Jeffrey Rykken

Intramedullary Spinal Cord Metastases: MRI and Relevant Clinical Features From a 13-Year Institutional Case Series

This article reviews the MRI and clinical findings in 70 spinal cord metastases; 20% of patients had multiple metastases and 8% were asymptomatic. Spinal cord metastases were the initial clinical presentation in 20% of patients. Nearly all metastases showed contrast enhancement and had extensive edema. Cysts and hemorrhage were, however, uncommon and nearly 60% of patients had other metastases to the CNS or that were seen in studies in other organs. Accompanying pial metastases were also common. BACKGROUND AND PURPOSE: Because intramedullary spinal cord metastasis is often a difficult diagnosis to make, our purpose was to perform a systematic review of the MR imaging and relevant baseline clinical features of intramedullary spinal cord metastases in a large series. MATERIALS AND METHODS: Consecutive patients with intramedullary spinal cord metastasis with available pretreatment digital MR imaging examinations were identified. The MR imaging examination(s) for each patient was reviewed by 2 neuroradiologists for various imaging characteristics. Relevant clinical data were obtained. RESULTS: Forty-nine patients had 70 intramedullary spinal cord metastases, with 10 (20%) having multiple intramedullary spinal cord metastases; 8% (4/49) were asymptomatic. Primary tumor diagnosis was preceded by intramedullary spinal cord metastasis presentation in 20% (10/49) and by intramedullary spinal cord metastasis diagnosis in 10% (5/49); 98% (63/64) of intramedullary spinal cord metastases enhanced. Cord edema was extensive: mean, 4.5 segments, 3.6-fold larger than enhancing lesion, and ≥3 segments in 54% (37/69). Intratumoral cystic change was seen in 3% (2/70) and hemorrhage in 1% (1/70); 59% (29/49) of reference MR imaging examinations displayed other CNS or spinal (non–spinal cord) metastases, and 59% (29/49) exhibited the primary tumor/non-CNS metastases, with 88% (43/49) displaying ≥1 finding and 31% (15/49) displaying both findings. Patients with solitary intramedullary spinal cord metastasis were less likely than those with multiple intramedullary spinal cord metastases to have other CNS or spinal (non–spinal cord) metastases on the reference MR imaging (20/39 [51%] versus 9/10 [90%], respectively; P = .0263). CONCLUSIONS: Lack of known primary malignancy or spinal cord symptoms should not discourage consideration of intramedullary spinal cord metastasis. Enhancement and extensive edema for lesion size (often ≥3 segments) are typical for intramedullary spinal cord metastasis. Presence of cystic change/hemorrhage makes intramedullary spinal cord metastasis unlikely. Evidence for other CNS or spinal (non–spinal cord) metastases and the primary tumor/non-CNS metastases are common. The prevalence of other CNS or spinal (non–spinal cord) metastases in those with multiple intramedullary spinal cord metastases is especially high.

Posterior Reversible Encephalopathy Syndrome

Posterior reversible encephalopathy syndrome (PRES) is a complex disorder, our understanding of which continues to evolve. PRES has many clinical associations, many causative factors, a variety of imaging manifestations, and its pathophysiology remains a topic of debate. There are also many other disorders that may mimic PRES. We present a concise review of PRES to enable the radiologist to more readily and easily recognize this treatable disorder with important clinical implications.

Rim and Flame Signs: Postgadolinium MRI Findings Specific for Non-CNS Intramedullary Spinal Cord Metastases

