal epidural fluid and to establish it as measure of spontaneous intracranial hypotension (SIH) syndrome diagnosis. Magnetic resonance imaging findings of the spine were analyzed in 37 patients, 24 women and 13 men (mean age 46.3 years), with SIH. Detection rate, thickness and patterns of the fluid collection were evaluated at every vertebral level. Follow-up spinal MRI findings were also analyzed for changes in epidural fluid collection and association with clinical symptoms. The MR images of the cervical spine were obtained in 30 patients, the thoracic spine in 36, and the lumbar spine in 17 patients. Epidural fluid collection was detected totally in 36 patients (97%) and was predominantly found at the mid-thoracic vertebrae. The fluid tended to locate dorsal to the dural sac at the thoracic spine and ventral at the cervical and lumbar spine. Patients with shorter duration of illness tended to have thicker fluid in the thoracic spine. In follow-up MRI, the findings of epidural fluid collection has disappeared in 32/36 cases within 3 months after treatment. Although residual fluid collection was found at the thoracic level in 4 cases, clinical symptoms were improved in all patients. This study suggested that the mid-thoracic spine should be chosen as the target of MRI in screening of SIH, and enlarged dorsal epidural space is strongly indicative of SIH.

The clinical management of pediatric medulloblastoma requires a multidisciplinary approach, which can be challenging, especially in low- and middle-income countries. The aim of this study was to identify current challenges and describe the treatment and outcomes of Iranian pediatric patients with medulloblastoma who were referred to our center in Tehran, Iran. Our retrospective review included 126 patient records from April 2007 to May 2015. The records were analyzed for epidemiologic features, treatment modalities, overall survival, and progression-free survival. Data were analyzed using SPSS 22.0 software. Median age at diagnosis was 6 years (male:female ratio, 2.3:1). At the time of diagnosis, 7 patients were 2 years or younger, and 76 (60.3%) were categorized as having high-risk disease. Overall, 100 patients had gross or near-total surgical resection. Cerebral spinal fluid involvement was detected in 22.2% of the patients tested, and spinal involvement was detected in 25% of the patients who underwent spinal MRI. Metastasis stages at the time of diagnosis were as follows: M0: 48.4% patients, M1: 16.7%, M2: 5.5%, and M3: 21.4%. Median times of follow-up and progression-free survival were 16 and 12 months, respectively. Probability of 7-year overall survival and progression-free survival were 59 and 53.8%, respectively. Results of the current retrospective study emphasize the need for implementing measures to improve outcome for our patients with medulloblastoma. Such measures include a multidisciplinary approach, unified national treatment guidelines, better disease and metastasis staging, twinning initiatives, and seeking a second opinion when needed.

A 49-year-old woman complained of right upper back pain for 1 month. Serum tests showed increased erythrocyte sedimentation rate. Thoracic spinal MRI showed an epidural lesion at the T1-T4 level with inhomogeneous signal intensity on T2-weighted images, slightly higher signal intensity than spinal cord on T1-weighted images, and remarkable enhancement on enhanced T1-weighted images. Epidural malignant tumor was suspected. FDG PET/CT was performed showing increased FDG uptake of the lesion extending to the right T1-T2 and T2-T3 neural foramina. A decompressive posterior thoracic laminectomy with the lesion resection was performed. Immunoglobulin G4-related spinal hypertrophic pachymeningitis was confirmed by pathology.

Magnetic resonance imaging (MRI) is the established technique for evaluating the spine. Unfortunately, the supine position of the patient during conventional MRI scanning does not truly reflect the physiological forces experienced by the discoligamentous structures during normal upright posture and ambulation. Upright MRI is a relatively new technique that allows the patient to be scanned in several different weight-bearing positions, which may potentially demonstrate occult pathology not visualised in the supine position. The imaging technique and current clinical indications of upright spinal MRI would be discussed.

Purpose To design a phantom capable of mimicking human respiration to serve as a testing platform for correction of the static and time-evolving magnetic field distortions typically encountered in MRI of the spinal cord. Methods An inflation system to mimic the air variation of the human lungs was constructed. The inflation system was linked to a phantom containing synthetic lungs and an ex vivo human spine. The relationship between air pressure and phantom lung volume was evaluated via imaging experiment. The geometric distortion (pseudo-displacement) caused by the B0 inhomogeneities was measured on echo planar imaging slices for different air volumes. Results Linear and quadratic relations linking air pressure to phantom lung volume were observed with a Pearson correlation coefficient of 0.99. Air distribution was uneven across the synthetic lungs, exhibiting a left-to-right lung volume ratio of up to 5/4. The pseudo-displacement artifact of the spine caused by the air-filled lungs was observed. Conclusion The proposed phantom can reproduce the lung volume variation of human respiration and thus can serve as a reliable testing platform for the correction of the associated time-varying B0 field distortions. Details of the construction and code for the inflation system microcontroller are available for download as open source. Magn Reson Med 79:600–605, 2017. © 2017 International Society for Magnetic Resonance in Medicine.

Background/aim: The variations and anomalies of the kidneys besides the variations of the inferior vena cava (IVC) and left renal vein (LRV) are mostly asymptomatic, but they carry potential risks particularly during retroperitoneal surgery and radiological interventions. Our aim was to find the frequencies, types, and sex distribution of renal anomalies and variations of the IVC and LRV utilizing magnetic resonance imaging (MRI). Materials and methods: Between November 2010 and April 2011, a retrospective study was conducted including lumbar spinal MRI of 3000 consecutive patients (1869 females and 1131 males) with a median age of 54 years (range: 9-78 years). Results: The percentages of renal anomalies and variations of the IVC and LRV were 0.9%, 0.07%, and 2.6%, respectively. Sex did not affect the distribution of renal anomalies (P = 0.2), IVC variations (P = 0.72), or LRV variations (P = 0.26).Conclusion: Lumbar spinal MRI is useful in detecting renal anomalies and variations of the IVC and LRV.

