Panuveitis caused by Borrelia burgdorferi sensu lato infection.

A 13-year-old boy who presented with a red left eye, painful eye movement, blurred vision, photophobia and increased lacrimation, was diagnosed with 1-sided panuveitis with optic disk edema. Diagnostic work-up revealed borrelial antibodies in serum. Diagnosis of Lyme borreliosis was substantiated by demonstration of lymphocytic pleocytosis, intrathecal borrelial antibody synthesis, improvement after treatment with ceftriaxone and exclusion of other causes.

Chromatic visual evoked potentials in young patients with demyelinating disease.

The purpose of this study was to evaluate color vision in young patients with demyelinating disease both clinically and electrophysiologically. Thirty young patients (8-28 years, mean age 19 years) with demyelinating disease with or without a history of optic neuritis (ON) were investigated. Color vision was evaluated clinically with the Ishihara test and the Farnsworth-Munsell 100 hue (FM 100 hue) test and electrophysiologically with chromatic visual evoked potentials (cVEPs). Color deficiency axis and error score (ES) obtained with the FM 100 hue test were analyzed. cVEPs to isoluminant red-green (R-G) and blue-yellow (B-Y) stimuli were recorded. The stimulus was a 7 deg circle composed of horizontal sinusoidal gratings with a spatial frequency of 2 cycles/deg and 90% chromatic contrast. Onset-offset mode of stimulation (ON:OFF=300∶700 ms) was used. Since the majority of the patients were adults (>18 years), the negative wave (N wave) of the cVEP respones is the prominent part and therefore was analyzed. Sixty eyes were studied-22 with at least one episode of ON (ON group) and 38 without any clinically evident episode of ON (nON group). The average ES in the ON group was 179.18±171.8, whereas in the nON group it was 87.60±65.34. The average N-wave latency in the ON group was 144±44 ms for the R-G stimulus and 146±56 ms for the B-Y stimulus, whereas in the nON group, it was 117±13 ms for the R-G stimulus and 121±22 ms for the B-Y one. The average N-wave amplitude in the ON group was 9.3±7.1 µV for the R-G stimulus and 5.1±3.9 µV for the B-Y one, whereas in the nON group, it was 10.8±8.3 µV for the R-G stimulus and 6.4±4.3 µV for the B-Y one. A significant difference between the ON and the nON group was found: in the ON group, ES was higher (p=0.01) and N-wave latency was longer (p=0.01) compared with those in the nON group. The study showed that color vision is expectedly more affected in the ON group, but also often in the nON group, which may indicate increased parvocellular visual pathway vulnerability in demyelinating diseases.

Chromatic visual evoked potentials in paediatric population.

BACKGROUND: The purpose of this study was to investigate chromatic visual evoked potential (cVEP) response characteristics during the first year of life and to collect as large database of healthy baby responses as possible. This study also complements our previous studies on cVEP in schoolchildren and preschool children. METHODS: Forty-four healthy babies aged 3-12 months were binocularly tested. cVEP were recorded to isoluminant red-green (R-G) and blue-yellow (B-Y) stimuli. The stimulus represented a circle composed of horizontal sinusoidal gratings with 90 % chromatic contrast and spatial frequency of 2 cycles/deg. Two stimulus sizes (7° and 21°) and onset-offset mode of stimulation (On-300 ms, Off-700 ms) were used. cVEP were recorded from Oz (mid-occipital) position with the reference at Fz. Waveform characteristics and its changes throughout the first year of life were studied. RESULTS: Chromatic visual evoked potential responses were reliably recorded in all but two youngest babies. Characteristic cVEP response consisted of negative-positive-negative complex, positive (P) wave being far more prominent than both negative waves (N1 and N2). cVEP response to larger stimulus size (21°) showed shorter latency and higher amplitude to both (R-G) and (B-Y) stimuli compared to smaller stimulus size (7°). The same was true when comparing R-G versus B-Y stimulus: R-G responses showed higher amplitude and shorter latency than B-Y response, for both stimulus sizes. P wave latency shortened with increasing age throughout the first year of life, both for R-G (R (2) = 0.59) and B-Y (R (2) = 0.41) 21° stimulation. P wave amplitude did not show significant changes throughout the first year of life. CONCLUSIONS: Chromatic visual evoked potential can be reliably recorded after the age of 3 months and show significant maturational changes throughout the first year of life.

