- Email: [email protected]
Spontaneous vertebral arteriovenous fistula causing cervical myelopathy and acute ischemic strokes treated by endovascular balloon–assisted coiling and Onyx embolization
Case Reports / Journal of Clinical Neuroscience 21 (2014) 167–170
consideration, at least, for genetic testing for CADASIL. This may be particularly important if there is a family history of stroke; given that stroke is so common, this family history could easily be dismissed as irrelevant in the assessment of suspected bvFTD. Finally, Patient 2 also suggests that CADASIL might possibly be an underrecognised diagnosis in apparent bvFTD. Screening Notch3 in a substantial and unselected cohort of FTD patients might be appropriate to investigate this possibility. Conflict of interest/disclosure The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. References
167
2. Joutal A, Vahedi K, Corpechot C, et al. Strong clustering and stereotyped nature of Notch3 mutations in CADASIL patients. Lancet 1997;350:1511–5. http:// dx.doi.org/10.1016/S0140-6736(97)08083-5. 3. Peters N, Opherk C, Danek A, et al. The pattern of cognitive performance in CADASIL: a monogenic condition leading to subcortical ischemic vascular dementia. Am J Psychiatry 2005;162:2078–85. http://dx.doi.org/10.1176/ appi.ajp.162.11.2078. 4. Brown J, Pengas G, Dawson K, et al. Self administered cognitive screening test (TYM) for detection of Alzheimer’s disease: cross sectional study. Br Med J 2009;338:b2030. http://dx.doi.org/10.1136/bmj.b2030. 5. Dichgans M, Mayer M, Uttner I, et al. The phenotypic spectrum of CADASIL: clinical findings in 102 cases. Ann Neurol 1998;44:731–9. http://dx.doi.org/ 10.1002/ana.410440506. 6. Amberla K, Wäljas M, Tuominen S, et al. Insidious cognitive decline in CADASIL. Stroke 2004;35:1598–602. http://dx.doi.org/10.1161/ 01.STR.0000129787.92085.0a. 7. Opherk C, Peters N, Herzog J, et al. Long-term prognosis and causes of death in CADASIL: a retrospective study in 411 patients. Brain 2004;127:2533–9. http:// dx.doi.org/10.1093/brain/awh282.
1. Rascovsky K, Hodges JR, Knopman D, et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain 2011;134:2456–77. http://dx.doi.org/10.1093/brain/awr179.
doi:http://dx.doi.org/10.1016/j.jocn.2013.02.025
Spontaneous vertebral arteriovenous fistula causing cervical myelopathy and acute ischemic strokes treated by endovascular balloon–assisted coiling and Onyx embolization Seby John a,⇑, Neda Jaffari b, Mei Lu b, Muhammad S. Hussain b, Ferdinand Hui b a b
Department of Neurology, Cleveland Clinic, 9500 Euclid Avenue, S100-A, Cleveland, OH 44195, USA Cerebrovascular Center, Cleveland Clinic Foundation, Cleveland, OH, USA
a r t i c l e
i n f o
Article history: Received 30 October 2012 Accepted 7 January 2013
Keywords: Cervical myelopathy Coil-assisted Onyx embolization Embolic ischemic stroke Vertebral arteriovenous fistulas
a b s t r a c t Vertebral arteriovenous fistulas (VAVF) are infrequent lesions characterized by abnormal communication of the extracranial vertebral artery or one of its branches to the surrounding venous plexuses, without the presence of any intervening vessels. We describe a rare occurrence of a patient with VAVF presenting with acute ischemic stroke, encephalomalacia from multiple prior embolic events, and cervical myelopathy, which was successfully treated by coil-assisted Onyx embolization (ev3 Endovascular, Plymouth, MN, USA) with balloon for flow arrest. Our patient demonstrates that point occlusion with embolization for VAVF can be a feasible, safe, and effective treatment option for complete obliteration of the fistula, with subsequent reduction in the volume of the intra-spinal canal venous plexus. Although it is postulated that thromboembolism is less common because of redirection of flow to the venous side of the fistula, our patient also illustrates the potential for to–fro flow in such a fistula to result in embolic injury to the distal circulation. Ó 2013 Elsevier Ltd. All rights reserved.
