“Idiopathic” intracranial hypertension caused by venous sinus thrombosis associated with contraceptive usage

BackgroundCerebral 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.CaseAn 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.ConclusionBecause 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.     

The effect of low-dose doxycycline therapy in chronic meibomian gland dysfunction

PURPOSE: The aim was to investigate the effect of low dose doxycycline (20 mg) therapy in patients with chronic meibomian gland dysfunction that were refractory to conventional therapy. METHODS: The randomized prospective study enrolled 150 patients (300 eyes) who have chronic meibomian gland dysfunction and who didn't respond to lid hygiene and topical therapy for more than 2 months. All topical therapy was stopped for at least 2 weeks prior to beginning the study. After conducting the tear break up time test (TBUT) and Schirmer test, the authors randomly divided the patients into three groups a high dose group (doxycycline, 200 mg, twice a day), a low dose group (doxycycline, 20 mg, twice a day) and a control group (placebo). After one month, the author repeated the TBUT and Schirmer tests, and analyzed the degree of symptomatic improvement. RESULTS: Compared to the control group, both the high and low dose group showed statistically significant differences after treatment in TBUT, Schirmer test, the number of symptoms reported and the degree of improvement of subjective symptoms. However, there was no statistically significant difference between the high and low dose group after treatment in TBUT (9.42 +/- 2.87 sec, 9.54 +/- 1.58 sec, p=0.726), Schirmer test (19.98 +/- 4.05 mm, 19.65 +/- 5.02 mm, p=0.624), the number of symptoms reported (1.45 +/- 0.62, 1.53 +/- 0.52, p=0.304), as well as the degree of improvement of subjective symptoms (p=0.288). The high dose group (18 patients, 39.13%) reported side effects more frequently than did the low dose group (8 patients, 17.39%) (P=0.002). CONCLUSIONS: Low dose doxycycline (20 mg twice a day) therapy was effective in patients with chronic meibomian gland dysfunction that were refractory to conventional therapy.     

Optic nerve involvement associated with the multiple evanescent white-dot syndrome

Five patients presenting with signs and symptoms of primary optic nerve disease were, after careful examination, diagnosed as having the multiple evanescent white-dot syndrome. Optic nerve findings in these patients included disc edema, central visual loss, enlarged blind spots, and other optic nerve field defects. The subtlety of the white spots in some cases made the diagnosis difficult. Multiple evanescent white-dot syndrome should be considered in the differential diagnosis of young, healthy patients who present with unilateral or bilateral optic nerve dysfunction.     

Correlation of nerve head and blind spot elliptical features

Ellipses were fitted to 129 nerve heads and corresponding blind spots in an attempt to determine how faithfully the elliptical nature of the nerve head is reflected in the blind spot. The blind spots are found to be 2 to 3 times more elliptical (based on the ratio of the long axis to the short axis) than the nerve heads. The correlation between the ratios mentioned above is found to be negligible (Pearson r = 0.07 for Goldmann 1-2 blind spots and 0.18 for Goldmann 1-4 blind spots).     

Multifocal choroiditis and choroidal neovascularization associated with the multiple evanescent white dot and acute idiopathic blind spot enlargement syndrome.

BACKGROUND: Several recent articles have described syndromes in which there is enlargement of the blind spot associated with retinal lesions. These have included the multiple evanescent white dot syndrome, acute macular neuroretinopathy, acute idiopathic blind spot enlargement syndrome, and multifocal choroiditis or pseudo presumed ocular histoplasmosis syndrome (pseudo POHS). METHODS: The authors reviewed the records of seven patients in whom signs and symptoms of acute enlargement of the blind spot and pseudo POHS developed. RESULTS: All seven patients had photopsia accompanying enlargement of the blind spot during their illness. Four had transient white spots as seen in the multiple evanescent white dot syndrome. All presented with or developed chorioretinal scars or neovascularization similar to that seen in multifocal choroiditis or pseudo POHS. In four of the seven patients, POHS-like scars developed only in the eye that was symptomatic with blind spot enlargement and photopsia. Five of the 7 had visual acuity of 20/25 or better at the last follow-up. CONCLUSION: It would appear that there is an overlap in the clinical findings of all of these syndromes and that there may be a common link in their etiology.     

