Spinal dysraphism with tripedus morphology: A case report

This is a rare case report of a 5-month-old child with a complex spinal dysraphic state, and an accessory limb (tripedus morphology), accessory genitalia, and anal dimple. The child was brought to the hospital with an accessory limb arising from the back. On clinical examination, an accessory limb arising from the lower back with a partially developed foot with the presence of toes and nails was noted. Spinal MRI was advised which revealed dysraphic features including spina bifida with the low lying and posteriorly tethered cord with diastematomyelia along with a supernumerary appendage attached to the vertebral column having rudimentary bones resembling those of extremities. The presence of an accessory limb with spinal dysraphism is quite a rare anomaly. The condition can be treated by surgical intervention and involves excision of the accessory limb with adequate dural and paraspinal muscle cover.

Spine Medical Image Segmentation Based on Deep Learning

The aim was to further explore the clinical value of deep learning algorithm in the field of spinal medical image segmentation, and this study designed an improved U-shaped network (BN-U-Net) algorithm and applied it to the spinal MRI medical image segmentation of 22 research objects. The application value of this algorithm in MRI image processing was comprehensively evaluated by accuracy (Acc), sensitivity (Sen), specificity (Spe), and area under curve (AUC). The results show that the image processing time of fully convolutional network (FCN) algorithm and U-Net algorithm is greater than 6 min, while the processing time of BN-U-Net algorithm is only 5–10 s, and the processing time is significantly shortened ( P < 0.05 ). The Acc, Sen, and Spe results of BN-U-Net segmentation algorithm were 94.54 ± 3.56%, 88.76 ± 2.67%, and 86.27 ± 6.23%, respectively, which were significantly improved compared with FCN algorithm and U-Net algorithm ( P < 0.05 ). In summary, the improved U-Net network algorithm used in this study significantly improves the quality of spinal MRI images by automatic segmentation of MRI images, which is worthy of further promotion in the field of spinal medical image segmentation.

New association between idiopathic scoliosis and Luckenschadel skull (lacunar skull)

Objective: Luckenschadel skull is a skull that is radiologically characterized by lacunae in the cranial vault. To date, although the association between neurological abnormalities and scoliosis is well-recognized, no relationship between idiopathic scoliosis and a lacunar skull has been defined. We explored the incidence and time courses of lacunar skulls in patients with idiopathic scoliosis. Materials and Methods: Spinal X-rays of 3,170 children aged 6 to 16 years with idiopathic scoliosis evaluated from October 2010 to August 2020 were examined for the presence of an irregular inner calvarial table indicative of a Luckenschadel skull. A total of 1,760 (55.5%) of the 3,170 images included the skull. We also explored the frequency of intraspinal abnormalities in children with lacunar skull images who underwent spinal magnetic resonance imaging. Results: The study population consisted of 1,760 children, 1,203 girls (68.4%) and 557 (31.6%) boys. A total of 268 (15.2%) clearly evidenced lacunar skulls in their radiographs; 186 (69.4%) girls(11.3±4.3 years) and 82 (30.6%) boys(12.6±3.3 years). In total, 2 of 56 patients (3.6%) who underwent spinal MRI had intraspinal abnormalities (isolated Chiari malformation-I). No additional neurological problems were detected in children with lacunar skulls. Conclusion: We are the first to report that the lacunar skull is very common in children with idiopathic scoliosis who lack any other neurological pathology. The lacunar skull does not disappear even in adolescence. Although previous publications have stated that lacunar skull disappears over time in radiographic images, we found that it became more noticeable over time in children with scoliosis.

Brighter spotty lesions on spinal MRI help differentiate AQP4 antibody-positive NMOSD from MOGAD

In a large acute myelitis cohort, we aimed to determine whether brighter spotty lesions (BSLs)—using the refined terminology—on spinal magnetic resonance imaging (MRI) help distinguish aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder (AQP4-NMOSD) from myelin oligodendrocyte glycoprotein antibody disease (MOGAD). An experienced neuro-radiologist and two neurologists independently analyzed 133 spinal MRI scans (65 from MOGAD and 68 from AQP4-NMOSD) acquired within 1 month of attacks. BSLs were observed in 18 of 61 (30%) participants with AQP4-NMOSD, while none of 49 participants with MOGAD showed BSL ( p < 0.001). BSL during the acute phase would be useful to differentiate AQP4-NMOSD from MOGAD.

