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Isolated amyloid deposition in an extraocular muscle is a rare event but can be a presenting feature of systemic amyloidosis. A 67-year-old woman with an acquired exotropia and hypertropia was found to have unilateral diffuse extraocular muscle enlargement on magnetic resonance imaging. Owing to the progressive nature of her strabismus and the negative laboratory testing for thyroid disease, she underwent an extraocular muscle biopsy that revealed amyloid deposition. Further workup demonstrated a monoclonal gammopathy consistent with systemic amyloidosis. This case demonstrates the need to consider amyloidosis in the differential diagnosis of patients presenting with an atypical acquired strabismus. We review other reports of isolated amyloid deposition in extraocular muscles and its association with systemic amyloidosis, emphasizing the importance of the ophthalmologist in the early recognition of this disease to prevent irreversible, life-threatening end organ damage.

A 16-year-old adolescent girl with multiple risk factors for thrombosis presented with acute onset of headache, decreased vision, and papilledema. Evaluation demonstrated cerebral venous thrombosis (CVT) involving the left transverse and sigmoid sinuses and left internal jugular vein. Following bilateral optic nerve sheath fenestration (ONSF), she experienced improvement in vision and resolution of papilledema. In selected cases, ONSF is an effective surgical option for the treatment of papilledema due to CVT after medical treatment has failed.

This Hoyt lecture is composed of 2 topics. First, a series of patients with idiopathic intracranial hypertension (IIH) is presented, emphasizing the importance of magnetic resonance venography (MRV). Study of the cerebral venous sinuses in IIH may demonstrate focal stenosis or venous gaps and represent a manifestation of elevated intracranial pressure. Conversely, the clinical picture of IIH may occur following cerebral venous sinus thrombosis, and MRV may be essential in establishing this diagnosis. In the future, evaluation of flow in the cerebral venous sinuses may play an important role in determining the potential for visual failure. Second, I will review patients with visual cognitive changes, which often go unrecognized. These patients suffer from visuoperceptual disturbances, recognizing parts of visual scenes but not the entire picture and are unable to comprehend their visual environment. These findings are often part of the syndrome of posterior cortical atrophy characterized by parieto-occipital atrophy, enlargement of the atrial portion of the ventricular system, and diminished metabolic activity in the posterior portion of the brain demonstrated with positron emission tomography.

We present two cases of delayed optic nerve complications following proton beam radiotherapy. Patient 1 received radiotherapy after surgery for clival chordoma and developed sequential radiation optic neuropathy at 5 and 9 months. Patient 2 developed optic nerve hemorrhage post radiotherapy for optic nerve sheath meningioma. These two cases of blindness demonstrate the risks associated with proton beam radiotherapy.

Background and Purpose: Endovascular transvenous embolization has been advocated as the treatment technique for dural carotid cavernous fistulas (dCCFs). Most centers use platinum coils primarily. The purpose of this study was to evaluate the technical aspects, efficacy, and safety of transvenous n-butyl cyanoacrylate (n-BCA) infusion in dCCFs as a primary alternative or adjunct to coil embolization., Methods: We retrospectively evaluated 14 patients with dCCFs who were treated at this institution from 1999 to 2004 by using n-BCA infusion alone or in combination with coils. The efficacy of treatment and safety aspects were studied in dCCFs of Barrow type B (4/14), C (2/14), and D (8/14). Six patients were treated with transvenous n-BCA infusion alone in the cavernous sinus, 7 with a combination of transvenous n-BCA and coil embolization, and one with transvenous n-BCA combined with transarterial polyvinyl alcohol (PVA)-particle embolization of the feeding arteries., Results: An angiographic obliteration and clinical cure was achieved in all patients. Technical complications were nonsymptomatic and included spillage of an n-BCA droplet into a middle cerebral artery branch retrograde through the arteriovenous fistulas in one patient and perforation of the inferior petrosal sinus during microcatheter placement in another. A third patient developed temporary palsy of the sixth cranial nerve a few days after the treatment., Conclusion: In this small series, the use of n-BCA either alone or in conjunction with detachable coils was a safe and effective technique for the treatment of symptomatic patients presenting with complex dCCFs.

