Downloaded from http://pn.bmj.com/ on October 6, 2017 - Published by group.bmj.com
NEUROLOGICAL RARITIES
Reversible brainstem dysfunction from spinal arterio-venous fistula M D Willis,1 T Amato-Watkins,2 M Zaben,2 A Baig,2 R Corkill,1 Y Joshi3
1
Department of Neurology, University Hospital of Wales, Cardiff, UK 2 Department of Neurosurgery, University Hospital of Wales, Cardiff, UK 3 Department of Radiology, University Hospital of Wales, Cardiff, UK Correspondence to Dr Mark Willis, Department of Neurology, University Hospital of Wales, Heath Park, Cardiff CF14 4XN, UK; [email protected] Published Online First 26 June 2014
ABSTRACT A 45-year-old man presented with subacute onset of ataxia, diplopia, urinary retention and paraparesis. MR scan of brain showed abnormal T2 hyperintense signal within the cervical cord, medulla and lower pons and vascular appearances suggesting an arterio-venous fistula. The fistula was surgically explored and successfully disconnected with good clinical outcome. Brainstem or cervical dural arteriovenous fistulae more typically present as a myelopathy; only a handful of cases have presented with brainstem dysfunction. This is a rare but reversible cause of subacute brainstem dysfunction.
CASE REPORT A 45-year-old man presented with a 2-day history of ataxia, diplopia and urinary retention. This had begun on awakening with generalised neck pain and abdominal discomfort. He then developed horizontal
To cite: Willis MD, AmatoWatkins T, Zaben M, et al. Pract Neurol 2014;14: 425–428.
Figure 1
diplopia in the primary gaze, with vomiting. The following morning the vomiting stopped but he still felt unsteady with continuing diplopia. Additionally, he could not pass urine, but there was no sensory or motor involvement of the limbs or trunk. Eight weeks before, he had experienced a ‘whooshing’ sound in his left ear at night, which eased with pressure over the left side of his neck; this had resolved 3 weeks before the presenting symptoms. Six months before, he had reported a left occipital headache; a CT scan had been normal. Two years before, he had been diagnosed with Hodgkin’s lymphoma (stage IV), with cervical lymphadenopathy and bony involvement on positron-emission tomography. He had been in remission for 12 months following successful treatment with doxorubicin, bleomycin, vinblastine and dacarbazine. During treatment, he
MR scan showing hyperintense signal in the brainstem and upper cervical cord.
Willis MD, et al. Pract Neurol 2014;14:425–428. doi:10.1136/practneurol-2014-000872
425
Downloaded from http://pn.bmj.com/ on October 6, 2017 - Published by group.bmj.com
NEUROLOGICAL RARITIES
Figure 2
Digital subtraction angiography showing the dural arterio-venous fistula (arrow). Left vertebral injection.
had developed a deep venous thrombosis of the left axillary, subclavian and brachio-cephalic veins secondary to a peripherally inserted central catheter; this required 9 months of treatment with low molecular weight heparin. On examination, there was nystagmus to lateral gaze and bilaterally limited abduction of eye movements. The remaining cranial nerve examination was normal. In the limbs, tone was normal but there was minimal weakness of hip flexion. Reflexes were symmetrical but brisk at brachioradialis and at the knee. Plantars were flexor bilaterally, and sensory examination was normal. There was appendicular ataxia and striking truncal ataxia. MR scan of the brain showed abnormal T2 hyperintense signal within the upper cervical cord, both sides of the medulla and in the lower pons. There were prominent vessels anterior to the spinal cord and vascular flow voids in the cervical spinal canal. Time-resolved MR angiography showed abnormal vessels anterior to the spinal cord with arterial-phase flow in the left sigmoid sinus, suggesting an arteriovenous fistula of the brainstem or upper cervical cord (figure 1). A digital subtraction angiography confirmed a dural arterio-venous fistula in the region of the left sigmoid sinus, with arterial feeders from the left ascending cervical artery—a dural branch from the left posterior 426
inferior cerebellar artery—and distal branches of the left middle cerebral artery. The venous drainage extended into the brainstem and spinal perimedullary veins. We classified the dural arterio-venous fistula as being Cognard type V, due to its venous drainage into the spinal canal (figure 2).1 Three days after the onset of ataxia, he developed a moderate asymmetrical paraparesis, prompting urgent neurosurgical intervention. Because of the tortuous vascularity of the lesion, we felt that a surgical
Figure 3 Intraoperative photograph showing the dural arterio-venous fistula (arrow). Willis MD, et al. Pract Neurol 2014;14:425–428. doi:10.1136/practneurol-2014-000872
Downloaded from http://pn.bmj.com/ on October 6, 2017 - Published by group.bmj.com
NEUROLOGICAL RARITIES repeat digital subtraction angiogram has shown no recurrence of the fistula.
