DO VISUAL EVOKED POTENTIALS DETECT NEURAL DAMAGE IN CHILDREN TREATED FOR CANCER?

Abnormal visual evoked potentials (VEPs) have been reported in children treated for acute lymphoblastic leukaemia (ALL), which suggests that VEPs may be useful in screening for toxicity. The authors investigated this by recording flash and pattern VEPs in a control group of 34 siblings of patients, a group of six children studied longitudinally during the early stages of treatment for ALL, and three other follow-up groups. In only three follow-up patients were VEP results outside the normal range and the six ALL patients did not develop new abnormalities during early treatment. Although differences were detected between the groups, there was no evidence of VEPs being a useful means of monitoring the treatment of individual patients.     

Serial pattern evoked potential recording in a case of toxic optic neuropathy due to ethambutol

Visual evoked potentials (VEPs) were studied in a patient who developed visual impairment during ethambutol treatment. The ERG and the flash VEP were normal at the time of maximal visual loss, whereas pattern reversal VEPs 2 and 5 months after onset revealed evidence of severe bilateral optic nerve involvement, especially affecting macular fibres. Seven months after onset paramacular PNP complexes with a late positivity (scotomatous response) were recorded after pattern reversal and half-field stimulation, suggesting involvement of fibres subserving central vision. At the time when visual acuity was normal there was still electrophysiological evidence of a mild involvement of the anterior visual pathway. The papillomacular bundle seems to be especially involved in ethambutol eye toxicity.     

Early diagnosis of optic glioma in children with neurofibromatosis type 1

Twenty-five asymptomatic patients with neurofibromatosis type 1 (NF 1), aged 6–21 years, underwent the following examinations: intracranial magnetic resonance testing (MRI), visual acuity testing, ophthalmoscopy, and visual field and pattern reversal visual evoked potentials (VEPs). MRI showed enlargement of one or both optic nerves in six children, with bilateral involvement in three. VEPs were normal in all these patients; two of them had abnormalities on other visual examinations, although there were no subjective visual disturbances. These results show that VEPs cannot be considered as a screening test for optic pathway lesions in children with NF 1, as previously stated, and that other types of visual function examination may be more sensitive. These data may contribute to the establishment of more precise guidelines for the evaluation and treatment of children with NF 1.     

A study of the relationship between neurological function and serum vitamin E concentrations in patients with cystic fibrosis.

A patient with cystic fibrosis and undetectable serum vitamin E concentrations is described who developed a progressive spinocerebellar syndrome and pigmentary retinopathy with abnormal somatosensory and visual evoked potentials (SSEPs and VEPs). In order to assess the relationship between neurological function and serum vitamin E concentrations in cystic fibrosis, 29 unselected patients who had no neurological symptoms were examined neurologically. Ten were randomly selected for neurophysiological assessment by recording SSEPs and VEPs. Electroretinograms (ERGs) were also performed in five cases. The findings were correlated with serum vitamin E concentrations which were unknown to the neurological investigators prior to completion of the study. Only one patient had definite reflex and sensory abnormalities, and the remaining 28 were clinically normal. The ERG was abnormal in two cases, one of whom had abnormal VEPs. SSEPs were normal in all 10 cases. Twenty six patients had serum vitamin E concentrations below the normal range. In two of the three patients who had definite neurological or electrophysiological abnormalities serum vitamin E concentrations were below the median value for the whole group.     

Clinical utility of electrophysiological evaluation in Crigler-Najjar syndrome

We evaluated the neurological and neurophysiological features in ten patients with genetically characterized Crigler-Najjar (CN) syndrome: four with typical type I CN had undergone orthotopic liver transplantation (OLT); six had type II CN, and three of them developed severe hyperbilirubinemia with a limited response to phenobarbital leading to an intermediate phenotype I/II. Clinical neurological and multimodal electrophysiological evaluations [electroencephalogram (EEG), visual (VEPs), motor (MEPs) and brainstem auditory (BAEPs) evoked potentials] were performed. Neurological examinations showed mild hand tremor in four patients (one pre-OLT and one post-OLT type I, two type I/II). EEG revealed high voltage paroxysmal discharges in four patients (three type I/II, and one type I with a marked improvement after OLT). VEPs showed P100 wave increased latency in five patients (three type I, and two type I/II considered for OLT evaluation). MEPs showed prolonged central motor conduction time in five patients (two type I; one type I/II; two type II). Only EEG and VEPs findings showed a correlation with high bilirubin levels. BAEPs were normal. In conclusion, VEPs and EEG contribute to identify and monitor bilirubin neurotoxic effects, and may play a decisional role in some cases of severe hyperbilirubinemia without overt neurologic damage.     