Purpose: To review the clinical characteristics, prevalence, and severity of retinopathy in diabetics with cranial nerve (CN) 3, 4, and/or 6 palsies, and to determine the relationship between type and duration of diabetes mellitus (DM), presence of retinopathy, and occurrence of CN palsy., Design: Retrospective, comparative cohort study., Participants: Chart reviews of 2229 patients with CN 3, 4, and/or 6 palsies were performed at the Bascom Palmer Eye Institute (BPEI) from January 1991 through December 1997 and at the Dean A. McGee Eye Institute (DMEI) from January 1994 through July 2001. A total of 306 patients qualified for the study group. The Wisconsin Epidemiologic Study of Diabetic Retinopathy (WESDR) was used as a control., Methods: Demographic and clinical data were extracted to determine characteristics of patients with diabetic ophthalmoplegia. The subsets of data regarding type of DM and level of diabetic retinopathy in the study population were compared with the WESDR control data for statistical analysis., Main Outcome Measures: The prevalence of diabetic retinopathy in patients with diabetic ophthalmoplegia., Results: Of 2229 patients at both institutions with ocular motor CN palsy, 306 (13.7%) were associated with DM. The frequency of CN involvement was 6 (50.0%), 3 (43.3%), and 4 (6.7%). There was a total of 12 patients (3.9%) with consecutive palsies and 8 patients (2.6%) with simultaneous palsies (5 unilateral and 3 bilateral). At both institutions, the prevalence of retinopathy controlling for duration of DM was lower in both insulin-dependent DM (IDDM) and non-IDDM (NIDDM) type II diabetics as compared with controls (BPEI, P = 0.009 and P = 0.005; DMEI, P = 0.004 and P = 0.29). When data from both locations were combined, the difference was even more significant (IDDM, P = 0.001 and NIDDM, P = 0.006). There were no significant differences between the two institutions in gender, type or duration of DM, age at presentation, or frequency of CN involvement., Conclusions: Diabetic ophthalmoplegia most commonly involves CN 3 and 6, with relative sparing of CN 4. Multiple cranial nerves are affected simultaneously in 2.6% of cases, and consecutive palsies occurred in 3.9% of cases. Type II diabetics with ocular motor CN palsy have significantly less diabetic retinopathy than do controls. This may imply a different pathophysiologic mechanism for these two microvascular complications of DM.

Purpose: To report infraorbital nerve dysfunction after laser in situ keratomileusis., Design: Observational case report., Methods: Neuro-ophthalmologic examination with brain and orbital magnetic resonance imaging (MRI) and orbital computed tomography (CT)., Results: During laser in situ keratomileusis, two healthy women, aged 42 and 46 years, experienced acute onset of sharp ipsilateral cheek pain. Both cases occurred during manipulation of the eyelid speculum. Postoperatively, ipsilateral numbness and tingling or pain of the upper cheek was reported, and examination showed decreased sensation in the distribution of the infraorbital nerve. In both cases, brain and orbit MRI and orbit CT were normal. Both patients were managed medically. In one patient, mild symptoms persisted 1 year postoperatively, and in the second patient, moderate discomfort persisted 8 months postoperatively., Conclusion: Infraorbital nerve palsy is a potential complication of laser in situ keratomileusis. Symptoms improve but may persist.

Ischemic optic neuropathy occurred in a patient following liposuction. Perioperative anemia and hypotension may be the cause of this complication. Correction of anemia with transfusion improved the hemoglobin and hematocrit but the right eye remained blind. Liposuction should be added to the list of the surgical procedures that may produce ischemic optic neuropathy as an isolated complication.

We report a case of tuberculum sellae meningioma with optic tract edema. Contrary to a prior report on this topic, edema along the optic tract is not only seen in craniopharyngiomas but may be seen (although rarely) in other common parasellar tumors, as in our case of a tuberculum sellae meningioma. The pathogenesis of this edema in meningioma is controversial.