Background:Magnetic resonance imaging (MRI) offers a non-invasive and objective method of early diagnosis and classification, monitoring disease burden and treatment response for patients (pts) with axial spondyloarthritis (axSpA) including ankylosing spondylitis (AS).1Numerous scoring schemes such as the AS Spine MRI Activity (ASspiMRIa) score are available for the quantitative assessment of MRI, but are subject to intra- and inter-rater variability, labor intensive and costly. Nevertheless, quantification of MRI changes has become an important tool to demonstrate treatment success of biologic drugs in axSpA.Objectives:To evaluate the performance of machine learning (ML) based software for automated Berlin grading of spinal MRI bone marrow oedema in pts with AS and compare with expert scoring.Methods:Fully automated ML software (Figure) was developed to detect and label 23 vertebrae, define vertebral units (VU) as per the Berlin modification of the ASspiMRIa score, and score each VU as either 0 (score of 0) or 1 (score of 1, 2 or 3). The ML algorithm was based on the previously developed SpineNet software.2Analysis included 108 pts from the secukinumab MEASURE 1 study3, in which imaging was done using T1 and STIR sagittal MRI at baseline and Weeks 16, 52, 104, 156 and 208. Two expert readers, blinded to treatment and visit, evaluated all images by ASspiMRIa score. The scores from Reader 2 (R2) were binned into two groups: 0 vs 1, 2, or 3. As a result of multiple pt time points and expert reading sessions, the complete dataset comprised of 10,988 VU. Ten-way cross-validation at per-VU was used to train and validate the ML software. The dataset was split into 10 randomly selected subsets, ensuring that each pt appears in only one subset, after which 8 subsets were used for training the ML software, 1 was used to check for correct training and 1 was used for validation. The process was repeated ten times such that all 10 subsets were used for validation. Accuracy weighted for the frequency of each category, sensitivity and specificity were calculated using scores from R2 as reference. Intra-reader accuracy was also calculated.Results:Accuracy of the software in relation to expert reader scores was 67% with a sensitivity of 0.63 and specificity of 0.70. The intra-reader accuracy was 71% and 77% for R1 and R2, respectively. Individual VU scoring of the Software vs. R2 are presented in the Table as a confusion matrix.Conclusion:Automated scoring of MR images in AS pts provided moderate agreement to that of expert reader-based assessments. ML software has potential to provide an automated guided-reading approach to scoring MR images, which may enable further clinical insights.References:[1]Lukas C, et al. J Rheumatol. 2007;34:862-70.[2]Jamaludin A, et al. Eur Spine J. 2017;26:1374-83.[3]Baeten D, et al. N Engl J Med. 2015;373,2534-48.Figure.Processing pipeline of automated Berlin scoring softwareTable.Confusion matrix between the software and R2SoftwareScore = 0SoftwareScore = 1, 2 or 3Total VU scoredR2 Score = 07199 (70%)3068 (30%)10,267R2 Score = 1, 2 or 3251 (35%)475 (65%)7267,4503,54310,993Percentages calculated as a fraction over the total in each row. Overall accuracy is the average of the highlighted percentages.Disclosure of Interests:Amir Jamaludin: None declared, Rhydian Windsor: None declared, Sarim Ather: None declared, Timor Kadir: None declared, Andrew Zisserman: None declared, Juergen Braun Grant/research support from: Abbvie (Abbott), Amgen, BMS, Boehringer, Celgene, Celltrion, Centocor, Chugai, Eli Lilly and Company, Medac, MSD (Schering Plough), Mundipharma, Novartis, Pfizer (Wyeth), Roche, Sanofi- Aventis, and UCB Pharma, Consultant of: Abbvie (Abbott), Amgen, BMS, Boehringer, Celgene, Celltrion, Centocor, Chugai, EBEWE Pharma, Eli Lilly and Company, Medac, MSD (Schering-Plough), Mundipharma, Novartis, Pfizer (Wyeth), Roche, Sanofi-Aventis, and UCB Pharma, Speakers bureau: Abbvie (Abbott), Amgen, BMS, Boehringer, Celgene, Celltrion, Centocor, Chugai, EBEWE Pharma, Eli Lilly and Company, Medac, MSD (Schering-Plough), Mundipharma, Novartis, Pfizer (Wyeth), Roche, Sanofi-Aventis, and UCB Pharma, Lianne S. Gensler Grant/research support from: Pfizer, Novartis, UCB, Consultant of: AbbVie, Eli Lilly, GSK, Novartis, UCB, Pedro Machado Consultant of: Abbvie, Celgene, Janssen, Lilly, MSD, BMS, Novartis, Pfizer, Roche and UCB, Speakers bureau: AbbVie, Centocor, Eli Lilly, Janssen, MSD, Novartis, Pfizer and UCB Pharma, Mikkel Ǿstergaard Grant/research support from: AbbVie, Bristol-Myers Squibb, Celgene, Merck, and Novartis, Consultant of: AbbVie, Bristol-Myers Squibb, Boehringer Ingelheim, Celgene, Eli Lilly, Hospira, Janssen, Merck, Novartis, Novo Nordisk, Orion, Pfizer, Regeneron, Roche, Sandoz, Sanofi, and UCB, Speakers bureau: AbbVie, Bristol-Myers Squibb, Boehringer Ingelheim, Celgene, Eli Lilly, Hospira, Janssen, Merck, Novartis, Novo Nordisk, Orion, Pfizer, Regeneron, Roche, Sandoz, Sanofi, and UCB, Denis Poddubnyy Grant/research support from: AbbVie, MSD, Novartis, and Pfizer, Consultant of: AbbVie, Bristol-Myers Squibb, Eli Lilly, MSD, Novartis, Pfizer, Roche, UCB, Speakers bureau: AbbVie, Bristol-Myers Squibb, Eli Lilly, MSD, Novartis, Pfizer, Roche, UCB, Thibaud Coroller Shareholder of: Novartis, Employee of: Novartis, Brian Porter Shareholder of: Novartis, Employee of: Novartis, Shephard Mpofu Shareholder of: Novartis, Employee of: Novartis, Aimee Readie Shareholder of: Novartis, Employee of: Novartis