Optic nerve sheath meningiomas. Clinical manifestations

A retrospective clinical study of optic nerve sheath meningiomas based on 22 patients showed that symptoms most commonly develop in women between the ages of 35 and 60 years. The most common presenting symptoms were decreased vision and transient visual obscurations. In the earliest stages, many patients presented with normal to mildly impaired acuity (despite subjectively decreased vision), optic disc edema and enlargement of the blind spot. Optic disc edema was frequently associated with refractile bodies indicative of chronic swelling. Optic disc edema preceded the development of optic atrophy. Another group of patients presented with a history of longstanding vision loss, visual acuity of 20/200 or worse and optic atrophy. Optociliary shunt vessels were late findings only seen in five patients. The most consistent visual field abnormality was peripheral constriction. Cecocentral scotomas were uncommon. Intracranial involvement was present in five patients. There were two patients with bilateral optic nerve sheath meningiomas without CT evidence of intracranial involvement. Computerized tomography was found to be indispensable in the diagnosis of optic nerve sheath meningiomas and the detection of intracranial involvement.     

Bilateral slowly progressive big blind spot syndrome

A 33-year-old woman was found with bilateral enlargement of the blind spot without optic disc edema. Her bilateral peripapillary retinas appeared slightly decolored and elevated with obscure margins and fluorescein angiography indicating only slight hyperfluorescence in the late venous phase. Four years after her initial visit, both of her blind spots became enlarged further and dark adaptation was remarkably difficult. Peripapillary retinal lesions appearing as retinal pigmentary degeneration were evident with very definitive margins. The lesions showed strong hyperfluorescence in the arteriovenous phase owing to window defects. From these findings, it appears that bilateral big blind spots without optic disc edema may possibly result from peripapillary retinal degeneration, which may become evident at a much later date.     

Fluctuation of the differential light threshold at the border of absolute scotomas. Comparison between glaucomatous visual field defects and blind spots

The outcome of repeated measurements of the differential light sensitivity fluctuates slightly. This fluctuation can be markedly increased in glaucoma patients but has also been described at the border of the blind spot of healthy subjects. The question arises, therefore, whether in glaucoma this increase may be due to the fact that many test locations may touch the border of (detected or undetected) scotomas. This study compares the behavior of the threshold at the border of glaucomatous defects and blind spots. The threshold was measured with program F8 of the Octopus 201 (Interzeag, Inc, Schlieren, Switzerland) automated static perimeter. The results revealed that the borders of glaucomatous defects are less steep than those of blind spots, the fluctuation is largest just at the border of the scotomas in both groups, and that the fluctuation is significantly larger at the edge of glaucomatous defects than at the edge of blind spots of normal subjects.     

The eye and sleep

BACKGROUND: Sleep-associated disorders of the eye are increasingly recognized. Disordered sleep has also been found in some blind patients. METHODS: Review of the current state of knowledge of sleep-related eye disorders and blindness-related sleep disorders. RESULTS: Incomplete eyelid closure during sleep (lagophthalmos) may be physiological but in some patients can cause significant symptoms. Nocturnal lagophthalmos may be a limiting factor in the surgical repair of some eyelid and orbital disorders. Normal eyelid closure has also been linked to the development of a number of ocular surface disorders. Sleep disorders are common, and obstructive sleep apnoea (OSA) the commonest. OSA is associated with a number of serious systemic diseases and also several eye disorders including floppy eyelid syndrome, optic neuropathy, glaucoma, anterior ischaemic optic neuropathy and papilloedema secondary to raised intracranial pressure. Treatment of OSA may help floppy eyelid syndrome, halt progression of associated glaucoma, and reduce intracranial pressure in patients with associated papilloedema. The diagnosis of OSA can only be made with formal sleep studies, but asking a small number of appropriate questions will help screen those patients who should be referred for sleep studies. Some blind patients have disordered sleep patterns due to disruption of the retinal input into the hypothalamic-pineal melatonin secreting apparatus. This can be alleviated by oral administration of melatonin. CONCLUSIONS: Ophthalmologists should be aware of sleep-associated eye disorders and refer appropriate patients for formal sleep studies. Sleep abnormalities in blind patients should be enquired about and patients offered assessment and treatment by a sleep physician.     