Area postrema syndrome in neuromyelitis optica spectrum disorder: diagnostic challenges and descriptive patterns

Background Although area postrema syndrome (APS) is one of the core clinical features of neuromyelitis optic spectrum disorder (NMOSD), it is frequently misdiagnosed as gastrointestinal or systemic disorders. In this study, we describe the diagnostic challenges in NMOSD patients with APS and their characteristic clinical and radiological features. All patients who attended our university hospitals during the period from March 2019 to August 2020 with a diagnosis of NMOSD according to the latest diagnostic criteria were admitted and evaluated clinically, radiologically with gadolinium-enhanced brain and spinal MRI, measures of serum Anti-Aquaporin 4 (Anti-AQP4) and clinical status using the Expanded Disability Status Scale (EDSS) scores. APS was diagnosed if there was a history of intractable nausea, vomiting, or hiccups (INVH) that had lasted longer than 1 week with the exclusion of other etiologies, or less than 48 h if associated with a lesion in the dorsal medulla on MRI scan. Results Twenty out of 90 (22.2%) identified patients with a diagnosis of NMOSD had a history of unexplained intractable nausea, vomiting or hiccoughs lasting an average of 20 days. Seventeen patients were anti-Aquaporin 4 seropositive. Seven patients (35%) presented initially with isolated clinical features of APS and were diagnosed only after subsequent relapse. Patients with APS preceding other core clinical presentations (13 cases, 65%) were diagnosed after development of motor manifestations. All patients developed acute myelitis during the course of illness. Brain and spinal MRI scans showed that 13 had a linear lesion in the dorsal tegmentum of the medulla oblongata adjacent to the fourth ventricle. Otherwise, longitudinally extensive transverse myelitis was found in 80%, while 35% showed extension of the cord lesion to the AP. Conclusions APS as a core clinical characteristic of NMOSD is not a rare presentation as was previously thought and can occur in both AQP4-seropositive and seronegative NMOSD.

NCMP-11. SPINAL CORD INFARCT AFTER CAR-T TREATMENT

Cortical and subcortical neurotoxicity from CAR-T therapy is a well described complication in literature, with over 40% of patients experiencing at least one neurologic side effect. However, spinal cord toxicity from CAR-T therapy is less well described. To our knowledge, this is the first reported case of a spinal cord infarct following CAR-T therapy. A 44 year old male with primary refractory DLBCL without CNS involvement, which was refractory to R-CHOP, R-ICE, and hyperCVAD part B underwent CD-19 CAR-T treatment. The day after infusion he developed grade 1 cytokine release syndrome (CRS) with fever and up trending inflammatory markers. Infectious work up was negative and he was treated with tocilizumab and dexamethasone. His fever resolved and markers down trended. On day 5 post CAR-T, he became encephalopathic, developed severe back pain, and was unable to move his bilateral lower extremities. He was treated with 2nd and 3rd doses of tocilizumab, dexamethasone and was started on anakinra. Patient’s mental status cleared by day 7 and he was found to have a dermatome sensory level at T10 with flaccid bilateral lower extremity paralysis. MRI Brain was unremarkable, but a spinal MRI showed longitudinally extensive cord edema and diffusion restriction at T10. Due to an initial question of transverse myelitis, he was treated with a 3-day course of IV methylprednisolone, with no improvement in symptoms. CSF studies were unable to be obtained due to his thrombocytopenia. Repeat MRI obtained 10 days after initial imaging showed resolution of cord edema, but continued areas of FLAIR hyperintensity at T10 through the conus. Despite aggressive rehabilitation services, four months later, patient remained paralyzed in his lower extremities with an indwelling foley catheter. He remains in a complete remission.

Tarlov cysts in back pain patients: prevalence, measurement method and reporting points

Objective: Determining the prevalence of Tarlov cysts in low back pain patients. Methods and materials: The picture archiving & communication system (PACS) & hospital information system (HIS) of a corporate hospital were retrospectively analyzed to determine the percentage of Tarlov cysts among patients undergoing spinal MRI for back pain over 3 years (January 2017 to December 2019). Results: 384 patients had undergone spinal MRI for back pain over the study period, and 25 of them (6.51%) had Tarlov cysts. Vast majority (15 cases) showed cysts located at S2/S3 level, and few were found at S1/S2 and other levels. Single cysts were found in most (=18) of the cases, while 7 cases of multiple / bilateral cysts were found. Cyst dimensions were higher in craniocaudal than anteroposterior or transverse directions. In case of multiple cysts, one or two cysts were noted as dominant, having higher dimensions than the others. The study data revealed no gender or age predilection. Conclusions: We conclude that including the entire sacrum with a T2 sagittal sequence in all MRI for low back pain can increase detection of Tarlov cysts, and thereby provide more data for further analysis. Advances in knowledge: We propose the concept of one “dominant” cyst when there are multiple Tarlov cysts. We recommend that diameter or size of Tarlov cysts be specified to their craniocaudal dimension. We also suggest reporting points for contextual structured reporting, viz. presence or absence of bony scalloping, neural foraminal narrowing, nerve root compression or extraforaminal extension.