espanolPaciente de 64 anos de edad, que acude a consulta por debilidad, astenia, anorexia y perdida de peso de 11 kg en un ano, sin motivo aparente. Al cabo de 3 semanas presenta disnea, lentitud en la marcha y somnolencia. Pasadas 3-4 semanas, en su domicilio, presenta de forma subita perdida de fuerza en los miembros inferiores y dificultad respiratoria. El paciente ingresa en la UCI por insuficiencia respiratoria y se inicia tratamiento con ventilacion mecanica. Se realiza electromiograma (EMG), en el que se observan datos sugestivos de afectacion de la segunda motoneurona, compatible con esclerosis lateral amiotrofica (ELA). La ELA es una enfermedad neuromuscular degenerativa que se manifiesta por debilidad muscular generalizada y disminucion de la movilidad, que afecta principalmente a las extremidades. La paralisis muscular afecta tambien a la musculatura respiratoria. Las pruebas necesarias para su diagnostico son EMG, RMN cerebral y medular… No existe tratamiento curativo, aunque el riluzol puede retrasar la progresion de la enfermedad. EnglishA 64-year-old patient comes to the health center for muscle weakness, asthenia, anorexia, and weight loss of 11 kg in a year with no apparent reason. After 3 weeks, it appears dyspnea, slow mobility and somnolence. After 3-4 weeks, staying at home, the patient suddenly shows important loss of strength in lower extremities and acute respiratory failure. The patient enters into the ICU due to acute respiratory failure and begins treatment with mechanical ventilation. EMG was performed observing data suggestive of second motor neuron involvement, compatible with amyotrophic lateral sclerosis (ALS). ALS is a degenerative neuromuscular disease manifested by generalized muscle weakness and decreased mobility that mainly affects extremities. Muscular paralysis also affects the respiratory muscles. The necessary tests for its diagnosis are EMG, brain and spinal MRI… There is no curative treatment, although riluzole may delay the progression of the disease.

This paper describes a novel automatic system for Modic changes classification of vertebral endplates. Modic changes are classes of vertebral degenerations visible as intensity variations in magnetic resonance images (MRI). The system operates on T1 and T2 MRI. We introduce three main novelties: 1. a vertebrae alignment scheme via precise bounding boxes obtained through corner localisation, 2. vertebral endplate classification in three dimensions, and 3. Modic changes classification. The system was trained and validated using a large dataset of 785 patients, containing MRIs sourced from a wide range of acquisition protocols. The proposed system achieved 87.8 % classification accuracy on our dataset.

Objective: We report a 63 years old female who presented with confusion, urinary retention and gait disturbances.Method: CT head shows communicating hydrocephalus. Spinal MRI demonstrated an L5- S1...

Spinal dural arteriovenous fistulas (DAVFs) are the most common type of spinal vascular disorders. Multiple spinal DAVFs are very rare, and the majority of them appear synchronously. True metachronous multiple spinal DAVFs occur even more rarely. We report a case of true metachronous multiple spinal DAVFs. A 61-year-old male presented with progressive paraparesis. Spinal MRI showed extensive edema of the spinal cord from T5 to the conus and prominent vascular flow voids. MR angiography showed a left T10 DAVF. The fistula was treated surgically and the clinical symptoms gradually resolved. Recurrence of symptoms occurred 4 months after surgery. A second angiography showed a new fistula located at the right L1. The second fistula was treated surgically and the symptoms again gradually improved. Metachronous multiple spinal DAVF may present within 4 months of each other.