1. Introduction Vertebral arteriovenous fistulas (VAVF) are rare lesions characterized by abnormal communication of the extracranial vertebral artery (VA) or one of its branches to the surrounding venous plexuses, without the presence of any intervening vessels or capillaries.1 They can be either spontaneous or traumatic in origin. Neurological sequellae from vertebral arteriovenous (AV) fistulas can arise from a number of mechanisms including mechanical compression or venous hypertension of the spinal cord and secondary arterial steal. These in turn depend on the pattern of venous drainage, flow velocity, and chronicity of the AV fistula.
⇑ Corresponding author. Tel.: +1 646 714 5220; fax: +1 216 444 0230. E-mail address: [email protected] (S. John).
Obliteration of the AV fistula with preservation of the parent artery is the primary goal of treatment. We describe a rare occurrence of a patient with VAVF presenting with acute and chronic ischemic strokes and cervical myelopathy which was successfully treated by coil-assisted Onyx embolization (ev3 Endovascular, Plymouth, MN, USA) with balloon for flow arrest.
2. Case report A right-handed hypertensive patient in their 60s presented with acute onset vertigo. Examination revealed left-appendicular ataxia, left C5 innervated muscle weakness, decreased left bicep reflex, and hyperreflexia in both legs. A review of systems was positive for pain and hyperesthesia in the left arm for 10 years.
168
Case Reports / Journal of Clinical Neuroscience 21 (2014) 167–170
2.1. Neuroimaging MRI of the brain showed scattered punctuate acute infarcts in the left cerebellum with background encephalomalacia (Fig. 1). MRI of the cervical spine revealed a lobulated, extramedullary enhancing structure adjacent to the left neural foramen at C2–3 with cord compression (Fig. 2). Digital subtraction angiography showed an ectatic left V3 segment with pseudoaneurysm measuring 3.8 3.4 2.3 cm, and slow drainage into the left suboccipital plexus and into an epidural vein within the spinal canal causing cord compression. Disconnection of the fistula was performed with temporary occlusion using an ASCENT occlusion balloon (DePuy, Raynham, MA, USA), followed by the deployment of four 0.018’’ bare platinum coils into the shunt followed by injection of Onyx LES-34 (eV3 Endovascular), which filled the coil frame selectively (Fig. 3).
Acetylsalicylic acid and atorvastatin were initiated for secondary stroke prevention. The cerebellar symptoms resolved and there was mild improvement in the left arm weakness. Follow-up electromyography (EMG) 2 months later showed normal sensory studies and severe chronic motor axon loss in the C5–7 myotomes bilaterally. There was active motor axonal loss in the left C5–7 myotomes suggesting ongoing compression of the spinal anterior horn cell or root. MRI after 4 months revealed continued prominent left sided epidural intra-canal flow voids, most notable at the C4–5 level. Repeat embolization was attempted, but minimal flow into the pouch was observed, and no significant change of angiographic flow could be achieved. Follow-up MRI after 11 months demonstrated involution of the epidural vein, with return of the spinal cord to near midline. There were no new areas of infarction when compared to the imaging at the onset of symptoms.
Fig. 1. (a) Axial diffusion weighted sequence MRI showing scattered acute ischemic infarcts in the superior cerebellar artery and posterior inferior cerebellar artery territory of the left cerebellar hemisphere. (b) Axial, (c) coronal and (d) sagittal CT angiography images showing a complex pseudoaneurysm (arrow) arising from the left vertebral artery between C1 and C2 on the left side.
Fig. 2. (a) Sagittal fat-suppressed short T1 inversion recovery MRI of the cervical spine showing a lobulated soft tissue mass arising adjacent to C2–3, compressing the thecal sac and spinal cord on the left as seen on (b) axial gradient echo and (c) axial T1-weighted sequences. (d) Sagittal fat-suppressed short T1 inversion recovery MRI of the cervical spine post coil and Onyx embolization (ev3 Endovascular, Plymouth, MA, USA) showing obliteration of the vascular mass.
169
Case Reports / Journal of Clinical Neuroscience 21 (2014) 167–170
Fig. 3. (a) Anteroposterior angiographic images of the vertebral arteriovenous fistula (VAVF) filling from the left vertebral artery. (b) Microcatheter angiography of VAVF showing filling of pouch and venous plexus. (c) An ASCENT balloon (DePuy, Raynham, MA, USA) was placed in the fistulous connection. Following (d) detachable coil placement and (e) Onyx (ev3 Endovascular, Plymouth, MN, USA) embolization, obliteration of the fistulous connection is shown (f) without residual filling of the VAVF.
