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The Pathogenesis and Treatment of Optic Disc Swelling in Neurosarcoidosis a Unique Therapeutic Response to Infliximab

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The Pathogenesis and Treatment of Optic Disc Swelling in Neurosarcoidosis a Unique Therapeutic Response to Infliximab OBSERVATION The Pathogenesis and Treatment of Optic Disc Swelling in Neurosarcoidosis A Unique Therapeutic Response to Infliximab Jeffrey M. Katz, MD; Michiko Kimura Bruno, MD; Jacqueline M. S. Winterkorn, MD, PhD; Nancy Nealon, MD Objective: To review the pathogenesis and treatment eye. A 57-year-old woman presented with bilateral, sub- of optic disc swelling in neurosarcoidosis, including a acute, painful visual loss and unilateral papillitis consis- novel therapeutic response to infliximab. tent with optic neuritis. Her visual loss responded rap- idly to intravenous corticosteroids. The funduscopic Design and Setting: Case reports from an inpatient examination findings in both patients prompted further neurology service. clinical investigation, culminating in the diagnosis of neu- rosarcoidosis. Patients: A 35-year-old woman presented with head- ache, chronic visual loss, papilledema, and optic atro- Conclusion: Understanding the multiple etiologic mecha- phy, characteristic of chronic intracranial hypertension. nisms that produce optic disc swelling in sarcoidosis can Magnetic resonance imaging showed bifrontal cerebral help neurologists tailor treatment for patients with neu- edema with en plaque frontal pachymeningeal enhance- rosarcoidosis who present with this symptom. ment. Her visual loss progressed despite conventional therapies. The use of the tumor necrosis factor ␣ antago- nist infliximab maintained functional vision in her right Arch Neurol. 2003;60:426-430 OSS OF VISION associated with 20/25 OD with a constricted visual field (VF) optic disc swelling (ODS) is and an inferonasal step. The left eye had no a rare initial presentation of light perception and an amaurotic pupil. neurosarcoidosis. Optic disc Funduscopic examination findings re- swelling is an important vealed right optic disc swelling (Figure 1A) Lclinical sign because it can herald central and left optic disc pallor and resolving swell- nervous system disease in an otherwise ing (Figure 1B). General and neurologic ex- neurologically intact patient. We report 2 amination results were unremarkable. cases of neurosarcoidosis in patients who A gadolinium-enhanced magnetic presented with ODS and bilateral visual resonance image of the brain (Figure 2) loss with otherwise normal neurologic ex- revealed bifrontal edema and en plaque amination results. Optic disc swelling can parafalcian and frontal lobe pachymenin- represent papilledema from increased in- geal enhancement. Serum angiotensin- tracranial pressure (ICP), papillitis from converting enzyme (ACE) level was el- optic neuropathy, or infiltration of the evated (61.1 U/L) as was her erythrocyte disc.1 We discuss the various pathophysi- sedimentation rate (33 mm/h). Lumbar ologic features of ODS in sarcoidosis. puncture showed a markedly elevated op- ening pressure of 40 cm H2O and a nega- REPORT OF CASES tive cerebrospinal fluid (CSF) ACE level (Ͻ4 U/L) but was otherwise unremark- PATIENT 1 able. Computed tomography of the chest revealed bilateral hilar and mediastinal A 35-year-old black woman presented with lymphadenopathy with clear lung fields. visual loss in the left eye progressing to no A hilar lymph node biopsy specimen con- From the Department of Neurology and Neuroscience, light perception. In the previous 5 months, firmed discrete epithelioid, noncaseating New York Presbyterian she had experienced bifrontal headaches, granulomata consistent with sarcoidosis. Hospital–Weill Medical transient visual obscurations of both eyes, The patient was treated with acet- Center of Cornell University, occasional diplopia, tinnitus, and blurred vi- azolamide and intravenous methylpred- New York, NY. sion in the left eye. Visual acuity (VA) was nisolone, without recovery of vision in her (REPRINTED) ARCH NEUROL / VOL 60, MAR 2003 WWW.ARCHNEUROL.COM 426 ©2003 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/24/2021 A B Figure 1. A, Fundus photograph of the right eye in patient 1 shows optic disc swelling. B, Fundus photograph of the left eye in patient 1 shows resolving optic disc swelling and atrophy. A B Figure 2. A, T2-weighted, fluid attenuated inversion recovery, noncontrast axial magnetic resonance image (MRI) of patient 1 shows bifrontal cerebral edema. B, T1-weighted, gadolinium-enhanced axial MRI of patient 1 shows en plaque pachymeningeal enhancement in the anterior cranial fossa and both sides of the anterior falx. left eye. The vision in her right eye deteriorated. She re- quence of immunosuppression. After 3 months of monthly fused optic nerve sheath fenestration. In conjunction with