Sleep apnea and intracranial hypertension in men

PURPOSE: To investigate sleep apnea as an associated finding in idiopathic intracranial hypertension (IIH) in men. DESIGN: Multicenter, retrospective, noncomparative interventional case series. METHODS: Retrospective review of all men with the diagnosis of IIH seen within the last 5 years at three tertiary care academic ophthalmologic institutions. Cases with sleep apnea (SA) and IIH were identified and reviewed. RESULTS: Thirty-two cases of IIH in men were reviewed. Six cases with SA met the modified Dandy criteria for the diagnosis of IIH. Of these six patients, one received acetazolamide alone, four received acetazolamide and continuous positive airway pressure (CPAP), and one was treated with CPAP alone. All patients had preserved central acuity (20/20 or better in both eyes), enlarged blind spots, and optic disc edema in both eyes. Five patients had normal visual fields after treatment, and one patient had residual visual field loss. Three patients had normal optic nerve examinations, with resolution of the optic disc edema at last follow-up. After resolution of the optic disc edema, these three patients were maintained on CPAP but discontinued acetazolamide. Two patients had persistent but improved papilledema and are under continued treatment with acetazolamide and CPAP. One patient had optic disc pallor in both eyes and is stable. CONCLUSIONS: SA was a common finding in men meeting the modified Dandy criteria for IIH in adults. Treatment of sleep apnea with nocturnal oxygenation may improve the signs and symptoms of IIH in affected men.     

Ophthalmologic involvement of intracranial tumors--report of a case

Bilateral papillary edema is considered to be the expression of a tumoral process that reduces the intracranial space resulting in elevation of the intracranial cerebrospinal fluid pressure. The authors present the case of a 21-year-old man consulting for mild visual loss of the left eye. The ophthalmologic examination showed bilateral papilledema and visual field defects in the left eye (enlargement of the blind spot), with no clinic or imagistic signs of increased intracranial pressure. Intracranial pathologic processes that bear like a tumor, reducing the intracranial space are not always associated with papilledema, but papilledema is always associated with intracranial tumor. Increased intracranial pressure is frequently associated with intracranial tumoral processes, but in this case was not showed increasing of intracranial pressure.     

Case of Primary Leptomeningeal Lymphoma Presenting with Papilloedema and Characteristics of Pseudotumor Syndrome

The authors describe an immunocompetent, 50-year-old man who complained of a daily transient blurring of his vision with bilateral papilloedema. His visual acuity was 20/20 OU, and the blind spot was enlarged bilaterally. There was intracranial hypertension, but imaging for systemic and brain tumours were negative. These findings suggested a diagnosis of the pseudotumor syndrome. However, MRI showed leptomeningeal enhancement, and acetazolamide successfully resolved his visual symptoms and papilloedema. Cytology and flow cytometry of the CSF led to the final diagnosis of primary leptomeningeal lymphoma (PLML). Clinicians need to be aware that a case of PLML may be misdiagnosed as peudotumor cerebri.     

Leber's congenital amaurosis associated with hyperthreoninemia

Two siblings had Leber's congenital amaurosis. The girl (Patient 1) showed blindness shortly after birth, absent pupillary light reflex, and multiple round, white spots in both fundi. Her serum threonine level was increased (2.0 to 5.3 mg/dl; normal, 0.78 to 1.82 mg/dl). She died of massive pericardial effusion four months after birth. Her brother (Patient 2) was nearly blind shortly after birth. He had a poor pupillary light reflex and a nearly extinguished electroretinographic response. He also had hyperthreoninemia, hyperthreoninuria, hepatomegaly, and mental and physical retardation. We suspect a close relationship between hyperthreoninemia and Leber's congenital amaurosis in these siblings.     

