Partial Recovery from Cortical Blindness Following Monoxide Intoxication

Patients with carbon monoxide (CO) intoxication may show neurological signs such as headache, seizures, extrapyramidal findings, and coma. However, cortical blindness is rare in these cases. This study describes a woman exhibiting confusion and axial rigidity after CO intoxication. Ten days after intoxication, her pupils were isonormocoric and reactive to light. A fundoscopic examination was normal, but visual acuity was light-perception in both eyes. There were diffuse EEG slow waves. Magnetic resonance imaging (MRI) demonstrated bilateral hyperintensity in the basal ganglia. The P100 latencies of visual evoked potentials (VEP) were increased and dispersed. One year later, the patient's visual acuity was almost normal and VEPs showed mild dispersion in P100 latencies. The authors found this case of interest because cortical blindness due to CO intoxication is only rarely seen with a relatively good outcome.     

Visual system function in patients after surgery for intracranial tumors

Visual function was studied in patients after operations for brain tumours. The study group comprised 7 cases. Visual acuity, field of vision by kinetic and static methods and visual evoked potentials were studied before and after operations. The follow up time was from 1 to 51 months, mean 20 months. In all patients decreased visual acuity, visual field defects and VEP abnormalities were found, before operation. The first control examination after operation showed improvement of visual acuity in 2 cases after removal of pituitary tumours, and worsening of vision in all the remaining ones. In two cases of tumours spreading to the basis of the frontal lobe blindness of one eye developed. Static and kinetic perimetry showed in all cases enlarged visual field defects. VEP confirmed that removal of pituitary tumours compressing visual tract can improve vision: P100 amplitudes increased and latencies become shorter. Further VEP improvement occurred even 6-20 months after achieving of good visual acuity. No improvement of vision developed if the visual pathway had been damaged during the operation. CONCLUSIONS: Pituitary tumours can be removed without damage to the surrounding structures and vision can improve after that. Meningiomas and gliomas lying in immediate vicinity of optic nerves and their chiasma or growing out from them are usually large and often their removal is associated with damage to the visual pathway leading to visual field defects to blindness. The assessment of vision should be based on static and kinetic perimetry and visual evoked potentials (VEP) since these methods are mutually complementary and only their comparison provides a full result.     

Visually evoked potentials in respiratory chain disorders

OBJECTIVES: Little is known about the frequency of abnormal visually evoked potentials (VEPs) in patients with respiratory chain disorders (RCDs). We thus wanted to investigate the frequency of abnormal VEPs in RCDs, how often VEPs are abnormal despite normal visual acuity, and which of the VEP variables are most often abnormal. MATERIAL AND METHODS: Reversal checkerboard VEPs of 26 patients with RCDs, aged 32-74 years, were evaluated. RESULTS: VEPs were abnormal in 17 of the 26 cases (65%). The P100 latency was prolonged at least unilaterally in 16 patients. The P100/N145 amplitude was decreased in a single patient. VEPs were abnormal without visual impairment in 9 cases (53%). CONCLUSION: VEPs prove useful to detect clinical or subclinical impairment of the optical tract in patients with RCDs. In the majority of the cases, the P100 latencies are prolonged while the P100/N145 amplitude remains normal.     

Impairment of visual recognition after a traumatic brain injury]

Impaired vision and cerebral blindness were observed in a patient who had suffered brain trauma. One year after the trauma, the impairment was characterized by low visual acuity, visual field restricted to central tunnel vision and impaired recognition of objects, line drawings, colors and faces. Vision improved six years after the brain trauma with more rapid recognition of objects and line drawings, increased visual acuity and broadening of the visual field. Reading was possible at this time. However, prosopagnosia remained very severe and was still the primary complaint of the patient. Improvement of visual function continuing for several years after a brain injury is discussed on the basis of cognitive and neurophysiological knowledge. The place of rehabilitation is discussed. Functional improvement is explained by extrastriate cortical afferences and the cortical network of visual pathways.     

Analysis of SMON at 30 years after its outbreak ending: special reference to those patients with blindness]

We analysed the present state of those SMON patients with severely disturbed visual acuity. In medical screening of SMON Research Group at 1999, 52 out of 1,083 patients and 549 out of 1128 had their histories of complete blindness and complete loss of locomotion ability at the onset of SMON, respectively. Eighty one out of 1,149 patients showed severe visual impairment, including 22 with complete blindness. The number of patients with complete loss of locomotion and that of gait with cane/assistance were 59 and 443, respectively. Then, a large number of SMON patients have been still suffering from sequelae of clioquinol intoxication. Those patients groups with blindness and severe visual acuity yet showed after 30 years more severe disorders in locomotive activity, muscle strength and spasticity of lower extremities, degree of dysesthesia as well as vibratory sensory deficit, and ADL score. Those patients with complete or near complete blindness were profoundly handicapped so that the ratio of patients with a life-long institutionalization was high compared to the groups with less severe visual disturbance. Thus, it seems necessary to make a more extensive and meticulous supports for those SMON patients with blindness as they are in need of more finance and care providers.     