Figure 4 Intraoperative indocyanine green fluorescence videography (still image) showing the dural arterio-venous fistula (arrow).
approach would more likely obliterate the complex fistula than endovascular treatment. He subsequently underwent a left retrosigmoid craniectomy to enter the cerebellopontine cistern. The surgeon used intraoperative indocyanine green fluorescence videography to locate the nidus (figures 3 and 4) near the left sigmoid sinus (which appeared thrombosed) before disconnecting the arterio-venous fistula. Postoperatively, his symptoms improved. On recent follow-up, he has made an excellent recovery and a
Figure 5
DISCUSSION We present a patient with subacute brainstem dysfunction due to venous congestion from a dural arteriovenous fistula. The presentation of dural arteriovenous fistulae depends largely on the type of venous drainage. Rarely, an arterio-venous fistula drains to the spinal perimedullary veins (Cognard type V— figures 5 and 6); these have a higher clinical risk of venous hypertension in the spinal cord, and typically present as a myelopathy. Initial brainstem dysfunction, as in this patient, occurs very rarely.2–7 Dural arterio-venous fistulae can follow venous sinus thrombosis.8 This patient’s dural arterio-venous fistula probably followed a left sigmoid sinus thrombosis, possibly from previous deep venous thrombosis of the left axillary, subclavian and brachio-cephalic veins. The disappearance of the ‘whooshing’ sound may reflect a change in the venous drainage pathway, which had congested vessels draining the spinal cord. The main points of interest are the unusual presentation and the rapidity of progression once the venous congestion entered a decompensated phase. As in previous cases, our patient made a good recovery, presumably because the urgent disconnection of the
Cognard classification of dural arteriovenous fistulae.
Willis MD, et al. Pract Neurol 2014;14:425–428. doi:10.1136/practneurol-2014-000872
427
Downloaded from http://pn.bmj.com/ on October 6, 2017 - Published by group.bmj.com
NEUROLOGICAL RARITIES
Figure 6
Venous drainage of the posterior fossa.
fistula relieved the venous congestion of the brainstem and upper cervical cord. Clinicians should consider dural arterio-venous fistulae in patients presenting with subacute brainstem dysfunction. Acknowledgements We thank Janice Sharp, Senior Medical Artist at the University Hospital of Wales for figures 5 and 6. Contributors MDW cared for the patient and wrote the manuscript. TA-W cared for the patient and edited the manuscript. MZ edited the manuscript. AB cared for the patient and edited the manuscript. RC cared for the patient and edited the manuscript. YJ cared for the patient and edited the manuscript. Competing interests None. Patient consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed. This paper was reviewed by Rick Nelson, Bristol, UK.