A patient with advanced AIDS presented with right conjugate gaze palsy and impaired adduction on left gaze (the one-and-a-half syndrome). The responsible pontine lesion involved the ipsilateral abducens nucleus and the adjacent medial longitudinal fasiculus (MLF), as demonstrated by magnetic resonance imaging (MRI). Tuberculosis (TB) was the etiology of the brainstem lesion. The patient had complete recovery after anti-tuberculosis treatment. The nuclear location of the tuberculoma is unusual and the reversal of this neuro-ophthalmologic syndrome is noteworthy.

The clinical course of four patients with visual loss in the postpartum period due to acute optic neuritis is described. Factors that disclosed the underlying etiology and expression of disease are discussed. The clinical records of four women examined and managed for visual loss after uncomplicated pregnancies and term deliveries were reviewed. Neurodiagnostic examination, treatment modalities, and outcomes were assessed. These four women with varied and confounding medical histories, all with optic neuropathy, eventually were demonstrated to harbor demyelinating disease. Although visual loss in the postpartum period evokes differential diagnostic considerations, the authors' experience suggests that puerperal immune-mediated changes are responsible for activation of optic neuritis associated with relapsing multiple sclerosis.

A patient with generalized inflammatory polyneuropathy and facial diplegia was studied with magnetic resonance imaging. Multiple cranial nerves showed signal enhancement without sensory or motor dysfunction.

A patient with typical pituitary apoplexy with documented intralesional hemorrhage and spontaneous resolution is presented. There was no evidence of a tumor at a 3-month follow-up.

A 42-year-old woman with a 6-year history of diabetes insipidus and progressive hypersomnolence presented with visual loss. Neuroimaging showed infiltration in the hypothalamus, the optic nerve, and the chiasm, as well as multiple lesions in other areas of the brain parenchyma. Biopsy showed Langerhans cell histiocytosis. This is an unusual presentation of Langerhans cell histiocytosis, involving the visual pathways without manifestations outside of the central nervous system. The differential diagnosis and the magnetic resonance imaging findings will be discussed.

Objective: To determine the incidence of positive neuroradiologic studies in consecutive patients with glaucoma associated with normal intraocular pressure and to compare the psychophysical and clinical characteristics of these eyes with eyes with disc cupping associated with intracranial masses., Design: Retrospective case-controlled study., Participants: Fifty-two eyes of 29 patients with glaucoma associated with normal intraocular pressure and 44 eyes of 28 control patients with compressive lesions were reviewed., Intervention: The medical records of consecutive glaucoma patients with normal intraocular pressure who underwent brain magnetic resonance imaging or computed tomography scanning as part of a diagnostic evaluation between January 1, 1985, and July 1, 1995, were reviewed. A masked reading of optic nerve photographs and visual fields was performed by one observer. A similar analysis was performed on a control group of consecutive patients with nonglaucomatous optic nerve cupping with known intracranial mass lesions., Main Outcome Measures: The neuroradiologic findings, clinical characteristics, optic nerve head appearance, and patterns of visual field loss were compared between groups., Results: None of the patients diagnosed with glaucoma had neuroradiologic evidence of a mass lesion involving the anterior visual pathway. Compared to control subjects, patients with glaucoma were older (P = 0.0001), had better visual acuity (P = 0.002), greater vertical loss of neuroretinal rim tissue (P = 0.0001), more frequent optic disc hemorrhages (P = 0.01), less neuroretinal rim pallor (P = 0.0001), and more nerve fiber bundle visual field defects aligned at the horizontal midline (P = 0.0001). Visual acuity less than 20/40, vertically aligned visual field defects, optic nerve pallor in excess of cupping, and age younger than 50 years were 77%, 81%, 90%, and 93% specific for nonglaucomatous cupping associated with compressive lesions, respectively., Conclusions: Anterior visual pathway compression is an uncommon finding in the neuroradiologic evaluation of patients with a presumptive diagnosis of normal-tension glaucoma. Younger age, lower levels of visual acuity, vertically aligned visual field defects, and neuroretinal rim pallor may increase the likelihood of identifying an intracranial mass lesion.

