ABSTRACT
@#Subacute sclerosing panencephalitis (SSPE) usually presents insidiously. Acute presentations with a fulminant course can occur. However hyper acute presentations with a non-progressive course have not been reported. Here we describe two cases that had hyper acute presentation with occipital involvement misdiagnosed initially as ischemic infarct. One case remained stable for 4 years and then had a fulminant course and patient succumbed; the other patient continued to remain stable at 2 year follow up. In countries where SSPE is prevalent, it is important to be aware of such a variant of SSPE.
ABSTRACT
Purpose: A pilot study was done to evaluate knowledge regarding 揷ortical visual impairment (CVI) in children� among ophthalmologists. Methods: This study was conducted during the annual conference of a zonal ophthalmological society. All ophthalmologists who attended the conference were requested to participate in this study. Those who agreed were given a validated questionnaire to assess knowledge regarding CVI. Cronbach's alpha of the questionnaire was 0.6. Participants were asked to respond to multiple choice questions by choosing the single best option. The responses obtained were then evaluated. Results: The total number of registered delegates in the conference was 448. A total of 103 ophthalmologists showed interest to participate in the study with a response rate of 22.9%. Only 89/103 interested delegates were included in the study as remaining were unaware of CVI. No participant gave correct answers to all questions. Although more than 80% of them knew the most common association (87%) and site of pathology (84%), only 52% were sure about clinical features and even lesser respondents (39%) knew that magnetic resonance imaging is the correct investigation of choice. The majority responded correctly that these children need eye examination (89%) and can be managed by rehabilitation through multidisciplinary approach (82%), but only 58% could recognize differential diagnoses and had a correct idea regarding the prognosis of CVI. There was no correlation between the number of patients diagnosed per month by the respondent with knowledge of the disease. Conclusion: In this pilot study, ophthalmologists were found to have limited knowledge regarding clinical features, investigation, differential diagnosis, and visual prognosis of CVI in children. There is a need to improve awareness regarding CVI among ophthalmologists.
ABSTRACT
Increasing rates of preterm births coupled with better survival of these infants have resulted in higher prevalence of systemic and ocular complications associated with prematurity. In addition to retinopathy of prematurity, infants who are born preterm may suffer from severe visual impairment as a result of hypoxic ischemic encephalopathy, hypoglycemia, and other metabolic imbalances. The effect of these processes on the anterior visual pathway may result in optic atrophy, optic nerve hypoplasia or optic disc cupping and affection of the posterior visual pathway leads to cortical visual impairment (CVI). Other ocular associations include strabismus, nystagmus, and ocular motor abnormalities such as tonic down gaze and defective saccades and pursuits. Cortical and subcortical involvement also manifests as defects in functional vision and these have not yet been completely understood. Children with CVI may have visual field defects, photophobia, defective visual processing, and deficient color vision. Since most of these children also suffer from additional systemic disabilities, evaluation, and management remains a challenge. However, early diagnosis and initiation of rehabilitation therapy can prove to be of significant benefit in these children.
ABSTRACT
Background and Aim: Children with periventricular leucomalacia (PVL) are known to have visual impairment of various forms starting from reduced vision, field defects, congnitive problems, and problems with hand eye coordination. There is very scant data/literature on the visual evoked potentials (VEPs) at an early age in children with PVL. We did a study to evaluate the flash visual evoked potentials (fVEPs) in children with PVL less than 1 year of age. Materials and Methods: A total of nine children diagnosed as having PVL on magnetic resonance imaging were included in the study. The mean age was 9.7± 3.5 months. All children underwent handheld fVEPs under sedation at two different flash frequencies 1.4 and 8 Hz. Results: The mean latency of N1 and P1 on stimulation with 1.4 Hz was 47.9± 15.2 and 77.7± 26.0 ms, respectively. However, on stimulation with 8 Hz the mean latency of N1 and P1 was 189.8± 25.6 and 238.4± 33.6 ms, respectively. The mean amplitude with 1.4 Hz and 8 stimulation frequency was 5.6± 4.5 and 5.59± 3 mV, respectively. Conclusion: We have found for the first time that there is a change in the latency and the delay occurs at 8 Hz frequency but not at 1.4 Hz. We also conclude that amplitudes by fVEPs may be normal even in presence of periventricular changes. The amplitudes of fVEPs are not reliable in children with PVL.
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PURPOSE: We performed this study to determine if ophthalmic tests and positron-emission tomography (PET) would have any useful role in the investigation of cortical visual impairment (CVI) in children. METHODS: Ophthalmic tests were performed in children with CVI. After children with CVI and normal volunteers were examined using PET, PET images were transformed spatially on template images using Statistical Parametric Mapping 99 for analysis. The PET images of the CVI patients were compared with those of the normal group to display pixels with significantly decreased metabolism. Glucose metabolism in each patient was compared to that of the normal group and the number of pixels with decreased metabolism in each lobe was counted for analysis. RESULTS: Nine children with CVI showed variable degree of visual acuity. There were two patients with strabismus and one patient with latent nystagmus. In CVI group (n=5), there was a decrease of glucose metabolism in comparison with that of normal group (n=22) in occipital lobe as well as other areas, and the degree of decrease varied widely. The extent of the areas with decreased glucose metabolism in the occipital lobe of each patient ranged widely from 4% to 59%. CONCLUSIONS: The cerebral glucose metabolism was decreased with a variable degree in children with CVI. This result suggests that PET may be helpful in the diagnosis of children with CVI.
Subject(s)
Child , Humans , Diagnosis , Glucose , Healthy Volunteers , Metabolism , Neuroimaging , Occipital Lobe , Positron-Emission Tomography , Strabismus , Vision Disorders , Visual AcuityABSTRACT
The epidemiology of children with cortical visual impairment(CVI)has been poorly defined till now, so the purposes of this study were to define the visual prognosis and prevalence of ophthalmologic problems in Korean children. The 20 children with follow-up of over 3 months were included out of 24 children diagnosed with CVI. The causes of CVI were periventricular leukomalacia in 7, congenital malformation in 4, and cerebral infarct or atrophy. Twelve of 19 children who had visual impairment at their initial visit showed visual improvement with follow-up. There were strabismus in 14, nystagmus in 6, and abnormal optic disc in 8 of the 20 children. All visualevoked response results were abnormal, but were not providing a more reliable prognosis in vision. The children who had abnormal electroencephalogram had poor visual prognosis as well as delayed developmental level. In conclusion, this study identified that children with CVI may be presented with a variety of ocular abnormalities, including strabismus, nystagmus, and abnormal optic disc. These findings suggest that a better understanding of the natural course will be required in children with CVI.