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REFERENCES 1 Cognard C, Gobin YP, Pierot L, et al. Cerebral dural arteriovenous fistulas: clinical and angiographic correlation with a revised classification of venous drainage. Radiology 1995;194:671–80. 2 Kulwin C, Bohnstedt BN, Scott JA, et al. Dural arteriovenous fistulas presenting with brainstem
428
8
dysfunction: diagnosis and surgical treatment. Neurosurg Focus 2012;32:E10. Li J, Ezura M, Takahashi A. Intracranial dural arteriovenous fistula with venous reflux to the brainstem and spinal cord mimicking brainstem infarction--case report. Neurol Med Chir 2004;44:24–8. Oishi H, Horinaka N, Shmizu T, et al. A case of intracranial dural arteriovenous fistula presenting with brainstem infarction. No Shinkei Geka 2005;33:1095–9. Satoh M, Kuriyama M, Fujiwara T, et al. Brain stem ischemia from intracranial dural arteriovenous fistula: case report. Surg Neurol 2005;64:341–5. Lagares A, Perez-Nuñez A, Alday R, et al. Dural arteriovenous fistula presenting as brainstem ischaemia. Acta Neurochir (Wien) 2007;149:965–7. Sugiura Y, Nozaki T, Sato H, et al. Sigmoid sinus dural arteriovenous fistula with spinal venous drainage manifesting as only brainstem-related neurological deficits without myelopathy: case report. Neurol Med Chir 2009;49:71–6. Sakaki T, Morimoto T, Nakase H, et al. Dural arteriovenous fistula of the posterior fossa developing after surgical occlusion of the sigmoid sinus. Report of five cases. J Neurosurg 1996;84:113–18.
Willis MD, et al. Pract Neurol 2014;14:425–428. doi:10.1136/practneurol-2014-000872
Downloaded from http://pn.bmj.com/ on October 6, 2017 - Published by group.bmj.com
Reversible brainstem dysfunction from spinal arterio-venous fistula M D Willis, T Amato-Watkins, M Zaben, A Baig, R Corkill and Y Joshi Pract Neurol 2014 14: 425-428 originally published online June 26, 2014
doi: 10.1136/practneurol-2014-000872 Updated information and services can be found at: http://pn.bmj.com/content/14/6/425
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To request permissions go to: http://group.bmj.com/group/rights-licensing/permissions To order reprints go to: http://journals.bmj.com/cgi/reprintform To subscribe to BMJ go to: http://group.bmj.com/subscribe/
NEUROLOGICAL RARITIES
Reversible brainstem dysfunction from spinal arterio-venous fistula M D Willis,1 T Amato-Watkins,2 M Zaben,2 A Baig,2 R Corkill,1 Y Joshi3
1
Department of Neurology, University Hospital of Wales, Cardiff, UK 2 Department of Neurosurgery, University Hospital of Wales, Cardiff, UK 3 Department of Radiology, University Hospital of Wales, Cardiff, UK Correspondence to Dr Mark Willis, Department of Neurology, University Hospital of Wales, Heath Park, Cardiff CF14 4XN, UK; [email protected] Published Online First 26 June 2014
ABSTRACT A 45-year-old man presented with subacute onset of ataxia, diplopia, urinary retention and paraparesis. MR scan of brain showed abnormal T2 hyperintense signal within the cervical cord, medulla and lower pons and vascular appearances suggesting an arterio-venous fistula. The fistula was surgically explored and successfully disconnected with good clinical outcome. Brainstem or cervical dural arteriovenous fistulae more typically present as a myelopathy; only a handful of cases have presented with brainstem dysfunction. This is a rare but reversible cause of subacute brainstem dysfunction.
CASE REPORT A 45-year-old man presented with a 2-day history of ataxia, diplopia and urinary retention. This had begun on awakening with generalised neck pain and abdominal discomfort. He then developed horizontal
To cite: Willis MD, AmatoWatkins T, Zaben M, et al. Pract Neurol 2014;14: 425–428.
Figure 1
diplopia in the primary gaze, with vomiting. The following morning the vomiting stopped but he still felt unsteady with continuing diplopia. Additionally, he could not pass urine, but there was no sensory or motor involvement of the limbs or trunk. Eight weeks before, he had experienced a ‘whooshing’ sound in his left ear at night, which eased with pressure over the left side of his neck; this had resolved 3 weeks before the presenting symptoms. Six months before, he had reported a left occipital headache; a CT scan had been normal. Two years before, he had been diagnosed with Hodgkin’s lymphoma (stage IV), with cervical lymphadenopathy and bony involvement on positron-emission tomography. He had been in remission for 12 months following successful treatment with doxorubicin, bleomycin, vinblastine and dacarbazine. During treatment, he
MR scan showing hyperintense signal in the brainstem and upper cervical cord.