Table 1 Endovascular modalities of vertebral arteriovenous fistula treatment Sl. number
Modality
Reference
1.
Detachable balloons
2.
Detachable coils
3. 4. 5.
Onyx (ev3 Endovascular, Plymouth, MN, USA) liquid embolic system embolization with concomitant detachable coil embolization Nitinol vascular occlusion plug and detachable coil embolization Stent graft
R.L. Beaujeux et al.2 G. Debrun et al.6 G. Guglielmi et al.7 S. Miralbes et al.8 H. Tenjin et al.9 Q. Wang et al.10 R.W. Crowley et al.11 A. Sadato et al.12 A. Gonzalez et al.13
3. Discussion To our knowledge, this is the first reported patient with VAVF presenting with acute ischemic stroke and concomitant findings of multiple old strokes and chronic compressive myelopathy. VAVF are shunts that occur either spontaneously or from trauma.2 Traumatic causes frequently involve penetrating neck injuries3 while neurofibromatosis, fibromuscular dysplasia, and Ehlers–Danlos syndrome predispose patients to spontaneous fistulae.4 Ischemic strokes in VAVF occur from vascular steal resulting in compromised flow or from embolic phenomenon.3 Thromboembolism is thought to be less common because flow is predisposed towards the venous outlet. Given the encephalomalacia and scattered pattern of acute infarction, slow flow into the venous outlet may have resulted in reflux of venous clot, possibly during valsalva. The degree of encephalomalacia in the left cerebellum was an indication that the patient had been having multiple embolic strokes from the VAVF for a long time. Contribution from vascular steal was thought to be less likely because there was no shunting through the fistula. Myelopathy from VAVF can occur due to arterialization of spinal pial veins or direct compression, and radiculopathy due to compression of the roots from engorged spinal veins.5 However, myelopathy and radiculopathy are rare and occur less frequently
when compared to dural AV fistulas or malformations. The patient’s cervical myelopathy was chronic, as confirmed electrodiagnostically. The long-standing left arm hyperesthesia may have been caused by cord compression from the epidural vein. An EMG carried out 10 years earlier had recorded chronic axon loss changes in the C5 muscles alone. The main goal of treatment is disconnection of the fistula with preservation of the parent artery.2 Multiple options are available for treatment and the choice depends mainly on the location of the fistula and its flow dynamics (Table 1). The use of coils as an anchor point for liquid embolic agents to occlude the arteriovenous fistula produces excellent results, though this is not commonly used10 due to the risk of unintentional distal venous migration. Detachable coils can be used to decrease blood flow in the fistula and reduce distal embolization.14,15 With balloon flow arrest, Onyx can be delivered more accurately than n-butyl cyanoacrylate.16 Combining both approaches, precise occlusion was achieved and we were also able to preserve the parent VA. 4. Conclusions This patient demonstrates that point occlusion with embolization for VAVF can be a feasible, safe, and effective treatment option for complete obliteration of the fistula, with subsequent reduction
170
Case Reports / Journal of Clinical Neuroscience 21 (2014) 170–172
in the volume of the intra-spinal canal venous plexus. This patient also illustrates the potential for to–fro flow in such a fistula to result in intermittent embolic injury to the distal circulation. Point occlusion with coil and Onyx embolization resulted in cessation of embolic phenomenon, and decompression of the spinal canal. Conflicts of interest/disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. References 1. Halbach VV, Higashida RT, Hieshima GB. Treatment of vertebral arteriovenous fistulas. AJR Am J Roentgenol 1988;150:405–12. 2. Beaujeux RL, Reizine DC, Casasco A, et al. Endovascular treatment of vertebral arteriovenous fistula. Radiology 1992;183:361–7. 3. Reizine D, Laouiti M, Guimaraens L, et al. Vertebral arteriovenous fistulas: clinical presentation, angiographical appearance and endovascular treatment— a review of twenty-five cases in English, French. Ann Radiol (Paris) 1985;28:425–38. 4. Goyal M, Willinsky R, Montanera W, et al. Spontaneous vertebrovertebral arteriovenous fistulae clinical features, angioarchitecture and management of twelve patients. Interv Neuroradiol. 1999;5:219–24. 5. De Bray JM, Bertrand P, Bertrand F, et al. Spontaneous arteriovenous fistulas of the vertebral artery: apropos of a case—review of the literature in French. Rev Med Interne 1986;7:133–9.
