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Cortical blindness as a rare presentation of cerebral venous thrombosis

Cortical blindness as a rare presentation of cerebral venous thrombosis JSCR 2013; 5 (3 pages) doi:10.1093/jscr/rjt035 Case Report Cortical blindness as a rare presentation of cerebral venous thrombosis 1,2,* 3 2,4 2,4 Bonnie Wang , Seong-Jin Moon , William C. Olivero and Huan Wang Department of Internal Medicine, University of Illinois, College of Medicine at Urbana-Champaign, Urbana, IL, USA, 2 3 Carle Foundation Hospital, Urbana, IL, USA, University of Illinois, College of Medicine at Urbana-Champaign, Urbana, IL, USA and Department of Neurological Surgery, University of Illinois, College of Medicine at Urbana- Champaign, Urbana, IL, USA *Correspondence address. University of Illinois, College of Medicine at Urbana-Champaign, Carle Foundation Hospital, 611 West Park Street, Urbana, IL 61801. Tel: þ1-(217)-383-3110; Fax: þ1-(217)-244-0621; E-mail: wsu4@illinois.edu Received 27 February 2013; accepted 7 April 2013 Cerebral venous thrombosis (CVT) remains a diagnostic and therapeutic challenge for clini- cians. Manifesting in a remarkably wide spectrum of symptoms and signs, CVT often presents in a misleading fashion—if unrecognized or misdiagnosed, it carries potentially fatal conse- quences. Visual loss is quite rare as the initial presentation of CVT and is typically a finding more frequent in chronic cases with associated papilledema on funduscopy Ferro, Lopes, Rosas and Fontes (Delay in Hospital Admission of Patients with Cerebral Vein and Dural Sinus Thrombosis. Cerebrovasc Dis 2005;19:152 – 6). We report a rare case of acute cortical blind- ness as the initial presentation of CVT in an 18-year-old female patient and review the current literature. INTRODUCTION 4þ in the lower extremities. No ankle clonus was noted, and plantar reflexes were equivocal. Cerebral venous thrombosis (CVT) remains a diagnostic and Cranial computed tomography (CT) demonstrated subtle therapeutic challenge for clinicians. Visual loss is quite rare as hypodensity mainly in the left occipital lobe, with a character- the initial presentation of CVT and is typically a finding more istic empty delta sign (Fig. 1). Computed tomography angiog- frequent in chronic cases with associated papilledema on fun- raphy demonstrated normal filling of the posterior cerebral duscopy [1]. We report a case of acute cortical blindness as arteries (PCA). Cranial magnetic resonance imaging (MRI) the initial presentation of CVT in an 18-year-old female evaluations demonstrated bilateral ischemic changes involv- patient. ing the occipital and parietal lobes, more extensively on the left (Fig. 2). Magnetic resonance venography (MRV) demon- strated complete occlusion of the left transverse and sigmoid CASE REPORT sinuses, as well as near occlusion of the posterior superior sa- An 18-year-old female with a three-week history of headaches gittal sinus (Fig. 3C). The patient was treated emergently with endovascular mech- presented with an acute onset of blindness in both eyes after anical thrombolysis with technical success (Fig. 3Aand B), her afternoon nap. Physical examination demonstrated normal pupils and funduscopic exam, but without light perception in followed by systemic anticoagulation therapy. Post-intervention MRV demonstrated persistent recanalization of the left trans- either eye. Cranial nerve examinations were otherwise unre- verse and sigmoid sinuses, along with much improved flow in markable. Speech was intact with no naming or repetition def- icits. Although alert, she was mildly confused. No isolated the posterior superior sagittal sinus (Fig. 3D). She was subsequently diagnosed with mixed connective motor or sensory deficits were identified in the extremities. tissue disease. Clinically, she recovered the left visual field Deep tendon reflexes were 3þ in the upper extremities and Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved.# The Author 2013. