Imaging Review of Paraneoplastic Neurologic Syndromes.

Paraneoplastic syndromes are systemic reactions to neoplasms mediated by immunologic or hormonal mechanisms. The most well-recognized paraneoplastic neurologic syndrome, both clinically and on imaging, is limbic encephalitis. However, numerous additional clinically described syndromes affect the brain, spinal cord, and peripheral nerves. Many of these syndromes can have imaging findings that, though less well described, are important in making the correct diagnosis. Moreover, imaging in these syndromes frequently mimics more common pathology, which can be a diagnostic challenge for radiologists. Our goal is to review the imaging findings of paraneoplastic neurologic syndromes, including less well-known entities and atypical presentations of common entities. Specifically, we discuss limbic encephalitis, paraneoplastic cerebellar degeneration, paraneoplastic brain stem encephalitis, cranial neuropathy, myelitis, and polyneuropathy. We also demonstrate common diagnostic pitfalls that can be encountered when imaging these patients.

The Continued Rise in Professional Use of Social Media at Scientific Meetings: An Analysis of Twitter Use during the ASNR 2018 Annual Meeting.

Professional use of social media continues to increase. We analyzed Twitter use of our own American Society of Neuroradiology (ASNR) 2018 annual meeting, reviewing all Twitter posts (3020 tweets from 523 participants) containing the hashtag #ASNR18 from May 21, 2018, to June 12, 2018, extracting the transcripts from Symplur. Then, each tweet was categorized by the role of user, type of tweet, and topic. The dominant user category was neuroradiologist/radiologist (63%). The keynote address, "The Radiology Renaissance: Shaping the Future of Healthcare," presented by Andy DeLao @Cancergeek was the most frequently tweeted topic (10%). Comment on a session was the major type of tweet. When we compared the data with a similar analysis in 2014, our data analysis showed a growth in the use of Twitter in only 4 years.

Evaluation of Lower-Dose Spiral Head CT for Detection of Intracranial Findings Causing Neurologic Deficits.

BACKGROUND AND PURPOSE: Despite the frequent use of unenhanced head CT for the detection of acute neurologic deficit, the radiation dose for this exam varies widely. Our aim was to evaluate the performance of lower-dose head CT for detection of intracranial findings resulting in acute neurologic deficit. MATERIALS AND METHODS: Projection data from 83 patients undergoing unenhanced spiral head CT for suspected neurologic deficits were collected. Cases positive for infarction, intra-axial hemorrhage, mass, or extra-axial hemorrhage required confirmation by histopathology, surgery, progression of findings, or corresponding neurologic deficit; cases negative for these target diagnoses required negative assessments by two neuroradiologists and a clinical neurologist. A routine dose head CT was obtained using 250 effective mAs and iterative reconstruction. Lower-dose configurations were reconstructed (25-effective mAs iterative reconstruction, 50-effective mAs filtered back-projection and iterative reconstruction, 100-effective mAs filtered back-projection and iterative reconstruction, 200-effective mAs filtered back-projection). Three neuroradiologists circled findings, indicating diagnosis, confidence (0-100), and image quality. The difference between the jackknife alternative free-response receiver operating characteristic figure of merit at routine and lower-dose configurations was estimated. A lower 95% CI estimate of the difference greater than -0.10 indicated noninferiority. RESULTS: Forty-two of 83 patients had 70 intracranial findings (29 infarcts, 25 masses, 10 extra- and 6 intra-axial hemorrhages) at routine head CT (CT dose index = 38.3 mGy). The routine-dose jackknife alternative free-response receiver operating characteristic figure of merit was 0.87 (95% CI, 0.81-0.93). Noninferiority was shown for 100-effective mAs iterative reconstruction (figure of merit difference, -0.04; 95% CI, -0.08 to 0.004) and 200-effective mAs filtered back-projection (-0.02; 95% CI, -0.06 to 0.02) but not for 100-effective mAs filtered back-projection (-0.06; 95% CI, -0.10 to -0.02) or lower-dose levels. Image quality was better at higher-dose levels and with iterative reconstruction (P < .05). CONCLUSIONS: Observer performance for dose levels using 100-200 eff mAs was noninferior to that observed at 250 effective mAs with iterative reconstruction, with iterative reconstruction preserving noninferiority at a mean CT dose index of 15.2 mGy.

Low-Dose CT for Craniosynostosis: Preserving Diagnostic Benefit with Substantial Radiation Dose Reduction.