6. Debrun G, Legre J, Kasbarian M, et al. Endovascular occlusion of vertebral fistulae by detachable balloons with conservation of the vertebral blood flow. Radiology 1979;130:141–7. 7. Guglielmi G, Vinuela F, Duckwiller GR, et al. Highflow, small-hole arteriovenous f istulas: treatment with electrodetachable coils. AJNR Am J Neuroradiol 1995;16:325–8. 8. Miralbes S, Cattin F, Andrea I, et al. Vertebral arteriovenous fistula: endovascular treatment with electrodetachable coils. Neuroradiology 1998;40:761–2. 9. Tenjin H, Kimura S, Sugawa N. Coil embolization of vertebro-vertebral arteriovenous fistula: a case report. Surg Neurol. 2005;63:80–3. 10. Wang Q, Song D, Chen G. Endovascular treatment of high-flow cervical direct vertebro-vertebral arteriovenous fistula with detach- able coils and Onyx liquid embolic agent. Acta Neurochir (Wien). 2011;153:347–52. 11. Crowley RW, Medel R, Dumont AS. Traumatic high flow vertebral-venous fistula presenting with delayed ischemic stroke: endovascular management with detachable coils and amplatzer vascular plugs. Neurosurg Focus 2009;26:E5. 12. Sadato A, Satow T, Ishii A, et al. Large vertebral arteriovenous f istula treated with stent-grafts. Neurol Med Chir (Tokyo) 2003;43:250–4. 13. González A, Mayol A, Gil-Peralta A, et al. Endovascular stent-graft treatment of an iatrogenic vertebral arteriovenous fistula. Neuroradiology 2001;43: 784–6. 14. Luo CB, Teng MM, Chang FC, et al. Endovascular treatment of intracranial high-flow arteriovenous fistulas by Guglielmi detachable coils. JCMA 2006;69:80–5. 15. Spiotta AM, Sivapatham T, Teng Q, et al. Balloon-augmented carotid artery sacrifice with Onyx: a proof of concept study in a swine model. J Neurointerv Surg 2011;3:390–4. 16. Jan van Rooij W, Sluzewski M. Endovascular occlusion of high-flow intracranial arteriovenous shunts: technical note. Neuroradiology 2007;49:1029–31.
doi:http://dx.doi.org/10.1016/j.jocn.2013.01.016
Guillain–Barré syndrome–like–onset neurosarcoidosis positive for immunoglobulin G anti-N-acetylgalactosaminyl-GD1a antibody H. Chatani a,⇑, M. Tanaka a, T. Nagata a, T. Araki a, S. Kusunoki b a b
Department of Neurology, Hiroshima Red Cross Hospital and Atomic-bomb Survivors Hospital, 1-9-6 Sendamachi, Naka-ku, Hiroshima 730-0052, Japan Department of Neurology, School of Medicine, Kinki University, Osaka, Japan
a r t i c l e
i n f o
Article history: Received 24 September 2012 Accepted 3 January 2013
Keywords: Ganglioside Guillain–Barré syndrome Mononeuritis multiplex Sarcoidosis Steroids
a b s t r a c t Anti-ganglioside antibodies have been reported in various peripheral neuropathies, including Guillain–Barré syndrome (GBS), chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy, Fisher syndrome, monoclonal gammopathy-associated neuropathy, and other idiopathic neuropathies. To our knowledge, there has been no report of anti-ganglioside-positive sarcoidosis. We report a 62-year-old man with acute weakness of the limbs and sensory disturbance of the right arm and trunk resembling GBS. Soluble interleukin-2 receptor and angiotensin-converting enzyme levels were elevated. Anti-ganglioside antibodies (immunoglobulin G anti-N-acetylgalactosaminyl-GD1a antibody [IgG anti-GalNAc-GD1a antibody]) were detected. Neurophysiological examination demonstrated axonal neuropathy. Bilateral hilar lymphadenopathy was demonstrated on a chest CT scan, and abnormal uptake of 67Gallium was detected by scintigraphy. The ratio of CD4 to CD8 was elevated in bronchoalveolar lavage fluid. Noncaseating epithelioid cell granulomas were detected in a specimen obtained via transbronchial lung biopsy. Because intravenous immunoglobulin did not improve the symptoms, we commenced steroid pulse therapy followed by oral prednisolone therapy. After steroid therapy, he recovered fully. Because the findings in our patient fulfilled the criteria for neurosarcoidosis, we diagnosed his illness as probable neurosarcoidosis. To the best of our knowledge, this is the first patient with GBS–like– onset neurosarcoidosis positive for anti-IgG anti-GalNAc-GD1a antibody. Ó 2013 Elsevier Ltd. All rights reserved.