Hypotensive synergism of propranolol and adrenalin

The effect of combined treatment with an oral beta-blocking agent (propranolol tablets 40 mg twice a day) and a local alpha- and beta-agonist (epinephrine eyedrops twice a day) was studied in 10 healthy volunteers. Propranolol was given in a double-blind randomized manner, while adrenalin was given unmasked. Propranolol and adrenalin proved to have an additive reducing effect on the intraocular pressure. The effect of propranolol alone was stronger than that of adrenalin.     

Bilateral optic disk edema caused by sarcoidosis mimicking pseudotumor cerebri

PURPOSE: To present a case of retrobulbar optic nerve and chiasm sarcoidosis that mimicked pseudotumor cerebri. METHODS: A 34-year-old, thin, black woman presented with transient visual obscurations, normal visual acuity, bilateral optic disk edema, and enlarged blind spots. Clinical, medical, and radiologic evaluations were consistent with pseudotumor cerebri. The patient improved while taking acetazolamide, but 6 months later her symptoms worsened. Neuroimaging disclosed enhancement of the optic nerve and chiasm. RESULTS: Despite administration of intravenous corticosteroids, the patient's vision worsened. Bilateral optic nerve sheath fenestrations were performed, and pathology disclosed sarcoidosis. CONCLUSION: Sarcoidosis of the optic nerves and chiasm may mimic pseudotumor cerebri.     

Therapeutic follow-up using automatic perimetry in chronic cerebroretinal ischemia in elderly patients. Prospective double-blind study with graduated dose ginkgo biloba treatment (EGb 761)]

The chronic cerebral retinal insufficiency syndrome in elderly patients is an organ specific expression of a generalized vascular cerebral deficiency. The progress of the disease is characterized by complex symptoms, variation in course, spontaneous remissions and, until recently inadequate diagnostic measurement methods. The new method of automated perimetry with the octopus 2000 P offers a patient-friendly procedure for indirect non-invasive diagnosis of circulatory state in limited cerebral retinal perfusion. In the present study measurements were made with this method on 24 patients (4 men and 20 women with an age of 74.9 +/- 6.9 years). The effect of the extract of Ginkgo biloba (EGb 761) on the reversibility of visual field disturbances was tested using a randomized and double blind study-design in two phases and with two dose levels. The main parameter investigated in this study was the change in the luminous density difference threshold after therapy with EGb 761. In group B (EGb 761 dose 160 mg/day) a significant increase in retinal sensitivity was seen within 4 weeks (p less than 0.05). In the lower dose (80 mg EGb 761/day) group (A), this change in retinal sensitivity was first seen after increasing the dose to 160 mg/day (p less than 0.01). The relative sensitivity of damaged retinal areas was more strongly influenced than "healthy" areas. The assessment by both doctors and patients of the general condition of the patients showed a significant improvement after the course of therapy. The results presented here show that damage to the visual field by chronic lack of bloodflow are significantly reversible.(ABSTRACT TRUNCATED AT 250 WORDS)     

Inhibition of PKC beta by oral administration of ruboxistaurin is well tolerated and ameliorates diabetes-induced retinal hemodynamic abnormalities in patients

PURPOSE: To assess ocular and systemic safety and pharmacodynamic effects of the oral PKC beta selective inhibitor ruboxistaurin (RBX; LY333531) mesylate in patients with diabetes. METHODS: This was a double-masked, placebo-controlled, parallel, randomized, single-center clinical study evaluating the effect of oral administration of RBX (8 mg twice a day, 16 mg per day, or 16 mg twice a day) or placebo for 28 days in patients with no or very mild diabetic retinopathy on mean retinal circulation time (RCT), retinal blood flow (RBF), treatment-emergent adverse events, and other safety parameters. RESULTS: Twenty-nine persons aged 18 to 65 years with type 1 or 2 diabetes were evaluated. The only treatment-emergent adverse event with a statistically significant difference among treatment groups was abdominal pain, which was more common in placebo-treated subjects (P = 0.049). Statistically significant effects of RBX were observed on several hematologic and laboratory parameters, but values were within the normal reference range and none of the changes was deemed clinically meaningful. In patients receiving 16 mg RBX twice daily, the diabetes-induced increase in RCT was ameliorated, with a baseline-to-endpoint difference of -0.84 seconds (P = 0.046) relative to placebo. Increasing RBX dose was linearly associated with greater effect on RCT (P = 0.03). Similar results were observed with RBF. CONCLUSIONS: RBX was well tolerated at doses up to 16 mg twice daily for 28 days in patients with diabetes. It ameliorated diabetes-induced RCT abnormalities. No serious safety problems were identified in this patient population. Compared with prior published data, these findings represent the first direct human evidence of both bioavailability of RBX to retinal vessels and amelioration of diabetes-induced retinal hemodynamic abnormalities by an oral PKC beta inhibitor.     