BACKGROUND AND PURPOSE: Given the positive impact of early intervention for craniosynostosis, CT is often performed for evaluation but radiation dosage remains a concern. We evaluated the potential for substantial radiation dose reduction in pediatric patients with suspected craniosynostosis. MATERIALS AND METHODS: CT projection data from pediatric patients undergoing head CT for suspected craniosynostosis were archived. Simulated lower-dose CT images corresponding to 25%, 10%, and 2% of the applied dose were created using a validated method. Three neuroradiologists independently interpreted images in a blinded, randomized fashion. All sutures were evaluated by using 3D volume-rendered images alone, and subsequently with 2D and 3D images together. Reference standards were defined by reader agreement by using routine dose and 2D and 3D images. Performance figures of merit were calculated based on reader response and confidence. RESULTS: Of 33 pediatric patients, 21 had craniosynostosis (39 positive sutures and 225 negative sutures). The mean volume CT dose index was 15.5 ± 2.3 mGy (range, 9.69-19.38 mGy) for the routine dose examination. Average figures of merit for multireader analysis ranged from 0.92 (95% CI, 0.90-0.95) at routine pediatric dose to 0.86 (95% CI, 0.79-0.94) at 2% dose using 3D images alone. Similarly, pooled reader figures of merit ranged from 0.91 (95% CI, 0.89-0.95) at routine pediatric dose to 0.85 (95% CI, 0.76-0.95) at 2% dose using 2D and 3D images together. At 25% and 10% dose, 95% CI of the difference in figures of merit from routine dose included 0, suggesting similar or noninferior performance. CONCLUSIONS: For pediatric head CT for evaluation of craniosynostosis, dose reductions of 75%-90% were possible without compromising observer performance.

Trigeminal and concurrent glossopharyngeal neuralgia secondary to lateral medullary infarction.

A 69-year-old woman developed acute pain in the left trigeminal and glossopharyngeal nerve distributions. MR imaging demonstrated a left lateral medullary infarction (LMI) involving the left spinotrigeminal nucleus and tract, nucleus ambiguus, and solitary nucleus. Most patients presenting with trigeminal neuralgia will have disease involving the trigeminal nerve or ganglion or the primary sensory nucleus in the pons. We discuss the unusual finding of LMI associated with concurrent trigeminal and glossopharyngeal neuralgia.

Dorsal Lumbar Disc Migrations with Lateral and Ventral Epidural Extension on Axial MRI: A Case Series and Review of the Literature.

Dorsal epidural migration of lumbar disc extrusion is rare and commonly misdiagnosed. Our purpose was to retrospectively analyze soft-tissue abnormalities on axial MR imaging in both the ventral and lateral epidural space in such dorsal epidural migrations. The presence of each component required complete concordance by 3 independent neuroradiologist readers. In a case series (n = 6) of surgically proved dorsal lumbar disc migrations, in which the radiologist's favored prospective diagnosis had not been correct, each case demonstrated epidural soft-tissue abnormality that had components both laterally and ventrally, abutting the parent disc. Similarly, in previously published cases for which axial MR imaging was available, the lateral component was demonstrated in 23/24 cases (96%). Ventral abutment of the parent disc was evident, in addition, in 17/18 cases (94%) with available disc-level axial images. Both ventral and lateral epidural soft-tissue abnormalities are typically present in dorsal lumbar disc herniations and may help radiologists suggest this rare diagnosis in appropriate cases.

SAPHO Syndrome: Imaging Findings of Vertebral Involvement.

BACKGROUND AND PURPOSE: Imaging findings in patients with a combination of synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) are often misinterpreted as discitis/osteomyelitis or metastases, resulting in multiple biopsies and delayed diagnosis. We have incidentally noted a semicircular morphology in vertebral body imaging in several cases of SAPHO syndrome with vertebral involvement. Our goal was to evaluate the prevalence of this distinctive morphology in these patients. MATERIALS AND METHODS: A retrospective review of patients with SAPHO syndrome diagnosed between July 1998 and August 2013 was conducted. A descriptive analysis of MR imaging, CT, radiography, bone scanning, and PET imaging was performed for the presence and distribution of vertebral body signal intensity or attenuation changes and/or enhancement; contiguous vertebral body involvement; vertebral body collapse; endplate irregularity; disc space, facet, and spinous process involvement; subligamentous thickening; and paraspinal soft-tissue involvement. RESULTS: Eighteen patients (16 women [89%]; mean age, 52.9 years) with SAPHO and spine involvement were included. Contiguous involvement of ≥2 vertebral bodies was found in 16 patients (89%), with a curvilinear or "semicircular" pattern involving portions of adjacent vertebral bodies in 10 (63%, P = .14). Most intervertebral discs demonstrated absence of abnormal T2 hyperintensity (73%) and enhancement (89%). Subligamentous thickening was present in 12 (67%). Paraspinal soft-tissue involvement was present in 6 (33%). CONCLUSIONS: SAPHO syndrome should be included in the differential diagnosis in a patient with a curvilinear or semicircular pattern of vertebral involvement, contiguous vertebral body involvement, and absence of intervertebral disc edema and enhancement.

