Kara M. Schwartz

Pattern of T2 hypointensity associated with ring-enhancing brain lesions can help to differentiate pathology.

Ring-enhancing lesions seen on MR images can occur with a variety of etiologies. Some ring-enhancing lesions have hypointense rims peripherally on T2-weighted MR images. In this study, we examined whether T2 hypointense rims were associated with specific pathologies. A search for ring-enhancing lesions on MR images obtained from 1996 to 2004 was performed, and revealed 221 patients with MRI findings of ring enhancement. The pattern of T2 hypointensity (arc or rim) corresponding with ring enhancement was recorded. In addition, we analyzed other imaging characteristics, including signal on diffusion-weighted images, central homogeneity on T2 and multiplicity of lesions. We then reviewed clinical data on the patients to ascertain the diagnosis for each examination. The most common associated pathologies in our study were gliomas (40%), metastases (30%), abscesses (8%) and multiple sclerosis (MS; 6%). Hypointense borders on T2-weighted images were present in 67% of lesions in the form of a rim in 40% and an arc in 60%. Abscesses had the highest percentage of hypointense rims. Metastases and gliomas more commonly had arcs, and MS lesions were divided between rims and arcs. Abscesses and MS lesions were more commonly homogeneous centrally, compared to gliomas and metastases. Additionally, abscesses were more often bright on diffusion imaging than the other pathologies. As expected, abscesses and MS lesions were usually multiple, whereas metastases were typically multiple in approximately 50% of the patients; gliomas were generally solitary. Trends in T2 hypointensity may aid in distinguishing among etiologies of ring-enhancing lesions, although there is overlap between the MR appearance of these various pathologies.

Pseudotumor of Infancy and Congenital Muscular Torticollis: 170 Cases†

Objectives To review pseudotumor of infancy (POI) and congenital muscular torticollis (CMT) and to suggest an algorithm for treatment.

The role of MR myelography with intrathecal gadolinium in localization of spinal CSF leaks in patients with spontaneous intracranial hypotension.

BACKGROUND AND PURPOSE: Localization of spinal CSF leaks in CSF hypovolemia is critical in directing focal therapy. In this retrospective review, our aim was to determine whether GdM was helpful in confirming and localizing spinal CSF leaks in patients in whom no leak was identified on a prior CTM. MATERIALS AND METHODS: Forty-one symptomatic patients with clinical suspicion of SIH were referred for GdM after undergoing at least 1 CTM between February 2002 and August 2010. A retrospective review of the imaging and electronic medical records was performed on each patient. RESULTS: In 17 of the 41 patients (41%), GdM was performed for follow-up of a previously documented leak at CTM. In the remaining 24 patients (59%), in whom GdM was performed for a suspected CSF leak, which was not identified on CTM, GdM localized the CSF leak in 5 of 24 patients (21%). In 1 of these 5 patients, GdM detected the site of leak despite negative findings on brain MR imaging, spine MR imaging, and CTM of the entire spine. Sixteen of 17 patients with previously identified leaks underwent interval treatment, and leaks were again identified in 12 of 17 (71%). CONCLUSIONS: GdM is a useful technique in the highly select group of patients who have debilitating symptoms of SIH, a high clinical index of suspicion of spinal CSF leak, and no demonstrated leak on conventional CTM. Intrathecal injection of gadolinium contrast remains an off-label use and should be reserved for those patients who fail conventional CTM.

When Should I Do Dynamic CT Myelography? Predicting Fast Spinal CSF Leaks in Patients with Spontaneous Intracranial Hypotension

Which patients with intracranial hypotension will need dynamic CT myelography? The authors assessed brain and spine MRI studies and opening pressure measurements in 150 consecutive patients with intracranial hypotension to see if these can predict fast CSF spine leaks that will require dynamic myelography. Fast leaks were present in 21% and correlated well with the presence of spinal extra-arachnoid fluid. No correlation between fast leaks and brain MRI findings was found. Thus, when spinal extra-arachnoid fluid is documented on MRI studies there is no need for conventional CT myelography but the patient should be directly studied with dynamic CT myelography. BACKGROUND AND PURPOSE: Some patients with SIH have fast CSF leaks requiring dynamic CTM for localization; however, patients generally undergo conventional CTM before a dynamic study. Our aim was to determine whether findings on head MR imaging, spine MR imaging, or opening pressure measurements can predict fast spinal CSF leaks. MATERIALS AND METHODS: A retrospective review was performed on 151 consecutive patients referred for CTM to evaluate for spinal CSF leak. Head MR imaging was evaluated for diffuse dural enhancement and “brain sag,” and spine MR imaging for presence of an extradural fluid collection. The opening pressure was recorded. The CTM was scored as no leak, slow leak localized on conventional CTM, or fast leak that required dynamic CTM. RESULTS: Fast CSF leaks were identified in 32 (21%), slow leaks in 36 (24%), and no leak in 83 (55%) of 151 patients on initial CTM. There was significant association between spinal extra-arachnoid fluid on MR imaging and the presence of a fast leak (sensitivity 85%, specificity 79%, P < .0001). There was not significant association between fast leak and findings on head MR imaging (P = .27) or opening pressure (P = .30). CONCLUSIONS: If all patients with spinal extra-arachnoid CSF on MR imaging had been sent directly to dynamic CTM, repeat myelography would have been avoided in most patients with fast leaks (23 of 27; 85%). However, a minority of patients with slow or no leaks would have been converted from conventional to dynamic CTM (16 of 77; 21%). Spinal MR imaging is helpful in premyelographic evaluation of SIH.