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.     

Cytidine-5'-diphosphocholine (Citicoline): a pilot study in patients with non-arteritic ischaemic optic neuropathy

Background and purpose:  Our work evaluates visual function before and after treatment with cytidine-5-diphosphocholine (Citicoline) in patients with non-arteritic ischaemic optic neuropathy (NION).
Methods:  Twenty-six patients in which at least 6 months elapsed from NION, were randomly divided into two age-similar groups: 14 patients had Citicoline (Cebrolux-Tubilux, Italy, 1600 mg/diem for 60 days, followed by a 120-day period of wash out, days 60–180) (T-NION); 12 patients had no treatment during the same period (NT-NION). At day 180, in T-NION a second period of treatment (days 181–240) followed by a wash-out (days 241–360) was performed. Fourteen age-matched healthy subjects provided normative data. In all patients, pattern-electroretinogram (PERG), visual evoked potentials (VEPs) and visual acuity (VA) measurements were performed at baseline and at days 60 and 180. In T-NION, further measurements were achieved at days 240 and 360.
Results:  At baseline, NT-NION and T-NION patients showed abnormal PERGs and VEPs, and reduced VA, compared to controls. At the end of treatment (days 60 and 240), T-NION patients showed improvement ( P  < 0.01) of PERGs, VEPs parameters and VA, compared to pre-treatment values. After wash out, functional improvements persisted compared to baseline. No changes in NT-NION patients were observed.
Conclusions:  Our results suggest a beneficial effect of oral Citicoline in NION.     

Visual impairment in children with epilepsy treated with vigabatrin

Vigabatrin is an anti-epileptic drug particularly useful for drug-resistant partial seizures and infantile spasms. Recently, vigabatrin-induced visual field constriction (VFC) and abnormal ocular electrophysiological studies were reported. In this study, we assessed visual fields, visual evoked potentials (VEPs), and electroretinography (ERG) in children treated with vigabatrin. Twenty-four visually asymptomatic children underwent a clinical ophthalmological examination, perimetry when appropriate, and VEP and ERG. Thirteen patients had at least one abnormal study. VFC was seen in 11 of 17 patients who had perimetry; 5 of 15 patients who underwent VEP testing and 4 of 11 who underwent ERG testing had abnormal examinations. For the most part, abnormal VEPs and ERGs were found in children who also had VFC. There was a consistent trend for longer treatment periods to correlate with VFC, abnormal ERGs, and VEPs. In summary, over half of the children treated with vigabatrin demonstrated VFC or abnormal ocular electrophysiological studies. Perimetry seemed to be the most sensitive modality for identifying vigabatrin toxicity. Abnormal ERGs and VEPs were primarily seen in children with VFC and may be useful in monitoring children who are not appropriate candidates for perimetry. Although the incidence of vigabatrin-induced VFC is worrisome, in the context of intractable seizures or infantile spasms, therapeutic benefits must be weighed against risks.     

Subclinical VEP abnormalities in patients on chronic deferoxamine therapy: longitudinal studies

As deferoxamine (DFO) appeared to have certain toxic effects on the sensory pathways in some of our patients on nightly subcutaneous deferoxamine (DFO) for transfusion-dependent anemia, treatment was stopped in all of these patients to obtain a comprehensive baseline assessment of sensory function. Visual evoked potentials (VEPs) were studied in all patients; the 77 described in this report all had normal ophthalmological examinations. Abnormally prolonged VEP latencies were found in 21%. The patients remained off DFO for 2-6 months, and most of those with abnormal VEPs who were retested showed improvement in their VEPs over this period with the VEPs returning to within normal range in half the cases; two showed no change. Since restarting DFO, VEP latencies in 10 of these patients have increased again beyond normal limits, as have the VEPs in 7 who had previously normal VEPs. Although most of the 77 patients have VEPs that are currently normal and stable while on DFO, a significant sub-group have abnormal VEPs that appear sensitive to the administration of DFO and may reflect a vulnerability to DFO neurotoxicity. These data suggest that the VEPs can detect subclinical toxic effects of DFO on the visual system and should be considered as a monitor for patients receiving chronic DFO therapy.     

