A clinical case report of left homonymous hemianopia

prevalent in 20–57% of stroke patients, visual field defects have been shown to impact quality of life. Studies ([8][1]) have shown increased risk of falling, ambulatory difficulties, impaired reading ability, and feelings of panic in crowded or unfamiliar places in patients with visual field

To investigate whether persistent visual field defects among patients exposed once to the antiepileptic drug vigabatrin (VGB) were associated with peripapillary retinal nerve fibre layer thickness (RNFLT) attenuation. Nine individuals with partial epilepsy and VGB-attributed visual field loss (group 1; 18 eyes) and seven age- and gender-matched individuals with epilepsy and no previous VGB exposure (group 2; 14 eyes) were included in the study. Full-field 120 point screening perimetry out to 60 degrees from central fixation using the Humphrey Field Analyzer was performed. RNFLT was quantified by optical coherence tomography (OCT) using Fast RNFLT protocol, Stratus OCT (3.0) after pupillary dilation. The results from the right eye are presented in this article. Among the patients with VGB-attributed visual field loss, five patients had only peripheral field defect (group 1a) and the remaining four had advanced field defects both in the periphery and within 30° from central fixation (group 1b). None of the patients in the control group had manifest visual field loss. The mean RNFLT among the patients with VGB-attributed visual field loss was significantly attenuated compared to the controls [mean total RNFLT: group 1: 75.6 ± 12.7 μm, group 2: 103.5 ± 9.7 μm, mean difference 27.9 μm, (CI 15.9-39.9; p < 0.001)]. RNFLT values classified as borderline according to normative database (Stratus OCT) occurred more frequently among individuals with VGB-attributed visual field loss than in controls (frequency in group 1: 6/9; group 2: 0/7, p = 0.011). The nasal, superior and inferior quadrants of RNFLT in individuals with VGB-attributed visual field loss were significantly attenuated, while no difference was detected in temporal quadrants compared to controls. Both individuals with peripheral and those with advanced visual field losses in the VGB group had attenuated mean total RNFLT compared to controls (p = 0.006, p = 0.002, respectively). Occurrence of borderline classification of total RNFLT ≤5th percentile was more frequent among individuals with advanced visual field loss than among controls (p = 0.048). Persistent visual field loss attributed to VGB is associated with reduced peripapillary RNFLT and was detected both among patients with advanced and among patients with only peripheral visual field defects. Measurements of RNFLT with OCT might be considered as a diagnostic supplement in the follow-up of patients exposed to vigabatrin.