infusions, her VA improved to 20/40 OD. an oral prednisone taper, cyclophosphamide therapy was initiated at a dose of 200 mg orally once daily and rapidly PATIENT 2 increased to 200 mg twice daily, effecting a white blood cell response ranging from 2200 to 5000/µL. This re- A 57-year-old black woman with a history of diabetes sulted in stabilization of her VA at 20/200 OD. However, mellitus complained of ocular tenderness and progres- 2 months later her VA declined to 20/800 OD. One dose sive visual loss in both eyes over 7 weeks. Her VA was of the tumor necrosis factor ␣ (TNF-␣) antagonist inflix- light perception in the right eye and 20/200 OS. Ameri- imab (3-mg/kg intravenous infusion) was administered can Optical Hardy Rand Rittler (AOHRR) color plates were without adverse effects. The cyclophosphamide and pred- not seen with either eye, and a red object was seen as dark. nisone taper was continued. Her VA improved to 20/200 The VF testing revealed a nasal island in the right eye OD during the next 3 weeks and remained stable for 6 and a superior altitudinal defect in the left eye. Pupils months. Then the patient’s VA fluctuated between 20/200 showed a right relative afferent pupillary defect. The right OD and 20/400 OD. A second dose of infliximab (3- disc appeared normal (Figure 3A), and the left disc mg/kg intravenous infusion) was given 10 months after the showed chronic swelling (Figure 3B). There was no sign first, with monthly infusions thereafter. Of note, the pa- of diabetic retinopathy. Her general and neurologic ex- tient developed an uncomplicated herpes zoster dermati- amination results were normal except for erythema no- tis 3 weeks after the second treatment, a likely conse- dosum on the anterior aspect of her legs. (REPRINTED) ARCH NEUROL / VOL 60, MAR 2003 WWW.ARCHNEUROL.COM 427 ©2003 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/24/2021 A B Figure 3. A, Fundus photograph of the right eye of patient 2 shows a pink and sharp optic disc. B, Fundus photograph of the left eye of patient 2 shows optic disc swelling. COMMENT Pathogenesis of Optic Disc Swelling in Neurosarcoidosis Papilledema Granulomatous mass Intra-axial mass Optic disc swelling represents obstruction of axoplas- lesion Extra-axial mass mic flow at the lamina cribrosa, resulting in accumula- Hydrocephalus Obstructive or tion of axoplasm at the disc.2 The patients described herein communicating both presented with predominantly unilateral ODS but Meningoencephalitis Acute, subacute, or chronic bilateral visual loss, and both were proven to have sar- Venous sinus thrombosis coidosis. The pathophysiologic features of the ODS and Macroinfiltrative Optic disc granuloma Optic nerve and visual loss differed in the 2 patients and prompted us to optic chiasm infiltration* review the differential diagnosis of ODS in sarcoidosis neuropathy Orbital apex mass Microscopic infiltration (Table). Patient 1 reminded us that although papill- Retro-orbital infiltration Perineural vasculitis edema may manifest more in one eye, owing to ana- Papillitis Optic neuritis Retinal periphlebitis tomic asymmetry of the nerve sheaths, resolution of ODS Severe posterior uveitis in a patient with chronic papilledema may also reflect op- tic atrophy. Patient 2 reminded us that optic neuropa- *A form of optic neuropathy that may clinically mimic idiopathic thy can present with or without ODS. Although visual intracranial hypertension. loss was bilateral, the optic neuropathy in the right eye was retrobulbar and only the left disc was swollen. Cor- The findings from gadolinium-enhanced magnetic rect interpretation of disc findings may reveal the de- resonance images of brain and orbits were normal. Op- gree of central nervous system involvement and have con- ening pressure was 25 cm H2O, and CSF analysis was con- sequences for therapy. sistent with aseptic meningitis (white blood cell count, 69/µL; 87% lymphocytes; high protein level, 0.11 g/dL; PAPILLEDEMA normal glucose level; and negative findings on serologic tests, microbial stains, and cultures). Oligoclonal bands Papilledema is ODS caused by elevated ICP. In neuro- were negative in CSF and serum. The CSF ACE was nor- sarcoidosis, elevated ICP can have several origins. Intra- mal, but serum ACE level was elevated (64.1 U/L). Chest cranial, noncaseating granulomata of sufficient size or at computed tomography disclosed bilateral hilar lymph- significant locations may mimic intracranial tumors on adenopathy and pulmonary nodules. A hilar lymph node neuroimaging3 and can raise ICP, causing papilledema.4 biopsy specimen demonstrated granulomatous lymph- Granulomatous meningoencephalitis can elevate ICP adenitis, and a liver needle biopsy specimen showed non- sufficiently to cause papilledema and may have an acute, caseating, epithelioid granulomatous hepatitis, consis- subacute, or chronic course. Patients
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