Acute multifocal placoid pigment epitheliopathy and central nervous system involvement: nine new cases and a review of the literature.

OBJECTIVE: The authors describe nine new cases of acute multifocal placoid pigment epitheliopathy (AMPPE) with associated central nervous system (CNS) involvement and permanent visual sequelae. The study includes a review of the literature and discussion of evaluation, management, and treatment options. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Nine patients were identified with AMPPE and CNS involvement in addition to 22 patients reviewed in the literature. MAIN OUTCOME MEASURES: A review of nine patients with AMPPE and CNS involvement was performed. Charts were reviewed for age, gender, preceding viral prodromes, visual acuity, ophthalmologic examination findings, CNS findings, and treatment. RESULTS: Thirty-one patients (nine new patients) were diagnosed with AMPPE and various degrees of CNS involvement. Ages ranged from 8 to 54 years, with an average of 27 years. Twenty-one males (68%) and 10 females (32%) were identified. Eleven patients (35%) had antecedent viral illnesses. Visual acuity was variable and ranged from 20/20 to count fingers. The spectrum of CNS findings ranged from headaches to sagittal sinus thrombosis. CONCLUSIONS: Acute multifocal placoid pigment epitheliopathy can be associated with CNS abnormalities and permanent visual deficits. Neuroimaging, lumbar puncture, and cerebral angiography analysis provide useful diagnostic tools when CNS involvement is suspected. Intravenous corticosteroids and collaboration with neurovascular colleagues should be considered in these situations. In cases complicated by CNS arteritis, immunosuppressive agents can be a beneficial adjunct to corticosteroids.

Giant cavernous carotid aneurysms: clinical presentation in fifty-seven cases.

OBJECTIVES: To review the presenting symptoms and ophthalmic findings of 57 patients with cavernous carotid aneurysms of giant size (> or = 2.5-cm diameter). MATERIALS AND METHODS: Hospital charts of 57 patients with giant cavernous carotid aneurysms who presented to University Hospital in London, Ontario, Canada between 1961 and 1993 were reviewed. All patients were proven by cerebral angiography to have unruptured giant cavernous carotid aneurysms. RESULTS: Forty-six patients (81%) were women (mean age, 54 years). The most common presenting symptoms were diplopia (89%), retroorbital pain (61%), headache (19%), diminished or blurred vision (14%), and photophobia (4%). The most common clinical sign was partial or complete ophthalmoplegia (93%). Trigeminal nerve involvement was found in 37% of patients. Other clinical signs included ptosis, decreased visual acuity, proptosis, and visual field defects. CONCLUSIONS: This study characterizes a large group of patients with giant cavernous carotid aneurysms seen over a 30-year period at a single institution. As in previous studies, diplopia and retroorbital pain were the most common symptoms. The high incidence of ophthalmoplegia observed in this study may be explained by a greater compressive and/or ischemic effect of giant aneurysms compared with their smaller counterparts.

BOLD fMRI response of early visual areas to perceived contrast in human amblyopia.

In this study, we used a temporal two-alternative forced choice psychophysical procedure to measure the observer's perception of a 22% physical contrast grating for each eye as a function of spatial frequency in four subjects with unilateral amblyopia and in six subjects with normal vision. Contrast thresholds were also measured using a standard staircase method. Additionally, blood-oxygenation-level-dependent (BOLD) functional magnetic resonance imaging (fMRI) was used to measure the neuronal response within early visual cortical areas to monocular presentations of the same 22% physical contrast gratings as a function of spatial frequency. For all six subjects with normal vision and for three subjects with amblyopia, the psychophysically measured perception of 22% contrast as a function of spatial frequency was the same for both eyes. Threshold contrast, however, was elevated for the amblyopic eye for all subjects, as expected. The magnitude of the fMRI response to 22% physical contrast within "activated" voxels was the same for each eye as a function of spatial frequency, regardless of the presence of amblyopia. However, there were always fewer "activated" fMRI voxels during amblyopic stimulation than during normal eye stimulation. These results are consistent with the hypotheses that contrast thresholds are elevated in amblyopia because fewer neurons are responsive during amblyopic stimulation, and that the average firing rate of the responsive neurons, which reflects the perception of contrast, is unaffected in amblyopia.

Erdheim-Chester disease: two cases of orbital involvement.

Erdheim-Chester disease (ECD) is an increasingly recognised form of fibro-inflammatory process characterised by xanthomatous histiocytes containing large amounts of ingested lipid, plasma cells, macrophages and Touton-type giant cells. Ophthalmic involvement in ECD has been reported in only 22 cases. We describe two patients, one presenting with diabetes insipidus and subsequently developing orbital pseudotumours and retroperitoneal fibrosis, the other presenting with exophthalmos and diplopia. The first patient was treated with cladribine and subsequently developed sudden onset of bilateral blindness while the second required radiation therapy for the retro-orbital process and developed radiation retinopathy. These cases typify the variable presentation and course in patients with ECD.