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/ by-nc/3.0/), which permits non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com. Page 2 of 3 B. Wang et al. but continued to have a dense right homonymous hemianopsia substance abuse; infections and head trauma [3]. In 20–35% at discharge. She returned to her home state and was unfortu- of CVT cases, however, the etiology is unknown. nately lost to further follow-up. Patients with CVT commonly present with headache, papil- loedema, motor or sensory deficits, seizures or conscious dis- turbance. Headache is the most common symptom, present in more than 90% of patients either in a gradual manner or as a DISCUSSION sudden onset of severe headache (thunderclap headache). CVT comprises 0.5–1% of all strokes [2]. First described in Only half (40–60%) of CVT patients develop subsequent 1825, CVT occurs much less frequently than arterial throm- cerebral lesions and neurologic deficits. Focal seizures have bosis but can present in an atypical fashion leading to mis- been noted to develop in 30–40% of CVT cases, which is diagnosis or delayed diagnosis [1]. Cortical blindness much higher than in patients with arterial stroke [3]. represents a rare presentation of CVT, and has not been well Diagnosis of CVT is substantiated with neuroimaging. An reported in the literature. abnormal sinus signal coupled with an absence of flow on While CVT can present at all ages, it is more common in MRV strengthens the diagnosis. If diagnosis is still uncertain young adults and neonates. CVT primarily affects females at this stage, cerebral angiography may be attempted as it pro- (typically women of childbearing age), and recent reports in- vides definitive information of the cerebral arterial and venous dicate an increased frequency attributed to use of oral contra- system. An empty delta sign is the most commonly observed ceptives [3]. Major causes of CVT constitute prothrombotic sign of CVT on CT imaging (such as our patient), but this is conditions such as thrombophilia, pregnancy and puerperium; only present in 20% of cases. the use of certain medications such as oral contraceptives or Main treatment options are anticoagulation, fibrinolytic therapy, thrombolysis (either directly or mechanically) and/or management of intracranial pressure [3]. Heparinization offered mortality rates typically ,10%, which was often attributed to the underlying disease rather than CVT. If the patient experiences a raised intracranial pressure or worsened clinical state after anticoagulation, thrombolytic therapy is suggested. Presenting without light perception in either eye, our patient was treated emergently with mechanical thromb- olysis followed by anticoagulation therapy (intravenous heparin bridging to coumadin), resulting in the successful recanalization of the left transverse sinus and a much improved flow in the superior sagittal sinus. Visual loss is quite rare as the initial presentation of CVT and occurs more frequently in chronic cases with associated papilledema on funduscopy. Cortical blindness denotes a loss of vision due to damage on the geniculocalcarine tract, par- ticularly the primary visual cortex (V1) [4]. This causes con- scious vision deficit that may become permanent without timely intervention. It is often associated (40–90% of all cases) with a bilateral PCA occlusion [4]. Bilateral infarctions, edema, hemorrhage, thrombosis, trauma, meningioma, glioma and neurological disease involving the occipital cortex are some primary causes of cortical blindness in patients. In cor- tical blindness with vascular origins, loss of vision is often Figure 1: Head CT with contrast: note empty delta sign. precipitous with some patients experiencing visual agnosia, Figure 2: Axial MRI demonstrating cerebral tissue injuries from venous hypertension with the associated ischemia: (A) Diffusion-weighted imaging (DWI) MRI. (B) Fluid-attenuated inversion recovery (FLAIR) imaging. (C)T -weighted MRI. (D) Gradient-echo (GRE) imaging. 