CT Metal Artifact Reduction in the Spine: Can an Iterative Reconstruction Technique Improve Visualization?

BACKGROUND AND PURPOSE: Metal-related artifacts from spine instrumentation can obscure relevant anatomy and pathology. We evaluated the ability of CT images reconstructed with and without iterative metal artifact reduction to visualize critical anatomic structures in postoperative spines and assessed the potential for implementation into clinical practice. MATERIALS AND METHODS: We archived CT projection data in patients with instrumented spinal fusion. CT images were reconstructed by using weighted filtered back-projection and iterative metal artifact reduction. Two neuroradiologists evaluated images in the region of spinal hardware and assigned a score for the visualization of critical anatomic structures by using soft-tissue and bone windows (critical structures totally obscured, n = 0; anatomic recognition with high diagnostic confidence, n = 5). Using bone windows, we measured the length of the most pronounced linear artifacts. For each patient, neuroradiologists made recommendations regarding the optimal use of iterative metal artifact reduction and its impact on diagnostic confidence. RESULTS: Sixty-eight patients met the inclusion criteria. Visualization of critical soft-tissue anatomic structures was significantly improved by using iterative metal artifact reduction compared with weighted filtered back-projection (median, 1 ± 1.5 versus 3 ± 1.3, P < .001), with improvement in the worst visualized anatomic structure in 88% (60/68) of patients. There was not significant improvement in visualization of critical osseous structures. Linear metal artifacts were reduced from 29 to 11 mm (P < .001). In 87% of patients, neuroradiologists recommended reconstructing iterative metal artifact reduction images instead of weighted filtered back-projection images, with definite improvement in diagnostic confidence in 32% (22/68). CONCLUSIONS: Iterative metal artifact reduction improves visualization of critical soft-tissue structures in patients with spinal hardware. Routine generation of these images in addition to routine weighted filtered back-projection is recommended.

MRI findings in autoimmune voltage-gated potassium channel complex encephalitis with seizures: one potential etiology for mesial temporal sclerosis.

BACKGROUND AND PURPOSE: Autoimmune voltage-gated potassium channel complex encephalitis is a common form of autoimmune encephalitis. Patients with seizures due to this form of encephalitis commonly have medically intractable epilepsy and may require immunotherapy to control seizures. It is important that radiologists recognize imaging characteristics of this type of autoimmune encephalitis and suggest it in the differential diagnosis because this seizure etiology is likely under-recognized. Our purpose was to characterize MR imaging findings in this patient population. MATERIALS AND METHODS: MR imaging in 42 retrospectively identified patients (22 males; median age, 56 years; age range, 8-79 years) with seizures and voltage-gated potassium channel complex autoantibody seropositivity was evaluated for mesial and extratemporal swelling and/or atrophy, T2 hyperintensity, restricted diffusion, and enhancement. Statistical analysis was performed. RESULTS: Thirty-three of 42 patients (78.6%) demonstrated enlargement and T2 hyperintensity of mesial temporal lobe structures at some time point. Mesial temporal sclerosis was commonly identified (16/33, 48.5%) at follow-up imaging. Six of 9 patients (66.7%, P = .11) initially demonstrating hippocampal enhancement and 8/13 (61.5%, P = .013) showing hippocampal restricted diffusion progressed to mesial temporal sclerosis. Conversely, in 6 of 33 patients, abnormal imaging findings resolved. CONCLUSIONS: Autoimmune voltage-gated potassium channel complex encephalitis is frequently manifested as enlargement, T2 hyperintensity, enhancement, and restricted diffusion of the mesial temporal lobe structures in the acute phase. Recognition of these typical imaging findings may help prompt serologic diagnosis, preventing unnecessary invasive procedures and facilitating early institution of immunotherapy. Serial MR imaging may demonstrate resolution or progression of radiologic changes, including development of changes involving the contralateral side and frequent development of mesial temporal sclerosis.

Cervical spine MR imaging findings of patients with Hirayama disease in North America: a multisite study.

BACKGROUND AND PURPOSE: Most studies of HD have been conducted in Asia, particularly Japan. To characterize the MR imaging findings of North American patients with HD, we reviewed neutral and flexion cervical MR imaging examinations performed for possible HD at 3 academic medical centers located in the Southeastern, Southwestern, and Midwestern regions of the United States. MATERIALS AND METHODS: Three neuroradiologists assessed the MR imaging examinations in a blinded fashion and reached a consensus rating for LOA of the posterior dura to the spine, lower spinal cord atrophy, spinal cord T2 hyperintensity, loss of cervical lordosis, anterior dural shift with flexion, and confidence of imaging diagnosis. Final reference diagnosis was established separately with a retrospective chart review by a neurologist. RESULTS: Twenty-one patients met the criteria for HD, all were North American males and all who reported their race were white. Seventeen patients did not meet the criteria and served as controls. Four imaging attributes, LOA, dural shift with flexion, consensus diagnosis of neutral images, and consensus diagnosis of combined neutral and flexion images were all able to discriminate the group with HD from the group without HD (P < .05 for each). Findings of HD were often present on neutral images, but the addition of flexion images increased diagnostic confidence. CONCLUSIONS: MR imaging findings in white North American patients with HD include LOA on neutral images and forward displacement of the dura with flexion. Findings are often present on neutral MR images and, in the appropriate clinical scenario, should prompt flexion MR imaging to evaluate anterior dural shift.

