Ameliorating Hemianopia with Multisensory Training.

Hemianopia (unilateral blindness), a common consequence of stroke and trauma to visual cortex, is a debilitating disorder for which there are few treatments. Research in an animal model has suggested that visual-auditory stimulation therapy, which exploits the multisensory architecture of the brain, may be effective in restoring visual sensitivity in hemianopia. It was tested in two male human patients who were hemianopic for at least 8 months following a stroke. The patients were repeatedly exposed to congruent visual-auditory stimuli within their blinded hemifield during 2 h sessions over several weeks. The results were dramatic. Both recovered the ability to detect and describe visual stimuli throughout their formerly blind field within a few weeks. They could also localize these stimuli, identify some of their features, and perceive multiple visual stimuli simultaneously in both fields. These results indicate that the multisensory therapy is a rapid and effective method for restoring visual function in hemianopia.SIGNIFICANCE STATEMENT Hemianopia (blindness on one side of space) is widely considered to be a permanent disorder. Here, we show that a simple multisensory training paradigm can ameliorate this disorder in human patients.

Partial Balint's syndrome and left homonymous hemianopsia presenting after resection of a right occipito-parietal glioblastoma.

A 66-year-old left-handed male was admitted to our acute inpatient rehabilitation (AIR) unit following a resection of the right occipito-parietal glioblastoma. He presented with symptoms of horizontal oculomotor apraxia, contralateral optic ataxia and left homonymous hemianopsia. We diagnosed this patient with partial Bálint's syndrome (BS)- oculomotor apraxia, optic ataxia but not simultanagnosia. BS is typically caused by bilateral posterior parietal lesions, but we here describe a unique case due toresection of a right intracranial tumor. A short AIR stay allowed our patient to learn how to compensate for visuomotor and visuospatial deficits, and improved his quality of life significantly.

Acute visual impairment as a main presenting symptom of non-convulsive status epilepticus: a case report.

BACKGROUND: Nonconvulsive status epilepticus (NCSE) is a state of ongoing seizure activity without convulsions. The heterogeneous and subtle clinical features of NCSE make diagnosis and treatment challenging. Here, we report a patient with NCSE who showed a main presenting symptom of acute visual impairment, which is a rare and atypical clinical symptom of NCSE. CASE PRESENTATION: A 62-year-old man was admitted to the neurology department after complaining of an inability to see in the right eye for 2 days and progressive headache. He had a history of poststroke epilepsy and vascular dementia. Physical examination revealed right visual field hemianopia, visual neglect and cognitive impairment. T2 and diffusion-weighted magnetic resonance imaging showed high signal intensity in the left temporal, parietal and occipital lobes. Electroencephalography monitoring was performed, which found continuous sharp wave discharges, especially in the regions of the left temporal, parietal and occipital lobes. These findings were most consistent with the diagnosis of NCSE. Thus, a treatment of intravenous pumping of diazepam and an oral antiepileptic drug was added immediately. After that, the visual loss in the patient recovered quickly, and electroencephalography did not find epileptiform waves. On day 11, a follow-up MRI was performed, which showed that the abnormal signals of the left temporal, parietal and occipital lobes were markedly attenuated, and the patient returned to his premorbid state with a modified Rankin Scale score of 3. CONCLUSIONS: Acute visual impairment can be seen in NCSE, and it can be reversed by administering effective antiepileptic treatment. Meanwhile, transient peri-ictal MRI abnormalities can be observed in NCSE.

The Difficult Diagnosis of Posterior Cortical Atrophy in a 62-Year-Old Woman.

Posterior cortical atrophy (PCA) describes a rare heterogenous neurodegenerative syndrome with early visuospatial and visuoperceptual deficits due to atrophy of parieto-occipital brain regions. Here, we describe the case of a 62-year-old woman showing severe cognitive impairments as well as hemianopsia and all core symptoms of Bálint's syndrome. Years ago, the patient had complained about a "tunnel view" and concentration problems. The diagnostic results point to a case of PCA with underlying Alzheimer pathology. The disease course until diagnosis lasted for 7 years, reflecting the diagnostic difficulties with this still largely unknown syndrome. The unfamiliar symptom presentation including fluctuations in cognitive performance, affective symptoms, cerebrospinal fluid (CSF) biomarkers, which were at first inconspicuous, and a former suspected diagnosis of dissociative pseudodementia, altogether brought considerable uncertainty to the involved health-care professionals. We conclude that cases of "atypical dementia" presenting with visual symptoms, even if appearing unspecific at first, are suspect of PCA. This case report provides an ostensive overview of PCA, including imaging data, CSF-findings, original drawings and handwriting samples from the patient.