1. Introduction Sarcoidosis is a multisystem granulomatous inflammatory disease. Peripheral neuropathies occur in 15% of neurosarcoidosis ⇑ Corresponding author. Tel.: +81 82 241 3111; fax: +81 82 246 0676. E-mail address: [email protected] (H. Chatani).
patients.1 Mononeuritis multiplex is seen in 10% of patients with limb sarcoidosis neuropathy,2 and neuropathies rarely present in a fashion similar to Guillain–Barré syndrome (GBS). We report, to our knowledge, the first patient with GBS–like–onset neurosarcoidosis positive for anti-ganglioside antibodies (immunoglobulin G anti N-acetylgalactosaminyl-GD1a antibody [IgG anti-GalNAcGD1a antibody]).
consideration, at least, for genetic testing for CADASIL. This may be particularly important if there is a family history of stroke; given that stroke is so common, this family history could easily be dismissed as irrelevant in the assessment of suspected bvFTD. Finally, Patient 2 also suggests that CADASIL might possibly be an underrecognised diagnosis in apparent bvFTD. Screening Notch3 in a substantial and unselected cohort of FTD patients might be appropriate to investigate this possibility. Conflict of interest/disclosure The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. References
167
2. Joutal A, Vahedi K, Corpechot C, et al. Strong clustering and stereotyped nature of Notch3 mutations in CADASIL patients. Lancet 1997;350:1511–5. http:// dx.doi.org/10.1016/S0140-6736(97)08083-5. 3. Peters N, Opherk C, Danek A, et al. The pattern of cognitive performance in CADASIL: a monogenic condition leading to subcortical ischemic vascular dementia. Am J Psychiatry 2005;162:2078–85. http://dx.doi.org/10.1176/ appi.ajp.162.11.2078. 4. Brown J, Pengas G, Dawson K, et al. Self administered cognitive screening test (TYM) for detection of Alzheimer’s disease: cross sectional study. Br Med J 2009;338:b2030. http://dx.doi.org/10.1136/bmj.b2030. 5. Dichgans M, Mayer M, Uttner I, et al. The phenotypic spectrum of CADASIL: clinical findings in 102 cases. Ann Neurol 1998;44:731–9. http://dx.doi.org/ 10.1002/ana.410440506. 6. Amberla K, Wäljas M, Tuominen S, et al. Insidious cognitive decline in CADASIL. Stroke 2004;35:1598–602. http://dx.doi.org/10.1161/ 01.STR.0000129787.92085.0a. 7. Opherk C, Peters N, Herzog J, et al. Long-term prognosis and causes of death in CADASIL: a retrospective study in 411 patients. Brain 2004;127:2533–9. http:// dx.doi.org/10.1093/brain/awh282.
1. Rascovsky K, Hodges JR, Knopman D, et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain 2011;134:2456–77. http://dx.doi.org/10.1093/brain/awr179.
doi:http://dx.doi.org/10.1016/j.jocn.2013.02.025
Spontaneous vertebral arteriovenous fistula causing cervical myelopathy and acute ischemic strokes treated by endovascular balloon–assisted coiling and Onyx embolization Seby John a,⇑, Neda Jaffari b, Mei Lu b, Muhammad S. Hussain b, Ferdinand Hui b a b
Department of Neurology, Cleveland Clinic, 9500 Euclid Avenue, S100-A, Cleveland, OH 44195, USA Cerebrovascular Center, Cleveland Clinic Foundation, Cleveland, OH, USA
a r t i c l e
i n f o
Article history: Received 30 October 2012 Accepted 7 January 2013
Keywords: Cervical myelopathy Coil-assisted Onyx embolization Embolic ischemic stroke Vertebral arteriovenous fistulas
a b s t r a c t Vertebral arteriovenous fistulas (VAVF) are infrequent lesions characterized by abnormal communication of the extracranial vertebral artery or one of its branches to the surrounding venous plexuses, without the presence of any intervening vessels. We describe a rare occurrence of a patient with VAVF presenting with acute ischemic stroke, encephalomalacia from multiple prior embolic events, and cervical myelopathy, which was successfully treated by coil-assisted Onyx embolization (ev3 Endovascular, Plymouth, MN, USA) with balloon for flow arrest. Our patient demonstrates that point occlusion with embolization for VAVF can be a feasible, safe, and effective treatment option for complete obliteration of the fistula, with subsequent reduction in the volume of the intra-spinal canal venous plexus. Although it is postulated that thromboembolism is less common because of redirection of flow to the venous side of the fistula, our patient also illustrates the potential for to–fro flow in such a fistula to result in embolic injury to the distal circulation. Ó 2013 Elsevier Ltd. All rights reserved.