BACKGROUND AND PURPOSE: No highly specific MR imaging features distinguishing ISCMs from primary cord masses have been described. Our purpose was to retrospectively compare peripheral enhancement features on postgadolinium MR imaging of ISCMs with primary intramedullary cord masses. MATERIALS AND METHODS: A consecutive group of patients with firmly diagnosed ISCM (45 patients with 64 ISCMs) and a comparison group with consecutive pathologically proved primary intramedullary spinal cord masses (64 patients with 64 primary spinal cord masses: ependymoma, astrocytoma, hemangioblastoma, ganglioglioma, and cavernous malformation) were included. MR images were evaluated for 2 specific signs on postgadolinium images: a “rim” sign (more intense thin rim of peripheral enhancement around an enhancing lesion) and “flame” sign (ill-defined flame-shaped region of enhancement at the superior/inferior lesion margins). The frequency of rim and/or flame signs in ISCMs and primary cord masses was compared (χ2 test). For ISCMs, the maximal dimension of the enhancing lesion was correlated with the presence of rim or flame signs (t test). RESULTS: Rim and flame signs, alone and in combination, were seen more frequently in ISCMs than in primary cord masses (P < .0001 for each). Specificity and sensitivity, respectively, for diagnosing ISCMs among spinal cord masses on a per-patient basis were the following: rim sign, 97%, 47%; flame sign, 97%, 40%; at least 1 sign, 94%, 60%; and both signs concurrently, 100%, 27%. In the ISCM group, the presence of either a rim or flame sign correlated with a larger measured maximum enhancing lesion size (P = .0065 and P = .0012, respectively). CONCLUSIONS: The rim and flame signs are common in and specific for ISCM and are rare in primary spinal cord masses.

Utility and significance of gadolinium-based contrast enhancement in posterior reversible encephalopathy syndrome

The authors report on a cohort of 135 patients with clinically confirmed PRES who received gadolinium-based contrast and evaluate symptoms, etiology, and clinical follow-up. The most common pattern seen was leptomeningeal (17.8*) or leptomeningeal plus cortical (15.6*). No association was found between the presence or pattern of enhancement and any of the variables, which included sex, age, symptoms, blood pressure, and outcome. BACKGROUND AND PURPOSE: Posterior reversible encephalopathy syndrome is a clinicoradiologic syndrome. Literature regarding associated factors and the prognostic significance of contrast enhancement in posterior reversible encephalopathy syndrome is sparse. This study set out to evaluate an association between the presence of enhancement in posterior reversible encephalopathy syndrome and various clinical factors in a large series of patients with this syndrome. MATERIALS AND METHODS: From an MR imaging report search that yielded 176 patients with clinically confirmed posterior reversible encephalopathy syndrome between 1997 and 2014, we identified 135 patients who had received gadolinium-based contrast. The presenting symptoms, etiology, clinical follow-up, and maximum systolic and diastolic blood pressures within 1 day of MR imaging were recorded. MRIs were reviewed for parenchymal hemorrhage, MR imaging severity, and the presence and pattern of contrast enhancement. Statistical analyses evaluated a correlation between any clinical features and the presence or pattern of enhancement. RESULTS: Of 135 included patients (67.4% females; age range, 7–82 years), 59 (43.7%) had contrast enhancement on T1-weighted MR imaging, the most common pattern being leptomeningeal (n = 24, 17.8%) or leptomeningeal plus cortical (n = 21, 15.6%). Clinical outcomes were available in 96 patients. No significant association was found between the presence or pattern of enhancement and any of the variables, including sex, age, symptom, MR imaging severity, blood pressure, or outcome (all P > .05 after Bonferroni correction). CONCLUSIONS: The presence or pattern of enhancement in posterior reversible encephalopathy syndrome is not associated with any of the tested variables. However, an association was found between MR imaging severity and clinical outcome.

Intramedullary Spinal Cord Metastases: Visibility on PET and Correlation with MRI Features

BACKGROUND AND PURPOSE: Studies systematically evaluating the detection of intramedullary spinal cord metastasis with PET are lacking. Our purpose was to evaluate the visibility of intramedullary spinal cord metastasis on PET in a single institutional series and to correlate PET and MR imaging features. MATERIALS AND METHODS: Patients were included if pretreatment MR imaging identifying an intramedullary spinal cord metastasis and an [18F] FDG-PET examination near the time of MR imaging were available. PET examinations were retrospectively reviewed, with reviewers blinded and then unblinded to the PET report and MR imaging findings. PET intramedullary spinal cord metastasis features were compared with and correlated with previously analyzed MR imaging lesion characteristics. Original clinical PET reports were reviewed. RESULTS: The final study sample was 10 PET examinations in 10 patients with 13 intramedullary spinal cord metastases. In 7 (70%) patients, retrospective blinded review demonstrated convincing evidence of 10 (77%) intramedullary spinal cord metastases. Three MR imaging features correlated with intramedullary spinal cord metastases being visible on PET compared with those nonvisible, respectively: larger lesion enhancement size: mean size: 32.1 mm versus 6.0 mm (P = .038); larger longitudinal extent of T2 signal abnormality: mean 5.6 versus 1.0 segments (P = .0081); and larger ratio of extent of T2 signal abnormality to contrast enhancement: 3.8 versus 1.0 (P = .0069). Intramedullary spinal cord metastasis was confidently reported clinically in 2 (20%) patients, accounting for 5 (38%) intramedullary spinal cord metastases. CONCLUSIONS: Most intramedullary spinal cord metastases can be detected on PET when performed near the time of pretreatment MR imaging. However, intramedullary spinal cord metastases may not be clinically reported on PET. Larger lesions with more edema are more likely to be visible. The spinal cord should be specifically and carefully assessed on PET for evidence of intramedullary spinal cord metastases to provide timely diagnosis.