Visual evoked potentials in adrenolukodystrophy: A trial with glycerol trioleate and Lorenzo oil

Adrenoleukodystrophy is an X-linked metabolic disorder with very-long-chain fatty acid (VLCFA) accumulation and multifocal nervous system demyelination, often with early involvement of visual pathways. Dietary therapy with glycerol trioleate and glycerol trieucate (Lorenzo oil) diminishes VLCFA levels. In a study of patients with the adrenomyeloneuropathy phenotype of adrenolukodystrophy, we used pattern-reversal visual evoked potentials to evaluate visual pathways before and after treatment. Of 108 patients tested, all 26 women and 68 of the 82 men had normal potentials at baseline. Seventy patients were retested at 1 year, at which time VLCFA levels were markedly diminished. Of them, the responses in the 10 men who showed abnormalities at baseline remained abnormal; the latencies in 4 men with initially normal responses became abnormal. No patients improved. There were no correlations between disease duration prior to treatment, baseline P100 latencies, VLCFA levels, or the change in P100 latencies and VLCFA levels after dietary treatment for 1 year. Pattern-reversal visual evoked potentials were abnormal in 17% of the men with adrenoleukodystrophy, and there was no evidence that reduction of VLCFA levels improved or retarded visual pathway demyelination.     

Cortical blindness in children: a study of etiology and prognosis

Thirty-four children (20 boys, 14 girls) with congenital and acquired cortical blindness were analyzed for visual outcome in relation to etiology, visual evoked potentials, electroencephalography, and cranial computed tomography. All 7 children with congenital cortical blindness remained blind on subsequent examination. Of the 27 children with acquired blindness, 16 (59%) had poor visual outcome. Poor visual outcome occurred in those with cardiac arrest, hypoxia, status epilepticus, intracranial hemorrhage, cerebral thrombosis, and head trauma. Good visual outcome occurred in children with hypotensive episodes after cardiac surgery. Of the 12 children with recovery of vision, the interval from acute loss of vision to partial or total recovery was 2 weeks to 5 months. Seven children had complete recovery of vision with no residual visual field defect. The majority of children (87%) had focal or multifocal spike-and-waves and slow sharp-wave discharges on electroencephalography. None had photic recruitment response or occipital spike-and-wave discharges. Flash visual evoked potential studies performed during acute episodes of cortical blindness documented 11 with absent response, 10 with bilateral increases in latency, and 6 with normal responses. There was no correlation between normal visual evoked potentials and a good visual outcome. Only 2 of 6 children with normal responses had normal vision. Abnormal or absent responses are more predictive of a poor recovery of vision because only 3 of 21 (14%) had normal vision on subsequent examination. Abnormal electroencephalographic findings with focal or multifocal spike-and-wave discharges or cerebral atrophy on cranial computed tomography are also poor prognostic signs.     

Flash Visual Evoked Potentials of Various Derivations in Dementia of the Alzheimer Type and Vascular Dementia

Background : Recently, evoked potentials have been used for objective monitoring of the cortical function in dementia. The aim of this study was to distinguish dementia of the Alzheimer type (DAT) from vascular dementia (VaD) using flash visual evoked potentials of various derivations.
Methods : A total of 70 patients consulting Tachikawa Medical Center Kashiwazaki Kosei Hospital were divided into four groups, normal adult (29.5 ± 8.5 years, n=16), normal elderly (77.2 ± 4.7 years, n=17), VaD (81.1 ± 8.1 years, n=17) and DAT (77.4 ± 5.6 years, n=20). Red flash stimulation was provided by a pair of goggles. Visual evoked potentials (VEPs) to flash were recorded in accordance with the International 10–20 Electrode System.
Results : The latencies of N130 and P190 of frontal and central derivations were significantly reduced in DAT compared with all other groups (p<0.05). The N130 and P190 latencies were markedly delayed in the normal elderly group compared with the normal adult group (p<0.05). The P3 (P100) latency of occipital derivation was significantly delayed in normal elderly, VaD and DAT groups compared with the normal adult group (p<0.05). In the DAT group, the P190 latency was shortened at central and frontal derivations compared with that at occipital derivation (p<0.05).
Conclusions : The N130 and P190 latencies decreased at frontal and central derivations in DAT. While the reason is still unclear, dysfunction in the central visual system induced by the degeneration of neuronal populations in DAT may cause the apparently reduced latency. Flash VEPs of frontal and central derivations may be useful for the differential diagnosis of DAT from VaD.     