There were 1,405 scientific papers and poster presentations at the 57th Annual Meeting of the American Academy of Neurology held in Miami Beach, Florida from April 9 to 16, 2005. Abstracts are published in Neurology 2005;64:Suppl 1. We have summarized the material of most interest to neuro-ophthalmologists. OPTIC NEURITIS AND MULTIPLE SCLEROSIS Brain MRI is an established biomarker of disease in multiple sclerosis (MS), and data on the impact of MS and acute optic neuritis on retinal nerve fiber layer (RNFL) thickness, as measured by optical coherence tomography (OCT), are beginning to emerge. A recent investigation examined the relation of visual function to RNFL thickness and brain MRI parameters in an MS cohort. Scores for low-contrast letter acuity (Sloan charts) and contrast sensitivity (Pelli-Robson), the leading candidates for visual components for inclusion in the MS functional composite, were significant predictors of average overall RNFL thickness (P < 0.001) and of lesion burden on T2-weighted MRIs (P = 0.001 for Sloan charts; P = 0.03 for Pelli-Robson) accounting for age. The greatest reductions in RNFL thickness were noted among eyes of MS patients with a history of acute optic neuritis. However, MS eyes without an optic neuritis history also demonstrated abnormalities. These results not only confirm a role for axonal loss in the anterior visual pathways of MS patients but demonstrate correlations of low-contrast letter acuity and contrast sensitivity with structural biomarkers, supporting their validity as clinical trial outcome measures (Wu GF, Philadelphia, PA, P01.025). Visual dysfunction is a common manifestation of MS and may therefore have important effects on health-related quality of life (HRQOL). The 25-Item National Eye Institute Visual Function Questionnaire (VFQ-25) has been used in a variety of ocular conditions and ophthalmic clinical trials. A study was performed to examine whether a 10-Item Neuro-Ophthalmic Supplement could increase the capacity of the VFQ-25 to capture self-reported visual dysfunction in patients with neuro-ophthalmic disorders, particularly those associated with diplopia or optic neuropathy. Patients included those with optic neuritis, MS, idiopathic intracranial hypertension, ischemic optic neuropathy, ocular myasthenia gravis, thyroid eye disease, and ocular motor palsies. Scores for the VFQ-25 + 10-Item Neuro-Ophthalmic Supplement (combined scores) had a greater capacity to distinguish neuro-ophthalmically impaired patients from disease-free controls (receiver operating characteristic[ROC] curve area, 0.77) than did the VFQ-25 alone (ROC curve area, 0.73; P = 0.001). VFQ-25 + Supplement scores correlated significantly with binocular and with worse eye visual acuities (rs = 0.38 - 0.41; P < 0.0001), and Neuro-Ophthalmic Supplement items demonstrated appropriate levels of reliability. The 10-Item Supplement is a valid measure of self-reported visual dysfunction in neuro-ophthalmically impaired patients. This new scale captures symptoms and limitations related to diplopia and will be used to complement the VFQ-25 in clinical trials and other research (Balcer LJ, Philadelphia, PA, P01.031). Sixty MS patients were studied to establish the relationship of infections to MS exacerbations. MS attacks occurring between two weeks before and five weeks after an infection were considered temporally related to infection. A total of 127 infections were recorded in 53 patients, and 124 exacerbations occurred in 49 of the 53 patients. There was an increased risk of relapses temporally related to infection (odds ratio, 3.2; P < 0.0001). Exacerbations that were temporally related to infection were more frequently associated with severe and sustained deficits than those that were not temporally related to infection. The number of Gd-enhancing lesions was higher during exacerbations temporally related to infection than in those that were not temporally related to infection. This study demonstrates a significant association between systemic infection, risk of MS clinical relapse, and degree of MRI activity (Correale JD, Buenos Aires, Argentina, P03117). NEUROMYELITIS OPTICA Neuromyelitis optica (NMO) is a severe inflammatory central nervous system (CNS) disorder with a predilection for the optic nerves and spinal cord. Because of the potential overlap with MS with regard to clinical and neuroimaging findings, the diagnostic criteria for NMO have been recently re-examined and revised. These new criteria have modified those published in 1999 (Wingerchuk et al., Neurology 1999;53:1107-14) to update MRI findings and to incorporate a novel serum autoantibody marker, NMO-immunoglobulin (Ig)G. Investigations for these studies calculated sensitivities and specificities for each component of the 1999 NMO diagnostic criteria using a new 118-patient cohort. Combinations of individual criteria, including NMO-IgG, were tested to optimize the potential for correct diagnosis. Among patients with NMO (n = 84) versus MS (n = 34), criteria that had the greatest capacity to distinguish NMO from MS were the presence of NMO-IgG (sensitivity, 75.3%; specificity, 93.3%) and spinal cord T2-lesion length extending beyond three vertebral segments (sensitivity, 97.0%; specificity, 79.0%). Neurological symptoms indicating disease outside the optic nerves and spinal cord were seen in 17 NMO patients (20.2%). Based on these findings, the authors suggested criteria for diagnosis of NMO. Absolute criteria: presence of optic neuritis and acute myelitis (but disease in other areas of CNS allowed); supportive criteria: 1) spinal cord MRI with lesion(s) extending over three or more vertebral segments or 2) NMO-IgG seropositivity (Wingerchuk DM, Scottsdale, AZ, P01.033). As emphasized by studies refining the diagnostic criteria for NMO (P01.033 above), an NMO diagnosis is no longer precluded by the presence of clinical or imaging abnormalities outside the optic nerves or spinal cord as long as the patient meets the new NMO criteria described the previous paragraph. Importantly, the former (1999) criteria required a normal brain MRI. The brain MRI findings for 60 patients who met revised criteria for NMO (P01.033 above) were described in a recent study. In 26 (43%) of 60 patients, brain MRI was normal at a median of 1.6 years (range, 0-29 years) after the onset of symptoms. Seventeen patients (28%) had non-specific abnormalities that were generally located in the deep white matter and few in number (only four [6.5%] patients had more than four high T2 signal foci). Typical MS-like lesions were found in only six patients (10%), five of whom were seropositive for NMO-IgG. The remaining 11 patients (19%) had MRI findings considered atypical for MS; brainstem lesions were seen mostly among children. Because brain MRI abnormalities were noted among ~50% of patients who otherwise had classic findings for NMO, diagnostic criteria have been revised to allow for brain involvement (Pittock SJ, Scottsdale, AZ, P01.036). Because brain MRI techniques have continued to evolve, atypical findings have been noted more frequently in the brains of NMO patients. Given the contiguity of the hypothalamic regions to the anterior visual pathways, hypothalamic involvement in patients with NMO is perhaps not unexpected. Two patients with otherwise classic features of NMO were described as having hypersomnolence, hyponatremia, and hypothermia. MRI scans revealed new areas of hyperintense signal in the hypothalamus in the absence of other brain parenchymal lesions. Additional similar cases were identified from the literature, and this series again supports revision of the NMO diagnostic criteria to include involvement beyond the optic nerves and spinal cord (Poppe AY, Montreal, QC, Canada, P01.037). GENETIC OPTIC NEUROPATHIES Patients with Friedreich ataxia (FA) develop optic neuropathies, most frequently after development of gait and limb dysfunction. Sixteen molecularly defined Italian FA patients with quantified GAA were assessed with visual acuity, fundus photos, 30-degree Humphrey visual field, Ishihara color plates, OCT, visual evoked potentials (VEP), and electroretinography (ERG). All patients showed optic nerve damage as assessed by OCT and visual fields. Fiber loss was typically peripheral; decreased visual acuity was present only when central fibers were lost. VEP was altered in eight (56%) of the patients. In six, they were significantly delayed with reduced amplitudes; in three, the responses were absent. ERGs were normal in all patients. The degree of fiber loss, expressed as thickness of the nerve fiber layer as measured by OCT, was significantly correlated with the GAA expansion in the smaller allele of the frataxin gene (P = 0.002; r = −0.69 for the peripheral sectors). OCT was the most sensitive test in showing optic neuropathy (Fortuna F, Milan, Italy, S01.003). Investigators designed a battery of clinical measures that may be used for outcomes assessment in FA therapeutic trials. FA candidate performance measures, including the timed 25-foot walk, 9-hole peg test, low-contrast letter acuity, and PATA test (a test of speech) were evaluated in a cohort of 131 FA patients at six academic medical centers. Scores for individual components (including vision) and for the combination of performance measures correlated significantly with neurologic disability, activities of daily living, and disease duration (rs = 0.40 - 0.89; P < 0.0001). Visual function scores were also found to be lower in the FA cohort compared with disease-free controls, further supporting the inclusion of low-contrast letter acuity in an FA outcomes assessment battery. Given the high proportion of FA patients who progress to a non-ambulatory status in adulthood, inclusion of measures that capture vision, arm function, and speech will ensure sensitivity and applicability throughout the course of disease (Lynch DR, Philadelphia, PA, P01.104). To further development of an animal model for Leber Hereditary Optic Neuropathy (LHON), mice received intraocular injections of an adeno-associated virus-ribozyme directed against the murine mitochondrial ND4 subunit of complex I. Their ganglion cells showed hyperchromatic cytoplasm and nuclear condensation suggestive of apoptosis. Optic nerves showed secondary demyelination with axonal loss. Transmission electron microscopy revealed swelling and vacuolization of mitochondria with dissolution of cristae. Allotopic expression of a mutant R340H ND4 results in histopathology similar to that of patients with LHON harboring the G11778A mutation in mitochondrial DNA (mtDNA). This is a step further in development of an animal model of LHON (Qi X, Gainesville, FL, S01.001). In addition to the three most common mtDNA mutations that occur in patients with LHON, novel pathogenetic mutations continue to be discovered. Overlap with other mitochondrial disease phenotypes, such as mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS), has also been described for mutations involving the ND5 subunit of complex I. An Italian family with a maternally inherited LHON phenotype demonstrated an ND5 mtDNA mutation (G13042A/ND5) that was pathogenetic for LHON yet had been previously described in a case of MELAS. This report underscores the potentially variable clinical expression that may occur in the setting of mtDNA mutations and reinforces the need for consideration of a variety of mutations in patients with optic neuropathies consistent with LHON (Valentino ML, Bologna, Italy, P01.029). An attempt to use neuroprotection to prevent second eye involvement with LHON was reported. In an open-label prospective pilot study, nine patients with one-eye vision loss for less than six months and normal visual function in the other eye were treated with brimonidine purite 0.15% (Alphagan P) four times daily in the unaffected eye for up to two years. Visual acuity was the primary outcome measure. Secondary measures included changes on automated perimetry and quantification of the relative afferent pupillary defect. There were eight men and one woman enrolled, aged 13-54 years (mean, 32 years); eight had the 11,778 mtDNA mutations, and one the 3,460 mutations. Despite normal visual acuity and visual field mean deviations at baseline, seven patients had minimal changes in the central visual field of the study eye. All patients had deterioration of their second eye vision. Topical brimonidine purite in this dosage was unsuccessful in preventing second-eye involvement (Newman NJ, Atlanta, GA, S01.002). Horizontal gaze palsy with progressive scoliosis (HGPPS) is a rare syndrome with congenital absence of conjugate horizontal eye movements from birth and a severe progressive scoliosis. It is caused by mutations in the human axon guidance gene ROBO3 that is important in midline crossing during neurodevelopment. Two genetically defined HGPPS patients were studied. One patient underwent structural MRI, and one underwent Brain Stem Auditory Evoked Responses (BAERs). A T1-weighted three-dimensional volume MRI provided anatomical information and was co-registered to the diffusion tensor imaging (DTI) data. Diffusion tensors were calculated from diffusion-weighted images using standard methods. The structural MRI showed reductions in the size of the pons, medulla, and cerebellar peduncles and flattening of the rostral medulla. Color-coded DTI showed absence of the decussation of the superior cerebellar peduncles and absence of the major crossing fibers representing the trapezoid body at the pontine level, confirmed by the BAER results in the second patient. Supratentorial crossing fibers appeared normal. Mutations in ROBO3 resulted in DTI-defined abnormal midline crossing of several white matter tracts in the hindbrain. This is the first report of the lack of decussation of both the superior cerebellar peduncles and the auditory/vestibular pathways (Sicotte NL, Los Angeles, CA, S01.005). Two children with severe microphthalmia and one with anophthalmia developed progressive severe white matter degeneration. The patients were unrelated, but parents originated from the same Pakistani village. Workup for known causes of white matter degeneration was negative. The authors propose that this is a novel example of a leukoencephalopathy in which the abnormal cerebral white matter disappears over time (vanishing white matter) (Kanavin OJ, Amsterdam, Netherlands, S65.001). IDIOPATHIC INTRACRANIAL HYPERTENSION Obesity and weight gain are known risk factors for idiopathic intracranial hypertension (IIH). However, the profiles of body mass index (BMI; = weight [kg]/height2 [meters]) and weight change that are most associated with IIH have not been defined. A recent case-control study designed to pilot a new risk factor questionnaire and to examine patterns of weight gain and obesity has provided new insight. This study included 34 newly diagnosed IIH patients and 41 age-matched controls. As expected, higher levels of BMI were associated with progressively greater risk of IIH in this cohort (BMI, 25-30; odds ratio in favor of IIH = 6.5; BMI 30-35: odds ratio = 19.5; BMI >35; odds ratio = 26.0; P < 0.01, χ2 for trend). Higher percentages of weight gain during the year before diagnosis (or corresponding reference time for controls) were also associated with IIH (5%-10% weight gain: odds ratio = 3.5; 10%-15%: odds ratio = 10.2; >15%: odds ratio = 15.2; P < 0.01). Most interesting was the finding that among patients with moderate weight gain (5%-15%), those with BMIs <30 (ideal or slightly overweight) were at greatest risk for IIH. Results of this study support clinical observations that associate IIH with increasing degrees of weight gain and indicate that weight gain-associated IIH may develop even among patients of ideal or slightly overweight BMI profile (Daniels AB, Philadelphia, PA, P01.032). VESTIBULO-CEREBELLAR PATHWAY DYSFUNCTION Whereas skew deviation is typically attributed to brain stem lesions that disrupt otolithic vestibular projections to the third and fourth nerve nuclei, it may also occur in the setting of isolated focal cerebellar lesions. Focal cerebellar lesions were identified in five patients with vertical ocular misalignment who had no evidence of brainstem involvement (or neuromuscular disease). After detailed testing of the vestibulo-ocular reflex, patterns were found that implicate imbalance of the utriculo-ocular reflex as the mechanism for skew deviation (Wong MF, Toronto, ON, Canada, P01.013). Benign paroxysmal positional vertigo (BPPV) is a common cause of vertigo encountered by neurologists and neuro-ophthalmologists. The assessment and treatment of BPPV has been systematically investigated by several research groups. BPPV is unique among other causes of vertigo in that it results from misplaced otoconia in the semicircular canals. Treatment, therefore, centers on repositioning of the otoconial debris within the canals. Among 524 consecutive patients who presented to an emergency department in Italy with symptoms of dizziness, 243 were found to have BPPV that localized by Dix-Hallpike maneuver to the posterior or anterior canals (n = 202), the horizontal canal (n = 35), or the posterior and horizontal canals combined (n = 6). Canalith repositioning treatment (modified in cases of horizontal canal BPPV) produced resolution of vertigo symptoms in all but six patients (Del Colle R, Legnago, Italy, P01.019). The efficacy of a new treatment maneuver for posterior canal BPPV was also investigated. This new treatment, the Gans Repositioning Maneuver (GRM), is a hybrid of the Semont Liberatory Maneuver (SLM) and the Canalith Repositioning Maneuver (CRM). Although these established maneuvers are well tolerated and easily performed on most patients, those with hip problems that may be aggravated by brisk lateral motion during the SLM, and individuals with a contraindication to neck hyperextension (as required in the CRM), need a modified version of these treatments. The GRM consists of the following: 1) the patient is placed in a side-lying position (used in SLM); 2) the patient rolls onto the shoulder opposite the involved ear (with head turned away from involved ear throughout rotation); and 3) the patient is seated upright. Patients with posterior canal BPPV treated with GRM in a recent series (n = 207) experienced resolution in 80.5% of cases after a single treatment; 95.5% resolved with two treatments. Symptom were only within a and results were in with previous data for the and The GRM was to be in posterior canal BPPV and may to repositioning maneuvers for of patients with hip and neck problems that FL, One of the most important in an emergency is peripheral vestibular dysfunction from more brain stem causes or of vertigo and An abnormal head on vestibular testing is to clinical evidence in favor of a peripheral vestibular To the the patient a the the head to each The of a during this maneuver is a of decreased from the ear to the head is decreased the eye with the head during the and a is required to the Among study were diagnosed with peripheral and were diagnosed with cerebellar in posterior cerebellar superior cerebellar and anterior cerebellar patients had a lateral syndrome one had a and patients had in multiple The was normal in of of and in all patients who presented with isolated vestibular In the three patients with and abnormal the abnormal was attributed to involvement of vestibular or peripheral vestibular nerve The authors that a normal to brain in patients with non-specific acute onset symptoms of vertigo and ataxia The National and cohort data on a of the The of self-reported among men and aged years who in from 1999 to was with the relationship to risk and to treatment for in this within the year was by of the The symptoms less than two weeks in two weeks to three months in and more than three months in of the with The to was for for and for years and with who did not report dizziness, with were more to be versus P < and to have a higher mean versus P < was in of the with It of (n = ear (n = other (n = vestibular (n = or other (n = after treatment was among of the treated patients. The demonstrates that is a common that over aged years and in the will have symptoms that over three A is required to and among Patients with ocular myasthenia diplopia and that may be was to consecutive patients with at to a of Patients had been on and were on of to treatment, or disease was during and all were after of symptoms. One patient with underwent an average of of patients on including and were but infections and were not and was generally well of the patients who continued on of developed or limb within years of and occurred in of patients, and they were treated with trials are to further examine the potential for to and to or prevent onset of symptoms A case of primary CNS was after months of treatment with for This is as the first such patient in a is as a clinical of of the In a series of patients with sensitivity of was found to be consistent with Whereas was the was in one of four patients diagnosed with that other for may be associated with nerve was performed on the nerve from the in normal controls and patients with who had a clinical history and consistent with the diagnosis of and A was found after than versus of demonstrated on without significant on is a visual manifestation of or head of is to occur within six months of but the course of such has not been examined in A series of cases in and in in in in in and in demonstrated or resolution in The of did not with of or of resolution was seen more with Importantly, of visual field was noted to occur after six months in eight patients, evidence for that may be when the and of such of visual field function or increasing capacity to visual field testing to be investigated X, Atlanta, GA, A study at the of neuroimaging with of was Among patients, had were and were There were no significant between the two with to and of the visual field defect. of and of were (P = 0.001). was more common in and than in other (P = 0.001). after was more than after or demyelination (P = 0.001). lesions resulted in the most lesions produced in was also seen in of of of of optic and of lateral lesions. the cases with and the cases with and the most information from this study is that at of secondary to lesions of the optic and optic will be Atlanta, GA, patients treated with for primary CNS developed The clinical of included vision visual loss and One patient was at diagnosis of The median from of to diagnosis of was in these patients included retinal and patient had previous or other disease to an to changes in visual of a to information visual A study of in a in disease patients, and was of the trials a that in and of the and no had to whether a change and reduced the to and to changes in of increased The authors attributed the increased in and to over the of items or decreased The in this study were also more than to report a change when had with impaired caused by and in which may have for information in a variety of including To test the that visual and with motor function in disease patients were tested on and higher visual patients with disease were impaired in and higher visual as well as including and compared with controls. The higher visual and dysfunction correlated with motor in with and may to of the dysfunction in patients A study compared visual in patients