Intensity of MRI Gadolinium Enhancement in Cerebral Adrenoleukodystrophy: A Biomarker for Inflammation and Predictor of Outcome following Transplantation in Higher Risk Patients

BACKGROUND AND PURPOSE: Outcomes following hematopoietic stem cell transplantation for higher risk childhood-onset cerebral adrenoleukodystrophy are variable. We explored whether a brain MR imaging gadolinium intensity scoring system improves prediction of neurologic outcome. MATERIALS AND METHODS: We developed a 4-point scale of gadolinium intensity relative to the choroid plexus: 0 = no enhancement; 1 = hypointense; 2 = isointense; 3 = hyperintense. The interobserver concordance of the scale was assessed on 30 randomly chosen studies. Scores were generated for 64 evaluable patients and compared with CSF chitotriosidase levels, a known inflammatory marker correlating with outcomes following transplantation. For 25 evaluable higher risk patients (Loes ≥10), the gadolinium intensity score was compared with longer term posttransplantation clinical change. RESULTS: The gadolinium intensity scoring system showed good interobserver reproducibility (κ = 0.72). Of 64 evaluable boys, the score positively correlated with average concomitant CSF chitotriosidase activity in nanograms/milliliter/hour: 0: 2717, n = 5; 1: 3218, n = 13; 2: 6497, n = 23; and 3: 12,030, n = 23 (P < .01). For 25 evaluable higher risk patients, more intense pretransplantation brain MR imaging gadolinium enhancement predicted greater average loss on the adrenoleukodystrophy neurologic function scale following transplantation: 0/1: adrenoleukodystrophy neurologic function scale score difference = 4.3, n = 7; 2/3: adrenoleukodystrophy neurologic function scale score difference = 10.4, n = 18 (P = .05). CONCLUSIONS: Gadolinium enhancement intensity on brain MR imaging can be scored simply and reproducibly for cerebral adrenoleukodystrophy. The enhancement score significantly correlates with chitotriosidase. In boys with higher risk cerebral disease (Loes ≥10), the enhancement score itself predicts neurologic outcome following treatment. Such data may help guide treatment decisions for clinicians and families.

Multiple Myeloma of the Thyroid Cartilage

A 60-year-old male presented with hoarseness. His past medical history was remarkable for a plasmacytoma of the left maxillary sinus having been resected without systemic evidence of plasma cell myeloma (PCM), also known as multiple myeloma (MM), at the time. This maxillary sinus disease recurred and was treated with radiation. Workup for PCM was conducted. Treatment included melphalan and autologous stem cell transplant. Because of the therapeutic and prognostic implications, a Plasma cell neoplasm (PCN) in a neck mass must be carefully evaluated by clinical and pathological criteria in order to distinguish plasmacytoma from PCM. PCN involvement of the thyroid cartilage is very rare, with only 5 previously reported cases.