1. Introduction Vertebral arteriovenous fistulas (VAVF) are rare lesions characterized by abnormal communication of the extracranial vertebral artery (VA) or one of its branches to the surrounding venous plexuses, without the presence of any intervening vessels or capillaries.1 They can be either spontaneous or traumatic in origin. Neurological sequellae from vertebral arteriovenous (AV) fistulas can arise from a number of mechanisms including mechanical compression or venous hypertension of the spinal cord and secondary arterial steal. These in turn depend on the pattern of venous drainage, flow velocity, and chronicity of the AV fistula.
⇑ Corresponding author. Tel.: +1 646 714 5220; fax: +1 216 444 0230. E-mail address: [email protected] (S. John).
Obliteration of the AV fistula with preservation of the parent artery is the primary goal of treatment. We describe a rare occurrence of a patient with VAVF presenting with acute and chronic ischemic strokes and cervical myelopathy which was successfully treated by coil-assisted Onyx embolization (ev3 Endovascular, Plymouth, MN, USA) with balloon for flow arrest.
2. Case report A right-handed hypertensive patient in their 60s presented with acute onset vertigo. Examination revealed left-appendicular ataxia, left C5 innervated muscle weakness, decreased left bicep reflex, and hyperreflexia in both legs. A review of systems was positive for pain and hyperesthesia in the left arm for 10 years.
168
Case Reports / Journal of Clinical Neuroscience 21 (2014) 167–170
2.1. Neuroimaging MRI of the brain showed scattered punctuate acute infarcts in the left cerebellum with background encephalomalacia (Fig. 1). MRI of the cervical spine revealed a lobulated, extramedullary enhancing structure adjacent to the left neural foramen at C2–3 with cord compression (Fig. 2). Digital subtraction angiography showed an ectatic left V3 segment with pseudoaneurysm measuring 3.8 3.4 2.3 cm, and slow drainage into the left suboccipital plexus and into an epidural vein within the spinal canal causing cord compression. Disconnection of the fistula was performed with temporary occlusion using an ASCENT occlusion balloon (DePuy, Raynham, MA, USA), followed by the deployment of four 0.018’’ bare platinum coils into the shunt followed by injection of Onyx LES-34 (eV3 Endovascular), which filled the coil frame selectively (Fig. 3).
Acetylsalicylic acid and atorvastatin were initiated for secondary stroke prevention. The cerebellar symptoms resolved and there was mild improvement in the left arm weakness. Follow-up electromyography (EMG) 2 months later showed normal sensory studies and severe chronic motor axon loss in the C5–7 myotomes bilaterally. There was active motor axonal loss in the left C5–7 myotomes suggesting ongoing compression of the spinal anterior horn cell or root. MRI after 4 months revealed continued prominent left sided epidural intra-canal flow voids, most notable at the C4–5 level. Repeat embolization was attempted, but minimal flow into the pouch was observed, and no significant change of angiographic flow could be achieved. Follow-up MRI after 11 months demonstrated involution of the epidural vein, with return of the spinal cord to near midline. There were no new areas of infarction when compared to the imaging at the onset of symptoms.
Fig. 1. (a) Axial diffusion weighted sequence MRI showing scattered acute ischemic infarcts in the superior cerebellar artery and posterior inferior cerebellar artery territory of the left cerebellar hemisphere. (b) Axial, (c) coronal and (d) sagittal CT angiography images showing a complex pseudoaneurysm (arrow) arising from the left vertebral artery between C1 and C2 on the left side.