Background In patients (pts) with axial spondyloarthritis (axSpA), inflammation of the spine is believed to trigger a repair mechanism that results in syndesmophyte formation.1Fatty lesions (FLs) in the bone marrow and erosions in the axial skeleton, both visible on magnetic resonance imaging (MRI) T1 sequences, are post–inflammatory changes that have been shown to contribute significantly to models predicting new bone formation.2 It has previously been shown that resolution of inflammatory lesions (INFLs) in pts with axSpA treated with anti–TNF therapy may be associated with an increase in FLs.3,4 RAPID–axSpA was a long–term study in pts with radiographic (r)–axSpA (also known as ankylosing spondylitis) or non–radiographic (nr)–axSpA treated with certolizumab pegol (CZP), which rapidly suppressed active inflammation of the spine, with pts showing limited spinal radiographic progression over 4 years.5 However, it is not known whether CZP treatment coincides with changes in FLs, sclerosis, and erosions. Objectives To report the incidence and association of active inflammation and chronic lesions in the spine of pts with axSpA treated with CZP over 4 years Methods RAPID–axSpA (NCT01087762) was double–blind and placebo (PBO)–controlled to Week (Wk)24, dose–blind to Wk48, and open–label to Wk204. CZP–randomised pts (either 200mg every 2 weeks [Q2W] or 400 mg Q4W) continued their assigned dose throughout; PBO–randomised pts received CZP from Wk24, or if non–responders, from Wk16 onwards. Blinded spinal MRI scans at baseline (BL) and Wk12, 48, 96, and 204 were assessed by 2 central readers to evaluate the presence/absence of active INFLs (Short T1 Inversion Recovery [STIR] sequence) and FLs, sclerosis and erosions (T1 sequence) in vertebral edges (VEs) (present if recorded so by both readers). Pts (r–axSpA and nr–axSpA combined) with valid assessments at BL and at least once post–BL were included. Mean lesion counts at the pt level were estimated from mixed models with repeated measures (MMRM), fitted on observed data from CZP–randomised pts. Associations between INFLs and FLs at the VE level for CZP–and PBO–randomised pts were described using cross–tabulations. Results Of 325 randomised pts, 136 were eligible for these analyses. In pts randomised to CZP at Wk0 (n=89), active INFLs were reduced, and FL counts only slightly increased by Wk12; both were sustained at a low level to Wk204 (Table A). Very few VEs with sclerosis and erosions were observed at BL, and no changes in their frequency were observed over 4 years’ treatment (Table A). Over 4 years’ CZP treatment, the risk of developing a new FL was greater in VEs with vs without INFLs at BL, regardless of changes to INFLs in these VEs post–BL (Table B). The prevalence of FLs was greater in pts with >3 vs ≤3 years’ symptom duration and there were more new FLs in the >3 years subgroup during CZP treatment (data not shown) Conclusion Long–term CZP treatment in axSpA pts was associated with rapid and sustained reduction in active inflammation and a negligible increase in FLs in VEs. More FLs developed in VEs with INFLs at BL than without, but this was not affected by resolution of INFLs. There was no increase in sclerosis and erosions in VEs over 4 years’ CZP treatment. Reference: [1] Appel H. Curr Rheumatol Rep2008;10:356–63; 2. Poddubnyy D. Arthritis Rheum 2016;68:1899–903; 3. Maksymowych W. Ann Rheum Dis 2013;72:23–8; 4. Song I. Ann Rheum Dis 2011;70:1257–63; 5. van der Heijde D. Ann Rheum Dis2018;77:699–705. Acknowledgement We thank the patients who participated. This study was funded by UCB Pharma, medical writing by Hinal Tanna, Costello Medical, UK. Disclosure of Interests Xenofon Baraliakos Grant/research support from: AbbVie, Boehringer Ingelheim, Bristol-Myers Squibb, Celgene, Centocor, Chugai, Janssen, MSD, Novartis, Pfizer Inc, Roche and UCB, Grant/research support from: AbbVie, Pfizer, Merck Sharp & Dohme, UCB Pharma, Novartis, Consultant for: AbbVie, Bristol-Myers Squibb, Boehringer Ingelheim, Celgene, Chugai, Janssen Biologics, Novartis, Pfizer, UCB Pharma, Galapagos, Speakers bureau: AbbVie, Chugai, Janssen, Novartis, Pfizer, UCB Pharma, Sebastian Kruse: None declared, Simone Auteri Employee of: Employee of UCB Pharma, Natasha de Peyrecave Employee of: Employee of UCB Pharma, Tommi Nurminen Employee of: Employee of UCB Pharma, Thomas Kumke Employee of: UCB Pharma, Bengt Hoepken Employee of: Employee of UCB Pharma, Juergen Braun Shareholder of: AbbVie, BMS, Celgene, Chugai, Merck, Novartis, Pfizer, UCB, Grant/research support from: AbbVie, BMS, Celgene, Chugai, Merck, Novartis, Pfizer, UCB, Grant/research support from: Abbott, Bristol Myers Squibb, Celgene, Celltrion, Chugai, Johnson & Johnson, MSD, Novartis, Pfizer, Roche, UCB Pharma, Grant/research support from: AbbVie, BMS, Celgene, Chugai, Merck, Novartis, Pfizer, UCB, Grant/research support from: Abbvie (Abbott), Amgen, Baxter, Biogen, BMS, Boehringer, Celgene, Celltrion, Centocor, Chugai, Hexal, Janssen, Lilly, Medac, MSD (Schering-Plough), Mylan, Mundipharma, Novartis, Pfizer (Wyeth, Hospira), Roche, Sanofi-Aventis and UCB, Consultant for: Abbvie (Abbott), Amgen, Baxter, Biogen, BMS, Boehringer, Celgene, Celltrion, Centocor, Chugai, Hexal, Janssen, Lilly, Medac, MSD (Schering-Plough), Mylan, Mundipharma, Novartis, Pfizer (Wyeth, Hospira), Roche, Sanofi-Aventis and UCB, Consultant for: AbbVie, BMS, Celgene, Chugai, Merck, Novartis, Pfizer, UCB, Consultant for: Abbott, Bristol Myers Squibb, Celgene, Celltrion, Chugai, Johnson & Johnson, MSD, Novartis, Pfizer, Roche, UCB Pharma, Speakers bureau: AbbVie, BMS, Celgene, Chugai, Merck, Novartis, Pfizer, UCB, Speakers bureau: Abbvie (Abbott), Amgen, Baxter, Biogen, BMS, Boehringer, Celgene, Celltrion, Centocor, Chugai, Hexal, Janssen, Lilly, Medac, MSD (Schering-Plough), Mylan, Mundipharma, Novartis, Pfizer (Wyeth, Hospira), Roche, Sanofi-Aventis and UCB, Speakers bureau: AbbVie, BMS, Celgene, Chugai, Merck, Novartis, Pfizer, UCB