Skeletal-Related Events (SRE) in Prostate Cancer: A Report of Two Cases

A skeletal-related event (SRE) is an event occurring due to bone metastasis in prostate cancer. SREs are usually marked by pain, pathological fractures, spinal cord compression, hypercalcemia, or bone metastasis requiring radiotherapy or operation. Case I: A 64-year-old male was diagnosed with a pathological fracture of the left femur. Thoracal CT scan showed osteoblastic lesions in the thoracal vertebrae, sternum, clavicle, and humeral head. Spinal MRI showed destruction of the cervical to sacral vertebral bodies. The histopathological result with Adenocarcinoma Gleason scores 8 (4+4) and an initial prostate-specific antigen (PSA) level of 689,7 ng/dL. Afterward, subcapsular orchiectomy was performed. However, his PSA level was still high (>100 ng/dL) even after serum testosterone had reached a castration level. The patient died during the first chemotherapy using docetaxel. Case II: A 61-year-old male was diagnosed with inferior paraplegia and neurogenic bladder, paraparesis, urinary retention, and pain in the flank area. Spinal MRI showed a pathological compression fracture of the 8th thoracic vertebrae. Thoracal CT scan showed costal and 8th thoracal vertebrae destruction as well as multiple nodules in the lungs. Histological results with Adenocarcinoma Gleason score 9 (4+5) and an initial PSA level of 750 ng/dL. Afterward, subcapsular orchiectomy was performed. Serum testosterone reached castration level with the lowest PSA concentration of 21.6 g/dL. The patient declined chemotherapy and agreed to palliative treatment. He died one year after diagnosis. A high PSA level (>500 ng/dL) could potentially be used as a predictor for severe SRE.

Enthesitis Related Arthritis: A Single Center Experience

Objective: Enthesitis-related arthritis is a subtype of juvenile idiopathic arthritis category, characterized by enthesitis, arthritis, and the risk of axial involvement. We aimed to summarize the demographics, clinical, and laboratory findings of enthesitis-related arthritis patients and to identify the distinguishing features of enthesitis-related arthritis patients with HLA B27 positive compared to the patients who were HLA B27 negative. Materials and Methods: This retrospective study included patients with Enthesitis-related arthritis who followed up between 2015 and 2018. Demographical, clinical, and laboratory data were retrospectively reviewed from the patient files and computerized medical charts. Results: A total of 72 patients diagnosed with enthesitis-related arthritis were included in the study. The male/female ratio was 2.1/1. Fifty-three (73%) of them presented with peripheral arthritis. The most commonly affected joint was knee (81.1%), followed by the ankle (43%), hips (32%), and wrist (5%). HLA B27 was positive in 36 (50%) patients. During follow-up, the number of patients who developed enthesitis-related arthritis -associated uveitis was 8 (11.1%). During follow-up, 56 patients with inflammatory back pain and/or sacroiliac tenderness underwent spinal MRI. Ten (17.8%) patients had only thoracal and/or lumbar involvement, 18 (32%) had only sacroiliitis, and 9 (16%) patients had both of them on spinal MRI. In comparison with HLA-B27-negative children, HLA-B27-positive patients were more likely to have enthesitis (16 (44.4%) vs 8 (22.2%), p=0.046), MRI proven sacroiliitis (19 (52.7%) vs 8 (22.2%), p=0.031), MRI proven spinal involvement (13 (36.1%) vs 6 (16.6%), p=0.031), and uveitis (8 (100%) vs 0(0%), p=0.014). During follow up, 65/72 (90.2 %) of them needed disease-modifying antirheumatic drugs (DMARD), and 51/72 (70.8%) needed anti-tumor necrosis factor-α (TNF-α) therapy. Conclusion: We found that patients who were HLA-B27- positive had significantly more enthesitis, MRI-proven sacroiliitis, MRI-proven spinal involvement, and acute anterior uveitis, in comparison to patients who were HLA B27 negative. It is crucial to carefully assess those patients with concern for enthesitis-related arthritis to determine the expected prognosis and make therapeutic decisions appropriately.