Abstract&#x0D; Introduction : Stroke are generally associated with peripheral visual field loss. Three out of four stroke patients report some form of disability, one of which is visual impairment. Approximately 16% of these patients have a homonymous visual field defect poststroke. These vision problems can lead to difficulty carrying out a lot of daily tasks and activities, such as driving. This report tries to define the diagnostic flow to determine the exact type and lesion.&#x0D; Case Illustration : Male, 61 years old, came to Universitas Sumatera Utara Hospital with visual field loss in both eyes. Two months ago, he was diagnosed with ischemic stroke and treated with Aspirin 80 mg, with no limbs disability right now. He also had hypertension and treated with Amlodipine 10 mg. VOD: 6/60 with BCVA S-2.00 6/7,5, VOS: 6/60 with BCVA S-2.00 6/7.5. With confrontation test and perimetry, defect was found in medial visual field OD and lateral visual field OS. Hess screen and Amsler Grid was normal. Head CT-Scan was performed with multiple infarcts, mainly in right occipital lobe. The patient was diagnosed with Incomplete Hemianopia, Left Congruous Homonymous Hemianopia with Macular Sparing, and treated with Citicholine 1000 mg once daily.&#x0D; Discussion&#x0D; Conclusion : In stroke patients with homonymous visual field loss, visual field testing with detailed evaluation of visual field defect can determine the lesion location, and neuro-radiological imaging was necessary to assess visual pathway. It’s important for educate the patients, diagnostic and prevent the stroke recurrent.