Position-related variability of CSF opening pressure measurements.

BACKGROUND AND PURPOSE: Normative data for CSF OP have previously been established with patients in the LD position. During fluoroscopically guided LP procedures, radiologists frequently obtain these OP measurements with patients prone. In this prospective study, our goal was to determine the variability of OP measurements as a function of patient positioning and to assess whether there is a relationship with patient BMI. MATERIALS AND METHODS: Consecutive patients reporting for fluoroscopically guided LP or myelography were enrolled. OP was measured with the patient in 3 positions, with the order of the technique randomized: prone with table flat, prone with table tilted until the hub of the needle was at the level of the right atrium, and LD with the needle hub at the level of the spinal canal. The BMI of each patient was calculated. The Wilcoxon signed-rank test and linear regression analysis with bivariate fit of difference were used for analysis. RESULTS: OP measurements with the patient in the prone position were significantly elevated compared with those in the LD position, with mean differences of 2.7 (P<.001) and 1.6 cm H2O, (P=.017) for prone flat and prone tilted, respectively. There was no significant difference in OP measurements for the prone flat versus prone tilted positions (P=.20). There was no correlation between BMI and observed differences (LD-flat: R2=0.00028; LD-tilt: R2=0.00038; prone-tilt: R2=0.00000020). CONCLUSIONS: Measuring OP with the patient in the prone position may result in overestimation of CSF pressure. Table tilt did not significantly impact mean prone OP. Radiologists should specify exact patient positioning when reporting OP measurements.

Interaction of H-2Eb with an IAP retrotransposon in the A20/2J B cell lymphoma.

In the A20/2J BALB/c B cell lymphoma, Southern analysis revealed an insertion of approximately 6 kilobases of DNA into the first intron of one Ebd-allele. Two observations suggest that the rearrangement did not occur recently in the A20/2J subline. Firstly, normal and altered Ebd-alleles are present in equal numbers, and secondly, the LB 27.4 and LS 102.9 somatic cell hybrids formed at an earlier date both possess the rearrangement. Sequences of two cDNA clones, lambda Eb-7 and lambda Eb-125, selected from an A20/2J cDNA library prepared from poly [A+] RNA indicate that the rearranged Ebd-allele directs the synthesis of atypical Eb transcripts. The clones contain Eb sequence linked to a portion of retroviral-like intracisternal A-particle (IAP) genomic sequence, and they appear to be copies of mRNA produced by splicing between a 5' donor site in the retroviral transcript and the 3' acceptor site of the Eb gene's first intron. The longer lambda Eb-125 insert corresponds to RNA that initiated in the 5'-untranslated region of the Eb gene. The 3'-end of the first Eb exon joins to long terminal repeat sequence, and retroviral sequence extends up to the splice junction with the second Eb exon; 3' of the junction, the lambda Eb-125 sequence corresponds to that of a correctly spliced Eb transcript. It seems feasible that the cDNA clones represent hybrid RNA synthesized by read-through transcription of the Eb coding region and an IAP element inserted into the first intron of the rearranged Ebd-allele.

Routine use of gradient-echo MRI to screen for cerebral amyloid angiopathy in elderly patients.

OBJECTIVE: The objective of this study was to evaluate the routine use of gradient-refocused echo MRI sequences in the detection of cortical cerebral microbleeding suggestive of cerebral amyloid angiopathy in elderly patients (> 70 years old). CONCLUSION: The addition of gradient-refocused echo sequences to routine brain MRI resulted in the identification of cerebral amyloid angiopathy-related microbleeding in 15.5% of elderly patients. In most (86.7%) of these patients with positive findings, cerebral amyloid angiopathy was not suspected clinically, and 46.7% of these patients were undergoing anticoagulant or aspirin therapy, placing them at an increased risk of recurrent intracranial hemorrhage and catastrophic stroke.

Abnormal diffusion-weighted MRI in medulloblastoma: does it reflect small cell histology?

A 12-year-old boy presented with the classic CT and MRI findings of medulloblastoma and the unusual finding of increased signal on diffusion MRI. The small-cell histology of medulloblastoma may account for the increased signal seen on diffusion MRI. Diffusion MRI with echoplanar technique may be useful in evaluation of these tumors and metastatic disease.