2 Cortical blindness as a rare presentation Page 3 of 3 Figure 3: Dural sinus venogram (A) Prior to endovascular mechanical thrombectomy (B) Post mechanical thrombectomy. (C) Pre-intervention MRV demonstrat- ing complete occlusion of the left transverse sinus and near occlusion of the posterior portion of the superior sagittal sinus. (D) Post-intervention MRV demonstrat- ing persistent recanalization of the left transverse and sigmoid sinuses, along with much improved flow in the posterior superior sagittal sinus. Notethe recanalization of the left transverse sinus and the much improved flow in the superior sagittal sinus. hallucinations, denial or disorientation. Homonymous hemi- Conflict of interest statement anopsia, both unilateral and bilateral in nature, have been The authors report no conflict of interest concerning the mate- described in patients with cortical blindness. rials or methods used in this study or the findings specified in In our patient, the combined occlusion of the posterior third this paper. of the superior sagittal sinus and the left transverse sinus likely resulted in severe venous hypertension affecting the bi- lateral visual cortices with subsequent incomplete infarction. REFERENCES Incomplete bilateral infarction of the visual cortices can present with a remarkable variety of syndromes, with some 1. Ferro JM, Lopes MG, Rosas MJ, Fontes J. Delay in hospital admission of cases having begun as complete blindness. Although patients patients with cerebral vein and dural sinus thrombosis. Cerebrovasc Dis 2005;19:152–6. with complete cortical blindness commonly volunteer no 2. Melamed E, Abraham FA, Lavy S. Cortical blindness as a manifestation of complaints and are unaware of the deficit [5], our patient basilar artery occlusion. Eur Neurol 1974;11:22–9. acutely presented with the primary complaint of bilateral 3. Sassi SB, Mizouni H, Nabli F, Kallel L, Kefi M, Hentati F. Cerebral venous thrombosis presenting with cerebellar ataxia and cortical blindness. visual loss. J Stroke Cerebrovasc Disc 2010;19:507–9. CVT presents in a remarkably wide spectrum of symptoms 4. Das A, Huxlin KR. New approaches to visual rehabilitation for and remains a diagnostic and therapeutic challenge for clini- cortical blindness: outcomes and putative mechanisms. Neuroscientist 2010;16:374–87. cians. Although cortical blindness is most commonly caused 5. Spector RH, Glaser JS, David NJ, Vining DQ. Occipital lobe by bilateral PCA occlusion, it can be a rare initial presentation infarctions: Perimetry and computed tomography. Neurology 1981;31: of CVT. 1098–106. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of Surgical Case Reports Oxford University Press

Cortical blindness as a rare presentation of cerebral venous thrombosis

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Oxford University Press
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Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author 2013.
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Abstract

JSCR 2013; 5 (3 pages) doi:10.1093/jscr/rjt035 Case Report Cortical blindness as a rare presentation of cerebral venous thrombosis 1,2,* 3 2,4 2,4 Bonnie Wang , Seong-Jin Moon , William C. Olivero and Huan Wang Department of Internal Medicine, University of Illinois, College of Medicine at Urbana-Champaign, Urbana, IL, USA, 2 3 Carle Foundation Hospital, Urbana, IL, USA, University of Illinois, College of Medicine at Urbana-Champaign, Urbana, IL, USA and Department of Neurological Surgery, University of Illinois, College of Medicine at Urbana- Champaign, Urbana, IL, USA *Correspondence address. University of Illinois, College of Medicine at Urbana-Champaign, Carle Foundation Hospital, 611 West Park Street, Urbana, IL 61801. Tel: þ1-(217)-383-3110; Fax: þ1-(217)-244-0621; E-mail: wsu4@illinois.edu Received 27 February 2013; accepted 7 April 2013 Cerebral venous thrombosis (CVT) remains a diagnostic and therapeutic challenge for clini- cians. Manifesting in a remarkably wide spectrum of symptoms and signs, CVT often presents in a misleading fashion—if unrecognized or misdiagnosed, it carries potentially fatal conse- quences. Visual loss is quite rare as the initial presentation of CVT and is typically a finding more frequent in chronic cases with associated papilledema on funduscopy Ferro, Lopes, Rosas and Fontes (Delay in Hospital Admission of Patients with Cerebral Vein and Dural Sinus Thrombosis. Cerebrovasc Dis 2005;19:152 – 6). We report a rare case of acute cortical blind- ness as the initial presentation of CVT in an 18-year-old female patient and review the current literature. INTRODUCTION 4þ in the lower extremities. No ankle clonus was noted, and plantar reflexes were equivocal. Cerebral venous thrombosis (CVT) remains a diagnostic and Cranial computed tomography (CT) demonstrated subtle therapeutic challenge for clinicians. Visual loss is quite rare as hypodensity mainly in