Early onset West syndrome with severe hypomyelination and coloboma-like optic discs in a girl with SPTAN1 mutation.

Recent study has shown that mutations in the alpha-II-spectrin (SPTAN1) gene cause early onset intractable seizures, severe developmental delay, diffuse hypomyelination, and widespread brain atrophy. We report a Slovene girl with hypotonia, lack of visual attention, early onset epileptic encephalopathy, and severe developmental delay. The patient presented with segmental myoclonic jerks at the age of 6 weeks, and infantile spasms at the age of 3.5 months. Her seizures were resistant to treatment. Multiple electroencephalography recordings showed deterioration of the background activity, followed by multifocal abnormalities before progressing to hypsarrhythmia. Ophthalmologic examination revealed bilateral dysplastic, coloboma-like optic discs. Brain magnetic resonance imaging showed diffusely reduced white matter and brainstem volumes with hypomyelination. A de novo heterozygous in-frame deletion was detected in SPTAN1: c.6619_6621delGAG (p.E2270del). This report supports the causative relationship between SPTAN1 mutations and early onset intractable seizures with severe hypomyelination and widespread brain volume reduction. Coloboma-like optic discs might be an additional feature observed in patients with SPTAN1 mutations.

Chromatic visual evoked potential responses in preschool children.

The purpose of the study was to analyze chromatic visual evoked potential (VEP) responses to isoluminant red-green (R-G) and blue-yellow (B-Y) stimuli in 30 preschool children (1.5-6 years). The predominant part of the response consisted of a positive (P) wave, which showed age-related latency changes (linear decrease). P wave latency was shorter when using 21° compared to 7° R-G (p=0.004) and B-Y (p=0.044) stimulus and also when using 21° R-G compared to 21° B-Y stimulus (P=0.000). P wave amplitude did not show age-related changes. However, a lower amplitude was recorded when using 7° R-G stimulus (p=0.0013) and also when using B-Y compared to R-G stimulus. We may conclude that chromatic VEP to R-G and B-Y stimuli is reliably recorded in preschool children and that P wave to R-G stimulation shows a higher amplitude and shorter latency than to B-Y stimulus.

Visual evoked potentials to red-green stimulation in schoolchildren.

The aim is to study chromatic visual evoked potentials (VEP) to isoluminant red-green (R-G) stimulus in schoolchildren. Sixty children (7-19 years) with normal color vision were examined, 30 binocularly and 30 monocularly. The isoluminant point was determined for each child subjectively by using heterochromatic flicker photometry, and objectively from recordings. The stimulus was a 7 degrees circle composed of horizontal sinusoidal gratings, with spatial frequency 2 cycles/degrees and 90% contrast, presented in onset-offset mode. VEP were recorded from Oz (mid-occipital) position. Age-dependent waveform changes and changes of the positive and negative wave were studied to both binocular and monocular R-G stimulation. Age-dependent waveform changes were observed to binocular and monocular R-G stimulation. In younger children the positive wave was prominent, whereas in older children also the negative wave became more evident. The latency of the positive wave decreased linearly with age to R-G binocular stimulation. To monocular stimulation no significant changes of the latency were observed. The amplitude of the positive wave dropped exponentially with age to binocular and monocular stimulation. The latency of the negative wave increased linearly with age to binocular and monocular stimulation, whereas the amplitude did not show age-dependent changes. These findings suggest that the chromatic VEP response undergoes evident age-dependent changes during the school-age period.