Unexplained bilateral occipital calcification and reduced vision

An eight year-old girl, investigated because of convulsions, was found to have isolated bilateral presumably cortical and subcortical calcification, reduced visual acuity and prolonged visual evoked response latencies. There were no clinical manifestations of a phakomatosis.     

Blind und sehend in einer Person

We present a patient with dissociative identity disorder (DID) who after 15 years of diagnosed cortical blindness gradually regained sight during psychotherapeutic treatment. At first only a few personality states regained vision, whereas others remained blind. This was confirmed by electrophysiological measurement, in which visual evoked potentials (VEP) were absent in the blind personality states but normal and stable in the seeing states. The switch between these states could happen momentarily. As a neural basis of such psychogenic blindness, we assume a top-down modulation of activity in the primary visual pathway, possibly at the level of the thalamus or the primary visual cortex. Therefore VEPs do not allow distinction of psychogenic blindness from organic disruption of the visual pathway. In summary, psychogenic blindness seems to suppress visual information at an early neural stage.     

To read or not to read: a neurophysiological study

Pure alexia (PA) has been associated with visual deficits or a failure to activate the visual word form area (VWFA). We report a patient with pure alexia due to posterior cortical atrophy, in whom event-related potentials revealed a delay in the P100 component and an absent N170 compared with controls. Furthermore, there was a tendency for a larger delay in P100 latencies associated with incorrectly read words. This suggests that some cases of PA might result from deficits in visual perception, signaled by the P100 early potential which could lead to an inability to consistently activate the VWFA, marked by the absent N170.     

A sequential study of visual evoked potential in patients with vitamin B12 deficiency neurological syndrome.

OBJECTIVE: Visual pathways are vulnerable to vitamin B(12) deficiency but there is paucity of studies evaluating visual evoked potential (VEP) changes following vitamin B(12) supplementation. Our aim was to evaluate the visual evoked potential changes in patients with vitamin B(12) deficiency neurological syndrome and their changes after vitamin B(12) therapy. METHODS: Seventeen patients with vitamin B(12) deficiency neurological syndromes diagnosed on the basis of megaloblastic bone marrow or low serum vitamin B(12) level or both were subjected to testing of visual acuity, field of vision, colour vision and neurological examination. Cranial magnetic resonance imaging was done in 9 patients and pattern reversal VEP was carried out on admission. P(100) latency and amplitude were measured. Visual function and VEP studies were repeated at 3 and 6 months after vitamin B(12) therapy. RESULTS: The patients' age ranged between 17 and 69 years; 7 were females and 16 were lactovegetarians. The duration of symptoms ranged between 10 days and 10 years. Visual acuity, colour vision, field of vision and fundus oculi were normal. VEP revealed prolongation of P(100) latency in 10 patients (17 eyes) which was mild in 2, moderate in 10 and marked in 5 eyes. Six months after treatment, P(100) latency improved to normal in all except 4 eyes. VEP abnormality was related to duration of illness and antiparietal cell antibodies. CONCLUSIONS: VEP is frequently prolonged in patients with vitamin B(12) deficiency neurological syndrome although asymptomatic. It usually returns to normal after treatment.     

Visual acuity evaluated by pattern-reversal visual-evoked potential is affected by check size/visual angle