Pituitary Stalk Lesions: The Mayo Clinic Experience

CONTEXT Pituitary stalk lesions have various etiologies, often not clinically apparent. Pathological samples from these lesions are rarely obtained, because of the critical location and function of the hypophyseal stalk. OBJECTIVES The purpose of this study was to characterize the etiological spectrum of pituitary stalk lesions seen at Mayo Clinic Rochester over 20 years and to determine whether specific magnetic resonance imaging (MRI) characteristics could provide clinician guidance with regard to the etiology of infundibular lesions. DESIGN A retrospective review of patients with pituitary stalk lesions seen at Mayo Clinic Rochester between 1987 and 2006 was conducted. Demographic, clinical presentation, imaging, laboratory, operative, and pathology data were reviewed and are reported using descriptive statistics. RESULTS Of the 152 pituitary stalk lesions included, 49 (32%) were neoplastic, 30 (20%) were inflammatory, 13 (9%) were congenital anomalies, and 60 (39%) were of unclear etiology. Diabetes insipidus was diagnosed in 43 (28%) of the 152 patients, and 49 (32%) patients had at least one anterior pituitary hormone deficit. Secondary hypogonadism was the most common endocrine deficiency. Eleven of 13 congenital lesions were round in appearance and 5 of 7 patients with neurosarcoidosis confirmed by pathology had a uniformly thickened pituitary stalk on MRI. There were no statistically significant correlations between hypopituitarism and the pattern of enhancement or size of the lesion. CONCLUSIONS Findings on MRI remain key in guiding the diagnosis of pituitary stalk lesions, particularly when used in conjunction with other clinical clues. There are no good imaging predictors for hypopituitarism, making clinical evaluation of all patients with pituitary stalk lesions crucial.

Neuroimaging and clinical features in type II (late-onset) Alexander disease.

Objective: To describe the imaging and clinical features in type II (late-onset) Alexander disease (AxD). Methods: We retrospectively identified all cases of type II AxD evaluated at Mayo Clinic, Rochester from January 1996 to February 2012. Clinical and neuroimaging data abstracted from the record included age at onset of symptoms, age at diagnosis, first symptom, neurologic symptoms, physical/neurologic findings on examination, genetic testing and/or biopsy (if performed), and MRI findings. Results: Thirteen patients with type II AxD were identified. Median age at onset was 38 years (range: 12–63). Five patients were female. Eleven of 13 patients had atrophy of the medulla while all 13 had medullary T2 hyperintensity. In 7 patients, these brainstem regions showed patchy enhancement. Five subjects had T2 signal change in the middle cerebellar peduncle, with associated contrast enhancement in 4 subjects. Eleven of 12 patients with T2 fluid-attenuated inversion recovery (FLAIR) imaging had pial FLAIR signal change in the medulla. Nine of 12 patients with spinal cord imaging had cord atrophy, and 3 of 9 of these evaluated with contrast had cervical cord enhancement. Conclusions: Our study confirms prior reports of atrophy and signal change of the medulla and spinal cord in late-onset AxD. We expand on previous imaging studies by identifying middle cerebellar peduncle and pial FLAIR signal changes as important diagnostic clues. Variable patchy enhancement may occur in regions of T2 hyperintensity, leading to diagnostic uncertainty. In addition, we demonstrate that previously emphasized clinical features such as palatal tremor may not be common. We affirm that age at onset predicts clinical phenotype and imaging findings.

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.

Immunoglobulin G4–Related Disease of the Orbit: Imaging Features in 27 Patients

BACKGROUND AND PURPOSE: Immunoglobulin G4–related disease is a systemic fibroinflammatory process of unknown etiology, characterized by tissue infiltration by immunoglobulin G4 plasma cells. The purpose of this study was to retrospectively identify the spectrum of imaging features seen in immunoglobulin G4–related disease of the orbit. MATERIALS AND METHODS: This study included 27 patients with biopsy-proved immunoglobulin G4–related disease of the orbit and either a CT or MR imaging of the orbits. These CT or MR imaging examinations were evaluated for the following: extraocular muscle size, extraocular muscle tendon enlargement, lacrimal gland enlargement, infiltrative process in the orbital fat (increased attenuation on CT or abnormal signal on MR imaging), infraorbital nerve enlargement, mucosal thickening in the paranasal sinuses, and extension of orbital findings intracranially. RESULTS: Extraocular muscles were enlarged in 24 of 27 (89%) patients, 21 (88%) bilaterally. In 32 of 45 (71%) affected orbits, the lateral rectus was the most enlarged muscle. In 26 (96%) patients, the tendons of the extraocular muscles were spared. Nineteen (70%) patients had lacrimal gland enlargement. Twelve (44%) patients had an infiltrative process within the orbital fat. Infraorbital nerve enlargement was seen in 8 (30%) patients. Twenty-four (89%) patients had sinus disease. Cavernous sinus or Meckel cave extension was seen in 3 (11%) patients. CONCLUSIONS: In patients with extraocular muscle enlargement, particularly when the tendons are spared and the lateral rectus is the most enlarged, and even more so when other noted findings are present, immunoglobulin G4–related disease should be a leading differential consideration, even over more commonly known etiologies of extraocular muscle enlargement.

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.

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.