Jeffrey G. Odel

The multifocal electroretinogram.

The multifocal electroretinogram (mfERG) technique allows local ERG responses to be recorded simultaneously from many regions of the retina. As in the case of the full-field ERG, the ganglion cells contribute relatively little to the response, which originates largely from the outer retina. The mfERG is particularly valuable in cases in which the fundus appears normal, and it is difficult to distinguish between diseases of the outer retina and diseases of the ganglion cells and/or optic nerve. The mfERG can also help to differentiate among outer retinal diseases, to follow the progression of retinal diseases, and, with the addition of the mfVEP, to differentiate between organic and nonorganic causes of visual loss. However, because the difficulties encountered in recording and analyzing mfERG responses are greater than those involved in full-field ERG testing, mfERG testing is best left to centers with an electrophysiologist familiar with the mfERG test. Although this technique is relatively new and standards are still being developed, centers capable of recording reliable mfERG responses can be found in hundreds of locations around the world.

The multifocal visual evoked potential

With the multifocal technique, visual evoked potentials (VEPs) can be recorded simultaneously from many regions of the visual field. For the multifocal VEP (mfVEP), the patient views a display that typically contains 60 sectors, each with a checkerboard pattern. The display covers about the same retinal area as the 24-2 Humphrey visual field (HVF). However, due to the scaling of the sectors of the mfVEP display, the fields are sampled differently by the mfVEP and HVF. To assess local defects in the visual field, the mfVEP responses must be compared with normal controls. These comparisons require relatively sophisticated analyses and software. Whereas the mfVEP can be recorded relatively easily with the same equipment used to record multifocal electroretinograms (mfERGs), the software needed to perform the analysis is not yet widely available. The mfVEP is valuable for ruling out non-organic visual loss, diagnosing and following patients with optic neuritis/multiple sclerosis, evaluating patients with unreliable or questionable HVFs, and following disease progression. When combined with the mfERG, diseases of the outer retina (before the retinal ganglion cells) can be distinguished from diseases of the ganglion cells and/or optic nerve. The difficulties encountered in recording and analyzing mfVEP responses are greater than those involved in full-field VEP testing. Thus, in its current form, the mfVEP is best recorded and interpreted by ophthalmologists and electrophysiologists experienced with the technique. However, this technique is developing rapidly; advances in commercial hardware and software are expected in the near future.

Cytomegalovirus Retinitis in a Young Homosexual Male with Acquired Immunodeficiency

A case is reported of histopathologically documented CMV retinitis. It is part of a recently appreciated syndrome in young homosexual men, in which cellular immune deficiency has been documented and in which CMV infection may play a role. This case demonstrates that CMV retinitis is not excluded by negative CMV serology or cultures.

A comparison of multifocal and conventional visual evoked potential techniques in patients with optic neuritis/multiple sclerosis

Purpose To compare conventional visual evoked potential (cVEP) and multifocal visual evoked potential (mfVEP) methods in patients with optic neuritis/multiple sclerosis (ON/MS). Methods mfVEPs and cVEPs were obtained from eyes of the 19 patients with multiple sclerosis confirmed on MRI scans, and from eyes of 40 normal controls. For the mfVEP, the display was a pattern-reversal dartboard array, 48° in diameter, which contained 60 sectors. Monocular cVEPs were obtained using a checkerboard stimulus with check sizes of 15′ and 60′. For the cVEP, the latency of P100 for both check sizes were measured, while for the mfVEP, the mean latency, percent of locations with abnormal latency, and clusters of contiguous abnormal locations were obtained. Results For a specificity of 95%, the mfVEP(interocular cluster criterion) showed the highest sensitivity (89.5%) of the 5 monocular or interocular tests. Similarly, when a combined monocular/interocular criterion was employed, the mfVEP(cluster criterion) had the highest sensitivity (94.7%)/specificity (90%), missing only one patient. The combined monocular/interocular cVEP(60′) test had a sensitivity (84.2%)/specificity (90%), missing 3 patients, 2 more than did the monocular/interocular mfVEP(cluster) test. Conclusion As the cVEP is more readily available and currently a shorter test, it should be used to screen patients for ON/MS with mfVEP testing added when the cVEP test is negative and the damage is local.

Microangiopathy of brain, retina, and inner ear.

Microangiopathy of brain, retina, and inner ear is a rare syndrome manifesting as arteriolar occlusions of the brain, retina, and inner ear, with resultant encephalopathy, visual, and hearing loss. Despite exhaustive laboratory examinations in these patients, no evidence of a systemic disease can be found. We treated and followed an adolescent with this disorder who initially presented with a branch retinal artery occlusion. A unique finding in this case was retinal vessel wall hyperfluorescence noted five days prior to retinal infarction. The patient developed recurrent branch artery occlusions, sensorineural hearing loss, and central nervous system infarctions despite anticoagulation and immunosuppressive treatment.