Do visual evoked potentials give relevant information to the neuro-ophthalmological examination in optic nerve lesions?

The visual evoked potentials (VEPs) and neuro-ophthalmological examinations of 134 patients were compared. The VEPs were abnormal in 95 % of the eyes with optic neuritis. Defective color vision was found in 99 %, visual field defects in 88 %, decreased vision in 66 % and an afferent pupillary defect in 55 %. 29 patients with optic neuritis were followed up with repeated tests. VEPs and color vision recovered more slowly than visual acuity and visual field.
Abnormal VEPs were observed in 68 % of 50 MS patients. An analysis of symptomatic and asymptomatic eyes showed that testing of color vision, visual field and red-free ophthalmoscopy were equally as useful diagnostic tools as VEPs. 4 (8 %) of the MS patients had abnormal VEPs despite a normal neuro-ophthalmological examination; 94 % of MS patients with symptoms and 47 % of MS patients without visual symptoms had abnormal VEPs.
VEPs were pathological in 59 % of 24 patients with traumatic or compressive optic nerve diseases or optic atrophies of unknown etiology. The neuro-ophthalmological examination was more sensitive than VEPs in the diagnosis of these disorders. A neuro-ophthalmological examination is in most cases sufficient to diagnose optic nerve lesions. VEPs are of diagnostic aid especially in mild optic nerve lesions.     

Symptomatic retrochiasmal lesions in multiple sclerosis: clinical features, visual evoked potentials, and magnetic resonance imaging.

We have studied 18 patients with relapsing-remitting multiple sclerosis (MS) who had symptomatic visual field defects due to retrochiasmal lesions. In 17, the lesion responsible was identified by magnetic resonance imaging (MRI), computed x-ray tomography (CT), or both. The lesion responsible involved the posterior optic radiations in eight cases, the optic tract and lateral geniculate nucleus in six, and the posterior limb of the internal capsule in three. The prognosis for recovery of the field defect was good; complete recovery occurred in 14 patients, and only two showed no recovery at all. The striking characteristic of the lesions was that most were unusually large; indeed, many were detectable on CT as well as MRI. Half-field asymmetries of either amplitude or latency of the visual evoked potentials (VEPs), consistent with a postchiasmal lesion, were present in only five out of 13 patients acutely. In only three of these did the abnormality persist at follow-up. We conclude that only large postchiasmal lesions are likely to cause symptomatic homonymous field defects in MS, usually characterized by rapid recovery. Hemifield VEPs have a low sensitivity for the detection of postchiasmal as compared with prechiasmal abnormalities.     

Early visual processing in neglect patients: A study with steady-state VEPs

Reliable steady-state visual evoked potentials (VEPs) were recorded in a group of 19 right brain-damaged patients with visuospatial hemineglect (Neglect), and two control groups: 15 left brain-damaged (LBD) patients and 12 right brain-damaged (RBD) patients without neglect. Moreover, VEPs were recorded in two rare cases of left brain damage and right visuospatial hemineglect. Stimuli were gratings phase-reversed at various temporal frequencies presented in the left and right visual field. In the Neglect group, VEPs to stimuli displayed in the left visual field (contralesional stimuli) had longer latencies. The delay was not present for the two control groups. As regards the VEP amplitudes, the Neglect group data showed a less distinctive pattern than in the case of latency. VEPs to stimuli contralateral to the lesion were smaller than those recorded for stimuli ipsilateral to the lesion in both Neglect and RBD groups. On the contrary, the VEP amplitudes for the two hemifields were comparable in the LBD group. In the case of left brain damage and neglect, VEPs to right visual field stimuli had longer latencies and lower amplitudes compared to the ipsilesional responses in both patients. Overall, the data support the view that, in most cases, early visual processing is not intact in the neglected hemifield.     

