Posterior Reversible Encephalopathy Syndrome and Subarachnoid Hemorrhage After Lumboperitoneal Shunt for Fulminant Idiopathic Intracranial Hypertension

A 20-year-old woman presented with headache, decreased vision, eye pain, and urinary retention. During her clinical course, visual acuity declined to 20/800, right eye, and 20/50, left eye, associated with bilateral optic disc edema. Brain magnetic resonance imaging revealed enhancement of the leptomeninges, right optic nerve, and right side of the optic chiasm. Extensive evaluation of the central nervous system (CNS) for an infectious cause was negative. Brain biopsy showed a pattern consistent with vasculitis. The patient was treated with prednisone and cyclophosphamide, resulting in improvement of her vision and systemic symptoms. Primary CNS vasculitis is a rare condition that may affect the anterior visual pathways.

Clinical Observation Posterior Reversible Encephalopathy Syndrome and Subarachnoid Hemorrhage After Lumboperitoneal Shunt for Fulminant Idiopathic Intracranial Hypertension Anthony Fok, FRACP, Ronil V. Chandra, FRANZCR, Matthew Gutman, MBBS, Matthew Ligtermoet, MBBS, Udaya Seneviratne, FRACP, Peter Kempster, FRACP Abstract: A 33-year-old woman presented with severe visual loss from fulminant idiopathic intracranial hypertension. Her lumbar puncture opening pressure was 97 cm H2O. Soon after lumboperitoneal shunt surgery, she had a generalized tonic-clonic seizure. Magnetic resonance imaging demonstrated frontal subarachnoid hemorrhage (SAH) and neuroimaging findings consistent with posterior reversible encephalopathy syndrome (PRES). We hypothesize that an abrupt drop in intracranial pressure after lumboperitoneal shunting led to maladjustment of cerebral vascular autoregulation, which caused SAH and PRES. Journal of Neuro-Ophthalmology 2016;36:164-166 doi: 10.1097/WNO.0000000000000355 © 2016 by North American Neuro-Ophthalmology Society I n fulminant idiopathic intracranial hypertension (IIH), urgent surgical interventions, such as optic nerve sheath fenestration, venous stenting, and cerebrospinal fluid (CSF) diversion procedures (lumboperitoneal or ventriculoperitoneal shunt) may prevent or minimize vision loss. CSF diversion procedures lower CSF pressure, improving both headache and visual outcome. We report a patient with fulminant IIH in whom lumboperitoneal shunting was followed by subarachnoid hemorrhage (SAH) and posterior reversible encephalopathy syndrome (PRES). Departments of Neuroscience (AF, ML, US, PK); Diagnostic Imaging (RVC), Monash Health, Melbourne, Australia; Department of Medicine (RVC, US), Monash University, Melbourne, Australia; and Department of Neurosurgery (MG), Monash Health, Melbourne, Australia. A. Fok has received a Doctor's in Training Grant from Medical Insurance Group Australia. The other authors report no conflicts of interest. Address correspondence to Anthony Fok, MBBS, Department of Ophthalmology and Visual Sciences, Faculty of Medicine, Eye Care Centre (Vancouver General Hospital), 2550 Willow Street, Vancouver, British Columbia, Canada V5Z 3N9; E-mail: [e-mail] 164 CASE REPORT Over 2 weeks, a 33-year-old woman developed worsening headache, visual blurring, diplopia, and vomiting. Her vision abruptly deteriorated on the day that she presented to hospital. Visual acuity was light perception bilaterally, with sluggish pupillary responses, bilateral sixth and partial right third nerve palsies and bilateral optic disc edema. Her body mass index was 41 kg/m2, and she was taking no medications regularly. Lumbar puncture opening pressure was 97 cm H2O, with normal CSF composition. Magnetic resonance imaging (MRI) of the brain showed the optic discs protruding into the vitreous cavity, prominent CSF sleeves around the orbital optic nerves and an empty sella (Fig. 1). No transverse venous sinus narrowing was evident and the ventricular size was normal. The patient was diagnosed with fulminant IIH, and emergency bilateral optic nerve sheath fenestrations were performed. Her vision improved to counting fingers bilaterally but her headache persisted, and a repeat lumbar puncture showed that the opening pressure was still above 37 cm H2O. Two days later, a lumboperitoneal shunt was inserted. She developed orthostatic headache suggestive of low CSF pressure. Her vision recovered to 20/70 in each eye with severely constricted visual fields. On Day 7 of hospitalization, shortly after complaining of acute frontal headache, the patient had a left-sided focal motor seizure with secondary generalization. Her blood pressure had averaged 125/75 and the highest reading was 142/90. Computed tomography and MRI of the brain showed right frontal SAH, and bilateral parieto-occipital and subcortical white matter changes typical of PRES (Fig. 2). There were no signs of transtentorial or tonsillar herniation. By Day 10, her vision had deteriorated to light perception in the left eye and she reported formed visual Fok et al: J Neuro-Ophthalmol 