A 29-year-old woman underwent brain MRI for increasing frequency of migraine headaches. The MRI report recommended further evaluation for septo-optic dysplasia. The patient reported no visual deficits. Her examination showed normal visual acuities, color vision, pupillary function, and a normal optic disc in the right eye with mild temporal disc pallor in the left eye. Automated perimetry revealed an incongruous left homonymous hemianopia (Fig. 1). Optical coherence tomography (OCT) showed thinning of the retinal nerve fiber layer to 60 µm in the right eye and 54 µm in the left eye. There was ganglion cell layer thinning in a hemianopic pattern with temporal thinning in the right eye and nasal thinning in the left eye. This corresponded with the left homonymous hemianopia (Fig. 2).FIG. 1.: Automated visual field testing reveals an incongruous left homonymous hemianopia.FIG. 2.: Optical coherence tomography of the macular ganglion cell layer shows a hemianopic pattern of thinning with temporal thinning in the right eye and nasal thinning in the left eye. OD, right eye; OS, left eye.Endocrine studies included a normal pituitary hormone panel with the exception of a TSH of 0.36 µIU/mL (normal:0.49–4.70 µIU/mL). Free T4 level was 1.1 ng/dL (normal: 0.9–2.2 ng/dL). Clinically, the patient was euthyroid. A dedicated 3-T MRI of the orbits demonstrated absent septum pellucidum, thinning of both optic nerves, and asymmetric thinning of the right optic tract (Figs. 3, 4). The pituitary gland measured 4 mm in craniocaudal height, which was relatively small for the patient's age and sex (1). There also was a closed-lip schizencephaly of the right lateral ventricle atrium with associated overlying polymicrogyria (Fig. 4). Further evaluation with high-resolution reduced field of view diffusion tensor imaging more precisely delineated asymmetry of the right optic radiation superior to the temporal horn (Fig. 4B) and more posteriorly along the inferolateral wall of the lateral ventricle atrium (Fig. 4C). Diffusion tractography was not used because it is highly variable and lacks reproducibility across individuals particularly for the visual pathways. Clinical and radiographic findings were consistent with the diagnosis of congenital homonymous hemianopia involving the right optic tract and radiation.FIG. 3.: MRI and diffusion tensor imaging. Coregistered axial T1 image (left) and direction-encoded diffusion tensor image (right; red = left-right; blue = craniocaudal; green = anterior–posterior fiber orientation) demonstrate asymmetry in the optic tracts along the anterolateral margins of the rostral cerebral peduncles. Compared with the left optic tract (arrowhead), the right optic tract (arrows) is thin and less bright on the T1 scan. There is absence of green anterior–posterior tubular structure in the expected location of the right optic tract on the direction-encoded diffusion tensor images (arrows) compared with the left optic tract (arrowheads).FIG. 4.: MRI and diffusion tensor imaging. A. Coronal T2 imaging through the optic chiasm demonstrates marked thinning of the right lateral aspect of the optic chiasm (arrow). There also is absence of the septum pellucidum. Corresponding diffusion tensor imaging confirms right chiasmal asymmetry (arrow) as compared to the more normal appearance of the left lateral aspect of the optic chiasm (green fibers, arrowhead). B. Coronal T2 MRI shows a closed-lip schizencephaly with associated polymicrogyria (arrows). Corresponding diffusion tensor imaging demonstrates an asymmetry in the size of the proximal right optic radiation (arrow) compared with the normal contralateral left optic radiations (green fibers, arrowhead). Note the fimbria/fornix is a much smaller structure with a similar orientation located inferior to this position that is not detectable with diffusion tensor imaging in vivo due to partial volume effects. C. Coronal T2 scan reveals closed-lip schizencephaly extending to the region of the optic radiations (arrows). On diffusion tensor imaging, there is loss of the right optic radiation (arrows) compared with the normal left optic radiation (green fibers, arrowheads).Initially, we suspected a right optic tract lesion based on the findings of a left incongruous homonymous hemianopia, OCT abnormalities, and optic nerve pallor. However, in addition to optic tract thinning, MRI demonstrated schizencephaly, polymicrogyria, and thinning of the right optic radiation. Optic tract lesions are commonly, although not always, associated with an afferent pupillary defect (APD). Lesions of the optic tract without an APD have been reported with incongruous homonymous hemianopia, where there is less dense involvement of the temporal hemifield in the eye contralateral to the tract lesion (2), as seen in our patient. The visual field defect also potentially could localize to the right superior optic radiation; this may explain may explain the denser visual field deficit in the inferior left quadrant. Such retrogeniculate lesions also typically do not cause an APD. The few reported patients with congenital homonymous hemianopia typically are asymptomatic at the time of diagnosis (3). Early injury to the retrochiasmatic visual pathway may occur in utero, perinatally, or early in life (4). OCT, particularly of the ganglion cell layer, is useful to assess retrograde transynaptic degeneration in congenital homonymous hemianopia due to retrogeniculate hemianopia, as well as in primary optic tract hypoplasia (5). Previously cited causes of congenital homonymous hemianopia, as well as classic septo-optic dysplasia with acuity and color vision loss, have included porencephalic cysts and brain malformations (6–8). The periventricular location of the optic radiations also makes the visual pathway susceptible to periventricular leukomalacia as a result of in utero ischemia (4). Our patient's clinical examination suggested an optic tract lesion; however, the region of polymicogyria and schizencephaly in the expected location of the superior right optic radiations could potentially represent the underlying etiology for this patient's congenital homonymous hemianopia (Fig. 4C) and may have secondarily led to optic tract retrograde degeneration and/or maldevelopment. The other possibilities are that the optic tract hypoplasia induced the retrogeniculate injury and cortical malformations, or that true primary visual pathway hypoplasia with coexistent but noncausative cortical malformations were induced by the same in utero or perinatal insult (7,8). Our case highlights the utility of diffusion tensor imaging in evaluation of the visual pathways. Color-encoded fractional anisotropy maps better demonstrate asymmetry and anatomy of the retrochiasmatic optic pathway compared with conventional MRI (9). The clear left–right asymmetry in the direction-encoded fractional anisotropy maps detected in our patient not only allowed for localization of the anatomical abnormality but also the extent of visual pathway involvement. STATEMENT OF AUTHORSHIP Category 1: a. conception and design: J. Rispoli, M. Seay, M. Sum, J. Rucker, and T. M. Shepherd; b. acquisition of data: J. Rispoli, M. Seay, M. Sum, J. Rucker, and T. M. Shepherd; c. analysis and interpretation of data: J. Rispoli, M. Seay, M. Sum, J. Rucker, and T. M. Shepherd. Category 2: a. drafting the manuscript: J. Rispoli, M. Seay, M. Sum, J. Rucker, and T. M. Shepherd; b. revising it for intellectual content: J. Rispoli, M. Seay, M. Sum, J. Rucker, and T. M. Shepherd. Category 3: a. final approval of the completed manuscript: J. Rispoli, M. Seay, M. Sum, J. Rucker, and T. M. Shepherd.