Background: In radiographic axial spondyloarthritis (r-axSpA), whole spine low-dose CT (ldCT) is superior in detecting syndesmophytes to conventional radiography (CR), which is limited to the lumbar and cervical spine. As facet joints are difficult to visualize on CR, CT has been used to study facet joint ankylosis in r-axSpA in parts of the spine. However, facet joints in the whole spine have never been studied. Objectives: To assess visibility and interreader reliability of facet joint ankylosis as detected by whole spine ldCT and to describe the prevalence of facet joint ankylosis in patients with r-axSpA. Methods: In an observational cohort, 60 r-axSpA patients with 1-18 syndesmophytes on CR and at least one inflammatory lesion on spinal MRI, underwent ldCT (approximately 4 mSV) of the whole spine. Images were assessed independently by two trained readers and left and right C2-C3 to L5-S1 facet joints were scored as ankylosis present (1) or absent (0). The percentages of missing joint scores per reader were calculated. Ankylosis scores were summed per patient for the whole spine and per segment and presented as mean scores per reader. Interreader reliability was assessed by calculating intraclass correlation coefficients (ICCs), two-way average, absolute agreement. Results: 60 r-axSpA patients were analyzed (mean age 47.7 years, 85% male, 80% HLA-B27+). Reader 1 had between 0% and 18.3% missings per joint, reader 2 had between 0% and 51.7% missings per joint (figure). Most missings occurred in the cervicothoracic junction for both readers and mostly at both left and right facet joints of the same level. Means (SD) of sum-scores for the whole spine and cervical, thoracic and lumbar segments for both readers as well as ICCs are presented in the Table. The lowest ICC was found in the cervical segment. After excluding facet joints with more than 15% missings (C5-C6 to T1-T2), ICCs were 0.93, 0.84 and 0.91 for the whole spine and cervical and thoracic segments, respectively. A striking finding is that while patients with >18 syndesmophytes were excluded from our study, very high scores of ankylosis were reported, suggesting that facet joint ankylosis may occur without syndesmophytes at the same level. Conclusion: In patients with r-axSpA and at least one syndesmophyte, facet joint ankylosis was detected in all spinal segments. Facet joints around the cervicothoracic junction were difficult to score in a relatively high percentage of patients. The interreader reliability of the remaining levels was good to excellent. These results show that ldCT can be used to study facet joint ankylosis in axSpA in all segments of the spine except the cervicothoracic junction. Disclosure of Interests: Rosalinde Stal: None declared, Floris A. van Gaalen: None declared, Alexandre Sepriano: None declared, Juergen Braun Shareholder of: AbbVie, BMS, Celgene, Chugai, Merck, Novartis, Pfizer, UCB, Grant/research support from: AbbVie, BMS, Celgene, Chugai, Merck, Novartis, Pfizer, UCB, Grant/research support from: Abbott, Bristol Myers Squibb, Celgene, Celltrion, Chugai, Johnson & Johnson, MSD, Novartis, Pfizer, Roche, UCB Pharma, Grant/research support from: AbbVie, BMS, Celgene, Chugai, Merck, Novartis, Pfizer, UCB, Grant/research support from: Abbvie (Abbott), Amgen, Baxter, Biogen, BMS, Boehringer, Celgene, Celltrion, Centocor, Chugai, Hexal, Janssen, Lilly, Medac, MSD (Schering-Plough), Mylan, Mundipharma, Novartis, Pfizer (Wyeth, Hospira), Roche, Sanofi-Aventis and UCB, Consultant for: Abbvie (Abbott), Amgen, Baxter, Biogen, BMS, Boehringer, Celgene, Celltrion, Centocor, Chugai, Hexal, Janssen, Lilly, Medac, MSD (Schering-Plough), Mylan, Mundipharma, Novartis, Pfizer (Wyeth, Hospira), Roche, Sanofi-Aventis and UCB, Consultant for: AbbVie, BMS, Celgene, Chugai, Merck, Novartis, Pfizer, UCB, Consultant for: Abbott, Bristol Myers Squibb, Celgene, Celltrion, Chugai, Johnson & Johnson, MSD, Novartis, Pfizer, Roche, UCB Pharma, Speakers bureau: AbbVie, BMS, Celgene, Chugai, Merck, Novartis, Pfizer, UCB, Speakers bureau: Abbvie (Abbott), Amgen, Baxter, Biogen, BMS, Boehringer, Celgene, Celltrion, Centocor, Chugai, Hexal, Janssen, Lilly, Medac, MSD (Schering-Plough), Mylan, Mundipharma, Novartis, Pfizer (Wyeth, Hospira), Roche, Sanofi-Aventis and UCB, Speakers bureau: AbbVie, BMS, Celgene, Chugai, Merck, Novartis, Pfizer, UCB, Monique Reijnierse Grant/research support from: Funding from the Dutch Arthritis Foundation. The funding source had no role in the design and conduct of the study., Desiree van der Heijde Consultant for: AbbVie, Amgen, Astellas, AstraZeneca, Bristol-Myers Squibb, Boehringer Ingelheim, Celgene, Daiichi, Eli-Lilly, Galapagos, Gilead, GlaxoSmithKline, Janssen, Merck, Novartis, Pfizer, Regeneron, Roche, Sanofi, Takeda, Union Chimique Belge, Xenofon Baraliakos Grant/research support from: AbbVie, Boehringer Ingelheim, Bristol-Myers Squibb, Celgene, Centocor, Chugai, Janssen, MSD, Novartis, Pfizer Inc, Roche and UCB, Grant/research support from: AbbVie, Pfizer, Merck Sharp & Dohme, UCB Pharma, Novartis, Consultant for: AbbVie, Bristol-Myers Squibb, Boehringer Ingelheim, Celgene, Chugai, Janssen Biologics, Novartis, Pfizer, UCB Pharma, Galapagos, Speakers bureau: AbbVie, Chugai, Janssen, Novartis, Pfizer, UCB Pharma

Background Radiographic axial spondyloarthritis (r-axSpA) is a chronic inflammatory disease of the axial skeleton, and interleukin (IL)-17 plays an important role in the pathogenesis. Elevated C-reactive protein (CRP) levels in serum predict response to tumor necrosis factor inhibitors (TNFi)1-4 but not to secukinumab.5 The role of baseline spine magnetic resonance imaging (MRI) as a predictor of response has not been investigated for IL-17 inhibitors. Objectives To evaluate response rates at week (wk) 16 with ixekizumab (IXE), an IL-17A antagonist, in patients with ankylosing spondylitis (AS)/r-axSpA and elevated or normal/low inflammation as measured by CRP or spinal MRI. Methods Two Phase 3, randomized, double-blind, placebo (PBO)-controlled trials (COAST-V, NCT02696785; COAST-W, NCT02696798) enrolled patients who were biologic-naive or TNFi-experienced, respectively, with active disease (Bath Ankylosing Spondylitis Disease Activity Index [BASDAI] ≥4 and spinal pain ≥4 on a numeric rating scale) and an established diagnosis of r-axSpA and fulfilling Assessment of SpondyloArthritis international Society (ASAS) criteria (sacroiliitis on radiograph by modified New York [mNY] criteria and ≥1 spondyloarthritis feature). All patients fulfilling ASAS criteria also fulfilled mNY criteria for AS. Patients were treated with IXE (80 mg every 2 or 4 wks [Q2W, Q4W]) or PBO; adalimumab (40 mg Q2W) was an active reference arm in COAST-V. We examined ASAS 40% (ASAS40) response rates at wk 16 for the intent-to-treat population by CRP (≤5 or >5 mg/L) or MRI spine inflammation (Spondyloarthritis Research Consortium of Canada [SPARCC] spine score, Results In the COAST-V/W integrated dataset that combined biologic-naive and TNFi-experienced populations, significantly more patients treated with IXE achieved ASAS40 response at wk 16 than with PBO in the elevated (>5 mg/L) baseline CRP group (39.3%, 42.5%, and 16.7% for IXE Q4W, IXE Q2W and PBO, respectively; p Conclusion IXE demonstrated rapid efficacy in the treatment of AS/r-axSpA at wk 16 irrespective of baseline serum CRP levels or spinal MRI score. References [1] Inman, et al. 2008 [2] Braun, et al. 2016 [3] de Vries, et al. 2009 [4] Vastesaeger, et al. 2011 [5] Braun, et al. 2018 Disclosure of Interests Walter P. Maksymowych Grant/research support from: Abbvie, Pfizer, Novartis, Consultant for: Abbvie, Boehringer, Celgene, Lilly, Novartis, Pfizer, UCB, Speakers bureau: Honoraria from Abbvie, Boehringer, Celgene, Lilly, Novartis, Pfizer, UCB for speaking events but I am not in any speaker’s bureau., Gaia Gallo Shareholder of: Eli Lilly and Company, Employee of: Eli Lilly and Company, Rebecca Bolce Shareholder of: Eli Lilly and Company, Employee of: Eli Lilly and Company, Fangyi Zhao Shareholder of: Eli Lilly and Company, Employee of: Eli Lilly and Company, Vladimir Geneus Shareholder of: Eli Lilly and Company, Employee of: Eli Lilly and Company, Mikkel Ǿstergaard Grant/research support from: Abbvie, Celgene, Centocor, Merck, Novartis, Consultant for: Abbvie, BMS, Boehringer-Ingelheim, Celgene, Eli Lilly, Hospira, Janssen, Merck, Novartis, Novo, Orion, Pfizer, Regeneron, Roche, and UCB, Speakers bureau: Abbvie, BMS, Boehringer-Ingelheim, Celgene, Eli Lilly, Hospira, Janssen, Merck, Novartis, Novo, Orion, Pfizer, Regeneron, Roche, and UCB, Kurisu Tada Grant/research support from: Eli Lilly, Atul Deodhar Grant/research support from: AbbVie, Amgen, Eli Lilly, GSK, Janssen, Novartis, Pfizer, and UCB, Consultant for: AbbVie, Amgen, BMS, Eli Lilly, Janssen, Novartis, Pfizer, and UCB, Lianne S. Gensler Grant/research support from: Abbvie, Amgen, UCB Pharma , Consultant for: Novartis, Lilly, Janssen

