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Background Cerebral venous sinus thrombosis (CVST) is a life-threatening condition that may present with symptoms and signs identical to idiopathic intracranial hypertension (IIH). Both conditions consist of increased intracranial pressure (ICP); however, IIH shows no evidence of contributory intracranial pathology. Oral hormonally based contraceptive usage has been associated with both conditions as well. Because disc edema often occurs in CVST, and IIH and is evident in other sight- and life-threatening conditions such as intracranial mass lesions, neuroimaging and other ancillary testing must be conducted for definitive diagnosis. Case An 18-year-old white woman with IIH previously diagnosed presented with headaches and severe visual loss in the left eye. Bilateral disc edema with macular edema in the left eye (O.S.) was present. Threshold perimetry found bilaterally enlarged blind spots as well as a central scotoma O.S. Her medical history was significant for a right transverse, sigmoid and jugular siphon thrombosis secondary to a hypercoagulable state and associated with her use of oral birth control medication. Subsequent magnetic resonance venography (MRV) found the presence of CVST. Despite anticoagulation therapy and acetazolamide, she had severe, nonresolving papilledema. Treatment with oral prednisone was initiated. She recovered full visual fields and excellent visual acuity. Conclusion Because of the similarity in clinical presentation of CVST and IIH, it is important to differentiate distinguishing characteristics of these diseases for correct diagnosis and prompt treatment.

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.

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 fasciculus (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.

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.

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.

Background: Occlusive vasculitis affecting the optic nerve or chiasm is an infrequent delayed complication of radiation treatment. Hyperbaric oxygen (HBO) has been used to treat such a complication...

Purpose To report two cases of progressive outer retinal necrosis occurring in immunocompetent individuals after treatment with corticosteroids for presumed optic neuropathy. Design Observational case report. Setting University–based tertiary eye hospital. Methods Retrospective review of existing clinical records. Results Two patients were treated empirically with systemic corticosteroids for suspected inflammatory papillopathy. Subsequently, both were diagnosed with necrotizing herpetic retinitis with features of progressive outer retinal necrosis. Anterior chamber paracentesis confirmed varicella-zoster infection. Both patients were human immunodeficiency virus negative; one patient with rheumatoid arthritis was taking etanercept. Both became completely blind in one eye despite intensive treatment with antiviral medication intravenously and intravitreally. Conclusions Progressive outer retinal necrosis is not confined to patients with underlying severe immunodeficiency, such as acquired immune deficiency syndrome. Initial treatment of acute, unexplained vision loss with systemic corticosteroids may lead to catastrophic visual loss in patients with evolving necrotizing herpetic retinopathy.

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.

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.

After transsphenoidal resection of a pituitary (or other) tumor, the remaining intrasellar cavity, and sphenoid sinus are usually packed with exogenous fat or muscle to prevent cerebrospinal leak and prolapse of the optic chiasm into an empty sella. We treated two patients in whom chiasmal compression occurred postoperatively because of packing of fat. In one patient, the expected visual improvement in the postoperative period was suboptimal. The subsequent removal of fat resulted in total visual recovery. In the other patient, chiasmal compression persisted from intrasellar fat and residual tumor. Iatrogenic compression of the optic nerves or chiasm should be considered in all patients in whom visual recovery is incomplete.

Hodgkin's disease is a rare cause of infiltrative optic neuropathy, which typically evolves late in the disease course. We managed an unusual case of isolated optic neuropathy in a 21-year-old man occurring during clinical remission from Hodgkin's disease. Radiotherapy and treatment with high-dose systemic corticosteroids resulted in dramatic improvement in vision. Even without other evidence of recurrent disease, acute-onset optic neuropathy in a patient with a history of a lymphoproliferative disorder should raise the question of a reemergence of the malignancy.

Dural sinus hypertension from cerebral venous outflow impairment is a cause of pseudotumor cerebri. The authors documented six such patients: two with unilateral neck dissection, one with surgical ligation of the dominant sigmoid sinus, two with thrombosed central intravenous catheterization, and one with dural sinus thrombosis. The site of cerebral venous outflow obstruction was variable and identified in three patients with computed tomography, conventional magnetic resonance imaging, magnetic resonance angiography, and/or angiography. Magnetic resonance angiography used in two patients characterized the venous flow pattern and identified the site of obstruction, confirming magnetic resonance angiography as an effective noninvasive blood flow technique in diagnosing and following these patients. Three patients were treated successfully with medical therapy and one patient with optic nerve fenestration. The two patients with thrombosed central venous catheters had serious systemic illnesses and suffered permanent visual loss.