Clinical relevance of phase of steady-state VEPs to P100 latency of transient VEPs.

Pattern visual evoked potentials (VEPs) to transient and steady-state stimulation were recorded in 10 normal subjects at 4 levels of luminance (180, 57, 22 and 11 cd/m2). VEPs were also recorded in 5 patients with optic neuropathy at a fixed luminance (180 cd/m2). The relationship between P100 latency of transient VEPs (T-VEPs) and the phase of steady-state VEPs (S-VEPs) was analyzed. As luminance decreased in normal subjects, P100 latency was prolonged and the phase lag increased. A significant linear relationship between the P100 latency and phase was found. Patients showed both the prolonged P100 latency and the delayed phase. The simple linear regression line of the phase-P100 latency function of normal subjects closely matched the patients' values. These results suggest that changes in the phase may be equivalent to changes in the P100 latency. S-VEPs, therefore, may be clinically useful in assessing visual function.     

Ethambutol neurophathy: clinical and electroneuromyographic studies

Clinical features including changes in the peripheral nerve conduction were analzyed in 10 cases with ethambutol neuropathy. There were abnormalities in the visual field in seven and optic atrophy in five of 10 cases. Seven of 10 cases complained of numbness in the lower limbs. The age of onset and dose of ethambutol the patients continue to take after the occurrence of visual impairment were found to be important in determining the severity of neurological symptoms. A functional disturbance was more conspicuous in ethambutol neuropathy, particularly in the sensory system than in the motor system so far as the peripheral nerve conduction was serially examined. Some cases still had serious optic disturbances even about seven years after the onset of the disease and the presence of irreversible lesions was suspected.     

Central effects of drugs used in migraine prophylaxis evaluated by visual evoked potentials

The present study used recordings of visual potentials evoked by pattern reversal (VEPs) to investigate the central effects of three drugs used in migraine prophylaxis: the calcium channel blocker nifedipine, the beta-1-selective blocker metoprolol, and the nonselective beta adrenoreceptor blocker propranolol. The study involved 58 patients with common or classical migraine who were treated in a double-blind randomized study over a period of 7 months, while the effectiveness of prophylactic treatment was recorded in headache diaries that were subjected to time series analysis. VEPs were recorded at the beginning of a 2-month baseline period without treatment, after 4 months of treatment, and at the end of a 3-month washout period. At baseline, migraine patients had significantly higher VEP amplitudes and longer latencies than did a group of 87 healthy control subjects. Patients were separated by statistical analysis into responders and nonresponders to each prophylactic treatment. Nifedipine had no effects on the frequency, intensity, and duration of migraine attacks, nor on amplitude and latency of the VEPs. In contrast, the use of beta blockers resulted in a significant decrease in VEP amplitude, both in responders and nonresponders, whereas VEP latency remained unchanged. VEP amplitudes returned to the initial values at follow-up in the nonresponders, but stayed at lower levels in responders. Beta blockers thus appear to have a significant effect on the increased excitability of the visual system in patients with migraine, although their action is not directly related to their reduction of migraine frequency.     

Ethambutol Neuropathy: Clinical and Electroneuromyographic Studies

Abstract: Clinical features including changes in the peripheral nerve conduction were analyzed in 10 cases with ethambutol neuropathy. There were abnormalities in the visual field in seven and optic atrophy in five of 10 cases. Seven of 10 cases complained of numbness in the lower limbs. The age of onset and dose of ethambutol the patients continue to take after the occurrence of visual impairment were found to be important in determining the severity of neurological symptoms. A functional disturbance was more conspicuous in ethambutol neuropathy, particularly in the sensory system than in the motor system so far as the peripheral nerve conduction was serially examined. Some cases still had serious optic disturbances even about seven years after the onset of the disease and the presence of irreversible lesions was suspected.     

Cortical blindness and residual vision: is the "second" visual system in humans capable of more than rudimentary visual perception?