A 29-year-old woman underwent brain MRI for increasing frequency of migraine headaches. The MRI report recommended further evaluation for septo-optic dysplasia. The patient reported no visual deficits. Her examination showed normal visual acuities, color vision, pupillary function, and a normal optic disc in the right eye with mild temporal disc pallor in the left eye. Automated perimetry revealed an incongruous left homonymous hemianopia (Fig. 1). Optical coherence tomography (OCT) showed thinning of the retinal nerve fiber layer to 60 µm in the right eye and 54 µm in the left eye. There was ganglion cell layer thinning in a hemianopic pattern with temporal thinning in the right eye and nasal thinning in the left eye. This corresponded with the left homonymous hemianopia (Fig. 2).FIG. 1.: Automated visual field testing reveals an incongruous left homonymous hemianopia.FIG. 2.: Optical coherence tomography of the macular ganglion cell layer shows a hemianopic pattern of thinning with temporal thinning in the right eye and nasal thinning in the left eye. OD, right eye; OS, left eye.Endocrine studies included a normal pituitary hormone panel with the exception of a TSH of 0.36 µIU/mL (normal:0.49–4.70 µIU/mL). Free T4 level was 1.1 ng/dL (normal: 0.9–2.2 ng/dL). Clinically, the patient was euthyroid. A dedicated 3-T MRI of the orbits demonstrated absent septum pellucidum, thinning of both optic nerves, and asymmetric thinning of the right optic tract (Figs. 3, 4). The pituitary gland measured 4 mm in craniocaudal height, which was relatively small for the patient's age and sex (1). There also was a closed-lip schizencephaly of the right lateral ventricle atrium with associated overlying polymicrogyria (Fig. 4). Further evaluation with high-resolution reduced field of view diffusion tensor imaging more precisely delineated asymmetry of the right optic radiation superior to the temporal horn (Fig. 4B) and more posteriorly along the inferolateral wall of the lateral ventricle atrium (Fig. 4C). Diffusion tractography was not used because it is highly variable and lacks reproducibility across individuals particularly for the visual pathways. Clinical and radiographic findings were consistent with the diagnosis of congenital homonymous hemianopia involving the right optic tract and radiation.FIG. 3.: MRI and diffusion tensor imaging. Coregistered axial T1 image (left) and direction-encoded diffusion tensor image (right; red = left-right; blue = craniocaudal; green = anterior–posterior fiber orientation) demonstrate asymmetry in the optic tracts along the anterolateral margins of the rostral cerebral peduncles. Compared with the left optic tract (arrowhead), the right optic tract (arrows) is thin and less bright on the T1 scan. There is absence of green anterior–posterior tubular structure in the expected location of the right optic tract on the direction-encoded diffusion tensor images (arrows) compared with the left optic tract (arrowheads).FIG. 4.: MRI and diffusion tensor imaging. A. Coronal T2 imaging through the optic chiasm demonstrates marked thinning of the right lateral aspect of the optic chiasm (arrow). There also is absence of the septum pellucidum. Corresponding diffusion tensor imaging confirms right chiasmal asymmetry (arrow) as compared to the more normal appearance of the left lateral aspect of the optic chiasm (green fibers, arrowhead). B. Coronal T2 MRI shows a closed-lip schizencephaly with associated polymicrogyria (arrows). Corresponding diffusion tensor imaging demonstrates an asymmetry in the size of the proximal right optic radiation (arrow) compared with the normal contralateral left optic radiations (green fibers, arrowhead). Note the fimbria/fornix is a much smaller structure with a similar orientation located inferior to this position that is not detectable with diffusion tensor imaging in vivo due to partial volume effects. C. Coronal T2 scan reveals closed-lip schizencephaly extending to the region of the optic radiations (arrows). On diffusion tensor imaging, there is loss of the right optic radiation (arrows) compared with the normal left optic radiation (green fibers, arrowheads).Initially, we suspected a right optic tract lesion based on the findings of a left incongruous homonymous hemianopia, OCT abnormalities, and optic nerve pallor. However, in addition to optic tract thinning, MRI demonstrated schizencephaly, polymicrogyria, and thinning of the right optic radiation. Optic tract lesions are commonly, although not always, associated with an afferent pupillary defect (APD). Lesions of the optic tract without an APD have been reported with incongruous homonymous hemianopia, where there is less dense involvement of the temporal hemifield in the eye contralateral to the tract lesion (2), as seen in our patient. The visual field defect also potentially could localize to the right superior optic radiation; this may explain may explain the denser visual field deficit in the inferior left quadrant. Such retrogeniculate lesions also typically do not cause an APD. The few reported patients with congenital homonymous hemianopia typically are asymptomatic at the time of diagnosis (3). Early injury to the retrochiasmatic visual pathway may occur in utero, perinatally, or early in life (4). OCT, particularly of the ganglion cell layer, is useful to assess retrograde transynaptic degeneration in congenital homonymous hemianopia due to retrogeniculate hemianopia, as well as in primary optic tract hypoplasia (5). Previously cited causes of congenital homonymous hemianopia, as well as classic septo-optic dysplasia with acuity and color vision loss, have included porencephalic cysts and brain malformations (6–8). The periventricular location of the optic radiations also makes the visual pathway susceptible to periventricular leukomalacia as a result of in utero ischemia (4). Our patient's clinical examination suggested an optic tract lesion; however, the region of polymicogyria and schizencephaly in the expected location of the superior right optic radiations could potentially represent the underlying etiology for this patient's congenital homonymous hemianopia (Fig. 4C) and may have secondarily led to optic tract retrograde degeneration and/or maldevelopment. The other possibilities are that the optic tract hypoplasia induced the retrogeniculate injury and cortical malformations, or that true primary visual pathway hypoplasia with coexistent but noncausative cortical malformations were induced by the same in utero or perinatal insult (7,8). Our case highlights the utility of diffusion tensor imaging in evaluation of the visual pathways. Color-encoded fractional anisotropy maps better demonstrate asymmetry and anatomy of the retrochiasmatic optic pathway compared with conventional MRI (9). The clear left–right asymmetry in the direction-encoded fractional anisotropy maps detected in our patient not only allowed for localization of the anatomical abnormality but also the extent of visual pathway involvement. STATEMENT OF AUTHORSHIP Category 1: a. conception and design: J. Rispoli, M. Seay, M. Sum, J. Rucker, and T. M. Shepherd; b. acquisition of data: J. Rispoli, M. Seay, M. Sum, J. Rucker, and T. M. Shepherd; c. analysis and interpretation of data: J. Rispoli, M. Seay, M. Sum, J. Rucker, and T. M. Shepherd. Category 2: a. drafting the manuscript: J. Rispoli, M. Seay, M. Sum, J. Rucker, and T. M. Shepherd; b. revising it for intellectual content: J. Rispoli, M. Seay, M. Sum, J. Rucker, and T. M. Shepherd. Category 3: a. final approval of the completed manuscript: J. Rispoli, M. Seay, M. Sum, J. Rucker, and T. M. Shepherd.

Visual field loss and visuospatial neglect are frequent consequences of cerebral stroke. They often have a strong impact on independence in many daily activities. Rehabilitation aiming to decrease these disabilities is therefore important, and several techniques have been proposed to foster awareness, compensation, or restitution of the impaired visual field. We here describe a rehabilitation intervention using adapted boxing therapy that was part of a pluridisciplinary intervention tailored for a particular case. A 58-year-old man with left homonymous hemianopia (HH) and mild visuospatial hemineglect participated in 36 sessions of boxing therapy six months after a right temporo-occipital stroke. Repeated stimulation of his blind and neglected hemifield, and training to compensate for his deficits through improved use of his healthy hemifield were performed through boxing exercises. The patient showed a stable HH before the beginning of the training. After six months of boxing therapy, he reported improved awareness of his visual environment. Critically, his HH had evolved to a left superior quadrantanopia and spatial attention for left-sided stimuli had improved. Several cognitive functions and his mood also showed improvement. We conclude that boxing therapy has the potential to improve the compensation of visuospatial impairments in individual patients with visual field loss.

NEURO-OPHTHALMOLOGY

Abstract&#x0D; Introduction : Homonymous hemianopia (HH) is a visual field loss in the same halves of the visual field of each eye that is caused by numerous lesion affecting the retrochiasmal visual pathway. Homonymous hemianopia in children is a rare disabling condition and might be found as an initial presentation of brain tumors.&#x0D; Case Illustration : A 12 years old boy came with chief complaint of partially vision loss on the left side of vision accompanied by intermittent headache. Ophthalmological examination showed visual acuity of 1.0 on both eyes. Visual field examination revealed left homonymous hemianopia (figure 1). These findings were relevant to the result of his brain Computed Tomography (CT) scan, which showed vascular mass in the right occipital lobe caused by suspected arteriovenous malformation mixed diffuse type (figure 2). The patient was referred to neurosurgeon for further management.&#x0D; Discussion : Visual field defect depends on location of the lesion and can be used to recognize optic pathway diseases. Homonymous hemianopia occurs in retrochiasmal lesions that could happened either in adult or children. Unusual presentation of HH in children caused by brain tumor such as arteriovenous malformation. It might occur in up to 27% and can be detected radiologically with either brain CT scan or Magnetic Resonance Imaging (MRI).&#x0D; Conclusion : Visual field loss in pediatric patient might be challenging. Proper and prompt diagnosis is important to detect underlying condition and determine further management of the patient.

Real-World Assessment of Physical Activity in Glaucoma Using an Accelerometer

PurposeTo report a sectoral analysis of circumpapillary retinal nerve fiber layer (cpRNFL) thinning and its association with visual field loss using spectral-domain optical coherence tomography (SD-OCT) in patients with homonymous hemianopia following acquired post-geniculate visual pathway damage.Patients and methodsSeven patients with homonymous hemianopia due to unilateral acquired post-geniculate visual pathway lesions were studied. The average duration from the onset of brain lesions to the initial visit was 49.8 months. Forty-nine normal control subjects without visual field defects, as confirmed using a Humphrey visual field analyzer, were also enrolled. Measurement of the cpRNFL thickness was performed at the initial visit and 24 months using SD-OCT (RTVue-100® OCT). The cpRNFL thickness was divided into eight sectors (superior temporal: ST, temporal upper: TU, temporal lower: TI, inferior temporal: IT, inferior nasal: IN, nasal lower: NL, nasal upper: NU, superior nasal: SN). The eye on the same side as the occipital lobe lesions was defined as the ipsilateral eye, and the eye on the opposite side was defined as the contralateral eye.ResultsThe average cpRNFL thickness in the homonymous hemianopic eyes was significantly reduced as compared with that seen in the normal controls, except for the ipsilateral eyes at the initial visit. Four of the eight sectors of the cpRNFL thickness in the homonymous hemianopic eyes were significantly reduced compared with that noted in the normal controls. In the ipsilateral eyes, the cpRNFL thickness in the ST, TU, TL, and IT sectors was significantly reduced at both the initial visit and 24 months. In the contralateral eyes, the cpRNFL thickness in the TU, TL, IT, and SN sectors was significantly reduced at both the initial visit and 24 months. The reduction of the quadrantic cpRNFL thickness significantly correlated with some of the visual field parameters, in accordance with the structure–function relationship. In the contralateral eyes, the T and I quadrant cpRNFL thickness correlated with the mean deviation and hemianopic field total deviation at 24 months. In the ipsilateral eyes, the S, T, and I quadrant cpRNFL thickness correlated with mean deviation. However, there were no correlations between the cpRNFL thickness and visual field parameters at the initial visit.ConclusionsA reduction of the cpRNFL thickness corresponding to the hemianopic visual field loss due to acquired post-geniculate visual pathway lesions was detected using SD-OCT, and the change was more evident at 24 months than at the initial visit. The latter finding suggests that this change is, at least partially, caused by transsynaptic retrograde degeneration.

