Unique Gadolinium Enhancement Pattern in Spinal Dural Arteriovenous Fistulas

Zalewski, Nicholas L.; Rabinstein, Alejandro A.; Brinjikji, Waleed; Kaufmann, Timothy J.; Nasr, Deena M.; Ruff, Michael W.; Flanagan, Eoin P.

doi:10.1001/jamaneurol.2018.2605

Cited by 44 publications

(34 citation statements)

References 11 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…There is no consensus on the treatment option for intracranial DAVFs with brainstem engorgement. Of note, premature administration of corticosteroid could be dangerous even fatal in case of undiagnosed DAVFs with brainstem or spinal cord engorgement (74,75). Hence, precise and comprehensive diagnosis is crucial for further treatment.…”

Section: Discussionmentioning

confidence: 99%

Intracranial Dural Arteriovenous Fistulas With Brainstem Engorgement: An Under-Recognized Entity in Diagnosis and Treatment

Hou

et al. 2020

Front. Neurol.

View full text Add to dashboard Cite

Background: In rare circumstances, patients with intracranial (dural arteriovenous fistulas) DAVFs could be complicated with brainstem engorgement, which might lead to delayed or false diagnosis and subsequent improper management. Methods: On July 2th, 2019, a systematic search was conducted in the PubMed database for patients with intracranial DAVFs complicated with brainstem engorgement. Results: Sixty-eight articles reporting of 86 patients were included for final analysis. The patients were aged from 20 to 76 years (57.10 ± 12.90, n = 82). The female to male ratio was 0.68 (35:51). Thirty-three (40.2%, 33/82) patients were initially misdiagnosed as other diseases. The specific location distributions were cranio-cervical junction, cavernous sinus, superior petrosal sinus, transverse and/or sigmoid sinus, tentorium, and other sites in 27 (32.5%), 11 (13.2%), 9 (10.8%), 10 (12.0%), 21 (25.3%), and 5 (6.0%) patients, respectively. The Cognard classification of DAVFs were II, III, IV, and V in 9 (10.7%, 9/84), 1 (1.2%, 1/84), 1 (1.2%, 1/84), and 73 (86.9%, 73/84) patients. Eighteen (22%, 18/82) patients were demonstrated to have stenosis or occlusion of the draining system distal to the fistula points. The mean follow-up period was 7.86 (n = 74, range 0-60 months) months. Fifty-four (70.1%, 54/77) patients experienced a good recovery according to the mRS score. Conclusions: Intracranial DAVFs complicated with brainstem engorgement are rare entities. Initial misdiagnosis and delayed definite diagnosis are common in the past three decades. The treatment outcome is still unsatisfactory at present. Early awareness of this rare entity and efficiently utilizing the up to date investigations are of utmost importance.

show abstract

Section: Discussionmentioning

confidence: 99%

Intracranial Dural Arteriovenous Fistulas With Brainstem Engorgement: An Under-Recognized Entity in Diagnosis and Treatment

Hou

et al. 2020

Front. Neurol.

View full text Add to dashboard Cite

show abstract

“…6 The "missing-piece" sign in spinal DAVF was not found among 144 patients with alternative myelopathies. 10 The tract-specific enhancement of paraneoplastic myelopathy, "trident" enhancement pattern of sarcoid myelopathy, and linear craniocaudal strip of enhancement in spinal cord infarct have been reported or illustrated independently in other publications from other investigators, although the frequency among control myelopathies has not been as well studied. [20][21][22] While the T2-patterns were overall less useful in determining the cause (Figures 1 and 2), they remain useful for some etiologies (Table 3), such as: DAVF where the presence of flow voids dorsal to the cord accompanying a thoracic longitudinally-extensive T2-signal abnormality is very suggestive (Figure 2C.a), SCI in which owl/snake eye T2appearance can assist (diffusion weighted imaging is also useful in this scenario), and spondylotic myelopathy where the degree of narrowing and spondylotic changes on T2-weighted sequences can be useful.…”

Section: Evaluation Of Neuroimaging Alone Is Insufficient For Diagnosismentioning

confidence: 99%

“…1 The pattern of gadolinium enhancement accompanying longitudinally-extensive T2hyperintensities can be a critical clue to diagnosis and guide further investigations to confirm the diagnosis. Several gadolinium enhancement patterns have been proposed to strongly suggest a specific etiology including: linear craniocaudal strip of enhancement in spinal cord infarction (SCI); 3 tract-specific enhancement in paraneoplastic myelopathy; 4 "pancakelike" transverse band of enhancement in spondylotic myelopathy; 5 dorsal subpial enhancement 6,7 and axial "trident" sign 8 in sarcoid myelopathy; ring/partial ring enhancement in neuromyelitis optica spectrum disorders (NMOSD); 9 "missing-piece" sign in spinal dural arteriovenous fistula (DAVF); 10 and "rim" and/or "flame" signs in intramedullary spinal cord metastases. 11 Whether these can be reliably identified or distinguished has not yet been established.…”