the left occipital lobe, with a character- the initial presentation of CVT and is typically a finding more istic empty delta sign (Fig. 1). Computed tomography angiog- frequent in chronic cases with associated papilledema on fun- raphy demonstrated normal filling of the posterior cerebral duscopy [1]. We report a case of acute cortical blindness as arteries (PCA). Cranial magnetic resonance imaging (MRI) the initial presentation of CVT in an 18-year-old female evaluations demonstrated bilateral ischemic changes involv- patient. ing the occipital and parietal lobes, more extensively on the left (Fig. 2). Magnetic resonance venography (MRV) demon- strated complete occlusion of the left transverse and sigmoid CASE REPORT sinuses, as well as near occlusion of the posterior superior sa- An 18-year-old female with a three-week history of headaches gittal sinus (Fig. 3C). The patient was treated emergently with endovascular mech- presented with an acute onset of blindness in both eyes after anical thrombolysis with technical success (Fig. 3Aand B), her afternoon nap. Physical examination demonstrated normal pupils and funduscopic exam, but without light perception in followed by systemic anticoagulation therapy. Post-intervention MRV demonstrated persistent recanalization of the left trans- either eye. Cranial nerve examinations were otherwise unre- verse and sigmoid sinuses, along with much improved flow in markable. Speech was intact with no naming or repetition def- icits. Although alert, she was mildly confused. No isolated the posterior superior sagittal sinus (Fig. 3D). She was subsequently diagnosed with mixed connective motor or sensory deficits were identified in the extremities. tissue disease. Clinically, she recovered the left visual field Deep tendon reflexes were 3þ in the upper extremities and Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved.# The Author 2013. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/ by-nc/3.0/), which permits non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com. Page 2 of 3 B. Wang et al. but continued to have a dense right homonymous hemianopsia substance abuse; infections and head trauma [3]. In 20–35% at discharge. She returned to her home state and was unfortu- of CVT cases, however, the etiology is unknown. nately lost to further follow-up. Patients with CVT commonly present with headache, papil- loedema, motor or sensory deficits, seizures or conscious dis- turbance. Headache is the most common symptom, present in more than 90% of patients either in a gradual manner or as a DISCUSSION sudden onset of severe headache (thunderclap headache). CVT comprises 0.5–1% of all strokes [2]. First described in Only half (40–60%) of CVT patients develop subsequent 1825, CVT occurs much less frequently than arterial throm- cerebral lesions and neurologic deficits. Focal seizures have bosis but can present in an atypical fashion leading to mis- been noted to develop in 30–40% of CVT cases, which is diagnosis or delayed diagnosis [1]. Cortical blindness much higher than in patients with arterial stroke [3]. represents a rare presentation of CVT, and has not been well Diagnosis of CVT is substantiated with neuroimaging. An reported in the literature. abnormal sinus signal coupled with an absence of flow on While CVT can present at all ages, it is more common in MRV strengthens the diagnosis. If diagnosis is still uncertain young adults and neonates. CVT primarily affects females at this stage, cerebral angiography may be attempted as it pro- (typically women of childbearing age), and recent reports in- vides definitive information of the cerebral arterial and venous dicate an increased frequency attributed to use of oral contra- system. An empty delta sign is the most commonly observed ceptives [3]. Major causes of CVT constitute prothrombotic sign of CVT on CT imaging (such as our patient), but this is conditions such as thrombophilia, pregnancy and puerperium; only present in 20% of cases. the use of certain medications such as oral contraceptives or Main treatment options are anticoagulation, fibrinolytic therapy, thrombolysis (either directly or mechanically) and/or management of intracranial pressure [3]. Heparinization offered mortality rates typically ,10%, which was often attributed to the underlying disease