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.

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.

Three patients with delayed radiation optic neuropathy after radiation therapy for parasellar neoplasms underwent magnetic resonance imaging. The affected optic nerves and chiasms showed enlargement and focal gadopentetate dimeglumine enhancement. The magnetic resonance imaging technique effectively detected and defined anterior visual pathway changes of radionecrosis and excluded the clinical possibility of visual loss because of tumor recurrence.

Three of ten children with optic gliomas satisfy the 1987 National Institutes of Health consensus criteria for neurofibromatosis type 1 (NF-1). The diagnosis in these cases is based on the presence of café-au-lait skin lesions in addition to the optic glioma. Two children without skin lesions have typical findings on magnetic resonance imaging (MRI) that also suggest a diagnosis of NF-1. A review of 281 NF-1 children with brain MRI results demonstrates the presence of these abnormalities in 57% of the cases. It is suggested that this MRI abnormality is sufficiently specific to add this feature to the list of diagnostic criteria. When MRI findings are present in a child with optic glioma and without skin lesions, counseling should be given for NF-1. It is noteworthy that these typical MRI findings in NF-1 are less common in adulthood, apparently because of spontaneous resolution.

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.

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.

AIMS: A variety of acute and chronic orbitopathies may be distinguished by standardised echography. Venous stasis orbitopathy (VSO) often presents with orbital signs when secondary to cavernous sinus and middle cranial fossa disorders. In this study, the aim was to assess whether differentiation between vascular and nonvascular causes of VSO could be made on the basis of clinical and echographic features at the time of presentation. METHODS: This study comprised 37 patients with echographic features of VSO (17 patients with arteriovenous fistulae, confirmed by computed tomography imaging or angiography, and 20 patients with non-vascular diseases). Excluded were patients with orbital mass lesions detected by echography and muscle enlargement due to other causes (for example, orbital myositis). Patients with a suspected mass involving the orbital apex and echographic features of VSO were included. After full neuro-ophthalmic and ocular examination, both orbits were examined to document maximal thickness and reflectivity of four recti muscles and compared with the normal contralateral orbit with standardised A-scan (Kretz-technik 7200MA or Ophthascan) and contact B-scan (Ultrascan or Ophthascan S). RESULTS: Cumulative ocular recti muscle thickness was significantly greater in patients with arteriovenous fistulae compared with the non-fistula group (23.3 (SD 3.7) and 17.8 (2) mm, p = 0.001). Clinically, the presence of a bruit and a uniocular rise in intraocular pressure were significantly greater in the fistula group of patients. CONCLUSIONS: Standardised echography is a safe and non-invasive method of diagnosing VSO in patients presenting with signs of proptosis, ophthalmoplegia, and inflammation of the conjunctiva. Furthermore, using these standard techniques the two major causes of VSO (arteriovenous fistulae and compressive mass lesions) could be differentiated.

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 or 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 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.

Homonymous visual field defects are rare in patients with MS and are usually caused by large lesions in the white matter of the posterior optic radiations and visual cortex. The postulate of Holmes and others, early this century, that optic radiation lesions could produce quad-rantic defects has recently been questioned. We report the full recovery of a patient with probable MS who presented with congruous quadrantanopia from a small white matter lesion in the contralateral trigone area.

Clinical data and fundus fluorescein angiograms were analyzed from 35 patients with acute (onset less than four weeks) anterior ischemic optic neuropathy. Nineteen of the 35 patients (54%) had nonarteritic disease, and 16 patients (46%) had giant cell arteritis confirmed by biopsy. Patients with arteritis had higher erythrocyte sedimentation rates, larger cup/disk ratios, and delayed fluorescein dye appearance and choroidal filling times. Three additional patients with cranial arteritis confirmed by biopsy, but without visual loss, had angiographic characteristics similar to patients with arteritic ischemic neuropathy. We consider fluorescein angiography a valuable diagnostic adjunct in identifying patients with giant cell arteritis.