Fig. 2. (a) Sagittal fat-suppressed short T1 inversion recovery MRI of the cervical spine showing a lobulated soft tissue mass arising adjacent to C2–3, compressing the thecal sac and spinal cord on the left as seen on (b) axial gradient echo and (c) axial T1-weighted sequences. (d) Sagittal fat-suppressed short T1 inversion recovery MRI of the cervical spine post coil and Onyx embolization (ev3 Endovascular, Plymouth, MA, USA) showing obliteration of the vascular mass.
169
Case Reports / Journal of Clinical Neuroscience 21 (2014) 167–170
Fig. 3. (a) Anteroposterior angiographic images of the vertebral arteriovenous fistula (VAVF) filling from the left vertebral artery. (b) Microcatheter angiography of VAVF showing filling of pouch and venous plexus. (c) An ASCENT balloon (DePuy, Raynham, MA, USA) was placed in the fistulous connection. Following (d) detachable coil placement and (e) Onyx (ev3 Endovascular, Plymouth, MN, USA) embolization, obliteration of the fistulous connection is shown (f) without residual filling of the VAVF.
Table 1 Endovascular modalities of vertebral arteriovenous fistula treatment Sl. number
Modality
Reference
1.
Detachable balloons
2.
Detachable coils
3. 4. 5.
Onyx (ev3 Endovascular, Plymouth, MN, USA) liquid embolic system embolization with concomitant detachable coil embolization Nitinol vascular occlusion plug and detachable coil embolization Stent graft
R.L. Beaujeux et al.2 G. Debrun et al.6 G. Guglielmi et al.7 S. Miralbes et al.8 H. Tenjin et al.9 Q. Wang et al.10 R.W. Crowley et al.11 A. Sadato et al.12 A. Gonzalez et al.13
3. Discussion To our knowledge, this is the first reported patient with VAVF presenting with acute ischemic stroke and concomitant findings of multiple old strokes and chronic compressive myelopathy. VAVF are shunts that occur either spontaneously or from trauma.2 Traumatic causes frequently involve penetrating neck injuries3 while neurofibromatosis, fibromuscular dysplasia, and Ehlers–Danlos syndrome predispose patients to spontaneous fistulae.4 Ischemic strokes in VAVF occur from vascular steal resulting in compromised flow or from embolic phenomenon.3 Thromboembolism is thought to be less common because flow is predisposed towards the venous outlet. Given the encephalomalacia and scattered pattern of acute infarction, slow flow into the venous outlet may have resulted in reflux of venous clot, possibly during valsalva. The degree of encephalomalacia in the left cerebellum was an indication that the patient had been having multiple embolic strokes from the VAVF for a long time. Contribution from vascular steal was thought to be less likely because there was no shunting through the fistula. Myelopathy from VAVF can occur due to arterialization of spinal pial veins or direct compression, and radiculopathy due to compression of the roots from engorged spinal veins.5 However, myelopathy and radiculopathy are rare and occur less frequently
when compared to dural AV fistulas or malformations. The patient’s cervical myelopathy was chronic, as confirmed electrodiagnostically. The long-standing left arm hyperesthesia may have been caused by cord compression from the epidural vein. An EMG carried out 10 years earlier had recorded chronic axon loss changes in the C5 muscles alone. The main goal of treatment is disconnection of the fistula with preservation of the parent artery.2 Multiple options are available for treatment and the choice depends mainly on the location of the fistula and its flow dynamics (Table 1). The use of coils as an anchor point for liquid embolic agents to occlude the arteriovenous fistula produces excellent results, though this is not commonly used10 due to the risk of unintentional distal venous migration. Detachable coils can be used to decrease blood flow in the fistula and reduce distal embolization.14,15 With balloon flow arrest, Onyx can be delivered more accurately than n-butyl cyanoacrylate.16 Combining both approaches, precise occlusion was achieved and we were also able to preserve the parent VA. 4. Conclusions This patient demonstrates that point occlusion with embolization for VAVF can be a feasible, safe, and effective treatment option for complete obliteration of the fistula, with subsequent reduction
170
Case Reports / Journal of Clinical Neuroscience 21 (2014) 170–172
in the volume of the intra-spinal canal venous plexus. This patient also illustrates the potential for to–fro flow in such a fistula to result in intermittent embolic injury to the distal circulation. Point occlusion with coil and Onyx embolization resulted in cessation of embolic phenomenon, and decompression of the spinal canal. Conflicts of interest/disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. References 1. Halbach VV, Higashida RT, Hieshima GB. Treatment of vertebral arteriovenous fistulas. AJR Am J Roentgenol 1988;150:405–12. 2. Beaujeux RL, Reizine DC, Casasco A, et al. Endovascular treatment of vertebral arteriovenous fistula. Radiology 1992;183:361–7. 3. Reizine D, Laouiti M, Guimaraens L, et al. Vertebral arteriovenous fistulas: clinical presentation, angiographical appearance and endovascular treatment— a review of twenty-five cases in English, French. Ann Radiol (Paris) 1985;28:425–38. 4. Goyal M, Willinsky R, Montanera W, et al. Spontaneous vertebrovertebral arteriovenous fistulae clinical features, angioarchitecture and management of twelve patients. Interv Neuroradiol. 1999;5:219–24. 5. De Bray JM, Bertrand P, Bertrand F, et al. Spontaneous arteriovenous fistulas of the vertebral artery: apropos of a case—review of the literature in French. Rev Med Interne 1986;7:133–9.