We report two patients with unique homonymous hemianopias from occipital lobe infarctions. One patient had vertical meridian sparing, and the other displayed horizontal meridian sparing. MRI correlation with the visual field defects confirmed that the vertical hemianopic meridian was represented along the border of the calcarine lips, and the horizontal meridian lies at the base of the calcarine banks deep within the calcarine fissure.

A 66-year-old woman presented with a 3-year history of predominantly right-sided blepharospasm and a 1-year history of progressive predominantly left-sided hemiparkinsonism manifested by a left upper extremity resting tremor and left-sided bradykinesia. Magnetic resonance imaging of the brain revealed a large right mesencephalic cyst with mass effect. Positron emission tomography revealed bilateral striatal hypometabolism consistent with nigrostriatal dopaminergic dysfunction. The association of predominantly ipsilateral blepharospasm and predominantly contralateral hemiparkinsonism is very rare, and its association with a posterior fossa space-occupying lesion has been reported only once. This is the second report of such an association and the first description of adult-onset symptomatology.

Cutaneous carcinomas of the face, and some nasopharyngeal carcinomas, may present with facial dysesthesias and/or facial nerve palsies in the absence of visible masses. Even with frank ophthalmoplegia, occult tumors that present in this matter may elude detection, for which reason specific diagnostic studies must be employed. We report seven cases of trigeminal nerve infiltration by occult tumors (five squamous cell carcinomas, one basal cell carcinoma, and one adenoid cystic carcinoma), and outline the clinical course, diagnostic investigations, and the subsequent management of these patients. The importance of establishing an early diagnosis before the tumor has transgressed the basal foramina is emphasized, as tumor infiltration of the cavernous sinus carries a more guarded prognosis. The use of magnetic resonance imaging to identify involved peripheral nerve branches that may then be biopsied is suggested. The patho-physiological mechanisms of neurotropic spread of tumor are reviewed.

The objective of the study was to determine the efficacy of optic nerve sheath fenestration (ONSF) in eyes with progressive visual or field loss in pseudotumor cerebri in spite of medical therapy with oral Diamox. Visual data on 29 eyes of patients with pseudotumor cerebri who underwent ONSF at Bascom Palmer Eye Institute from 1987 to 1995 were studied retrospectively. These patients had progressive visual loss despite medical therapy. Visual acuity and fields were compared before and after surgery (within 1 and 6 months). During a mean follow-up of 15.7 months (range, 1-50 months), visual acuity improved in four eyes (14%), was unchanged in 22 (76%) eyes, and worsened in three (10%) eyes. Visual fields improved in 10 (48%) eyes, remained unchanged in eight (38%) eyes, and worsened in three (14%) eyes (six were lost in long-term follow-up). There were four repeat surgeries in which vision was lost in one eye. Data from these patients indicate that ONSF improves or protects visual function in patients with pseudotumor cerebri who experience deteriorating visual acuity and fields in spite of medical therapy.

Background: Controversy exists regarding the cortical representation of the foveal region in man. The most plausible explanation for foveal sparing in homonymous hemianopia is dual blood supply to the occipital pole. However, bilateral cortical representation has also been suggested., Objective: To determine the representation of the foveal region in the primary human visual cortex., Design: A case series correlating magnetic resonance imaging to the visual field defects of 6 patients with occipital lobe injuries., Results: Four patients with foveal-sparing hemianopic defects demonstrated unilateral sparing of the posterior occipital cortex. In contrast, 2 patients with central homonymous defects showed isolated involvement of the posterior pole. One patient also demonstrated 5 degrees of field sparing along the horizontal meridian., Conclusions: On the basis of correlating the findings of magnetic resonance imaging and visual field defects in patients with occipital lobe injury, we conclude that the foveal region is unilaterally represented in the primary human visual cortex and that a major portion of the striate cortex is dedicated to the central 10 degrees of vision. The horizontal meridian of the visual field is represented deep within the calcarine banks at the fissure base.