An Analysis of Normal Variations in Retinal Nerve Fiber Layer Thickness Profiles Measured With Optical Coherence Tomography

PurposeTo assess the normal variations in retinal nerve fiber layer (RNFL) thickness measured with optical coherence tomography (OCT). Subjects and MethodsBoth eyes of 48 individuals (age 56.4±9.5 y) with normal vision and refractive errors between ±6.0 D were tested with the fast RNFL scan protocol of the OCT3 (Zeiss Meditech). Their 256-point RNFL profiles were exported for analysis. The location and peak amplitude of the maxima of the RNFL profiles were measured. Intersubject and interocular variations were assessed with a coefficient of determination, R2. An R2 of 1.0 indicated that the average profile from all 48 individuals (or of the 2 eyes) accounted for 100% of the variation of an individual eyes profile. ResultsThe R2 for the interocular comparison was good, with averages of 0.91±0.07 (right eye) and 0.92±0.05 (left eye). The R2 for the comparison of the individuals profile to the mean group profile was only 0.61±0.29 (right eye) and 0.65±0.24 (left eye), with 27% of the R2 values below 0.5. Even after normalizing each individuals profile by its mean, R2 was only 0.75±0.16 (0.75±0.16) for the right (left) eye. The location of the peaks for the right (left) eye ranged over 91 degrees (88 degrees) for the superior peak and over 64 degrees (66 degrees) for the inferior peak. The range of peak amplitudes for the right (left) eye spanned a factor of 1.7 (1.8) and 2.0 (1.7) for the superior and inferior peaks, respectively. ConclusionsThere was a wide variation in the amplitude and shape of the individual RNFL profiles. However, the RNFL profiles of the 2 eyes of an individual were extremely similar. Adding an interocular comparison with OCT RNFL tests should help identify some false positives.

A comparison of multifocal ERG and frequency domain OCT changes in patients with abnormalities of the retina

To compare the ability of the multifocal electroretinogram (mfERG) and frequency domain optical coherence tomography (fdOCT) to detect retinal abnormalities. A total of 198 eyes (100 patients) were referred by neuro-ophthalmologists to rule out a retinal etiology of visual impairment. All patients were evaluated with static automated perimetry (SAP) (Humphrey Visual Field Analyzer; Zeiss Meditec), mfERG (Veris, EDI) and fdOCT (3D-OCT 1000, Topcon). The mfERG was performed with 103 scaled hexagons and procedures conforming to ISCEV standards (Hood DC et al. (2008) Doc Ophthalmol 116(1):1–11). The fdOCT imaging included horizontal and vertical line scans through the fovea. Local mfERG and fdOCT abnormalities were compared to local regions of visual field sensitivity loss measured with SAP and categorized as normal/inconclusive or abnormal. 146 eyes were categorized as normal retina on both fdOCT and mfERG. The retina of 52 eyes (36 patients) was categorized as abnormal based upon mfERG and/or fdOCT. Of this group, 25 eyes (20 patients) were abnormal on both tests. However, 20 eyes (13 patients) were abnormal on mfERG, while the fdOCT was normal/inconclusive; and 7 eyes (7 patients) had normal or inconclusive mfERG, but abnormal fdOCT. Considerable disagreement exists between these two methods for detection of retinal abnormalities. The mfERG tends to miss small local abnormalities that are detectable on the fdOCT. On the other hand, the fdOCT can appear normal in the face of clearly abnormal mfERG and SAP results. While improved imaging and analysis may show fdOCT abnormalities in some cases, in others early damage may not appear on structural tests.

Magnetic resonance imaging of optic perineuritis

Optic perineuritis, an uncommon variant of orbital pseudotumor, may be clinically indistinguishable from retrobulbar optic neuritis. Because treatment and prognosis for these two entities are different, early diagnosis is important. We report a case of a 47 year-old woman with clinical findings suggestive of retrobulbar optic neuritis, but whose magnetic resonance images suggested optic perineuritis. A dramatic clinical response to oral corticosteroids was observed. Optic perineuritis should be considered in cases of presumed retrobulbar optic neuritis. MRI may differentiate these two entities in the acute stage, and should be considered before treatment is decided.

Magnetic Resonance Imaging of Optic Tract Involvement in Multiple Sclerosis

We studied two cases of optic tract involvement in multiple sclerosis with documentation by magnetic resonance imaging. In one, incongruous homonymous hemianopsia was accompanied by a decrease in visual acuity in one eye from chiasmal involvement. In the other, the involvement was restricted to the optic tract and the homonymous hemianoptic visual field defect was nearly congruous.

Varicella-zoster Virus Retrobulbar Optic Neuritis in a Patient With Human Immunodeficiency Virus

PURPOSE To determine the cause of bilateral retrobulbar optic neuritis followed by progressive outer retinal necrosis in a patient with human immunodeficiency virus (HIV). METHODS Extensive ophthalmologic, neurologic, infectious disease, rheumatologic, and radiologic examinations were performed. RESULTS Cerebrospinal fluid samples taken after the onset of bilateral retrobulbar optic neuritis and before the development of clinical progressive outer retinal necrosis disclosed varicella-zoster virus from polymerase chain reaction and viral culture. CONCLUSION Ophthalmologists and neurologists should consider varicella-zoster virus optic neuritis as a potential precursor of progressive outer retinal necrosis and as a cause of retrobulbar optic neuritis in patients infected with HIV.