rather than CVT. If the patient experiences a raised intracranial pressure or worsened clinical state after anticoagulation, thrombolytic therapy is suggested. Presenting without light perception in either eye, our patient was treated emergently with mechanical thromb- olysis followed by anticoagulation therapy (intravenous heparin bridging to coumadin), resulting in the successful recanalization of the left transverse sinus and a much improved flow in the superior sagittal sinus. Visual loss is quite rare as the initial presentation of CVT and occurs more frequently in chronic cases with associated papilledema on funduscopy. Cortical blindness denotes a loss of vision due to damage on the geniculocalcarine tract, par- ticularly the primary visual cortex (V1) [4]. This causes con- scious vision deficit that may become permanent without timely intervention. It is often associated (40–90% of all cases) with a bilateral PCA occlusion [4]. Bilateral infarctions, edema, hemorrhage, thrombosis, trauma, meningioma, glioma and neurological disease involving the occipital cortex are some primary causes of cortical blindness in patients. In cor- tical blindness with vascular origins, loss of vision is often Figure 1: Head CT with contrast: note empty delta sign. precipitous with some patients experiencing visual agnosia, Figure 2: Axial MRI demonstrating cerebral tissue injuries from venous hypertension with the associated ischemia: (A) Diffusion-weighted imaging (DWI) MRI. (B) Fluid-attenuated inversion recovery (FLAIR) imaging. (C)T -weighted MRI. (D) Gradient-echo (GRE) imaging. 2 Cortical blindness as a rare presentation Page 3 of 3 Figure 3: Dural sinus venogram (A) Prior to endovascular mechanical thrombectomy (B) Post mechanical thrombectomy. (C) Pre-intervention MRV demonstrat- ing complete occlusion of the left transverse sinus and near occlusion of the posterior portion of the superior sagittal sinus. (D) Post-intervention MRV demonstrat- ing persistent recanalization of the left transverse and sigmoid sinuses, along with much improved flow in the posterior superior sagittal sinus. Notethe recanalization of the left transverse sinus and the much improved flow in the superior sagittal sinus. hallucinations, denial or disorientation. Homonymous hemi- Conflict of interest statement anopsia, both unilateral and bilateral in nature, have been The authors report no conflict of interest concerning the mate- described in patients with cortical blindness. rials or methods used in this study or the findings specified in In our patient, the combined occlusion of the posterior third this paper. of the superior sagittal sinus and the left transverse sinus likely resulted in severe venous hypertension affecting the bi- lateral visual cortices with subsequent incomplete infarction. REFERENCES Incomplete bilateral infarction of the visual cortices can present with a remarkable variety of syndromes, with some 1. Ferro JM, Lopes MG, Rosas MJ, Fontes J. Delay in hospital admission of cases having begun as complete blindness. Although patients patients with cerebral vein and dural sinus thrombosis. Cerebrovasc Dis 2005;19:152–6. with complete cortical blindness commonly volunteer no 2. Melamed E, Abraham FA, Lavy S. Cortical blindness as a manifestation of complaints and are unaware of the deficit [5], our patient basilar artery occlusion. Eur Neurol 1974;11:22–9. acutely presented with the primary complaint of bilateral 3. Sassi SB, Mizouni H, Nabli F, Kallel L, Kefi M, Hentati F. Cerebral venous thrombosis presenting with cerebellar ataxia and cortical blindness. visual loss. J Stroke Cerebrovasc Disc 2010;19:507–9. CVT presents in a remarkably wide spectrum of symptoms 4. Das A, Huxlin KR. New approaches to visual rehabilitation for and remains a diagnostic and therapeutic challenge for clini- cortical blindness: outcomes and putative mechanisms. Neuroscientist 2010;16:374–87. cians. Although cortical blindness is most commonly caused 5. Spector RH, Glaser JS, David NJ, Vining DQ. Occipital lobe by bilateral PCA occlusion, it can be a rare initial presentation infarctions: Perimetry and computed tomography. Neurology 1981;31: of CVT. 1098–106.

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Published: May 9, 2013

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