A 15-year-old boy had onset of unilateral facial weakness. A few days later, he experienced mild vertigo, double vision, and headache. Examination confirmed a peripheral right seventh nerve weakness in addition to an internuclear ophthalmoplegia. The neurologic features suggested a pontine glioma. A T2-weighted MRI scan revealed demyelinating lesions in the pons and in several areas of the cerebrum, including the periventricular region. Subsequent history revealed that he had been diagnosed with Lyme arthritis 7 years earlier while living in Connecticut. The radiographic studies favored a diagnosis of multiple sclerosis. However, studies of blood and cerebrospinal fluid established a diagnosis of Lyme neuroborreliosis.

Purpose: We examined a 28-year-old woman who developed incongruous binasal and bitemporal visual field defects one week after having a febrile gastroenteritis characterized by severe diarrhea while traveling in Mexico., Methods: The patient was treated with intravenous methylprednisolone sodium succinate, 1 g daily for three days, followed by an eight-week course of oral prednisone., Results: Neuroradiologic examination demonstrated bilaterally increased signal intensity within the lateral geniculate bodies. Cerebrospinal fluid analysis disclosed isolated protein increase., Conclusion: Severe diarrhea may be associated with an aseptic bilateral lateral geniculitis resulting in hourglass-shaped visual fields. Corticosteroid therapy may provide mild improvement.

Purpose: Severe vistral acuity loss associated with pseudotumor cerebri is usually caused by chronic optic disk edema or a retinal abnormality., Methods: We treated a women, with known pseudotumor cerebri treated with a lumboperitoneal shunt, who developed acute pallied optic disk swelling and visual acuity of R.E.: no light perception and L.E.: 20/70 in association with lumboperitoneal shunt failure. There were no contributory retinal lesions., Results: The patient underwent optic nerve sheath fenestration and lumboperitoneal shunt revision. Visual acuity improved to 20/20 in both eyes. The papilledema resolved., Conclusion: The severe sudden visual loss was attributed to axoplasmic stasis and optic nerve ischemia associated with a sudden rise in intracranial pressure.

Traumatic chiasmal neuropathy results from injury to the face, sphenoid and clivus. Its pathogenesis remains enigmatic. Because of its close relationship to the pituitary gland, hypothalamus and internal carotid artery, a neuro-ophthalmic evaluation and imaging is needed in such cases. We present a patient who developed traumatic chiasmal syndrome after an automobile accident. Computed tomographic scan showed fracture of the sella turcica. A carotid angiography showed a traumatic pseudoaneurysm of the internal carotid at the base of the skull.

Background: Radiation optic neuropathy (RON) is a rare, unpredictable, late complication of radiotherapy secondary to obliterative endarteritis. Tumor recurrence has to be ruled out by a clinical and neuroradiological examination., Methods: Five patients with RON were investigated by magnetic resonance imaging (MRI) during 1992., Results: Radiation-induced lesions of the intracranial visual pathways were easily visible on MRI. Without Gadolinium, a sectorial swelling was detectable, which markedly enhanced with Gadolinium. Intracranial optic nerve was affected in 5/5 cases, optic chiasm in 3/5 cases, and optic tract in 2/5 cases., Conclusions: MRI is the examination of choice when RON is suspected: it will easily delineate the extent of the lesion, and compression/infiltration by a recurrent tumor will be formally ruled out. A segmental swelling of visual pathway with marked Gadolinium enhancement on MRI is highly suggestive of radionecrosis.

Neuroimaging with nuclear medicine techniques permits assessment of brain function by measurement of metabolism or blood flow. Such studies complement the anatomic information derived from computed tomography (CT) or magnetic resonance imaging (MRI). We describe two patients with occipital lesions who were initially diagnosed with functional visual loss. Neither CT scan nor MRI adequately demonstrated the source of visual dysfunction; however, single proton emission tomography (SPECT) scanning in a patient with carbon monoxide poisoning and positron emission tomography (PET) scanning in a patient with post-hypoxic delayed encephalopathy were helpful in confirming the organic substrate of their visual impairment. Functional imaging techniques such as SPECT and PET should be considered in patients with suspected cortical visual loss and normal CT or MR scans.