Background: Axial inflammation is a key feature in axial SpA (axSpA). There is lack of data relative to the persistence of BME in the same anatomical quadrant (Q, 8 in total for both SIJ together), regardless of the overall presence of BME. Objectives: This study aims to investigate particular patterns of distribution of SIJ-BME across quadrants over time, their persistence over time, and their impact on clinical and structural outcomes. Methods: Patients from the DESIR cohort (early axSpA according to the rheumatologist) with MRI-SIJ available at baseline, 2 and 5 years were included. Each image was scored by 3 trained central readers blinded to chronological order. BME was considered positive if detected in ≥1/6 slices in each of the 8 quadrants, according to each individual reader. Four different patterns of BME over time were defined (no BME, sporadic pattern, fluctuating BME and persistent BME) considering all 8 quadrants (Figure). The effect of BME patterns on 5-year structural (mNY, mSASSS, ≥5 erosions and/or fatty lesions in MRI-SIJ) and clinical outcomes (BASFI, BASMI and ASQoL) was evaluated using multilevel generalised estimating equations (GEE) models (taking the individual reader data into account) and linear regression (using the agreement of ≥ 2 out of 3), as appropriate. All models were adjusted for relevant confounders including treatment (Table). Results: In total, 136 patients were included (age 34 (SD 9) years, 50% male, and 63% HLA-B27 positive). ‘No BME’ was seen in 63 patients (46%), the ‘sporadic pattern’ in 34 patients (25%), the ‘fluctuating pattern’ was seen in 21 patients (15%) and the ‘persistent BME pattern’ was seen in 18 patients (13%). Compared to the ‘no BME’ pattern (reference), the ‘sporadic’ [OR (95% CI): 2.1 (1.0;4.5)], ‘fluctuating’ [OR: 5.6 (2.2;14.4)] and ‘persistent’ [OR: 7.5 (2.8;19.6)] patterns were associated with higher likelihood to be mNY positive at 5-years, suggesting a gradient between cumulative inflammation and damage. Similar findings were observed for mNY as a continuous outcome variable and for ≥5 erosions and/or fatty lesions on spinal MRI as outcomes, but not for mSASSS (Table). There was no association between the BME patterns and the clinical outcomes. Conclusion: Only 13% of the patients showed persistent inflammation in the same Q over a 5-year period and in 15% inflammation was fluctuating across different Qs. More structural damage was found in patients with increasing cumulative levels of local inflammation in the quadrant. Even when BME (temporarily) disappears there is an important effect on structural outcomes, and that effect t is independent of treatment. Disclosure of Interests: Santiago Rodrigues-Manica Grant/research support from: Novartis, MSD, Speakers bureau: Novartis, Alexandre Sepriano: None declared, Sofia Ramiro Grant/research support from: MSD, Consultant for: AbbVie, Lilly, MSD, Novartis, Pfizer, Sanofi, Speakers bureau: AbbVie, Lilly, MSD, Novartis, Pfizer, Sanofi, Robert B.M. Landewe: None declared, Pascal Claudepierre Consultant for: Honoraria from Novartis as steering committe of this survey, Anna Molto: None declared, maxime dougados Grant/research support from: Eli Lilly and Company, Pfizer, AbbVie, and UCB Pharma, Consultant for: Eli Lilly and Company, Pfizer, AbbVie, and UCB Pharma, Miranda van Lunteren: None declared, Desiree van der Heijde Consultant for: AbbVie, Amgen, Astellas, AstraZeneca, Bristol-Myers Squibb, Boehringer Ingelheim, Celgene, Daiichi, Eli-Lilly, Galapagos, Gilead, GlaxoSmithKline, Janssen, Merck, Novartis, Pfizer, Regeneron, Roche, Sanofi, Takeda, Union Chimique Belge

Objective: We aimed to evaluate magnetic resonance imaging (MRI) previously used criteria (Matthews’s criteria, MC) for differentiating multiple sclerosis (MS) from neuromyelitis optica spectrum disorders (NMOSD) in Caucasian and non-Caucasian populations (Argentina, Brazil and Venezuela) with positive (P-NMOSD), negative (N-NMOSD), and unknown (U-NMOSD) aquaporin-4 antibody serostatus at disease onset and to assess the added diagnostic value of spinal cord MRI in these populations. Methods: We reviewed medical records, and MRIs were assessed by two blinded evaluators and were scored using MC. Short-segment transverse myelitis (STM) was added as a new criterion. MC sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) were determined. Results: We included 282 patients (MS = 188 and NMOSD = 94). MC applied to the entire cohort showed 97.8% sensitivity, 82.9% specificity, 92.0% PPV, and 95.1% NPV for differentiating MS from NMOSD. A subanalysis applied only to non-Caucasian (MS = 89 and NMOSD = 47) showed 100% sensitivity, 80.8% specificity, 90.8% PPV, and 100% NPV. Similar sensitivity, specificity, PPV, and NPV of MC for MS versus P-NMOSD ( n = 55), N-NMOSD ( n = 28), and U-NMOSD ( n = 21) were observed. Conclusion: MC distinguished MS from NMOSD of all serostatus in a Latin American cohort that included non-Caucasian populations. Addition of STM to MC did not raise the accuracy significantly.

