Idiopathic Intracranial Hypertension Progressing to Venous Sinus Thrombosis Subarachnoid Hemorrhage and Stroke

Idiopathic intracranial hypertension (IIH) is a syndrome characterized by increased intracranial pressure (ICP), the absence of structural lesions on neuroimaging, and normal cerebrospinal fluid composition. Cerebral venous sinus thrombosis (CVST) is a common cause of increased ICP and can be differentiated from IIH with magnetic resonance venography. We describe a young woman with typical IIH who underwent lumbar puncture and was treated with a short course of high-dose corticosteroids followed by acetazolamide. She subsequently developed CVST, subarachnoid hemorrhage, and stroke. Risk factors that may have resulted in CVST are discussed.

Clinical Observation Idiopathic Intracranial Hypertension Progressing to Venous Sinus Thrombosis, Subarachnoid Hemorrhage, and Stroke Joshua S. Hardin, MD, Raghu H. Ramakrishnaiah, MD, John D. Pemberton, MD, Paul H. Phillips, MD, Joseph G. Chacko, MD Abstract: Idiopathic intracranial hypertension (IIH) is a syndrome characterized by increased intracranial pressure (ICP), the absence of structural lesions on neuroimaging, and normal cerebrospinal fluid composition. Cerebral venous sinus thrombosis (CVST) is a common cause of increased ICP and can be differentiated from IIH with magnetic resonance venography. We describe a young woman with typical IIH who underwent lumbar puncture and was treated with a short course of high-dose corticosteroids followed by acetazolamide. She subsequently developed CVST, subarachnoid hemorrhage, and stroke. Risk factors that may have resulted in CVST are discussed. Journal of Neuro-Ophthalmology 2018;38:60-64 doi: 10.1097/WNO.0000000000000540 © 2017 by North American Neuro-Ophthalmology Society I diopathic intracranial hypertension (IIH) is a syndrome characterized by increased intracranial pressure (ICP), no structural lesions on neuroimaging, and normal cerebrospinal fluid composition (1). IIH is a diagnosis of exclusion after ruling out other entities such as an intracranial mass, meningitis, hydrocephalus, and cerebral venous sinus thrombosis (CVST) (2-6). Distinguishing IIH from intracranial tumor and CVST is possible with MRI and magnetic resonance venography (MRV), respectively (2,3,5). We describe a woman with typical IIH who subsequently developed CVST, subarachnoid hemorrhage, and stroke. Jones Eye Institute (JSH, JDP, PHP, JGC), University of Arkansas for Medical Sciences (UAMS), Little Rock, Arkansas; and Departments of Ophthalmology (PHP) and Radiology (RHR), Arkansas Children's Hospital, Little Rock, Arkansas. The authors report no conflicts of interest. Address correspondence to Paul H. Phillips, MD, Arkansas Children's Hospital, 1 Children's Way, Slot 111, Little Rock, AR 72202; E-mail: [e-mail] 60 CASE REPORT A 32-year-old woman with no ocular history was referred to our clinic for evaluation of bilateral optic disc edema. She had severe headaches, lightheadedness, pulsatile tinnitus, intermittent horizontal diplopia, and transient visual obscurations for the past 3 weeks. Medical history was significant for obesity (BMI, 33.4 kg/m2), recurrent ear infections, tobacco use, and polycystic ovary syndrome (PCOS), for which she was treated for 2 days with birth control pills (norethindrone/ethynyl estradiol [1/20 mg]) 1 week before her presentation. Visual acuity was 20/60, right eye, and count fingers at 3 feet, left eye. Confrontational visual fields revealed a superotemporal defect in the right eye and superior and inferotemporal defects in the left eye. Color vision was reduced in both eyes (Ishihara pseudoisochromatic plates). Pupils, tonometry, and ocular motility were normal in both eyes. Funduscopic examination showed Frisen Grade 4 disc edema bilaterally (Fig. 1). Brain MRI showed findings consistent with elevated ICP (Fig. 2A, B), whereas MRV demonstrated a hypoplastic right transverse sinus without evidence of thrombosis (Fig. 2C, D). Opening pressure was 45 cm H2O on lumbar puncture (LP) and was reduced to 20 cm H2O at completion of the procedure. Cerebrospinal fluid analysis was normal. The patient was admitted to the hospital and treated with intravenous (IV) methylprednisolone (250 mg every 6 hours). After 3 days, the patient had improvement of her headaches, visual acuity at near was 20/40 in each eye, and there was resolution of diplopia. She was discharged and started on acetazolamide, 500 mg twice daily. Six days later, she returned to the clinic complaining of a severe, pounding headache with nausea, vomiting, malaise, vision loss, and diplopia. Visual acuity was count fingers at 1 foot in each eye, color vision remained poor, and ophthalmoscopy was unchanged. After readmission to the hospital, LP Hardin et al: J Neuro-Ophthalmol 2018; 38: 60-64 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Observation FIG. 1. There is a marked bilateral optic disc edema. showed an opening pressure of .55 cm H2O (reduced to 10 cm H2O), and the neurosurgery service placed a lumbar drain in preparation for shunting. The next morning, the patient was lethargic and repeat neuroimaging demonstrated extensive CVST and evidence of subarachnoid hemorrhage with cerebral edema (Fig. 3). On the fifth hospital day, the lumbar drain was clamped without worsening of headaches. The following day, the patient's speech worsened, she had numbness of her left upper extremity, and brain computed tomography was consistent with a right-sided cortical venous stroke. On the seventh day, cerebral