Purpose: To describe the MR characteristics of optic neuropathy caused by vasculitis., Methods: Nine cases of optic neuropathy with diagnosis of vasculitis (six with systemic lupus erythematosis and one each with rheumatoid arthritis, Sjögren disease, and radiation vasculitis) were reviewed retrospectively. Patients were 31 to 62 years old, and all but one were women. All patients had MR imaging through the orbits and anterior visual pathways, five with fat suppression, with and without gadopentetate dimeglumine. Five patients also had MR imaging of the entire brain. The size and enhancement of various segments of the optic nerve and anterior visual pathways were studied., Results: MR imaging with contrast material showed enhancement and enlargement of segments of the optic nerves and/or chiasm in six of the nine patients (all but three with systemic lupus erythematosis). Enlargement of a segment of the anterior visual pathway never occurred without enhancement, but enhancement alone did occur in three cases. Of the five patients who had MR imaging of the whole brain, abnormalities were seen in three: periventricular hyperintensity in two and a lacunar infarct in one; none had vessel abnormalities., Conclusion: Because the MR enhancement seen represents disruption of the blood-brain barrier within the optic nerve, MR imaging with gadopentetate dimeglumine and fat suppression should be performed to detect increased permeability of the blood-brain barrier in acute optic neuropathy.

Purpose/methods: Orthoclone OKT3 is a monoclonal murine IgG immunoglobulin used to treat acute cellular rejection of allografted organs. Aseptic meningitis and meningoencephalopathy are known adverse side effects from the drug. OKT3 caused additional ophthalmologic and neurologic complications in an 18-year-old woman who was treated for transplanted kidney graft rejection. Papilledema and bilateral abduction deficits developed., Results/conclusions: Results of magnetic resonance imaging and magnetic resonance angiography were unremarkable. Lumbar puncture disclosed increased intracranial pressure and sterile meningeal inflammation. Most of the symptoms resolved by one week after discontinuation of OKT3. Ophthalmologists and neurologists should be aware that optic disk swelling and abducens palsies can be associated with OKT3 when used in the treatment of transplanted kidney graft rejection.

Ten patients with migraine developed persistent positive visual phenomena lasting months to years. The complaints were similar in their simplicity and involvement of the entire visual field and usually consisted of diffuse small particles such as TV static, snow, lines of ants, dots, and rain. Neurologic and ophthalmologic examinations were normal, and EEGs were normal in eight of eight patients tested. MRI was normal in all patients except one who had nonspecific biparietal white matter lesions and another with a small venous angioma. Treatment of this unusual complication of migraine was unsuccessful.

Purpose: To characterize visual morbidity in giant cell arteritis and to assess prognosis with respect to treatment., Methods: Record review of 185 patients with coded diagnosis of giant cell (cranial) arteritis examined at the Bascom Palmer Eye Institute from January 1, 1980, to January 31, 1993., Results: Forty-five patients with biopsy-proven giant cell arteritis had visual symptoms, and 41 individuals (63 eyes) lost vision. The visual loss was unilateral in 19 patients (46%), sequential in 15 (37%), and simultaneous in 7 (17%). Anterior ischemic optic neuropathy developed in 88% of eyes, visual acuity was 20/200 or worse in 70%, 21% had no light perception, and the majority of field defects in testable eyes, aside from central scotomas associated with loss, showed altitudinal or arcuate patterns. Six patients lost vision during corticosteroid therapy for systemic symptoms of giant cell arteritis, whereas in 39 patients visual symptoms prompted steroid treatment. For visual symptoms, 25 patients received intravenous methylprednisolone, whereas 20 received oral prednisone alone. In the 41 patients with visual loss, vision was unchanged in 20 (49%), it worsened in 7 (17%), and it improved in 14 (34%). Subsequent fellow eye involvement was observed only with oral therapy, and a greater percentage of patients (9/23 [39%] versus 5/18 [28%]) improved after intravenous treatment., Conclusions: In the authors' series, patients with visual loss due to giant cell arteritis had a 34% chance for some improvement in visual function after corticosteroid treatment. Intravenous therapy may diminish the likelihood of fellow eye involvement and was associated with a slightly better prognosis for visual improvement.