Delayed presentation of morning glory disc anomaly and transsphenoidal encephalocele: A management dilemma.

Background Morning glory disc anomaly (MGDA) is a rare developmental abnormality of the optic disc that is associated with many other neurological and vascular conditions. Most cases are diagnosed in childhood. Case Report We report a 57-year-old woman who presented to the ophthalmology department for assessment of long-standing poor vision in the left eye and exotropia. Examination showed a left MGDA and bitemporal hemianopsia. These findings prompted magnetic resonance imaging, revealing a transsphenoidal basal meningoencephalocele with herniation of the optic chiasm and inferior hypothalamus into the dural sac. Due to the eloquence of the neurovascular structures it contained, a decision was made not to reduce the meningoencephalocele. Instead, a ventriculoperitoneal shunt was placed. The patient's ophthalmologic examination remained stable over the following year. Conclusion While rare, MGDA can be first diagnosed in late adulthood and a thorough evaluation should be completed to assess for midline cranial defects, vascular abnormalities, and other associated abnormalities. Patients presenting late in life with basal encephalocele, herniation of the optic chiasm, and bitemporal hemianopsia present a management dilemma. In this case, a ventriculoperitoneal shunt was placed with the intention of lowering intracranial pressure to prevent further herniation and reduce the risk of cerebrospinal fluid leak.

Clinical and Imaging Findings One Year Following Traumatic Chiasm Transection.

Traumatic chiasm transection is a rare condition characterized by bitemporal hemianopsia following head trauma. In a study of visual complications following head trauma, only 4.4% of patients suffered from chiasmal injuries. Complete transection of the chiasm is even more rare and best visualized using magnetic resonance imaging (MRI). We report the one-year follow-up of clinical, visual field, and MRI findings of a patient with complete transection of the optic chiasm following head trauma.

[The correlation of ganglion cell layer thickness with visual field defect in non-functional pituitary adenoma with chiasm compression].

Objective: To investigate the consequences of the thickness of ganglion cell layer (GCL) and visual field defect of non-functional pituitary adenoma with chiasm compression. Methods: A case control study. The study included 40 (80 eyes) non-functional pituitary adenoma patients in Peking Union Medical College Hospital from March 2015 to February 2017. Twenty patients (no visual field defect group, 40 eyes) of them were detected to be chiasm compressed or touched by the adenoma with no visual field defect detected, and the other 20 patients (visual field defect group, 40 eyes) were the sex-and-age matched pituitary adenoma patients with bitemporal heminopsia. This study also included 20 (control group, 40 eyes) sex-and-age matched healthy controls. The para-papillary retinal nerve fiber layer (RNFL) thickness in 6 quadrants including nasal, temporal, nasal superior, temporal superior, nasal inferior and temporal inferior as well as the macular GCL thickness and ganglion cell-inner plexiform layer (GCIPL) thickness in 4 quadrants including nasal superior, nasal inferior, temporal superior and temporal inferior were measured. The non-parametric test was used to compare the RNFL, GCL and GCIPL thickness among the three groups. Results: The mean age among the three groups was (46±10) years and the difference among the three groups was not significant (P=0.88). The sex ratio of the three groups was 9∶11 (male∶female) and the difference among the three groups was not significant. The mean axial length among the three groups was (23.22±0.90) mm and the difference among the three groups was not significant (P=0.51). The thickness of para-papillary RNFL of temporal superior, temporal, nasal superior, nasal, nasal inferior quadrants and whole circumference was significantly thinner in the visual field defect group than the control group [(129.88±28.64) µm, (63.63±26.84) µm, (88.08±32.16) µm, (50.68±19.99) µm, (92.48±25.06) µm, and (85.00±20.65) µm vs. (141.10±18.95) µm, (79.12±16.78) µm, (113.68±21.28) µm, (69.67±14.23) µm, (117.80±31.32) µm, and (102.80±9.68) µm, t=2.26, 3.06, 4.14, 4.84, 4.25, 4.88, all P<0.05]. In the nasal quadrant, the para-papillary RNFL of the no visual field defect group was significantly thinner compared with the control group [(61.45±9.83) µm vs. (69.67±14.23) µm, t=2.97, P<0.05]. The total GCL thickness was (30.48±5.42) µm in the visual field defect group, (31.35±2.77) µm in the no visual field defect group, thinner than that in the control group [(33.32±2.92) µm, t=2.92, 3.62; both P<0.05]. The total GCIPL thickness showed no significant difference among the three groups (P=0.07). In the superior and inferior temporal quadrants, the GCL and GCIPL thickness showed no significant difference among the three groups (all P>0.05). In the superior and inferior nasal quadrants, the GCL thickness was (29.41±5.97) µm, and (28.47±5.13) µm in the visual field defect group, (31.15±3.27) µm and (30.61±2.96) µm in the no visual field defect group, and (34.23±3.16) µm and (32.97±2.78) µm in the control group. The GCL thickness in the nasal quadrant was thinner in the visual field defect group (t=4.45, 4.82)and the no visual field defect group(t=4.23, 3.63) than in the control group (all P<0.01). However, no significant difference in GCL thickness was detected between the visual field defect group and the no visual field defect group (both P>0.05). In the superior and inferior nasal quadrants, the GCIPL thickness was (54.06±10.50) µm and (51.77±9.18) µm in the visual field defect group, (58.03±4.00) µm and (56.23±5.37) µm in the no visual field defect group, and (62.26±7.11) µm and (59.39±6.64) µm in the control group. The GCIPL thickness was thinner in the nasal quadrant in the visual field defect group than in the control group (t=3.95, 4.20, both P<0.01). Only in the Superior nasal quadrant, the GCIPL was significantly thinner in the no visual field defect group than the control group (t=3.25, P<0.01). Conclusion: The optic GCL may get thinner in pituitary nonfunctional adenoma with chiasm compression patients without the RNFL layer thinning and visual field defect. (Chin J Ophthalmol, 2019, 55: 186-194).