Willis MD, et al. Pract Neurol 2014;14:425–428. doi:10.1136/practneurol-2014-000872
425
Downloaded from http://pn.bmj.com/ on October 6, 2017 - Published by group.bmj.com
NEUROLOGICAL RARITIES
Figure 2
Digital subtraction angiography showing the dural arterio-venous fistula (arrow). Left vertebral injection.
had developed a deep venous thrombosis of the left axillary, subclavian and brachio-cephalic veins secondary to a peripherally inserted central catheter; this required 9 months of treatment with low molecular weight heparin. On examination, there was nystagmus to lateral gaze and bilaterally limited abduction of eye movements. The remaining cranial nerve examination was normal. In the limbs, tone was normal but there was minimal weakness of hip flexion. Reflexes were symmetrical but brisk at brachioradialis and at the knee. Plantars were flexor bilaterally, and sensory examination was normal. There was appendicular ataxia and striking truncal ataxia. MR scan of the brain showed abnormal T2 hyperintense signal within the upper cervical cord, both sides of the medulla and in the lower pons. There were prominent vessels anterior to the spinal cord and vascular flow voids in the cervical spinal canal. Time-resolved MR angiography showed abnormal vessels anterior to the spinal cord with arterial-phase flow in the left sigmoid sinus, suggesting an arteriovenous fistula of the brainstem or upper cervical cord (figure 1). A digital subtraction angiography confirmed a dural arterio-venous fistula in the region of the left sigmoid sinus, with arterial feeders from the left ascending cervical artery—a dural branch from the left posterior 426
inferior cerebellar artery—and distal branches of the left middle cerebral artery. The venous drainage extended into the brainstem and spinal perimedullary veins. We classified the dural arterio-venous fistula as being Cognard type V, due to its venous drainage into the spinal canal (figure 2).1 Three days after the onset of ataxia, he developed a moderate asymmetrical paraparesis, prompting urgent neurosurgical intervention. Because of the tortuous vascularity of the lesion, we felt that a surgical
Figure 3 Intraoperative photograph showing the dural arterio-venous fistula (arrow). Willis MD, et al. Pract Neurol 2014;14:425–428. doi:10.1136/practneurol-2014-000872
Downloaded from http://pn.bmj.com/ on October 6, 2017 - Published by group.bmj.com
NEUROLOGICAL RARITIES repeat digital subtraction angiogram has shown no recurrence of the fistula.
Figure 4 Intraoperative indocyanine green fluorescence videography (still image) showing the dural arterio-venous fistula (arrow).
approach would more likely obliterate the complex fistula than endovascular treatment. He subsequently underwent a left retrosigmoid craniectomy to enter the cerebellopontine cistern. The surgeon used intraoperative indocyanine green fluorescence videography to locate the nidus (figures 3 and 4) near the left sigmoid sinus (which appeared thrombosed) before disconnecting the arterio-venous fistula. Postoperatively, his symptoms improved. On recent follow-up, he has made an excellent recovery and a
Figure 5
DISCUSSION We present a patient with subacute brainstem dysfunction due to venous congestion from a dural arteriovenous fistula. The presentation of dural arteriovenous fistulae depends largely on the type of venous drainage. Rarely, an arterio-venous fistula drains to the spinal perimedullary veins (Cognard type V— figures 5 and 6); these have a higher clinical risk of venous hypertension in the spinal cord, and typically present as a myelopathy. Initial brainstem dysfunction, as in this patient, occurs very rarely.2–7 Dural arterio-venous fistulae can follow venous sinus thrombosis.8 This patient’s dural arterio-venous fistula probably followed a left sigmoid sinus thrombosis, possibly from previous deep venous thrombosis of the left axillary, subclavian and brachio-cephalic veins. The disappearance of the ‘whooshing’ sound may reflect a change in the venous drainage pathway, which had congested vessels draining the spinal cord. The main points of interest are the unusual presentation and the rapidity of progression once the venous congestion entered a decompensated phase. As in previous cases, our patient made a good recovery, presumably because the urgent disconnection of the
Cognard classification of dural arteriovenous fistulae.