• Optic nerve sheath fenestration was performed in 26 eyes for treatment of the progressive type of common (nonarteritic) anterior ischemic optic neuropathy. During a mean follow-up period of 21 weeks (range, 6 to 52 weeks), results were as follows: visual acuity increased by two or more lines on the Snellen chart in 7 eyes; visual acuity decreased by two or more lines in four eyes; some regression of visual field defects occurred in six eyes, including two eyes in which acuity also improved. These results, attributed to surgical decompression, do not exceed the spontaneous recovery rates reported in the literature pertaining to nonarteritic anterior ischemic optic neuropathy and fail to substantiate the sanguine visual outcome in recently reported series of patients undergoing optic nerve sheath decompression.

To establish the range of diameters of normal extraocular muscles, we used standardized A-scan echography to measure the superior recti/levator complex, medial, lateral, and inferior recti muscles in 38 subjects with healthy orbital tissues. The relationships of age, gender, height, and weight to axial length of the globe were considered. These factors and extraocular muscle diameters were not consistently associated. Our normative data were compared with previously reported studies of extraocular muscle diameter performed with standardized A-scan and contact B-scan echography and computed tomographic scanning.

Rhinocerebral mucormycosis is a rare but often fatal fungal infection. We present 2 patients with mucormycosis complicated by internal carotid artery thrombosis. Magnetic resonance imaging was superior to computed tomography in localizing the pathological process. Subtotal resection of devitalized tissue and intravenous amphotericin B therapy resulted in a successful outcome for both patients. Prompt recognition of this disorder by using modern diagnostic and therapeutic modalities promises to improve survival rates.

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.

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.

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.

A 13-year-old boy presented with bilateral panuveitis and a superior oblique palsy. Exhaustive laboratory workup was unremarkable, but magnetic resonance imaging (MRI) revealed an enhancing pontine mass. The mass was resected, and histopathology revealed a necrotizing granuloma. Although rare, particularly in the pediatric population, the combination of panuveitis and an intracranial mass likely represents sarcoidosis. Necrosis, although also rare, may similarly be seen in neurosarcoidosis.

• The mechanisms by which optic nerve-sheath fenestration is effective remain speculative. Possibilities include surgical production of a cerebrospinal fluid filtration outlet or scarring in the subarachnoid space around the nerve in the vicinity of the fenestration site, with shifting of the pressure gradient from the nerve head to the retrobulbar portion. Two patients who underwent successful optic nerve-sheath fenestration developed a cystlike structure contiguous to the fenestration site, apparently in direct communication with the optic nerve sheaths. This was shown on magnetic resonance imaging (one patient) and orbital echography (both patients). These previously unreported observations may support the hypothesis that fenestration works by creating a filtration apparatus that controls the intravaginal pressure in the subarachnoid space surrounding the orbital segment of the optic nerve.

The clinical situation of acute optic disc infarction in middle-aged and senescent patients is not uncommon, yet with the exception of those instances due to giant-cell arteritis, pathophysiological mechanisms remain obscure. In hopes of elucidating this syndrome, the clinical profile and natural history of 37 cases of non-arteritic ischaemic optic neuropathy were reviewed. For contrast, 13 cases of arteritic optic neuropathy were surveyed. The salient features of idiopathic optic neuropathy may be summarized as follows: (1) the syndrome occurs primarily in 55-70-year-old patients who, for the most part, are otherwise well; (2) mild hypertension is present in about half of the cases, but does not determine a separate variant of the disorder; (3) there is no significant association with extracranial carotid occlusive disease; (4) over long follow-up periods there appears to be no increased incidence of stroke; (5) the syndrome should be easily recognized on clinical grounds, consisting of sudden or rapidly progressive monocular visual deficit associated with optic disc swelling, with stable visual defects of variable degree; (6) after an interval of months to many years, the second eye is involved in about 40% of cases (old optic atrophy coupled with contralateral fresh disc infarction may be confused with the Foster-Kennedy syndrome); (7) no form of therapy has proved efficacious; (8) pathophysiological mechanisms remain speculative. It is the responsibility of the physician, be he ophthalmologist or neurologist, to distinguish the patient with occult arteritis (history, physical examination, sedimentation rate, arterial biopsy) and institute immediate high-dosage corticosteroid therapy. It is also incumbent upon the clinician to desist from unnecessary and unrewarding diagnostic procedures, in particular cerebral angiography, when confronted with an instance of non-arteritic ischaemic optic neuropathy.