angiography and percutaneous thrombectomy were performed with near complete recanalization of the venous sinuses (Fig. 4). However, the patient's vision declined to hand motions bilaterally, so right and left optic FIG. 2. Initial neuroimaging studies. A. Axial T2 orbital MRI shows distention of the optic nerve sheaths (white arrows) and flattening of the globes with bulging optic discs (black arrows). B. Postcontrast axial T1 orbital MRI with fat suppression reveals enhancement of the elevated optic discs (arrows). Axial (C) and coronal (D) magnetic resonance venography images show a hypoplastic right transverse sinus with a dominant left transverse sinus (arrowheads). Hardin et al: J Neuro-Ophthalmol 2018; 38: 60-64 61 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Observation FIG. 3. A. Sagittal T1 MRI shows hyperintensities in the superior sagittal sinus (arrows) and straight sinus (arrowheads) consistent with thrombosis. B. Noncontrast axial computed tomography reveals evidence of subarachnoid hemorrhage in the frontal sulcal spaces (arrows). C. Magnetic resonance venography shows near complete absence of flow within the cerebral venous sinuses. D. Axial fluid-attenuated inversion recovery image shows areas of hyperintensity in the right frontal and parietal lobes in a nonarterial distribution indicative of a venous stroke. FIG. 4. Endovascular thrombectomy. A. Prethrombectomy frontal fluoroscopic image from left internal carotid arteriogram in delayed venous phase shows cortical venous filling but absence of superior sagittal sinus or transverse sinus filling. B. Transfemoral catheter (arrows) is inserted into the left sigmoid, transverse, and superior sagittal sinuses. C. Postprocedure venous phase image after right carotid artery injection shows blood flow in the superior sagittal sinus (arrowheads) and dominant left transverse sinus (arrows). 62 Hardin et al: J Neuro-Ophthalmol 2018; 38: 60-64 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Observation nerve sheath fenestrations (ONSFs) were performed over the following 2 days. During the next 3 months, with rehabilitation the patient gained mobility, lost 20 lbs, her headaches resolved, and her visual acuity improved to 20/100, right eye and 20/60, left eye. Kinetic perimetry showed visual fields of 50° in each eye. Her papilledema resolved and the patient was left with bilateral optic atrophy. J. D. Pemberton; P. H. Phillips; and J. G. Chacko; c. Analysis and interpretation of data: J. S. Hardin; R. H. Ramakrishnaiah; J. D. Pemberton; P. H. Phillips; and J. G. Chacko. Category 2: a. Drafting the manuscript: J. S. Hardin; R. H. Ramakrishnaiah; J. D. Pemberton; P. H. Phillips; and J. G. Chacko; b. Revising it for intellectual content: J. S. Hardin; R. H. Ramakrishnaiah; J. D. Pemberton; P. H. Phillips; and J. G. Chacko. Category 3: a. Final approval of the completed manuscript: P. H. Phillips. DISCUSSION REFERENCES We report a unique case of IIH that progressed to CVST, subarachnoid hemorrhage, and stroke 1 week after lumbar puncture and a short 3-day course of IV corticosteroids. There are several important aspects of our patient's clinical course. First, she had multiple risk factors for CVST including tobacco and oral contraceptive use high-dose corticosteroids, PCOS, obesity, and lumbar puncture (4,7-24). Cases of CVST have been described after spinal dural injury (of which LP is most common) in patients without IIH (10-24). Canhao et al (23) and Honig et al (24) postulated that decreased ICP after LP causes venous sinus dilation resulting in reduction of venous sinus blood flow. This cascade of events may precipitate CVST followed by intracranial hemorrhage and stroke (11-13, 15, 18, 19, 21-27). Although post-LP CVST typically occurs with intracranial hypotension, the sudden reduction of ICP that occurs after LP and the use of a lumbar drain could precipitate CVST in patients with IIH via a similar mechanism (21, 28). Second, after our patient's initial presentation, she had a change in the quality and severity of her headaches. As CVST can cause a precipitous rise in ICP, an abrupt change in the pattern of headaches may be useful in differentiating CVST from postlumbar puncture headache or the headaches associated with IIH (11,28,29). Purvin et al (28) showed that patients with venous sinus thrombosis characteristically have abrupt onset of a severe headache and that this presentation enabled differentiation from patients with IIH that tend to have subacute onset of headaches. Third, although ONSF is a standard treatment for IIH, it is less frequently used to treat papilledema because of CVST (30,31). As our patient had IIH and later developed CVST with profound vision loss, urgent treatment with ONSF was justified to help prevent further vision loss. Patients with IIH undergo lumbar puncture and may be treated with a short course of high-dose corticosteroids. These are risk factors for venous thrombosis. For this reason, CVST should be considered in patients with IIH that develop an abrupt increase in the severity of their headaches. This should prompt repeat neuroimaging, as early diagnosis of CVST may allow for expeditious treatment and improved patient outcomes. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: J. S. Hardin; R. H. Ramakrishnaiah; J. D. Pemberton; P. H. Phillips; and J. G. Chacko; b. Acquisition of data: J. S. Hardin; R. H. Ramakrishnaiah; Hardin et al: J Neuro-Ophthalmol 2018; 38: 60-64 1. Friedman DI, Jacobson DM. 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