Utility of coronal contrast-enhanced fat-suppressed FLAIR in the evaluation of optic neuropathy and atrophy

Background and purpose Evaluating chronic sequelae of optic neuritis, such as optic neuropathy with or without optic nerve atrophy, can be challenging on whole brain MRI. This study evaluated the utility of dedicated coronal contrast-enhanced fat-suppressed FLAIR (CE-FS-FLAIR) MR imaging to detect optic neuropathy and optic nerve atrophy. Materials and methods Over 4.5 years, a 3 mm coronal CE-FS-FLAIR sequence at 1.5T was added to the routine brain MRIs of 124 consecutive patients, 102 of whom had suspected or known demyelinating disease. Retrospective record reviews confirmed that 28 of these 102 had documented onset of optic neuritis >4 weeks prior to the brain MRI. These 28 were compared to the other 22 (“controls”) of the 124 patients who lacked a history of demyelinating disease or visual symptoms. Using coronal CE-FS-FLAIR, two neuroradiologists separately graded each optic nerve (n = 50 patients, 100 total nerves) as either negative, equivocal, or positive for optic neuropathy or atrophy. The scoring was later repeated. Results The mean time from acute optic neuritis onset to MRI was 4.1 ± 4.6 years (range 34 days-17.4 years). Per individual nerve grading, the range of sensitivity, specificity, and accuracy of coronal CE-FS-FLAIR in detecting optic neuropathy was 71.4–77.1%, 93.8–95.4%, and 85.5–89.0%, respectively, with strong interobserver (k = 0.667 − 0.678, p < 0.0001), and intraobserver (k = 0.706 − 0.763, p < 0.0001) agreement. For optic atrophy, interobserver agreement was moderate (k = 0.437 − 0.484, p < 0.0001), while intraobserver agreement was moderate-strong (k = 0.491 − 0.596, p < 0.0001). Conclusion Coronal CE-FS-FLAIR is quite specific in detecting optic neuropathy years after the onset of acute optic neuritis, but is less useful in detecting optic nerve atrophy.

Intramedullary Spinal Cord Metastases: Prognostic Value of MRI and Clinical Features from a 13-Year Institutional Case Series

BACKGROUND AND PURPOSE: In patients with intramedullary spinal cord metastases, the impact of MR imaging and clinical characteristics on survival has not been elucidated. Our aim was to identify MR imaging and clinical features with prognostic value among patients with intramedullary spinal cord metastases from a large retrospective series. MATERIALS AND METHODS: The relevant MR imaging examination and baseline clinical data for each patient from a consecutive group of patients with intramedullary spinal cord metastases had previously been reviewed by 2 neuroradiologists. Additional relevant clinical data were extracted. The influence of clinical and imaging characteristics on survival was assessed by Kaplan-Meier survival curves and log-rank tests for categoric characteristics. RESULTS: Forty-nine patients had 70 intramedullary spinal cord metastases; 10 (20%) of these patients had multiple metastases. From the date of diagnosis, median survival for all patients was 104 days (95% CI, 48–156 days). One clinical feature was associated with decreased median survival: lung or breast primary malignancy (57 days) compared with all other malignancy types (308 days; P < .001). Three MR imaging features were associated with decreased median survival: multiple intramedullary spinal cord metastases (53 versus 121 days, P = .022), greater longitudinal extent of cord T2 hyperintensity (if ≥3 segments, 111 days; if ≤2, 184 days; P = .018), and ancillary visualization of the primary tumor and/or non-CNS metastases (96 versus 316 days, P = .012). CONCLUSIONS: Spinal cord edema spanning multiple segments, the presence of multifocal intramedullary spinal cord metastases, and ancillary evidence for non-CNS metastases and/or the primary tumor are MR imaging features associated with decreased survival and should be specifically sought. Patients with either a lung or breast primary malignancy are expected to have decreased survival compared with other primary tumor types.

All that bleeds is not black: susceptibility weighted imaging of intracranial hemorrhage and the effect of T1 signal

PURPOSE To determine if intracranial hemorrhages (ICH) are always hypointense on Susceptibility weighted imaging (SWI) and to determine the effect of T1-signal intensity on the appearance of ICH in SWI series. METHODS SWI and T1-signal intensities of ICH were retrospectively studied in a series of patients. SWI signal intensities were statistically correlated with T1-signal intensities. RESULTS In a series of 57 MRI scans from 40 patients, ICH was hypointense in 19, mixed-intensity in 21, and hyperintense in 17. Hyperintensity of ICH on SWI was significantly associated with increased T1 signal (P<.001). CONCLUSION ICH can have a varied appearance on SWI.