Advertisement

“Black butterfly” sign on T2*-weighted and susceptibility-weighted imaging: A novel finding of chronic venous congestion of the brain stem and spinal cord associated with dural arteriovenous fistulas

      Highlights

      • We report DAVFs with progressive myelopathy showing unique MR imaging findings.
      • T2*WI/SWI showed symmetrical dark signals of the central gray matter of spinal cord.
      • We named dark signals “black butterfly” sign.
      • “Black butterfly” sign was probably caused by prolonged venous congestion.
      • Identifying this “black butterfly” sign can facilitate the diagnosis of DAVF.

      Abstract

      A dural arteriovenous fistula (DAVF) with spinal perimedullary venous drainage can cause progressive myelopathy, and it is sometimes incorrectly diagnosed as another spinal cord disease. Here we report the cases of three individuals with a DAVF (one craniocervical junction DAVF and two tentorial DAVFs) with progressive myelopathy showing unique magnetic resonance (MR) imaging findings. MR T2*WI or susceptibility-weighted imaging (SWI) demonstrated symmetrical dark signal intensity lesions predominantly in the dorsal aspect of medulla and the central gray matter of cervical spinal cord that showed the “black butterfly” silhouette. Cerebral angiography revealed DAVFs draining into anterior and posterior spinal veins. Dark signals on T2*WI and SWI were presumed to be hemorrhages, which were probably caused by prolonged venous congestion. Identifying this “black butterfly” sign can facilitate the diagnosis of DAVF, differentiating DAVF from other spinal cord diseases such as demyelinating lesions and neoplasms.

      Keywords

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Journal of the Neurological Sciences
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Cognard C.
        • Gobin Y.P.
        • Pierot L.
        • Bailly A.L.
        • Houdart E.
        • Casasco A.
        • et al.
        Cerebral dural arteriovenous fistulas: clinical and angiographic correlation with a revised classification of venous drainage.
        Radiology. 1995; 194: 671-680
        • Jellema K.
        • Tijssen C.C.
        • van Gijn J.
        Spinal dural arteriovenous fistulas: a congestive myelopathy that initially mimics a peripheral nerve disorder.
        Brain. 2006; 129: 3150-3164
        • Bowen B.C.
        • Fraser K.
        • Kochan J.P.
        • Pattany P.M.
        • Green B.A.
        • Quencer R.M.
        Spinal dural arteriovenous fistulas: evaluation with MR angiography.
        AJNR Am. J. Neuroradiol. 1995; 16: 2029-2043
        • Gilbertson J.R.
        • Miller G.M.
        • Goldman M.S.
        • Marsh W.R.
        Spinal dural arterio-venous fistulas: MR and myelographic findings.
        AJNR Am. J. Neuroradiol. 1995; 16: 2049-2057
        • Jones B.V.
        • Ernst R.J.
        • Tomsick T.A.
        • Tew J.J.
        Spinal dural arteriovenous fistulas: recognizing the spectrum of magnetic resonance imaging findings.
        J. Spinal Cord Med. 1997; 20: 43-48
        • Hurst R.W.
        • Grossman R.I.
        Peripheral spinal cord hypointensity on T2-weighted MR images: a reliable imaging sign of venous hypertensive myelopathy.
        AJNR Am. J. Neuroradiol. 2000; 21: 781-786
        • Luetmer P.H.
        • Lane J.I.
        • Gilbertson J.R.
        • Bernstein M.A.
        • Huston 3rd, J.
        • Atkinson J.L.
        Preangiographic evaluation of spinal dural arteriovenous fistulas with elliptic centric contrast-enhanced MR angiography and effect on radiation dose and volume of iodinated contrast material.
        AJNR Am. J. Neuroradiol. 2005; 26: 711-718
        • Rizvi T.
        • Garg A.
        • Gupta V.
        • Gaikwad S.B.
        • Mishra N.K.
        Role of CISS MR sequence in detection of spinal dural arteriovenous fistula.
        Clin. Radiol. Extra. 2004; 59: 78-82
        • Terwey B.
        • Becker H.
        • Thron A.K.
        • Vahldiek G.
        Gadolinium-DTPA enhanced MR imaging of spinal dural arteriovenous fistulas.
        J. Comput. Assist. Tomogr. 1989; 13: 30-37
        • Igase K.
        • Kumon Y.
        • Shoda D.
        • Fukumoto S.
        • Ohue S.
        • Ohnishi T.
        Dural arteriovenous fistula at the craniocervical junction has a close relationship with the vertebral artery.
        J. Neurol. Sci. 2011; 28: 288-293
        • Foix C.
        • Alajouanine T.
        La myelite necrotique subaguie.
        Rev. Neurol. 1926; 2 (in French): 1-42
        • DeGirolami U.
        • Zivin J.A.
        Neuropathology of experimental spinal cord ischemia in the rabbit.
        J. Neuropathol. Exp. Neurol. 1982; 41: 129-149
        • Kasliwal M.K.
        • Moftakhar R.
        • O'toole J.E.
        • Lopes D.K.
        High cervical spinal subdural hemorrhage as a harbinger of craniocervical arteriovenous fistula: an unusual clinical presentation.
        Spine J. 2015; 15: e13-e17
        • Kai Y.
        • Hamada J.
        • Morioka M.
        • Yano S.
        • Mizuno T.
        • Kuratsu J.
        Arteriovenous fistulas at the cervicomedullary junction presenting with subarachnoid hemorrhage: six case reports with special reference to the angiographic pattern of venous drainage.
        AJNR Am. J. Neuroradiol. 2005; 26: 1949-1954
        • Brunereau L.
        • Gobin Y.P.
        • Meder J.F.
        • Cognard C.
        • Tubiana J.M.
        • Merland J.J.
        Intracranial dural arteriovenous fistulas with spinal venous drainage: relation between clinical presentation and angiographic findings.
        AJNR Am. J. Neuroradiol. 1996; 17: 1549-1554
        • Mitsuhashi Y.
        • Aurboonyawat T.
        • Pereira V.M.
        • Geibprasert S.
        • Toulgoat F.
        • Ozanne A.
        • et al.
        Dural arteriovenous fistulas draining into the petrosal vein or bridging vein of the medulla: possible homologs of spinal dural arteriovenous fistulas. Clinical article.
        J. Neurosurg. 2009; 111: 889-899
        • Wakhloo A.K.
        • Patel N.V.
        • DeLeo M.J.
        • Shaibani A.
        Vascular anatomy of the spine, imaging, and endovascular treatment of spinal vascular diseases.
        in: Herkowitz H.N. The Spine. 6th edition. Rothman-Simeone, Elsevier, Amsterdam, The Netherlands2011: 1655-1687
        • Miyasaka K.
        • Asano T.
        • Ushikoshi S.
        • Hida K.
        • Koyanagi I.
        Vascular anatomy of the spinal cord and classification of spinal arteriovenous malformations.
        Interv. Neuroradiol. 2000; 6: 195-198