Nedocromil sodium and astemizole, alone or combined, for treatment of seasonal allergic conjunctivitis (SAC)

SAC is caused by allergen interaction with IgE antibody on conjunctival mast cells, leading to local release of vasoactive inflammatory mediators such as histamine. Nedocromil sodium both stabilizes mast cells and has antiinflammatory activity against other cells involved in allergic inflammation. Astemizole is a second generation orally-active H(1)-receptor antagonist with reduced CNS effects such as drowsiness. This multicentre, double blind, double dummy trial compared efficacy and safety of qid 2% nedocromil sodium eye drops with once daily 10 mg oral astemizole, placebo, and combined active treatments for a four-week period. SAC patients (n=207, aged 6-70 years) recorded their symptoms each day on diary cards. Signs and symptoms were also evaluated by clinicians after one, two and four weeks and overall opinions were recorded at the end of treatment. Nedocromil sodium eye drops and astemizole, alone or combined, significantly reduced ocular symptoms compared to placebo (for diary card total symptom score and patients' opinion). Clinicians' opinion showed significantly decreased symptoms with nedocromil sodium, alone or combined, but not with astemizole alone. All treatments were well tolerated, with drowsiness the most frequent side effect observed in patients treated with astemizole. These results demonstrate the effectiveness of nedocromil sodium eye drops in the treatment of SAC.     

Imagen multimodal en el síndrome de aumento idiopático de mancha ciega

Two cases are reported of acute idiopathic blind spot enlargement syndrome (AIBSE) that presented with peripapillary white spots in the fundus. AIBSE belongs to the acute zonal occult outer retinopathy (AZOOR) complex. Conditions of this AZOOR complex may overlap. Typically, in AIBSE, a peripapillary hyperautofluorescence is seen in the autofluorescence. En-face OCT angiography at the ellipsoid level showed hyper-reflective spots around the optic disk in both cases. One case showed a reversible enlargement of the blind spot in visual field. AZOOR complex is an inflammatory disorder that affects the outer retina, and can now be confirmed with structural optical coherence tomography. In the cases presented, there was a reversible severe loss of the outer retina.     

Bilateral subretinal haemorrhage with Terson's syndrome

Purpose To present a case of Terson's syndrome with bilateral subretinal haemorrhage emanated from peripapilla, resulting in Mariotte blind spot enlargement.Methods Preoperative CT scan and postoperative eye examinations, including funduscopy, fluorescein angiography, optical coherence tomography (OCT), and Goldmann perimetry.Results A 41-year-old Japanese man had suffered a subarachnoid haemorrhage. Three months later, he recovered from disturbance of consciousness and was referred for decreased vision in both eyes. A CT scan, obtained on the day after the event, had revealed bilateral vitreous hemorrhage. The patient underwent a standard pars plana vitrectomy to clear vitreous haemorrhage. Surprisingly we found bilateral subretinal haemorrhage around peripapilla during surgery. Although subretinal haemorrhage was almost absorbed at six months after the operation, Mariotte blind spot enlargement corresponding to the area of subretinal haemorrhage was detected in both eyes.Conclusions In some population of the patients with Terson's syndrome, it was demonstrated that the disturbance of peripapillary structure, presumably due to intracranial hypertension, causes subretinal haemorrhage, resulting in irreversible visual field defect.