Vertebrobasilar dolichoectasia causing homonymous hemianopia: a case report and review of the literature.

A 73-year-old man was diagnosed with an incidental finding of right homonymous hemianopia and presyncopal spells. Magnetic resonance imaging revealed a vertebrobasilar dolichoectasia (VBD) with compression of the left optic tract. VBD is defined as an elongation and dilatation of the basilar artery. Only a few cases have been described in the literature of VBD causing optic tract compression. So far no treatment option has been described for these cases, while surgical options exist for compressions of other cranial nerves, most commonly the trigeminal nerve. VBD can further cause stroke or cerebral and subarachnoid bleeding.

Basal Encephalocele Presenting with Bitemporal Hemianopsia in an Adult Male.

Basal encephaloceles are uncommon malformations that are usually congenital in nature, although, occasionally, they can be traumatic. When they are congenital, they are associated with skull and facial midline defects, central nervous system abnormalities, and possible ocular and visual problems. The authors present a case of an adult male with a basal encephalocele who presented with a bitemporal hemianopsia. Ocular abnormalities, visual loss, and visual field deficits have previously been reported in patients with basal encephalocele; however, there does not appear to be any reported cases with formal visual field testing to demonstrate specific defects such as a bitemporal hemianopsia.

[Etiology and diagnostics of compressive optic neuropathies]. / Analiz prichin i vozmozhnosti diagnostiki kompressionnykh opticheskikh neiropatii.

PURPOSE: To determine the causes and study the clinical manifestations of compressive optic neuropathy (CON). MATERIAL AND METHODS: The study included 24 male and 36 female patients with CON (in total 60 patients, 97 eyes). All patients underwent standard ophthalmic examination, as well as computer perimetry, magnetic resonance imaging (MRI), and/or computed tomography (CT) of the brain and orbit and magnetic resonance angiography (MRA). RESULTS: Tumor or infiltrative lesions of the anterior optic pathway were detected in 60 patients (97 eyes). In 2/3 of cases, the brain and orbit diseases were established for the first time as the cause of optic neuropathy. Adenoma of the pituitary gland and meningioma as the most common cause of CON was diagnosed in 66%. According to the results of computed perimetry, in 65.9% of cases patients with CON are diagnosed with hemianopsia, while in other cases, other forms of scotomas characteristic for the defeat of any part of the optic nerve may be observed. CONCLUSION: Computed perimetry is the most important study for detection of optic neuropathy. An optic nerve disease of unknown origin is an absolute indication for radiological examination of the brain and eye orbits.

Trans-synaptic Retrograde Degeneration in the Human Visual System: Slow, Silent, and Real.