2016; 36: 164-166 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Observation optic discs became pale. Automated visual fields showed generalized depression. Five months later, the changes in the brain parenchyma completely resolved on a follow-up MRI. Residual hemosiderin staining was present over the frontal surface of the right cerebral hemisphere, and the optic nerves were noticeably thinned. DISCUSSION FIG. 1. A. Axial T2 magnetic resonance imaging shows prominent perioptic cerebrospinal fluid bilaterally with protrusion of the optic discs into the vitreous cavities. B. Precontrast sagittal T1 image reveals an empty sella. hallucinations. Opening pressure on lumbar puncture was 24 cm H2O. On Day 13, she had stereotactic insertion of a programmable ventriculoperitoneal shunt set to 14 cm H2O with an antisiphon device and the lumboperitoneal shunt was removed. Visual acuity steadily improved to 20/30, right eye, and 20/60, left eye. As her papilledema resolved, the Our case fulfills the revised diagnostic criteria for definite IIH (1) and the presentation was a fulminant one with early, severe visual loss. Five days after lumboperitoneal shunting, SAH and PRES developed concurrently. The clinical presentation of headache, visual hallucinations, and seizures accompanied by typical MRI findings were highly suggestive of PRES, and resolution of the parenchymal imaging abnormalities confirmed the diagnosis. In the absence of high or rapidly rising blood pressure, or of medical conditions or medications associated with PRES, we believe that these complications were related to the abrupt lowering of the CSF pressure after a period of severe intracranial hypertension. One previous report describes SAH after lumboperitoneal shunting in a patient with IIH (2). Similarities to our case were that the lumboperitoneal shunt was inserted for IIH, and that the SAH was nonaneurysmal. There was also intraparenchymal hemorrhage. Another report describes intraparenchymal without subarachnoid bleeding after a lumboperitoneal shunt (3). In neither case was an MRI performed, so coexistent PRES cannot be excluded. Both subarachnoid and parenchymal hemorrhage occur in PRES, with a combined prevalence of 15%-19% (4,5). A CSF pressure of 97 cm of water equates to 72 mm of mercury. At a mean arterial blood pressure of approximately 90 mm of mercury, brain capillary pressure would be dangerously close to tissue fluid pressure unless cerebral FIG. 2. Axial computed tomography (A) and magnetic resonance imaging (FLAIR) (B, C) demonstrate right frontal subarachnoid hemorrhage (oval) and bilateral parieto-occipital subcortical white matter edema (arrows). Fok et al: J Neuro-Ophthalmol 2016; 36: 164-166 165 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Observation autoregulation, acting through arteriolar dilation, maximized perfusion pressure to the capillary beds. We hypothesize that once the CSF pressure was lowered, there was a lag in the resetting of cerebral arteriolar tone. This led to high capillary transmembrane pressure and predisposed to bleeding and vasogenic edema. A similar phenomenon known as "normal perfusion pressure breakthrough" may develop after excision of a cerebral arteriovenous malformation (AVM) (6). AVMs hinder capillary perfusion because of chronic venous back pressure, which is compensated by arteriolar dilation. After AVM excision, a delay in the normalization of arteriolar autoregulatory capacity leads to overperfusion of capillary beds with similar effects of hemorrhage and edema. Although PRES after a CSF diversion procedure previously has not been reported, it has occurred from CSF leakage after inadvertent dural puncture (7-10) and after depletive lumbar puncture for hydrocephalus (11). It has been suggested that acute CSF hypotension may disrupt cerebral autoregulation (11). PRES has been reported to develop 4 to 15 days after dural puncture (7-10), similar to the delay in our patient. A standard lumboperitoneal shunt allows free flow of CSF, leading to a common complication of intracranial hypotension from overdrainage (12). Programmable shunt systems have been showed to reduce this adverse effect (13). Our experience supports the use of programmable valves to allow a graded reduction in intracranial pressure when extremely high CSF pressures are encountered in IIH patients. Ventricular and lumbar shunts in IIH have demonstrated generally comparable visual and symptomatic outcomes (14), yet in 1 case series, 4% of lumboperitoneal shunts were associated with tonsillar herniation (15). For this reason, stereotactic placement of a ventriculoperitoneal shunt with a programmable valve seems to be a prudent alternative. Avoidance of precipitous lowering of CSF pressure, together with careful postprocedure blood pressure control, should reduce the risk of PRES as a consequence of the treatment of fulminant IIH. STATEMENT OF AUTHORSHIP Category 1: a. 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