Bilateral anterior ischemic optic neuropathy as a presentation of Takayasu's disease.

Takayasu's disease is an arteritis with a predilection for the proximal branches of the aortic arch. Chronic retinal hypoxic changes and ischemic ocular inflammation, resulting from cervical vascular occlusion, have been described extensively. Anterior ischemic optic neuropathy has rarely been reported in this condition, and only in conjunction with other ophthalmic findings. Here we present the case of a patient who developed bilateral anterior ischemic optic neuropathy in the absence of other eye findings, and was subsequently found to have an aortic arch syndrome compatible with Takayasu's disease.

Task-dependent changes in the shape and thickness of Listing's plane.

We examined the 2D surface formed by 3D eye positions of normal subjects to determine whether the shape and thickness changed in tasks that differed in saccadic directions: random, horizontal, vertical, radial, clockwise and counter-clockwise. Eye positions during the random task did not lie precisely on Listing's plane but on a surface with a small twist. This twist was present before, during, and after saccades. The degree of twist changed with the task; becoming less twisted for horizontal tasks and more twisted in the vertical tasks. The surface thickness changed with the task becoming thicker for multidirectional tasks. This greater thickness may occur because surfaces obtained in multidirectional tasks are the composite of surfaces with slightly different shapes.

Left atrial myxoma causing ophthalmic artery occlusion.

A 45-year-old Caucasian woman presented with sudden onset right-sided hemiparesis, aphasia and a painful left eye. Examination revealed a bone-white fundus with no perfusion of either the retinal or choroidal circulations. Magnetic resonance imaging showed increased signal density of the left optic nerve sheath, orbital fat and extraocular muscles consistent with infarction of the ophthalmic artery distribution. An echocardiogram disclosed a mobile, multilobulated mass attached to the septal wall of the left atrium. Pathological examination of the resected tumour confirmed the diagnosis of endocardial myxoma. A colour Doppler study performed 1 month after surgery demonstrated absence of flow in the left ophthalmic artery. At 2 months, the left eye had no light perception and an intraocular pressure of 2 mmHg. This clinicopathological report describes the rare presentation of an acute ophthalmic artery obstruction secondary to atrial myxoma.

Orbital aspergillosis. A fatal masquerader.

Ocular manifestations of aspergillosis are emerging as increasingly more atypical, with a wide spectrum of presentations. The classic teaching is that this fungus presents as an endogenous endophthalmitis in immunocompromised hosts. In most other ocular conditions, the index of suspicion for this infection has typically been low, especially in apparently immunocompetent individuals. However, reports continue to appear that confirm involvement of Aspergillus species in a wide variety of primary ocular and orbital conditions characterized by rapid, uncontrollable progression and, not uncommonly, death. This report highlights a case of autopsy-proven orbital aspergillosis that masqueraded as temporal arteritis in terms of initial clinical presentation and response to standard therapy. Any ocular condition that manifests even subtle atypical features, including temporal arteritis, should be regarded as suspect, and an aggressive approach to secure a biopsy of any involved sites should follow.

Three-dimensional ultrasound imaging of the eye.

We assessed whether an inexpensive, three-dimensional (3D) ultrasound (US) imaging system could produce clinically useful 3D images, without causing patient discomfort. Five patients were examined. The 3D US system consisted of a transducer holder containing a mechanical motor, and a microcomputer. During data acquisition the transducer was mechanically rotated for 22 seconds, while 200 two-dimensional (2D) US images were collected and formed into a 3D image by the computer. The 3D image was viewed on the computer monitor. The 3D images correlated with the clinical and radiological findings. The new perspectives were helpful in diagnosing eye abnormalities and no patient discomfort occurred. The device was easy to use. It is concluded that, as good-quality 3D and 2D US images were produced quickly, with no patient discomfort, and the device is inexpensive, uncomplicated, and easily attached to existing ultrasound machines, it will probably be useful in clinical practice.

Separate neural pathways for the visual analysis of object shape in perception and prehension.

BACKGROUND: Earlier work with neurological patients has shown that the visual perception of object size and orientation depends on visual pathways in the cerebral cortex that are separate from those mediating the use of these same object properties in the control of goal-directed grasping. We present evidence suggesting that the same dissociation between perception and action is evident in the visual processing of object shape. In other words, discrimination between objects on the basis of their shape appears to be mediated by visual mechanisms that are functionally and neurally distinct from those controlling the pre-shaping of the hand during grasping movements directed at those same objects. RESULTS: We studied two patients with lesions in different parts of the cerebral visual pathways. One patient (RV), who had sustained bilateral lesions of the occipitoparietal cortex, was unable to use visual information to place her fingers correctly on the circumference of irregularly shaped objects when asked to pick them up, even though she had no difficulty in visually discriminating one such object from another. Conversely, a second patient (DF), who had bilateral damage in the ventrolateral occipital region, had no difficulty in placing her fingers on appropriate opposition points during grasping, even though she was unable to discriminate visually amongst such objects. CONCLUSIONS: This double dissociation lends strong support to the idea that the visual mechanisms mediating the perception of objects are functionally and neurally distinct from those mediating the control of skilled actions directed at those objects. It also supports the recent proposal of Goodale and Milner that visual perception depends on a ventral stream of projections from the primary visual cortex to the inferotemporal cortex, whereas the visual control of skilled actions depends on a dorsal stream from the primary visual cortex to the posterior parietal cortex.