The scientific sessions this year included 2021 platform presentations and posters. There were 11 educational courses in neuro-ophthalmology and neuro-otology given by the following faculty: Robert Baloh, Laura Balcer, Valerie Biousse, James Corbett, Wayne Cornblath, Fiona Costello, Kathleen Digre, Eric Eggenberger, Scott Eggers, Terry Fife, Steven Galetta, Christopher Glisson, Timothy Hain, Janet Helminski, Aki Kawasaki, David Kumpe, R. John Leigh, Grant Liu, Mark Moster, Nancy Newman, David Newman-Toker, Victoria Pelak, Sharon Polensek, Valerie Purvin, John Pula, Janet Rucker, Jonathan Trobe, Ronald Tusa, Gregory Van Stavern, and David Zee. The topics most relevant to neuro-ophthalmologists are summarized here. NEURODEGENERATIVE DISEASES Adam Boxer and colleagues (San Francisco, CA) used eye movement recordings to identify clinical differences in patients with frontotemporal lobar degeneration (FTLD) who had tau vs. TDP-43 autopsy pathology. The investigators recorded saccades in 11 patients with FTLD-TDP-43 and 10 patients with FTLD-tau. Horizontal and vertical saccade times were higher in the tau group than the TDP-43 group (P < 0.02). Using a receiver operating curve (ROC) analysis, the authors demonstrated that slowed saccades accurately distinguished TDP-43 from tau patients. Detailed examination of the brainstem ocular motor nuclei was conducted in 5 representative patients and revealed increased pathologic burden in the tau patients. The authors concluded that saccadic abnormalities are a useful predictor of pathologic subtypes of FTLD, possibly due to greater brainstem involvement in the tau subtype. (S42.006) Shawn Smyth and colleagues (Aurora, CO) conducted a retrospective study of computerized visual field (CVF) defects in 9 patients with clinically diagnosed posterior cortical atrophy (PCA). Eight patients were found to have homonymous hemianopia (HH) or quadrantanopia and 1 had bilateral visual field constriction. All patients progressed to dementia (8 had probable Alzheimer disease and 1 had probable dementia with Lewy bodies). The authors concluded that the characteristic visual field defects seen in PCA patients probably reflect pathology to the primary and visual association cortices. (P09.171) OPTIC NEURITIS AND MULTIPLE SCLEROSIS Noa Raz and colleagues (Jerusalem, Israel) conducted psychophysical tests of visual function in patients with acute optic neuritis (ON) to characterize the deficits with respect to parvocellular and magnocellular pathways. Ten patients and 10 control subjects were studied at presentation, after 1 month, and after 4 months with visual acuity, computerized visual fields, contrast sensitivity, color perception, static high-contrast object detection, motion detection, coherent moving noise perception, and object-from-motion (OFM) perception. One month after the acute phase, the initial deficits of visual acuity, contrast sensitivity, color perception, visual fields, and static object detection had returned to normal or near-normal levels in 9 of 10 patients. However, the patients had persistently defective motion processing, with reduced OFM perception, higher motion detection thresholds, and prolonged reaction times. The level of OFM impairment correlated with the conduction delay detected on visual evoked potentials. The authors suggested that abnormalities of the magnocellular pathway after ON may be responsible for the patients’ persistent visual complaints, particularly regarding motion processing, despite normal standard visual testing. Low-contrast acuity, which is probably mediated by the parvocellular pathway, was not assessed. (P04.077) Takafumi Hosokawa and colleagues (Osaka, Japan) reported a retrospective analysis of Goldmann visual field defects after ON in 15 patients with neuromyelitis optica (NMO) and 20 patients with multiple sclerosis (MS). Anti-aquaporin 4 antibodies were positive in all patients with NMO and negative in all patients with MS. Altitudinal hemianopia was seen in 33% of patients with NMO and in none of the patients with MS (P < 0.05). (The patients with MS all showed central scotomas.) The authors suggested that an altitudinal field defect may distinguish NMO from MS and postulated an ischemic mechanism for the optic neuropathy in NMO. The study was limited by a small sample size, and the results are at variance with the Optic Neuritis Treatment Trial, which found that 15% of typical patients with ON had altitudinal defects. (P04.093) Claire Riley and colleagues (New York, NY) conducted a meta-analysis to evaluate the prognostic value of cerebrospinal fluid (CSF) abnormalities in patients with a clinically isolated syndrome (CIS) and >2 MRI lesions to determine progression to clinically definite multiple sclerosis (cdMS). Using unpublished data from the trials of glatiramer acetate and interferon beta for patients with CIS (BENEFIT, IFNβ-1a ETOMS, and GA PreCISe), they examined the CSF results and clinical outcomes from patients receiving placebo. Abnormal CSF was defined as oligoclonal banding (OCBs) and/or an elevated IgG index, and patients without both sets of these data were excluded. The presence of a CSF abnormality did not predict the likelihood of progression to cdMS. In addition, this study demonstrated that most patients with CIS and MRI abnormalities also have CSF abnormalities, but that the presence of CSF abnormalities does not have significant additional prognostic value in predicting the risk of cdMS over 2 years. (P02.119) Jonathan McNulty and colleagues (Dublin, Ireland) conducted a diffusion tensor tractography study to evaluate the integrity of the medial longitudinal fasciculus (MLF) in patients with internuclear ophthalmoplegia (INO). They studied 12 patients with INO and 12 matched control subjects. Participants underwent conventional MRI and diffusion tensor imaging. Regions of interest approximating the MLF were identified. Qualitative MLF abnormalities were detected in all 12 subjects and in no control subjects. One shortcoming was that this method is not specific and produces extraneous fiber tracts apart from the MLF. (P04.082) Ari Green and colleagues (San Francisco, CA) reported the correlation between N-acetylaspartate (NAA) levels in normal-appearing gray matter (NAGM) and a measurement of retinal nerve fiber layer (RNFL) thickness performed 2 years later in a cohort of patients with MS and CIS. They studied 179 patients (aged 32.9 ± 9.5, 65% women) using proton spectroscopy imaging (1H-magnetic resonance spectroscopy [MRS]). NAGM NAA values correlated with average RNFL measures (R = 0.22, P < 0.0001). This study demonstrates that reduced cerebral NAA levels are predictive of future axonal loss in the anterior visual pathway. (P05.156) Esther Bisker and colleagues (Philadelphia, PA) conducted a longitudinal study to assess the degree of RNFL thinning associated with the loss of low-contrast acuity and high-contrast acuity in patients with MS. In their study, 365 patients (725 eyes) underwent optical coherence tomography (OCT)-3 imaging at baseline and at 6-month intervals during a mean follow-up period of 1.5 years (range 0.5-3.7 years) at three academic centers. Visual function testing was performed using low-contrast (2.5 and 1.25% levels) and Early Treatment Diabetic Retinopathy Study (ETDRS) acuity charts. Worsening of low-contrast acuity was noted in 33% of MS eyes. Of these eyes with visual loss, only one third had a known history of ON. Two-line (10-letter) losses of 2.5% low-contrast acuity were associated with a 1.6-μm decrement in RNFL thickness over time (P = 0.009), and losses of 1.25% contrast acuity were associated with a 3.7-μm decrement (P = 0.02). The authors concluded that visual loss may be present even in the absence of a history of ON and that reduced low-contrast acuity is associated with RNFL thinning over time. These findings suggest that gradual optic nerve axonal loss may be an important feature of MS. (S57.004) Deanna Cettomai and colleagues (Baltimore, MD) reported the concordance between RNFL thickness measured by OCT and the clinical findings of optic nerve pallor or afferent pupillary defect (APD) on 212 consecutive patients. The mean RNFL was 96 ± 15 m (n = 366) in eyes without pallor and 78.7 ± 20.6 m (n = 58) in eyes with pallor. Mean RNFL was 84.7±16.1 m (n = 41) for eyes with an APD was detected and 95.4±16.8 μm (n = 383) for eyes without an APD. In patients for whom the ratio of the mean RNFL between the two eyes was <90%, an APD was detected on clinical examination in 86% (sensitivity = 0.28, specificity = 0.93). There was wide variability across physicians in the accuracy of detecting pallor or an APD. The authors suggested that OCT is a more sensitive measure of subclinical optic nerve damage than clinical examination alone and that OCT may be a useful adjunct in the management of patients with MS. (P05.158) Salim Abboud and colleagues (Hinckley, OH) reported their findings on the reproducibility of serial OCT without pharmacologic pupillary dilation (PPD). They conducted 2 serial measurements at least 1 week apart of the peripapillary RNFL thickness and macular volume (MV) in 10 consecutive healthy volunteers by Stratus OCT without PPD. All studies were conducted by a single operator. Across subjects, the coefficient of variation (COV) for independent serial measures of RNFL was 2.86% and for MV was 1.90%. The authors concluded that serial measurements of RNFL and MV are sufficiently precise to use as outcome measures in longitudinal studies, even when implemented without PPD. (P05.164) Sally Chang and colleagues (Philadelphia, PA) evaluated the utility of measuring low-contrast acuity in addition to the standard Multiple Sclerosis Functional Composite (MSFC) by assessing the strength of correlations of these assessments with RNFL thickness measurements. They studied 164 patients (326 eyes, aged 47 ± 10 years), measuring low-contrast letter acuity (2.5% and 1.25% levels), high-contrast acuity (ETDRS charts), and standard MSFC. Scores for MSFC with low-contrast acuity added (MSFC-4) had greater correlations with RNFL thickness compared with the standard MSFC (P = 0.07 for MSFC, P = 0.005 for MSFC-4 with 2.5% low-contrast, and P = 0.007 for MSFC-4 with 1.25% contrast). The authors concluded that measurement of low-contrast acuity increases the capacity of the MSFC to capture the effects of axonal loss in the anterior visual pathway. (P04.076) Gurdesh Bedi and colleagues (Miami Beach, FL) conducted a retrospective study to evaluate the efficacy of rituximab on the relapse rate and disability in NMO. Among 19 patients treated with rituximab, relapses occurred in 5 patients. The authors concluded that rituximab leads to a significant reduction in relapses in patents with NMO. (P04.099) NONARTERITIC ANTERIOR ISCHEMIC OPTIC NEUROPATHY Edward Atkins and colleagues (Atlanta, GA) evaluated the treatment of nonarteritic anterior ischemic optic neuropathy (NAION) in the United Stated using a Web-based anonymous survey (n = 1595) of US neuro-ophthalmologists (US-NO = 350), Georgia ophthalmologists (GA-O = 340), Georgia neurologists (GA-N = 322), and Georgia optometrists (GA-OD = 583). For acute treatment, 63% of GA-N and GA-O and 80% of US-NO use antiplatelet agents, 10% of physicians use oral steroids, 19% of GA-N use high-dose intravenous steroids, 22% of US-NO and 13% of GA-O use topical brimonidine, and 7% of US-NO use intravitreal bevacizumab. For secondary prevention of fellow eye involvement, >74% physicians use antiplatelet agents, whereas 10%-15% of ophthalmology-trained US-NO and GA-O also use brimonidine in this setting. More than 80% of physicians manage vascular risk factors aggressively; 15% of US-NO obtain carotid ultrasound compared with 51% of GA-O and 72% of GA-N, and 16% of US-NO obtain neuro-imaging compared with 25% of GA-O and 84% of GA-N. The authors conclude that, despite insufficient evidence, most physicians currently use antiplatelet agents for acute treatment and secondary prevention of NAION. Other popular treatments include intravitreal bevacizumab, topical brimonidine, and steroids. Neurologists are less familiar with the management of NAION than ophthalmologists and neuro-ophthalmologists. (P04.079) VISUAL LOSS Wolfgang Heide and colleagues (Celle, Germany) investigated visual search patterns in patients with acute HH. They tested the hypothesis that visual search in HH is determined purely by the visual-sensory deficit by comparing 9 patients with HH due to acute occipital stroke with 9 healthy subjects with a simulated “virtual” HH (VHH) and 9 control subjects with normal visual fields. They recorded eye movements while subjects searched for targets among distractors. All patients, even those with small lesions restricted to the visual cortex, showed longer search durations than VHH subjects. Their longer search duration correlated with a higher number of both fixations and “re-fixations” (repeated scanning of fixated items). Scan-path strategies were similar in HH and VHH subjects. The authors concluded that pure visual input failure alone does not fully account for abnormal visual search in patients with isolated occipital lesions. They postulate that the longer search durations may result either from changes in visual attention due to disconnections of the visual cortex or from an early stage of compensatory eye movements. (S57.005) Sashank Prasad (Philadelphia, PA) received the S. Weir Mitchell award for excellence in basic science research from the American Academy of Neurology Alliance. He and his colleagues studied structural and functional changes of the visual pathway in patients with early-onset blindness. They studied 10 blind subjects and 10 sighted control subjects, collecting BOLD functional MRI (fMRI) data (during a language comprehension/semantic decision task), a volumetric anatomical scan, a resting perfusion scan, and diffusion tensor imaging. They found that during sentence comprehension, blind subjects demonstrated significant occipital activation in addition to left hemispheric language areas (BOLD Δ, blind 0.9% vs. sighted 0.0%; P < 0.05). Furthermore, they found a positive correlation across subjects between resting occipital perfusion and the amount of cross-modal task activation (R = 0.5; P < 0.05). In addition, white matter atrophy and a reduction in anisotropy were correlated (R = 0.7; P = 0.07). On the other hand, no structural measures predicted the amount of functional cross-modal activation (P > 0.1). The authors concluded that significant structural and functional differences exist between early-blind and sighted subjects. In addition, lack of correlation between structural and functional measures may suggest that these forms of plasticity are independent in the brain's response to early blindness. A larger study is necessary to explore that possibility. IDIOPATHIC INTRACRANIAL HYPERTENSION In a retrospective review of 230 consecutive patients with idiopathic intracranial hypertension (IIH) over 8 years, Sachin Kedar and colleagues (Jackson, MS) studied the effect of patient factors on the level of opening pressure (OP) in patients at presentation and the effect of OP on visual outcomes. They found an OP at presentation of 388 ± 93 mm H2O that negatively correlated with age (r = 0.2). Gender, race, initial body mass index (BMI), weight change, presenting symptom, and time interval to presentation were not associated with OP. Higher OP was associated with worse initial vision. Patients with visual acuity (VA) ≥20/100 had an OP of 382 ± 90 mm H2O and those with a VA <20/100 had an OP of 515 ± 77 mm H2O; patients with normal visual fields (VFs) had an OP of 390 ± 85 mm H2O and those with severely constricted VFs had an OP of 439 ± 110 mm H2O. Patients with normal-appearing optic nerves had an OP of 358 ± 94 mm H2O, whereas those with grade 4-5 papilledema had an OP of 439 ± 109 mm H2O. There was no significant association, however, between OP and visual outcome (improvement or worsening of VA or VFs or appearance of ON during follow-up). The authors concluded that a higher presenting OP in patients with IIH is associated with worse initial VA, VF loss, and ON appearance, but that OP is not predictive of the clinical course. (P04.080) J. Alexander Fraser and colleagues (Atlanta, GA) conducted a case-control study to evaluate potential risk factors for IIH in 24 men and matched control subjects. They administered a telephone questionnaire (including the Androgen Deficiency of Aging Men [ADAM] questionnaire for hypogonadism and the Berlin questionnaire for sleep apnea) and explored medical comorbidities, obesity patterns, endocrinologic problems, reproductive and and sleep for men with IIH were more than control subjects to have of The authors concluded that men with IIH have a higher of of and sleep and that these factors may be and colleagues GA) conducted a retrospective cohort study of consecutive with IIH and matched control subjects from three centers. In this group they 20 patients than years at and with a normal were more to be white (P = vs. than the of the IIH patients with IIH had a but were (P = vs. presentation, they were less to (P < vs. and more to of visual changes (P = vs. they were more to have persistent optic (P = but they had not visual outcomes than patients Among patients with normal IIH was more (P = vs. patient with IIH who had a normal had visual loss in either eye (P = vs. The authors concluded that patients and those with a normal a small of those with IIH but to have visual outcomes than typical patients with DISEASES and colleagues reported a patient with ophthalmoplegia and a in without associated visual loss or optic The patient was a who had loss, and in addition to There was no loss of visual acuity, optic visual field or optical coherence tomography visual evoked were revealed and revealed not Multiple were identified. The associated with multiple and were and no were A was in which reported in two patients with optic The the of associated with by multiple abnormalities in the absence of optic nerve and colleagues conducted a retrospective study of patients with ophthalmoplegia to the of to In a review of patients with who underwent and had they found that the of a was of and that of a was of of the subjects who had negative had positive The authors suggested that patients with a of that of higher and colleagues reported abnormalities of the and visual pathway in patients with studied by They studied 5 patients with and 10 healthy control subjects. They the correlations between diffusion tensor tractography and data with OCT measures and activation of the visual revealed optic nerve and as as a significant reduction of occipital gray in all patients with There was reduced anisotropy in the left optic which correlated across subjects with OCT RNFL measurements. The patients demonstrated reduced activation in The authors concluded that optic nerve damage in may structural and functional changes of the visual the study does not the that are also due to and colleagues visual disability in the of risk factors in = = and = from independent Of were clinically and of the subjects were received a questionnaire on and visual loss for and the Visual which impairment in of from to patients with the had higher compared with those with the and (P < 0.0001). was associated with visual loss in a ratio interval P = of on the other hand, did not with visual loss on analysis P = The authors concluded that the a useful in assessing visual in and that may to reduced visual The of is not and colleagues reported a correlation in between axonal number and the of in optic nerve Their were on a analysis of 2 patients eyes) and control subjects They found that the of the optic nerve was (P < than in the and They concluded that the of the are the most in and have the amount of The mechanism by which reduced number and are to be and colleagues the integrity of the that from the retinal in optic They studied 5 patients with 4 patients with optic and 9 control subjects. They optic neuropathy by A was collecting from to and a using between and The was by comparing the level to the baseline A significant of levels by was in control subjects ± and in patients ± ± without a between eyes of 2 patients with and 2 control subjects, analysis of retinal the optic nerve revealed a of in patients with compared with control subjects, despite the loss of The authors concluded that in and is of Their autopsy study in that this may to of Mark (New York, NY) conducted a retrospective analysis of patients with ocular to determine the effect of in and progression to Patients who had for longer than 4 years or who were identified. patients with received was present at the examination in of the group and of the the study is limited by retrospective the authors concluded that in patients with may the of delay and control and colleagues evaluated the outcomes of a cohort of patients with treated with trials have to of over alone as initial but these studies were limited by duration or sensitive outcome The authors the clinical outcomes of patients with receiving (n = or (n = for longer than Patients were by and follow-up was were Patients treated with to between and 12 80% of patients for months had an and of or pharmacologic Patients treated with had a reduction after 12 at had from and patients of The authors concluded that is an treatment as either or to The follow-up demonstrated a effect of during the and third year of that not be demonstrated by studies of This study, however, did not include patients treated with a of the of and colleagues reported the utility of the to In the the is by the while the patient on the is a deficit in the one a saccade at the of the The authors by measuring the of the at patients demonstrated an deficit of a from normal to bilateral The deficits occurred even at the Across subjects, correlated with testing = P = than with testing = P = The of saccades was times greater in patients than in control subjects. The authors concluded that the is a sensitive measure of and colleagues studied the value of in patients with acute syndrome in a study over 9 years. Their cohort of consecutive patients with who with and/or motion and had at least one stroke risk They underwent and testing for ocular of and were by MRI or A of patients with were of whom had a and had a brainstem ischemic 2 1 and 1 was present in 15% of the study and correlated with brainstem occurred in of with a of with pure and of with a brainstem (P = Of the presence of predicted the presence of stroke in 2 of the patients in whom positive results for a had suggested a The authors concluded that is an of central pathologic when is detected is specific for brainstem involvement among patients with David and colleagues (Baltimore, MD) evaluated the of decision to clinical for stroke among patients with in the are that use a and patient data to suggest that the The authors evaluated by and of and with to The was a patient in average without disability presenting either with persistent at risk for or at risk for ischemic The strategies were and measures were and The authors found that for persistent operating at and specificity or at be less and not be For operating at and specificity at and less for the and not be The authors concluded that among patients at and colleagues CA) evaluated the association between and by patients with a with a In their study, of patients the of for with and without did not for Among patients with or with or all of their the for definite The of patients did not and The age of and duration of did not between patients with ± years) and patients without ± In patients with the age of of the of by an average of years and by 8 years. The duration of was between 1 and 1 The authors concluded that the association between and as a However, patients with and have during the The of as of a syndrome may be by later to the of and and colleagues (Baltimore, MD) evaluated the correlation between retinal vascular abnormalities and in They studied a study in assessments included and were evaluated for small or to a greater degree in with than in those without to and to even after for and with had increased of the on There was no association between and retinal vascular The authors concluded that with measures in possibly by as a for cerebral vascular and colleagues conducted an retrospective review to assess the of with the of They examined other and outcomes. The included and with a mean age at presentation of years (range years) and mean age of years at of was most to by eye syndrome eye and other ocular was found in 25% of the patients, among whom most were Among these patients, reported that the with of and reported with were for of the patients, among whom a reported The authors concluded that a and is associated with a of and other and ocular and reduced of were among these patients. Sashank of of the of Mark Moster, of of