Degeneration of neuron and axons following injury to cells with which they synapse is termed trans-synaptic degeneration. This phenomenon may be seen in postsynaptic neurons (anterograde) or in presynaptic neurons (retrograde). Retrograde trans-synaptic degeneration (RTSD) of the retinal ganglion cells and retinal nerve fiber layer following injury to the occipital lobe has been well documented histologically in animal studies, but its occurrence in the human retina was, for many years, felt to be limited to cases of neonatal injury during a critical period of neuronal development. Over the last decade, imaging techniques such as MRI and optical coherence tomography have allowed us to visualize and quantify RTSD and analyze its time course and relationship to degree of vision loss and age of cortical injury. A deeper understanding of RTSD in the human visual system may allow us to interfere with its occurrence, potentially allowing for greater recovery following visual cortex injury.

Characteristics and surgical strategies for posterior clinoid process meningioma: two case reports and review of the literature.

Clinical and radiological features or characteristics of posterior clinoid process (PCP) meningiomas have rarely been described because of their extreme scarcity and terminological confusion. Therefore, the strategies in the surgical intervention for PCP meningiomas have not been well established. Moreover, the presence of deep and critical neuroanatomical structures and relatively high morbidity, which can be difficult to predict preoperatively, make their surgical excision more challenging. We report two surgical cases of PCP meningioma and discuss the appropriate assessment of preoperative features and surgical strategies with review of the literature. Our study suggests that PCP meningioma may be characterized by the anterior displacement of internal carotid artery, and infero-laterally shifted posterior communicating arteries, and homonymous hemianopsia, a distinctive clinical feature. One of the key issues in PCP meningioma surgery is preservation of the optic nerve. Unlocking the optic nerve by anterior clinoidectomy and dissection, the falciform ligament is the important step to preserve vision for larger tumors. Complication with the perforators is also hazardous of these challenging surgeries than anterior clinoid meningiomas for their specific neuroanatomical structures and might not be feasible to avoid even with additional techniques and critical monitoring. A combination and multi-staged-surgical approach can be options of tailor-made surgical strategy in cases with tumor adhesion to the perforators.

Homonymous Hemianopsia Due to Posterior Cortical Atrophy.

A 63-year-old woman presented to her ophthalmologist complaining of reading difficulties for two years. Ophthalmological examination revealed a homonymous hemianopsia. Brain magnetic resonance imaging (MRI) scan was interpreted as normal, but positron emission tomography (PET) showed areas of posterior brain hypometabolism. This case highlights the high diagnostic suspicion that ophthalmologists should have regarding posterior cortical atrophy (including the visual variant of Alzheimer disease) in patients complaining of reading difficulties in the setting of a normal ophthalmic examination.

Transcranial direct current stimulation (tDCS) combined with blindsight rehabilitation for the treatment of homonymous hemianopia: a report of two-cases.

[Purpose] Homonymous hemianopia is one of the most common symptoms following neurologic damage leading to impairments of functional abilities and activities of daily living. There are two main types of restorative rehabilitation in hemianopia: "border training" which involves exercising vision at the edge of the damaged visual field, and "blindsight training," which is based on exercising the unconscious perceptual functions deep inside the blind hemifield. Only border effects have been shown to be facilitated by transcranial direct current stimulation (tDCS). This pilot study represents the first attempt to associate the modulatory effects of tDCS over the parieto-occipital cortex to blindsight treatment in the rehabilitation of the homonymous hemianopia. [Subjects and Methods] Patients TA and MR both had chronic hemianopia. TA underwent blindsight treatment which was combined with tDCS followed by blindsight training alone. MR underwent the two training rounds in reverse order. [Results] The patients showed better scores in clinical-instrumental, functional, and ecological assessments after tDCS combined with blindsight rehabilitation rather than rehabilitation alone. [Conclusion] In this two-case report parietal-occipital tDCS modulate the effects induced by blindsight treatment on hemianopia.

Spectrum and Clinical Course of Visual Field Abnormalities in Ethambutol Toxicity.

Visual toxicities from ethambutol are rare but represent one of the few causes of non-glaucomatous and non-compressive bitemporal hemianopsia. The authors present a six-patient case series illustrating variable clinical presentation and reversibility of visual loss in patients treated with ethambutol for Mycobacterium avium complex, including four patients who presented with visual field defects suggestive of bitemporal hemianopsia. Two additional patients were being followed for glaucoma, developed visual field defects with ethambutol treatment, and subsequently recovered with cessation of drug. In patients being treated with ethambutol, reversible bitemporal hemianopsia was the most commonly noted presentation of ethambutol toxicity.