Spinal magnetic resonance imaging (MRI) is currently not recommended for the routine monitoring of clinically stable multiple sclerosis (MS) patients. We aimed to investigate the occurrence of asymptomatic spinal lesions (a-SL) in clinically stable MS patients, and their association with clinical and radiological outcomes, including the recurrence of spinal lesions. The hospital MS registry was searched for clinically stable MS patients (no relapses, no disability progression) with spinal MRIs performed at T1 (baseline) and T2 (9&ndash, 36 months after T1). Information on relapses, disability and new brain/spinal MRI lesions at T3 (&ge, 6 months after T2) was collected and analyzed. Out of 300 MS patients, 45 showed a-SL between T1 and T2. The presence of a-SL was not associated with the subsequent occurrence of relapses or disability progression at T3, but did correlate with the risk of new brain (rate ratio (RR) = 1.63, 95% CI = 1.16&minus, 2.25, p = 0.003) and recurrent spinal lesions (RR = 7.28, 95% CI = 4.02&ndash, 13.22, p &lt, 0.0001). Accounting for asymptomatic brain lesions (a-BL), the presence of either a-BL or a-SL was associated with subsequent risk for new brain (OR = 1.81, 95% CI = 1.25&ndash, 2.60, p = 0.001) or spinal (RR = 2.63, 95% CI = 1.27&ndash, 5.45, p = 0.009) lesions. Asymptomatic spinal demyelinating lesions occurred in 15% of clinically stable MS patients within a median period of 14 months and conferred an increased risk of future radiological activity at the brain and spinal level.

A 6 year old boy presented with low backache for the last 5 months. MRI of the spine showed a 1.5x1.5x4.7cm intradural mass extending from D10-D12, causing compression of the conus medullaris. With a preoperative diagnosis of intradural ependymoma, a gross total resection (GTR) of tumour was performed. Post-operative histopathology showed a markedly cellular, malignant tumour with frequent mitotic figures. Cells were round to polygonal with vesicular nuclei, prominent nucleoli and were immunopositive for CK,EMA,p53 and immunonegative for MIC2,desmin,SMA,GFAP,INI-1(MIB1 labeling index-35–40%). The overall impression was spinal atypical teratoid rhabdoid tumour(ATRT). Post-operative neuraxis MRI revealed post-operative changes(D10-D12) with a 9 mm enhancing lesion at L5-S1 junction suggesting drop metastasis. There was no brain lesion. CSF cytology did not show any malignant cell. The metastatic work-up was normal. He was started on chemotherapy with ICE regimen (Ifosfamide-2g/m2IVD1–D3,Carboplatin-500mg/m2IVD3,Etoposide-100mg/m2IVD1–D3q3weeks). Subsequently he received craniospinal irradiation (CSI)-36Gray/20fractions/4weeks→ focal boost to primary tumour bed and spinal drop metastasis-14.4Gray/8fractions/1.5 weeks. Thereafter he received 3 more cycles of ICE regimen. End-of-treatment MRI spine showed post-op changes(D10-D12) and 38.9%reduction of the L5-S1 lesion suggesting partial response. Six monthly spinal MRI showed serial reduction of the metastatic lesion leading to complete response (CR) 1 year after completion of treatment. On last follow-up (30 months from initial diagnosis), he was neurologically intact and in CR. Multimodality management comprising GTR,CSI followed by focal boost and multiagent chemotherapy(ICE) can lead to successful outcome in patients with this rare and aggressive spinal tumour.

Neuroimaging is an integral part of the work-up for children who may have experienced abusive head trauma (AHT). The initial exam is usually a head computed tomography (CT), which may be followed by magnetic resonance imaging (MRI) of the brain and/or spine, depending on clinical presentation and/or findings on head CT. Neuroimaging is important for both clinical care and prognosis, as well as the process of distinguishing AHT from accidental trauma. Brain CT and conventional MRI may provide evidence of more than one traumatic event. Diffusion-weighted and susceptibility-weighted imaging help with both acute clinical interventions and prognostication about neurodevelopmental outcomes. With more widespread use of spinal MRI, there is increased appreciation of the spectrum of clinically occult spinal soft tissue injuries associated with AHT. This review highlights clinical and radiologic findings in children evaluated for AHT and emphasizes the importance of a multidisciplinary evaluation.

We herein present a case of tubercular spinal epidural abscess (SEA) without osseous involvement that mimicked an acute bacterial abscess. This case manifested quite unusual findings not only radiographically, but also clinically compared with previously reported cases of tubercular SEA.

A 46-year-old man with stage IV triple-hit B-cell lymphoma diagnosed in February 2016 was treated with chemotherapy. He was followed classically with FDG PET/CT, which assessed the complete metabolic response in June 2016. In July 2016, he had autologous stem cell transplantation. Two months later, he underwent an FDG PET/CT for revaluation. It showed intense FDG uptake in the medullary canal from cervical 4 to thoracic 4, bilateral cervical 7 to thoracic 8, and right thoracic 9 to 12 nerve roots, leading to the diagnosis of neurolymphomatosis. A clinical cervical radiculopathy and spinal MRI results reinforced this diagnosis.