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doi:http://dx.doi.org/10.1016/j.jocn.2013.01.016
Guillain–Barré syndrome–like–onset neurosarcoidosis positive for immunoglobulin G anti-N-acetylgalactosaminyl-GD1a antibody H. Chatani a,⇑, M. Tanaka a, T. Nagata a, T. Araki a, S. Kusunoki b a b
Department of Neurology, Hiroshima Red Cross Hospital and Atomic-bomb Survivors Hospital, 1-9-6 Sendamachi, Naka-ku, Hiroshima 730-0052, Japan Department of Neurology, School of Medicine, Kinki University, Osaka, Japan
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Article history: Received 24 September 2012 Accepted 3 January 2013
Keywords: Ganglioside Guillain–Barré syndrome Mononeuritis multiplex Sarcoidosis Steroids
a b s t r a c t Anti-ganglioside antibodies have been reported in various peripheral neuropathies, including Guillain–Barré syndrome (GBS), chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy, Fisher syndrome, monoclonal gammopathy-associated neuropathy, and other idiopathic neuropathies. To our knowledge, there has been no report of anti-ganglioside-positive sarcoidosis. We report a 62-year-old man with acute weakness of the limbs and sensory disturbance of the right arm and trunk resembling GBS. Soluble interleukin-2 receptor and angiotensin-converting enzyme levels were elevated. Anti-ganglioside antibodies (immunoglobulin G anti-N-acetylgalactosaminyl-GD1a antibody [IgG anti-GalNAc-GD1a antibody]) were detected. Neurophysiological examination demonstrated axonal neuropathy. Bilateral hilar lymphadenopathy was demonstrated on a chest CT scan, and abnormal uptake of 67Gallium was detected by scintigraphy. The ratio of CD4 to CD8 was elevated in bronchoalveolar lavage fluid. Noncaseating epithelioid cell granulomas were detected in a specimen obtained via transbronchial lung biopsy. Because intravenous immunoglobulin did not improve the symptoms, we commenced steroid pulse therapy followed by oral prednisolone therapy. After steroid therapy, he recovered fully. Because the findings in our patient fulfilled the criteria for neurosarcoidosis, we diagnosed his illness as probable neurosarcoidosis. To the best of our knowledge, this is the first patient with GBS–like– onset neurosarcoidosis positive for anti-IgG anti-GalNAc-GD1a antibody. Ó 2013 Elsevier Ltd. All rights reserved.
1. Introduction Sarcoidosis is a multisystem granulomatous inflammatory disease. Peripheral neuropathies occur in 15% of neurosarcoidosis ⇑ Corresponding author. Tel.: +81 82 241 3111; fax: +81 82 246 0676. E-mail address: [email protected] (H. Chatani).
patients.1 Mononeuritis multiplex is seen in 10% of patients with limb sarcoidosis neuropathy,2 and neuropathies rarely present in a fashion similar to Guillain–Barré syndrome (GBS). We report, to our knowledge, the first patient with GBS–like–onset neurosarcoidosis positive for anti-ganglioside antibodies (immunoglobulin G anti N-acetylgalactosaminyl-GD1a antibody [IgG anti-GalNAcGD1a antibody]).