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From the Department of Neurology, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seoul, Korea.
Address correspondence and reprint requests to Dr. Ji Soo Kim, Department of Neurology, Seoul National University College of Medicine, Seoul National University Bundang Hospital, 300 Gumi-dong, Bundang-gu, Seongnam-si, Gyeonggi-do, 463-707, Korea jisookim{at}snu.ac.kr.
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Given the development of headache after neck massage, traumatic vertebral artery dissection should be considered. However, vertebral artery dissection mostly gives rise to dizziness/vertigo, posterior neck pain, and other focal neurologic deficits. Migraine is a common cause of headache in young women and rarely accompanies diplopia (ophthalmoplegic migraine).1 However, ophthalmoplegic migraine is an unlikely diagnosis in an adult since it usually develops before age 10. Furthermore, the interval of 1 week from the headache onset to diplopia in our patient is also unusual for ophthalmoplegic migraine.1
On admission, examination showed an overweight woman with a body mass index of 28.3. Her blood pressure was elevated at 192/122 mm Hg with normal heart rates and body temperature. Corrected visual acuities were 20/20 in both eyes with normal confrontation visual fields and pupillary responses without a relative afferent pupillary defect. However, funduscopic examination revealed optic disc swelling with peripapillary hemorrhages in both eyes, more severe in the left eye (figure 1). Both eyes were esotropic with limitation of abduction on attempted lateral gaze. Other findings of physical and neurologic examinations were normal.
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However, other causes of increased ICP should be considered, which include brain tumor, infectious, inflammatory, and malignant disorders involving the meninges, and a venous sinus thrombosis.2 In endemic areas, obstructive hydrocephalus due to neurocysticercosis is an important differential diagnosis. A markedly elevated blood pressure (malignant hypertension) could give rise to disc swelling in addition to headache, but bilateral abduction deficits are an exception.1,2 Diabetes mellitus (diabetic papillopathy) also could generate disc swelling without affecting optic nerve function in the presence of normal ICP.1,2
Since loss of central vision develops only during the advanced stage of papilledema (optic disc swelling from increased ICP), visual acuity is not a reliable indicator of visual loss, especially during the early stage of papilledema.2,3 If central visual acuity is reduced in a patient with acute papilledema, the cause is typically intraretinal fluid/edema.2 In contrast, visual field testing may disclose enlargement of the blind spot in almost all patients with papilledema.2,3 Other common visual field defects are generalized constriction and inferonasal loss (nasal step).2
In patients with suspected papilledema, neuroimaging should be performed immediately, prior to lumbar puncture, to exclude disorders with a risk for herniation during the procedure and to search for any secondary cause of increased ICP.2 MRI is superior to CT and enhanced imaging would aid in better detection of mass lesions including tumors and parasites, and meningitis of infectious, inflammatory, or carcinomatous origin.2 However, CT is adequate to detect mass lesions that are responsible for the increased ICP, especially when MRI is not readily available.4 CT or MR venography should be included to rule out venous sinus thrombosis.2,4 Rarely conventional catheter angiography with venous phase is required. In IIH, MRI is mostly performed to exclude other intracranial pathologies which may increase ICP.2,4 However, MRI may exhibit radiographic evidence of increased ICP, which includes slit ventricle, empty sella, flattening of the posterior sclera, distension of the perioptic subarachnoid space, tortuous optic nerve, protrusion and enhancement of the optic discs, and Chiari type 1 malformation.5 Scrutinized evaluation of MRI may assist in establishing the diagnosis of IIH.
If the neuroimaging does not show a mass lesion, obstructive hydrocephalus, or evidence of cerebral venous thrombosis, a lumbar puncture should be followed to confirm increased ICP (>250 mmH2O) and to rule out malignant, infectious, or inflammatory disorders simulating IIH.2 The CSF examination should include cytology, IgG index, viral and syphilis markers, and serology for parasites and fungi, if indicated.2 The profiles should be normal in IIH.2 Patients also should undergo blood tests including complete blood counts, erythrocyte sedimentation rate, coagulation battery, electrolytes, tests for syphilis, and thyroid function tests.2
In our patient, blood tests included complete blood counts, routine chemistry, erythrocyte sedimentation rate, C-reactive protein, coagulation panel, thyroid function tests, rheumatoid factor, antinuclear antibody, anti-ds-DNA antibody, and antineutrophil cytoplasmic antibodies, which were all normal. Goldmann perimetry showed enlarged blind spots in both eyes (figure 2A). MRI exhibited flattening of the posterior sclera, distension of the perioptic subarachnoid space, tortuous optic nerve, and protrusion and enhancement of the optic discs (figure 2B). MR venography was normal. Lumbar puncture revealed an elevated opening pressure of 430 mmH2O with normal CSF profiles, no white blood cell, 52.6 mg/dL of protein, 67 mg/dL of glucose, and IgG index of 0.3.