Abstract&#x0D; Introduction : Homonymous hemianopsia is a visual field defect characterized by loss of half of the visual field in each eye. Cerebral infarction in the occipital lobe is the most common cause of homonymous hemianopsia. Sudden visual field loss should be considered as a cerebral infarction for prompt management.&#x0D; Case Illustration : Male 43 years old, vision in both eyes appears dark on the left side since 3 months ago, complaints occur suddenly. Patients with a history of hypertension (+) hypercholesterolemia (+). Ophthalmological examination revealed visual acuity in both eyes 6/6 with normal pupillary reflexesand normal fundoscopy in both eyes. Humphrey's visual field perimetry test showed left homonymous hemianopsia. A brain CT scan examination showed multiple cerebral infarctions in the right occipital lobe, cerebellum and basal ganglia bilaterally. The patient was referred to the neurology department for further treatment.&#x0D; Discussion : The visual field defect pattern is used to predict the location of the lesion in the visual pathway. Neurologic symptoms that may be present in occipital lobe lesions are migraine with aura and visual hallucinations. The patient had left homonymous hemianopsia where the results of brain CT scan showed multiple cerebral infarctions in the right occipital lobe.&#x0D; Conclusion : Manifestations of homonymous hemianopsia can predict the presence of cerebral infarction so that in the presence of visual field defects it can be used as a predictor of intracranial abnormalities for further evaluation and collaboration with the neurology department.