Objective To systemically explore the range of visual angles that affect visual acuity,and to establish the relationship between the P1 component(peak latency～100 ms) of the pattern-reversal visual-evoked potential(PRVEP) and the visual acuity at particular visual angles.Methods Two hundred and ten volunteers were divided into seven groups, according to visual acuity as assessed by the standard logarithmic visual acuity chart(SLD-II).For each group,the PRVEP components were elicited in response to visual angle presentations at 8°,4°,2°,1°/60′30’,15’,and 7.5’,in the whiteblack chess-board reversal mode with a contrast level of 100%at a frequency of 2 Hz.Visual stimuli were presented monocularly, and 200 presentations were averaged for each block of trials.The early and stable component P1 was recorded at the mid-line of the occipital region(Oz) and analyzed with SPSS 13.00.Results(1) Oz had the maximum PI amplitude; there was no significant difference between genders or for interocular comparison in normal controls and subjects with optic myopia.(2) The P1 latency decreased slowly below 30’,then increased rapidly.The P1 amplitude initially increased with check size,and was maximal at～1°and～30’.(3) The PI latency in the group with visual acuity≤0.2 was significantly different at 8°,15’ and 7.5’,while the amplitude differed at all visual angles,compared with the group with normal vision.Differences in P1 for the groups with 0.5 and 0.6 acuity were only present at visual angles <1°.(4) Regression analysis showed that the P1 latency and amplitude were associated with visual acuity over the full range of visual angles. There was a moderate correlation at visual angles <30’.Regression equations were calculated for the P1 components and visual acuity,based on visual angle.Conclusion(1) Visual angle should be taken into consideration when exploring the function of the visual pathway,especially visual acuity.A visual angle -60’ might be appropriate when using PRVEP components to evaluate poor vision and to identify malingerers.(2) Increased P1 amplitude and decreased P1 latency were associated with increasing visual acuity,and the P1 components displayed a linear correlation with visual acuity,especially in the range of optimal visual angles.Visual acuity can be deduced from P1 based on visual angle.     

Sensory and cognitive evoked potentials in a case of congenital hydrocephalus

We studied auditory and visual evoked potentials in D.W., a patient with congenital stenosis of the cerebral aqueduct. Head CT scans revealed marked hydrocephalus with expanded ventricles filling more than 80% of the cranium and compressing brain tissue to less than 1 cm in thickness. Despite the striking neuroanatomical abnormalities, however, the patient functioned well in daily life and was attending a local community college at the time of testing. Evoked potentials provided evidence of preserved sensory processing at cortical levels. Pattern reversal visual evoked potentials had normal latencies and amplitudes. Brain-stem auditory evoked potentials (BAEPs) showed normal wave V latencies. Na and Pa components of middle-latency AEP had normal amplitudes and latencies at the vertex, although amplitudes at lateral electrodes were larger than at the midline. In contrast to the normal sensory responses, long-latency auditory evoked potentials to standard and target tones showed abnormal P3 components. Standard tones (probability 85%), evoked N1 components with normal amplitudes (-3.7 microV) and latencies (103 msec), but also elicited large P3 components (17 microV, latency 305 msec) that were never observed following frequent stimuli in control subjects. Target stimuli (probability 15%) elicited P3s in D.W. and controls, but P3 amplitudes were enhanced in D.W. (to more than 40 microV) and the P3 showed an unusual, frontal distribution. The results are consistent with a subcortical source of the P300. Moreover, they suggest that the substitution of controlled for automatic processes may help high-functioning hydrocephalics compensate for abnormalities in cerebral structure.     

Neurophysiological study in Pelizaeus-Merzbacher disease

The neurophysiological characteristics of Pelizaeus-Marzbacher disease (PMD) were studied in four Japanese patients aged between 5 and 13 years. Pendular spontaneous nystagmus was always recorded with a frequency between 2.5 and 4 Hz, and abnormal saccades with an almost twofold prolongation in onset time and 50% decrease in velocity were noted. Brainstem auditory evoked potentials consistently demonstrated severely altered waves II to V, following a normal wave I, despite normal hearing acuity. Somatosensory evoked potentials (SEPs) were always absent between brainstem components and early cortical responses. Late cortical components of SEPs and visual evoked potentials with significantly prolonged latencies were recorded in the three younger cases having normal sensory and visual acuity (N35 of SEP, 73.1 ± 2.1 ms; N75 of VEP, 129.0 ± 12.7 ms; mean ± S.D.), while these peaks were absent in the oldest case having the most severe handicap. In motor evoked potentials (MEPs), R1 of blink reflex with significantly prolonged latency (14.9 ± 1.48 ms) was always obtained, and no subsequent R2 was elicited. Magnetic transcortical stimulation elicited no MEPs of the thenar even in the facilitating condition on voluntary contraction despite mild weakness of the thenar, while normal MEPs were always elicited on cervical stimulation. These electrophysiological findings were consistent with extensive conduction slowing involving the brainstem to the cerebrum, which seemed to be accompanied by conduction block in motor systems rather than sensory systems. Although each of the results was not specific, in combination they suggested the characteristics of diffuse brain dysmyelination in PMD.     