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The pathophysiology of IIH remains unknown.2 The proposed hypotheses mostly concern deranged CSF homeostasis, and include diffuse brain edema, excessive CSF production, reduced CSF absorption, and increased cerebral venous pressure, which require further elucidation.2,4 Various conditions also have been implicated in IIH.2 However, apart from female sex, recent weight gain, and obesity, there are no proven associations.2–4
In IIH, the primary goals of treatment are preservation of vision and alleviation of the symptoms.2,6 Treatment options may depend on several factors including the severity of symptoms, presence and progression rate of visual loss, severity of disc swelling, and association with obesity or systemic hypertension.2,6
Medical treatments of IIH include carbonic anhydrase inhibitors (acetazolamide, methazolamide, topiramate) and diuretics (furosemide, chlorthalidone).2,4,6 Systemic corticosteroids may be used in urgent cases of impending or progressive visual loss while arranging a surgical procedure.2,6 Since weight reduction may relieve disc swelling, weight loss should be advised for patients with obesity.2 Coexisting systemic hypertension confers a poor visual prognosis in patients with IIH and should be controlled appropriately.7
Our patient began acetazolamide 500 mg and furosemide 20 mg twice a day and her headache improved over the following several days even though the papilledema and diplopia persisted. She was discharged with the medication and arranged for a weight reduction program. However, 2 weeks later, she reported transient visual obscuration on standing and hissing sound in the right ear. Her visual acuity had decreased to 20/30 in the left eye and funduscopic examination revealed a progression of the papilledema and newly developed macular star in both eyes. Goldmann perimetry also documented further aggravation of the enlarged blind spot.
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Some patients with IIH may show a rapid development of symptoms and precipitous visual decline.2 They often have significant visual field loss, decline in the visual acuity, and marked papilledema at presentation. A rapid deterioration requires urgent treatments to preserve vision, which include IV corticosteroids, IV acetazolamide, and surgery.2,6
Surgical procedure is indicated when patients present with severe papilledema and visual loss or when the medical treatments fail to control papilledema or prevent visual loss.2,6 Surgery also should be considered in patients with intractable headache or inability to perform visual function studies.6 CSF shunting and optic nerve sheath decompression (ONSD) are two major options and the choice of the procedure depends on the availability of a surgeon and the patient status.2,4 ONSD is preferred to treat significant visual symptoms, especially when severe papilledema extends into the macula, while CSF shunting is performed when headache is a major complaint.4 ONSD has little effect on ICP in most cases.2,4 Visual loss, diplopia, and infection may be complications of ONSD.2 Ventriculo- or lumboperitoneal shunting can effectively control ICP in IIH.2 Ventriculoperitoneal shunt may be technically difficult when the ventricle is not enlarged.2 Lumboperitoneal shunting can be rather easily performed but intracranial hypotension and tonsilar herniation are serious complications even though adopting a programmable valve may prevent many of the complications from lumboperitoneal shunts.2 CSF diversion surgery usually results in prompt normalization of ICP, resolution of papilledema, and improved visual function.2 However, shunting procedures require frequent revisions due to complications, which include shunt obstruction, intracranial hypotension with tonsilar herniation and lumbar radiculopathy, infection, and abdominal pain.2 Overall, shunt malfunction rate is approximately 50% in IIH.2 Infection is a rare (1%) but serious complication.8 Repeated lumbar puncture may be considered in selected patients with intermittent symptom exacerbations or in pregnant patients.2
In view of the aggravated papilledema with newly developed transient visual obscuration, tinnitus, and visual decline in the left eye despite the medical therapy, our patient was readmitted for close monitoring of her visual function and a shunt surgery to reduce her ICP. Three days later, her visual acuity deteriorated further to 20/30 in the right eye. She underwent a lumboperitoneal shunt operation. After the operation, she reported mild headache on standing for several days, probably due to low-pressure syndrome, but the tinnitus, visual obscuration, and diplopia disappeared over the following several days. Follow-up funduscopy 10 days after the operation showed a marked improvement of the papilledema (figure 3A). The enlarged blind spots on Goldmann perimetry also resolved (figure 3B) along with improvement of the bilateral abduction limitation.
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Since enlarged blind spots and peripheral field defects are early visual losses,2,3,7 and impaired central vision (visual acuity) is usually a late phenomenon in IIH, a careful evaluation and monitoring of visual field defects are required using quantitative perimetry, especially during the early stage.2 With progression of disease, ischemic optic neuropathy may occur, producing irreversible impairments of central vision2 even though visual loss may occur due to macular edema complicated by papilledema.2 Most visual defects in IIH are reversible if ICP is controlled before severe visual loss or optic nerve ischemia develops.2 Early central visual loss with rapid progression is a grave sign and requires prompt intervention.2,3,7
Even though IIH is usually a self-limiting condition,2,7 visual loss occurs in 4% to 31% and blindness in up to 5% of the patients.3,7–9 Furthermore, recurrence rate of IIH ranges from 10% to 40%, depending on the follow-up period,8 and some patients have delayed worsening or recurrences even several years after resolution of papilledema.7,10 Many patients with IIH also show increased ICP several years after onset of the disease.7 Accordingly, IIH may be a chronic condition, warranting long-term follow-up.10
Supported by a grant from the Korea Health 21 R&D Project, Ministry of Health & Welfare, Republic of Korea (A080750).
Disclosure: The author reports no disclosures.
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