Section: Introductionmentioning

confidence: 99%

Utility of MRI Enhancement Pattern in Myelopathies With Longitudinally Extensive T2 Lesions

et al. 2021

Self Cite

View full text Add to dashboard Cite

ObjectiveTo determine if MRI gadolinium enhancement patterns in myelopathies with longitudinally-extensive T2-lesions can be reliably distinguished and assist in diagnosis.MethodsWe retrospectively identified 74 Mayo Clinic patients (1/1/1996–12/31/2019) fulfilling the following criteria: 1) Clinical myelopathy; 2) MRI spine available; 3) Longitudinally-extensive T2-hyperintensity (≥3 vertebral segments); and 4) Characteristic gadolinium enhancement pattern associated with a specific myelopathy etiology. Thirty-nine cases with alternative myelopathy etiologies, without previously described enhancement patterns, were included as controls. Two independent readers, educated on enhancement patterns, reviewed T2-weighted and post-gadolinium T1-weighted images and selected the diagnosis based on this knowledge. These were compared to the true diagnoses and agreement was measured with Kappa coefficient.ResultsAmong all cases and controls (n=113), there was excellent agreement for diagnosis using post-gadolinium images (Kappa, 0.76) but poor agreement with T2-weighted characteristics alone (Kappa, 0.25). A correct diagnosis was more likely when assessing post-gadolinium image characteristics than with T2-weighted images alone (Rater 1: 100/113 [88%] vs 61/113 [54%] correct, p<0.0001; Rater 2: 95/113 [84%] vs 68/113 [60%] correct, p<0.0001). Of the 74 with characteristic enhancement patterns, 55 (74%) were assigned an alternative incorrect or non-specific diagnosis when originally evaluated in clinical practice, 12 (16%) received immunotherapy for non-inflammatory myelopathies, and 2 (3%) underwent unnecessary spinal cord biopsy.ConclusionsMisdiagnosis of myelopathies is common. The gadolinium enhancement patterns characteristic of specific diagnoses can be identified with excellent agreement between raters educated on this topic. This study highlights the potential diagnostic utility of enhancement patterns in myelopathies with longitudinally-extensive T2-lesions.

show abstract

“…1 Early identification of sDAVF via magnetic resonance angiography (MRA) or conventional angiography improves outcomes. 2 The clinical course and conus involvement make this a consideration, although signal in the upper thoracic cord is unusual.…”

Section: Sectionmentioning

confidence: 99%

Clinical Reasoning: A 70-year-old man with rapid stepwise paraparesis and sensory loss

2020

Self Cite

View full text Add to dashboard Cite

A 70-year-old man with a history of cardioembolic stroke, hypertension, hyperlipidemia, peripheral vascular disease, and recently diagnosed myelodysplastic syndrome presented to an outside facility with acute sensory loss in the left buttock and lower extremity without weakness. Spine MRI demonstrated nonenhancing T2-hyperintense lesions in the upper thoracic cord and conus medullaris. MRI brain showed areas of nonspecific T2-hyperintensity. CSF revealed 20 total nucleated cells (TNC)/μL; 11,000 red blood cells (RBC)/μL; normal glucose, protein, and immunoglobulin G (IgG) index; 0 unique CSF oligoclonal bands; negative Gram stain, herpes simplex virus (HSV) 1/2 PCR, and Borrelia PCR; cytology was normal. Pleocytosis was attributed to traumatic lumbar puncture (utilizing correction of 1 TNC/μL per 500 RBC/μL), and history was inconsistent with HSV or subarachnoid hemorrhage as a cause of elevated RBCs. Serum aquaporin-4-IgG was negative. Mild anemia and thrombocytopenia were apparent. He was discharged with a provisional diagnosis of demyelinating disease. Three weeks later, he developed acute paraparesis with bladder and bowel dysfunction within hours. Repeat MRI at the outside institution showed enlargement of the nonenhancing lesions in the upper thoracic cord, now longitudinally extensive (≥3 vertebral body segments), and the lower thoracic cord extending into the conus (figure, A and E). Given concern for progressive demyelination due to motor decline and expanding lesions, IV methylprednisolone was initiated. Despite treatment, his paraparesis worsened and he was transferred to our facility. Collateral history revealed 2 months of fatigue attributed to myelodysplastic syndrome, but no preceding infections, travel, or rashes. The patient was afebrile and hemodynamically stable. Neurologic examination demonstrated asymmetric flaccid paraparesis with plegia of the left lower extremity, saddle anesthesia, and loss of vibratory and pain sensation to the midthigh bilaterally with no truncal sensory level. He was areflexic in the lower extremities with mute plantar responses. Questions for consideration: 1. What is the best localization of the clinical presentation? 2. What diagnoses should be considered?

show abstract

Unique Gadolinium Enhancement Pattern in Spinal Dural Arteriovenous Fistulas

Cited by 44 publications

References 11 publications

Intracranial Dural Arteriovenous Fistulas With Brainstem Engorgement: An Under-Recognized Entity in Diagnosis and Treatment

Intracranial Dural Arteriovenous Fistulas With Brainstem Engorgement: An Under-Recognized Entity in Diagnosis and Treatment

Utility of MRI Enhancement Pattern in Myelopathies With Longitudinally Extensive T2 Lesions

Clinical Reasoning: A 70-year-old man with rapid stepwise paraparesis and sensory loss

Contact Info

Product

Resources

About