Objective: To determine the efficacy of optic nerve sheath fenestration in eyes with the progressive form of common anterior ischemic optic neuropathy. This investigation complements a previously reported initial series of 26 similar patients., Patients and Methods: Optic nerve sheath fenestrations were performed in 21 eyes for treatment of common (nonarteritic) ischemic optic neuropathy with documented progressive deterioration of visual function. All patients underwent thorough preoperative and postoperative ocular evaluations by the Neuro-Ophthalmology Service at the Bascom Palmer Eye Institute, Miami, Fla., Results: During a mean follow-up of 22.5 weeks (range, 3 to 104 weeks), results were as follows: visual acuity increased by 2 or more lines on the Snellen chart in two eyes (9.5%; the combined improvement rate for the two series was 14.9%); visual acuity decreased by 2 more lines in two eyes (9.5%)., Conclusions: Data from this series of 21 surgical procedures indicate no beneficial effect on visual morbidity in cases of common ischemic optic neuropathy and confirm the generally poor visual outcome implied from the results of optic nerve sheath decompression in our initial series. Based on this experience with a total of 47 eyes, we have discontinued optic nerve sheath decompression as a form of therapy for ischemic optic neuropathy.

We treated four patients who had acute, severe visual loss associated with pseudotumor cerebri with intravenous methylprednisolone (250 mg four times per day) for five days followed by an oral taper, in combination with acetazolamide and ranitidine. In addition to high-grade disk edema, one patient had serous detachment of both maculas and lipid deposition, one had a unilateral macular star, and one had a monocular branch retinal artery occlusion. These three patients experienced rapid and lasting improvement in visual acuity, visual field, papilledema, and symptoms. Vision of the fourth patient did not improve, requiring optic nerve sheath fenestration for chronic papilledema. Transient acne developed in one patient. This regimen is a safe, effective treatment of acute, severe visual loss associated with florid papilledema of pseudotumor cerebri. Lack of immediate improvement is an indication for optic nerve sheath decompression.

Background: Systemic lupus erythematosus (SLE) is a multisystemic inflammatory disease of unknown etiology. Physiopathology includes small vessel occlusion and auto-antibody production. Neuro-ophthalmic complications can be detected in 10-30% of SLE cases. Further, some patients can present with neuro-ophthalmic complications prior to diagnosis of SLE., Patients and Methods: We report seven cases of neuro-ophthalmic complications of SLE demonstrating lesions from the retina to the brain. The other possible manifestations of SLE will be reviewed., Results: Two cases were not yet diagnosed with SLE when neuro-ophthalmic disease occurred. Anti-double stranded DNA antibodies were detected at a very high titer, suggesting SLE, later confirmed by rheumatologists., Conclusions: Ophthalmologists should be aware of the unusual patient presenting with a neuroophthalmic disorder prior to the diagnosis of SLE. In such cases, autoantibodies should be sought. Antinuclear antibodies are very sensitive but non specific for SLE. Anti-double stranded DNA antibodies are specific for SLE and elevated titer should raise the suspicion of undiagnosed SLE.

We report three patients with chronic headaches and optic neuropathy due to widespread meningeal thickening shown on enhanced MRI; all had biopsy-proven intracranial pachymeningitis (fibrosclerosis of the meninges). Two patients had bilateral optic neuropathy, elevated CSF protein, and polyclonal serum hypergammaglobulinemia. They developed temporal lobe cortical necrosis or sagittal sinus thrombosis, presumably due to compromised dural venous drainage from extensive meningeal fibrosis. The other patient had multiple cranial nerve palsies and unilateral optic neuropathy with normal CSF. Corticosteroid therapy improved visual function in all three patients, although all had persisting visual deficits. Gadolinium-enhanced MRI was essential in identifying meningeal inflammation and locating suitable biopsy sites.