We studied 12 patients with static cortical blindness to evaluate residual vision after destruction of area 17 and to assess the visual capacity of the subcortical "second" visual system in humans. In each case, the cause was bilateral infarction of the occipital lobes. Five patients had total blindness, and four had residual rudimentary vision (RRV), characterized by homonymous areas of light perception in the peripheral field and ability to detect moving objects. Only three patients had the ability to read; two of these had spared macular vision, and the other had spared left homonymous hemimaculae and spared temporal crescent. Neuroimaging and visual evoked potentials (VEPs) correlated with the extent of the visual dysfunction. Total destruction of area 17 bilaterally was associated with total permanent visual loss. The larger the amount of spared visual cortex, the better the vision. Positron emission tomography (PET) or single photon emission computed tomography (SPECT) demonstrated retained metabolic activity in islands of preserved area 17 in patients with some residual vision. VEPs were present in totally blind individuals. We conclude that, in humans, useful visual function is preserved only when a critical amount of area 17 is spared. The subcortical second system may participate in the generation of VEPs, but is incapable of conscious visual perception.     

Color Responses of Flash Visual Evoked Potentials in Normal Adults, Normal Elderly Subjects and Dementia of the Alzheimer Type Using Light Emitting Diodes

Background : Since initially we could only employ the red light goggle-shaped stimuli equipment with flash light emitting diodes (LEDs), we had little information concerning different color responses of visual evoked potentials (VEPs). On our request, Nihon Kohden (Tokyo, Japan) developed green light goggle-shaped stimuli equipment with LEDs in 1999.
The purpose of this study was to determine the effects of green and red flash VEPs in normal adults, normal elderly subjects and patients with dementia of the Alzheimer type (DAT).
Methods : Subjects consisted of a normal adult group (n=30), normal elderly group (n=10), and DAT patients (n=7). Transient VEPs of 1 Hz were measured. The recording electrodes were placed at midline-occipital, left-occipital and right-occipital sites in accordance with the ten-twenty electrode system.
Results : In the normal adult group, peak latencies of green flash VEPs increased significantly compared to those of red flash VEPs (N2, P3: p<0.01, N3: p<0.05). The N2 and P3 latencies of both red and green flash VEPs were delayed in the normal elderly and DAT groups compared with those in the normal adult group. The delayed latencies of red flash VEPs were greater than those of green flash VEPs.
Conclusion : The difference in latency between green and red flash VEPs corresponds to the difference in the conducting time from three rods (each spectrum peak is 419–420 nm, 530–534 nm, and 558–564 nm) to the visual central system. Each channel from the three rods to the visual central system may have an individual speed reaction, individual ageing and pathological changes. The results indicate that the peak latency of red light reveal elderly and pathological change more easily than that of green light.     

Prognostic value of VEPs in young children with acute onset of cortical blindness

Visual evoked potentials (VEPs) were recorded in 32 children (ages 4 months to 5 years) who were clinically diagnosed as being cortically blind. None of the children had visual or neurologic abnormalities prior to the precipitating insult which included surgery (N = 15), trauma (N = 3), infectious disease (N = 5), hypoxia (N = 3), and other causes (N = 6). VEPs were recorded during the acute stage of cortical blindness in all children and were repeated in 24 of them. Either flash or pattern stimulation was used, depending upon the age and visual status of the child. All but one of the children who had normal flash VEPs while cortically blind, recovered normal visual function. All patients with abnormal VEPs had permanent visual impairment or blindness and all but one of those with absent VEPs remained blind. The recovery period was highly variable, ranging from 5 days to 3 years. Thus, flash VEPs recorded during the period of blindness were useful in predicting visual outcome, regardless of etiology. Repeat studies using pattern VEPs were valuable in monitoring recovery in many of these patients.     

Perimetry, visual evoked potentials and visual evoked spectrum array in homonymous hemianopsia

Confrontation field examination, perimetry, visual evoked potentials (VEPs) to pattern hemi-field stimulation and visual evoked spectrum array to flash stimulation were compared in 50 patients with homonymous hemianopsia. Visual field could be examined by confrontation in all patients and demonstrated the field defect in 96% of cases. Goldmann perimetry could only be performed in 60% of patients, but always quantitatively defined the margins of the defect. VEPs could only be tested in 77% of patients and demonstrated an abnormality in 79% of cases. VEPs were absent to stimulation of the affected hemifield in every case of homonymous field defects with macular splitting. Visual evoked spectrum array could be tested in 95% of patients and revealed abnormalities in 67% of cases. The relative value of each test is discussed.