Intracranial hypertension with papilledema occurred in two patients receiving danazol therapy for either cyclic neutropenia or immune hemolytic anemia. Results of clinical, laboratory, and neuroradiologic studies showed no apparent cause for the condition in Case 1 and the papilledema resolved one month after discontinuing danazol. Carotid angiography in Case 2 demonstrated cerebral venous sinus thrombosis; the papilledema showed gradual improvement after cessation of danazol. An additional seven cases of pseudotumor cerebri presumed secondary to danazol therapy have been reported to the Food and Drug Administration. The papilledema resolved in all seven cases soon after discontinuing danazol. A drug-induced complication should be suspected, and alternative therapy sought, in patients who develop intracranial hypertension associated with administration of danazol.

This study documents the results of standardized A-scan examinations performed in 59 cases of optic nerve lesions (15 perioptic meningiomas, four gliomas, 15 acute neuritides, ten optic atrophies, five ischemic optic neuropathies, five acute central retinal vein occlusions, five traumatic optic neuropathies), as compared with 73 normal optic nerves. Analysis included the assessment of reflectivity (spike height) and nerve width (maximal diameter) with the patient fixating in primary gaze and 30 degrees of eccentric gaze; measurements were obtained from the anterior one third and posterior one third of the optic nerves. Increased nerve diameters could be distinguished as noncompressible (a negative 30 degrees test) when due to tumor, or compressible (a positive 30 degrees test) when due to increased subarachnoid fluid, as exemplified by inflammatory optic neuritis or traumatic neuropathy. Moreover, reflectivity patterns regularly differentiated meningioma (medium reflectivity) from optic glioma (low reflectivity). Neither ischemic neuropathy nor vein occlusion altered optic nerve diameter. These results indicate that echographically defined optic nerve diameter, compressibility in eccentric gaze, and reflectivity patterns can be used to effectively distinguish among causes of chronic optic atrophy (tumor vs remote neuropathy) and disc edema (tumor vs neuritis vs ischemic neuropathy).

• In order to test the hypothesis that glaucomatous and nonglaucomatous optic disc cupping can be distinguished ophthalmoscopically, three ophthalmologists experienced in assessing optic dics were asked to view fundus stereophotographs as "unknowns." Of 29 eyes with nonglaucomatous optic atrophy, 13 (44%) were misdiagnosed as showing glaucoma by at least one observer. Of four optic disc features specifically analyzed, neuroretinal rim pallor proved to be 94% specific for nonglaucomatous atrophy, while focal or diffuse obliteration of the neuroretinal rim was 87% specific for glaucoma. These two signs are the most useful in making the distinction. Thinning of the rim was more common in glaucoma than in nonglaucomatous atrophy, but was only 47% specific for glaucoma. Laminar dots were present in both types of excavation.

In the case presented, bilateral optic canal meningiomas produced binocular visual loss. Correct diagnosis was delayed because of inadequate and misinterpreted radiological studies. Careful radiological and surgical examination of the planum sphenoidale later suggested this as the source of both canalicular masses. The pertinent aspects of this case are reviewed in relation to information from similar cases reported previously. In the future, increased clinical suspicion and more accurate neuroradiological studies should improve the detection and afford earlier surgical treatment of meningiomas of the optic canal.

In five patients with amaurosis fugax and visible retinal emboli, an additional finding was observed, consisting of focal arteriolar mural opacification. Typical atheromatous emboli were observed i...

The standardized A-scan (Kretztechnik 7200 MA) supplemented by contact B-scan and Doppler ultrasound is a time-tested, highly reliable, and noninvasive tool for the evaluation of patients presenting with signs or symptoms suggesting an orbital lesion. Diseases of the orbital adipose tissue, extraocular muscles, optic nerves and periorbital sinuses are detectable within minutes by the experienced examiner. More than 60 types of orbital lesions can then be differentiated, localized, measured, and schematized. 1 While the value of orbital CT scanning has been widely accepted, standardized echography is now also being recognized as an important modality in the diagnosis and management of orbital and periorbital disease. The use of standardized echography results in more frequent and more accurate tissue differentiation, specifically: (1) standardized tumor patterns closely correlate with histologic diagnoses, (2) vascular flow detection and compression testing (hard or soft), (3) detection and diagnosis of optic nerve sheath distention due to increased subarachnoid fluid (eg, benign intracranial hypertension), and (4) greater sensitivity in detecting mild extraocular muscle thickening (eg, Graves' disease). Additional advantages are the noninvasive nature of the procedure (no radiation) and portability of the equipment.