The purpose of this study was to assess how different MRI protocols (spinal vs. spinal plus pelvic vs. whole-body (wb)-MRI) affect staging in patients with smoldering multiple myeloma (SMM), according to the SLiM-CRAB-criterion &lsquo, &gt, 1 focal lesion (FL) in MRI&rsquo, In this retrospective study, a baseline cohort of 147 SMM patients with wb-MRI at initial diagnosis was investigated, including prognostic data regarding development of CRAB-criteria. Fifty-two patients formed a follow-up cohort with a median of three wb-MRIs. The locations of all FLs were determined and it was calculated how staging decisions regarding the criterion &lsquo, 1 FL in MRI&rsquo, would have been made if only a limited anatomic area (spine vs. spine plus pelvis) would have been covered by the MRI protocol. Furthermore, subgroups of patients selected by different cutoff-protocol-combinations were compared regarding their prognosis for development of CRAB-criteria. With an MRI protocol limited to spine/spine plus pelvis, only 28%/64% of patients who actually had &gt, 1 FL in wb-MRI would have been rated correctly as having &lsquo, Fifty-four percent/36% of patients with exactly 1 FL in spine/spine plus pelvis revealed &gt, 1 FL when the entire wb-MRI was analyzed. During follow-up, four more patients developed &gt, 1 FL in wb-MRI, both limited MRI protocols would have detected only one of these four patients as having &gt, 1 FL at the correct timepoint. Having &gt, 1 FL in spine/in spine plus pelvis/in the whole body was associated with a 43%/57%/49% probability of developing CRAB-criteria within 2 years. Patients with &gt, 3 FL in spine plus pelvis and patients with &gt, 4 FL in the whole body had an 80% probability to develop CRAB-criteria within 2 years. MRI protocols limited to the spine or to spine plus pelvis lead to substantial underdiagnoses of patients who actually have &gt, 1 FL in wb-MRI at baseline and during follow-up, which influences staging and treatment decisions according to the current SLiM-CRAB criteria. However, given the spatial distribution of FLs and the analysis on clinical course of patients indicates that the cutoff for the number of FLs should be adopted according to the MRI protocol when using MRI for staging in SMM.

Introduction Intradural spinal hematoma, which refers to subdural and/or subarachnoid hematomas, represent a small percentage of spinal hematoma cases, with both hematomas together being only 19.8% of all spinal hematomas. Taken individually, a traumatic cervical spine subdural hematoma is a rare presentation by itself with only 11 cases reported in literature so far. Presentation of case A 31 years old male presented to the ER with trauma to his neck after fall from height. ASIA (American Spinal Injury Association) impairment scale showed a grade A injury with no sensory or motor function preserved in the sacral segment S4-S5, upper paraparesis and lower paraplegia. Computed Tomography scan showed fracture of C6 vertebrae. Cervical spine T1 and T2 MRI showed intradural hematoma extending from C6 to craniocervical junction. Cervical traction and corpectomy were done. Discussion Traumatic Cervical spine intradural hematoma is a rare entity. Unlike spinal subarachnoid hematoma, spinal subdural hematomas pathophysiology is still unclear. Spinal MRI is the best imaging modality to reach the diagnosis. First line management is surgical evacuation of hematoma, but conservative management is a valid option in certain cases. Conclusion Knowing the risk factors that increase the chance of Intradural extramedullary spinal hematomas and clinically distinguishing those who need to be managed surgically or conservatively is an important step in managing Intradural extramedullary spinal hematomas cases.

Background Multiple sclerosis (MS) is often associated with fatigue, with an increased prevalence of sleep disorders compared to the general population, notably restless legs syndrome (RLS). The aim of this study was to evaluate the prevalence and severity of RLS as well the co-occurrence of spinal demyelination lesions in patients with MS in Lebanon. Methods In this cross-sectional study, we consulted the MS database of the Lebanese association against Multiple Sclerosis and sent out questionnaires to 300 MS patients to screen then confirm the presence of RLS. The final sample included 28 MS participants with confirmed RLS. We conducted further questionnaires to collect demographic data, screen for comorbidities, gather spinal MRI results, and evaluate the severity of both diseases (using the EDSS and the JHRLSS). Results Prevalence of RLS was 15% among MS patients in our study. 46.4% of RLS-affected MS patients had spinal cord demyelination lesions on their MRIs. Participants with MRI lesions had a lower severity score on the JHRLSS (p = 0.088). No association was found between the EDSS results and JHRLSS, demographic data, or comorbidities. Conclusion Restless legs syndrome is commonly found among patients with multiple sclerosis in Lebanon, is underdiagnosed, and ought to be systematically evaluated for in order to improve the patients’ quality of life.

Magnetic resonance imaging (MRI) plays an important role in the evaluation of patients with spine disorders, providing excellent evaluation of both normal and pathologic anatomy. Interpreting MRI findings can be difficult as anatomic abnormalities may not correlate with clinical symptoms and MRI may show abnormal findings in asymptomatic individuals. Using a systematic approach to interpreting a spinal MRI can help to achieve an accurate diagnosis and guide therapeutic decision-making, surgical intervention, and treatment monitoring.

The authors investigated the association between intraoperative motor-evoked potential (MEP) changes and the severity of spinal cord infarction diagnosed with magnetic resonance imaging (MRI) to clarify the discrepancy between them, which was observed in patients with postoperative motor deficits after thoracic and thoracoabdominal aortic surgery.A multicenter retrospective study.Motor-evoked potential25% of control values was deemed positive for spinal cord ischemia. The severity of spinal cord infarction was categorized into grades A to D based on previous studies using the most severe axial MRI slices. The associations between MRI grade, MEP changes, and motor deficits were examined using logistic regression.Twenty-three of 1,245 patients (from 1999 to 2013, at 12 hospitals in Japan) were extracted from medical records of patients who underwent thoracic and thoracoabdominal aortic repair, with intraoperative MEP examinations and postoperative spinal MRI.No intervention (observational study).Motor-evoked potential25% of control value was associated significantly with motor deficits at discharge (adjusted odds ratio [OR], 130.0; p = 0.041), but not with severity of spinal cord infarction (adjusted OR, 0.917; p = 0.931). Motor deficit at discharge was associated with severe spinal cord infarction (adjusted OR, 4.83; p = 0.043), MEP25% (adjusted OR, 13.95; p = 0.031), and combined deficits (motor and sensory, motor and bowel or bladder, or sensory and bowel or bladder deficits; adjusted OR, 31.03; p = 0.072) in stepwise logistic regression analysis.Motor-evoked potential25% was associated significantly with motor deficits at discharge, but not with the severity of spinal cord infarction.