Homonymous hemianopias: clinical-anatomic correlations in 904 cases

To describe the clinical characteristics and clinical-anatomic correlations of homonymous hemianopia (HH). Homonymous hemianopia impairs visual function and frequently precludes driving. Most knowledge of HH is based on relatively few cases with clinical-anatomic correlations. The authors reviewed medical records of all patients with HH seen in their service between 1989 and 2004. Demographic characteristics, characteristics of visual field defects, causes of visual field defects, neuroradiologic definition of lesion location, and associated neurologic deficits were recorded. A total of 904 HH were found in 852 patients. A total of 340 HH (37.6%) were complete and 564 HH (62.4%) were incomplete. Homonymous quadrantanopia (264 HH, 29%) was the most common type of incomplete HH, followed by homonymous scotomatous defects (116 HH, 13.5%), partial HH (114 HH, 13%), and HH with macular sparing (66 HH, 7%). A total of 407 HH (45.0%) were isolated. Causes of HH included stroke (629 HH, 69.6%), trauma (123, 13.6%), tumor (102, 11.3%), brain surgery (22, 2.4%), demyelination (13, 1.4%), other rare causes (13, 1.4%), and unknown etiology (2, 0.2%). The lesions were most commonly located in the occipital lobes (45%) and the optic radiations (32.2%). Every type of HH, except for unilateral loss of temporal crescent and homonymous sectoranopia, was found in all lesion locations along the retrochiasmal visual pathways. Homonymous hemianopia is usually secondary to stroke, head trauma, and tumors. Although the characteristics of visual field defects can be helpful in lesion location, specific visual field defects do not always indicate specific brain locations.