Willis MD, et al. Pract Neurol 2014;14:425–428. doi:10.1136/practneurol-2014-000872
427
Downloaded from http://pn.bmj.com/ on October 6, 2017 - Published by group.bmj.com
NEUROLOGICAL RARITIES
Figure 6
Venous drainage of the posterior fossa.
fistula relieved the venous congestion of the brainstem and upper cervical cord. Clinicians should consider dural arterio-venous fistulae in patients presenting with subacute brainstem dysfunction. Acknowledgements We thank Janice Sharp, Senior Medical Artist at the University Hospital of Wales for figures 5 and 6. Contributors MDW cared for the patient and wrote the manuscript. TA-W cared for the patient and edited the manuscript. MZ edited the manuscript. AB cared for the patient and edited the manuscript. RC cared for the patient and edited the manuscript. YJ cared for the patient and edited the manuscript. Competing interests None. Patient consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed. This paper was reviewed by Rick Nelson, Bristol, UK.
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REFERENCES 1 Cognard C, Gobin YP, Pierot L, et al. Cerebral dural arteriovenous fistulas: clinical and angiographic correlation with a revised classification of venous drainage. Radiology 1995;194:671–80. 2 Kulwin C, Bohnstedt BN, Scott JA, et al. Dural arteriovenous fistulas presenting with brainstem
428
8
dysfunction: diagnosis and surgical treatment. Neurosurg Focus 2012;32:E10. Li J, Ezura M, Takahashi A. Intracranial dural arteriovenous fistula with venous reflux to the brainstem and spinal cord mimicking brainstem infarction--case report. Neurol Med Chir 2004;44:24–8. Oishi H, Horinaka N, Shmizu T, et al. A case of intracranial dural arteriovenous fistula presenting with brainstem infarction. No Shinkei Geka 2005;33:1095–9. Satoh M, Kuriyama M, Fujiwara T, et al. Brain stem ischemia from intracranial dural arteriovenous fistula: case report. Surg Neurol 2005;64:341–5. Lagares A, Perez-Nuñez A, Alday R, et al. Dural arteriovenous fistula presenting as brainstem ischaemia. Acta Neurochir (Wien) 2007;149:965–7. Sugiura Y, Nozaki T, Sato H, et al. Sigmoid sinus dural arteriovenous fistula with spinal venous drainage manifesting as only brainstem-related neurological deficits without myelopathy: case report. Neurol Med Chir 2009;49:71–6. Sakaki T, Morimoto T, Nakase H, et al. Dural arteriovenous fistula of the posterior fossa developing after surgical occlusion of the sigmoid sinus. Report of five cases. J Neurosurg 1996;84:113–18.
Willis MD, et al. Pract Neurol 2014;14:425–428. doi:10.1136/practneurol-2014-000872
Downloaded from http://pn.bmj.com/ on October 6, 2017 - Published by group.bmj.com
Reversible brainstem dysfunction from spinal arterio-venous fistula M D Willis, T Amato-Watkins, M Zaben, A Baig, R Corkill and Y Joshi Pract Neurol 2014 14: 425-428 originally published online June 26, 2014
doi: 10.1136/practneurol-2014-000872 Updated information and services can be found at: http://pn.bmj.com/content/14/6/425
These include:
References Email alerting service
Topic Collections
This article cites 8 articles, 0 of which you can access for free at: http://pn.bmj.com/content/14/6/425#BIBL Receive free email alerts when new articles cite this article. Sign up in the box at the top right corner of the online article.
Articles on similar topics can be found in the following collections Ophthalmology (130)
Notes
To request permissions go to: http://group.bmj.com/group/rights-licensing/permissions To order reprints go to: http://journals.bmj.com/cgi/reprintform To subscribe to BMJ go to: http://group.bmj.com/subscribe/