A Case of Bilateral Optic Nerve Head Drusen-Induced Inferior Altitudinal Hemianopsia.

The current case involved a 59-year-old woman who was referred to our hospital with bilateral optic nerve abnormality and gradually progressive bilateral inferior visual field defects. An ophthalmological examination revealed superficial optic nerve head drusen (ONHD) and bilateral inferior altitudinal hemianopsia, but no intracranial lesions. The results of an angiogram revealed no evidence of retinal vascular occlusion or anterior ischaemic optic neuropathy (AION). Prior reports suggested that drusen-induced hemianopsia was slowly progressive but that ONHD-induced AION caused acute altitudinal hemianopsia, which led us to the diagnosis of bilateral drusen-induced altitudinal hemianopsia.

Bitemporal hemianopia; its unique binocular complexities and a novel remedy.

Bitemporal hemianopic visual field impairment frequently leads to binocular vision difficulties. Patients with bitemporal hemianopia with pre-existing exophoria complain of horizontal diplopia, sometimes combined with vertical deviation (with pre-existing hyperphoria). The symptoms are a result of the phoria decompensating into a tropia (hemi-slide) due to the lack of retinal correspondence between the remaining nasal fields of both eyes. We measured these effects using a dichoptic perimeter. We showed that aligning the eyes with prisms could prevent diplopia if the bitemporal hemianopia is incomplete. We also describe the successful use of a novel fusion aid - the 'stereo-typoscope' - that utilizes midline stereopsis to prevent diplopia resulting from hemi-sliding in patients with complete bitemporal hemianopia.

Use of yoked prisms in patients with acquired brain injury: a retrospective analysis.

PRIMARY OBJECTIVE: The purpose of this retrospective study was to determine the clinical practices for prescribing yoked prisms, as well as to assess related patient responses, in a sample of visually-symptomatic patients having acquired brain injury (ABI). METHODS: The clinical records of individuals with acquired brain injury (ABI) that were assessed for yoked prisms were reviewed retrospectively. This query resulted in 60 patient records for analysis between January 2011 and December 2012. The following diagnostic groups were analysed: homonymous hemianopsia (HH)/homonymous quadranopsia, abnormal egocentric localization (AEL) and visual neglect. RESULTS: HH/homonymous quadranopsia (58.3%) was the primary indication to prescribe yoked prisms, followed by visual neglect/unilateral spatial inattention (USI) (40.0%) and AEL (11.7%). The most common favourable patient responses were increased awareness of their blind visual field and improved gait, mobility and balance. The magnitude and direction of prisms prescribed were dependent upon the subjective responses in patients manifesting AEL. In contrast, base direction was dependent upon the direction of visual field loss in patients with HH/homonymous quadranopsia and visual neglect. CONCLUSIONS: Two-thirds of the present sample population responded favourably to the yoked prisms. The results of the present study should prove useful to clinicians for the successful prescription of yoked prisms as a treatment modality in patients presenting with the above three diagnoses.

Early cerebral venous drainage associated with focal seizures: a correlation of digital subtraction angiography and electroencephalography. Illustrative case.

BACKGROUND: Early venous drainage is a critical diagnostic feature of arteriovenous malformations (AVMs) and arteriovenous fistulas (AVFs). However, other conditions associated with early venous drainage can mimic AVMs and AVFs and mislead the treating physician team. OBSERVATIONS: The authors present a unique case of a 56-year-old man with new left hemianopsia. Workup with magnetic resonance imaging and magnetic resonance angiography had revealed cortical enhancement and early venous drainage, possibly caused by an AVF or AVM. Catheter angiography confirmed the presence of early venous drainage of specifically the right occipital lobe. The early venous drainage occurred as the result of local hyperperfusion limited to the right occipital lobe. Subsequent electroencephalography confirmed focal seizure activity in the right occipital lobe. After seizure control with multiple antiepileptic medications, the patient regained his vision. Repeat digital subtraction angiography revealed restoration of normal cerebral blood flow. LESSONS: Early venous drainage observed during catheter angiography can be associated with seizure activity. Differentiation from other conditions, such as AVM, AVF, or ischemic stroke, is critical to facilitate prompt and appropriate treatment.