Pattern-reversal visual evoked potentials in patients with newly diagnosed epilepsy

PURPOSE: The possible occurrence of evoked potential (EP) abnormalities in patients with newly diagnosed epilepsy has been little investigated. The main purpose of the present study was to investigate possible changes in pattern-reversal visual evoked potential (P-VEP) responses in newly diagnosed epilepsy patients. METHODS: By using P-VEPs, latency values of the N75 and P100 together with amplitude values of P100 were recorded in newly diagnosed idiopathic epilepsy patients. The patients comprised two groups; nonphotosensitive (non-PS), and photosensitive (PS) patients. RESULTS: Shortened N75 and normal P100 latencies of the P-VEP with higher than normal P100 amplitudes were detected in PS patients. In non-PS patients, N75 latencies of the P-VEPs were unaffected; however, P100 latencies were prolonged, and P100 amplitudes were unchanged. CONCLUSIONS: P-VEPs are different from those of controls in previously untreated idiopathic epilepsy patients. Results also indicate different P-VEP features in patients with and without photoparoxysmal responses. The changes might be the result of a disorder of one or more neurotransmitters or subtle morphologic damage such as microdysgenesis.     

Pattern reversal visual evoked potentials as a measure of visual pathway pathology in multiple sclerosis

BACKGROUND: Pattern reversal visual evoked potentials (PRVEPs) have a well-documented role in diagnosis of multiple sclerosis (MS), but their value as a visual function surrogate remains controversial. METHODS: We evaluated PRVEP in 37 patients with MS who were participating in a long-term follow-up study following a phase III trial of interferon beta-1a (Avonex). Patients were examined to determine the Kurtzke Extended Disability Status Score (EDSS), multiple sclerosis functional composite (MSFC), contrast letter acuity (CLA), and had cranial MRI scans to determine whole brain atrophy (BPF). PRVEP was evaluated for P100 latency, amplitude, and waveform morphology. Two summary scores were created: for Score A, abnormal latencies, morphologies, and amplitudes of each individual eye were added; for Score B, abnormal latencies, morphologies, and amplitude ratio between eyes was determined. Sixteen patients in this group also had PRVEP at the time they enrolled in the clinical trial, eight years previously. RESULTS: At the follow-up exam, over 75% of patients had abnormal PVEP parameters while visual acuity (VA) was abnormal only in 59%. Increased PRVEP latency over the eight-year period correlated with deterioration assessed by EDSS (P = 0.006), BPF (P = 0.0001), and MSFC (P = 0.0041). Score A was significantly correlated with EDSS, BPF, CLA, cognitive function, and quality of life assessed with the Sickness Impact profile. No correlation was seen with the MSFC. CONCLUSIONS: The results indicate that PRVEP measures MS-related pathology, and can provide not only diagnostic but also prognostic information during evaluation of MS patients.     

Plasticity of monocular and binocular vision following cerebral blindness: evoked potential evidence

Using monocular and dynamic random dot correlogram (DRDC) stimuli, sequential visual evoked potentials changes were demonstrated in 2 patients following cerebral blindness. The recovery of binocular vision was delayed in comparison to the recovery of monocular vision. The results are not due to simple acuity impairment or convergence deficiency, and thus provide evidence for the vulnerability of postsynaptic cortical mechanisms of human binocular vision.     

Ictal Cortical Blindness with Permanent Visual Loss

Cortical blindness is rarely an ictal manifestation. We report the case of a man who developed transient cortical blindness followed by permanent visual deficits during repeated partial seizures. Intermittent visual impairment began at age 14 years. After he had the first generalized seizure at age 28 years, neurologic, ophthalmologic, angiographic, and brain computed tomographic (CT) examinations were normal. Several EEGs showed almost continuous biposterior spike-waves. Over the next several years, frequent partial seizures were associated with transient visual loss and left body twitching or paresthesias. When he was 32, transient blindness occurred during several days of repeated occipital seizures. Permanent left homonymous hemianopia, right homonymous central scotoma, dyschromatopsia, and altered stereopsis followed these seizures. Brain CT demonstrated a new right occipital lesion. Partial seizures arising posteriorly may cause transient cortical blindness and result in permanent visual deficits.     

Migraine patients exhibit abnormalities in the visual evoked potential.

An experiment is described which compared visual evoked potential (VEP) amplitudes and latencies in normal subjects and migraine patients. Several VEP abnormalities were found: at vertex and to a lesser extent at temporal sites, P100-N120 amplitudes were larger in patients; at vertex and temporal sites N120 amplitudes were larger in patients; at temporal sites patents had larger N120-P200 amplitudes but smaller P100 amplitudes. Peak latencies of the VEP were also found to be abnormal in patients. At vertex, patients had delayed N120 peak latencies while at temporal sites delays were found in the P200 latencies. Patients were subdivided according to side of headache. Right-sided headache patients showed larger temporal P100 amplitudes and larger left temporal P100-N120 amplitudes than bilateral headache patients.