Findings of magnetic resonance and colour-flow Doppler imaging of orbital embryonal rhabdomyosarcoma.

The authors describe a case of embryonal rhabdomyosarcoma of the orbit evaluated with magnetic resonance imaging (MRI) and colour-flow Doppler imaging. MRI demonstrated a markedly enhancing tumour with no discrete vessels. Colour-flow Doppler ultrasonography showed evidence of perfusion; however, discrete vascular channels were difficult to visualize, and no evidence of large vessels within the tumour was found at surgery. The differential diagnosis of this condition is discussed, as well as the potential role of colour-flow Doppler imaging in the assessment of orbital tumours, a role in which this method has not yet been widely applied.

Colour-flow Doppler imaging of a carotid-cavernous fistula.

The authors describe a case of carotid-cavernous fistula evaluated with both conventional carotid angiography and colour-flow Doppler ultrasonography. The findings in the colour-flow Doppler images correlated well with those from angiography; in addition, Doppler imaging supplied extra information about the direction and the velocity of flow. After embolization, colour-flow Doppler imaging demonstrated a marked decrease in the flow. The authors conclude that colour-flow Doppler imaging may be useful for noninvasively detecting and evaluating a carotid-cavernous fistula, as well as for monitoring the patient's progress after therapy.

Terson's syndrome: a reversible cause of blindness following subarachnoid hemorrhage.

Terson's syndrome refers to the occurrence of vitreous hemorrhage with subarachnoid hemorrhage (SAH), usually due to a ruptured cerebral aneurysm. Although it is a well-described entity in the ophthalmological literature, it has been only rarely commented upon in the neurosurgical discussion of SAH. Fundus findings are reported in a prospective study of 22 consecutive patients with a computerized tomography- or lumbar puncture-proven diagnosis of SAH. Six of these patients had intraocular hemorrhage on initial examination. In four patients vitreous hemorrhage was evident on presentation (six of eight eyes). In the subsequent 12 days, vitreous hemorrhage developed in the additional two patients (three of four eyes) due to breakthrough bleeding from the original subhyaloid hemorrhages. The initial amount of intraocular hemorrhage did not correlate with the severity of SAH. Two of the six patients with intraocular hemorrhage died, whereas five of the 16 remaining SAH patients without intraocular hemorrhage died. Of the four survivors with intraocular hemorrhage, three showed gradual but significant improvement in their visual acuity by 6 months. The fourth underwent vitrectomy at 8 months after presentation and had a good visual result. With modern and aggressive medical and microsurgical management, Terson's syndrome should be recognized as an important reversible cause of blindness in patients surviving SAH.

Sarcoidosis of the eyelid skin.

A 64-year-old white woman presented with nodular lesions on the arms and face, including both medial canthi and the left upper eyelid. Histopathological examination of the biopsied eyelid lesion and other facial lesions disclosed the presence of noncaseating granulomas strongly suggestive of sarcoidosis. No other evidence of ocular sarcoidosis was present. The patient had clinical, radiologic and laboratory findings consistent with a diagnosis of systemic sarcoidosis. Therapy with chloroquine phosphate led to regression of the cutaneous lesions.

Bilateral blindness in temporal arteritis with skip areas.

A 73-year-old woman received systemic corticosteroids for suspected temporal arteritis after blindness developed in the right eye during the previous day. Because a right temporal artery biopsy specimen was reported as normal, a diagnosis of arteriosclerotic ischemic optic neuropathy was made and the corticosteroid therapy was discontinued. Two weeks later the patient rapidly lost vision in her left eye. A left temporal artery biopsy specimen showed granulomatous arteritis adjacent to normal artery (skip area). Deeper sections of the original right temporal artery biopsy specimen also demonstrated a small focus of granulomatous arteritis adjacent to normal artery. In patients with suspected temporal arteritis, numerous step sections of a long segment of temporal artery should be examined; if the results are normal, the contralateral temporal artery should be studied. A nonspecific inflammatory reaction within or adjacent to the artery should alert the pathologist to the possible presence of a nearby focus of granulomatous arteritis.

Clinical application of multiplanar computerized tomography of the orbit.

Multiplanar image reconstruction involves the reconstruction of sagittal, coronal or oblique views form a series of transverse computerized tomography images without exposing the patient to more radiation. The technique, especially when used in conjunction with high-resolution scanning, is invaluable in the diagnosis of lesions within the orbit. This paper deals with the clinical application of this new technique.