Development of visual function in hemihydranencephaly.

This study reports on the findings of longitudinal follow-up of visual function in a 12-year-old girl affected by congenital right hemihydranencephaly. This extremely rare unilateral brain malformation allowed the authors to gather new information on neuronal plasticity and functional compensations of the visual system across a period of 10 years. An extension of the preserved right visual hemifields above the middle line and strategical eye or head positions developed to increase visual functions are discussed. In addition, ophthalmological and orthoptical findings, as well as the development of monocular grating and linear acuity, are described.

Visual behaviours of neurologically impaired children with cerebral visual impairment: an ethological study.

BACKGROUND/AIMS: Visual functions of neurologically impaired children with permanent cerebral visual impairment (CVI) can be difficult to determine. This study investigated the behavioural profile of CVI children by means of ethological observations in order to gain a better understanding of their visual functions. METHODS: Video registrations of nine subjects who were unable to undergo more orthodox methods of visual function testing were observed and analysed by an ethologist. RESULTS: A series of behaviours (direct signs) and supportive or confirming behavioural elements (indirect signs) indicating some visual perception in the children were found. CONCLUSION: Detailed ethological observations of visual behaviour were shown to be useful for analysing visual functions of children with permanent CVI.

Longitudinal follow-up of grating acuity in children affected by cerebral palsy: results of a 5 year study.

PURPOSE: To analyse the natural course of visual acuity in subjects affected by cerebral palsy. METHODS: During the past 5 years, 16 children underwent repeated grating acuity measurements using the acuity card procedure. At the end of the follow-up final grating acuity was compared with linear acuity obtained using standard optotypes. RESULTS: A good or moderate improvement in grating acuity was shown by 14 subjects. However, the general development of grating acuity showed a protracted course and early values did not correlate with final grating acuity (r = 0.20). Moreover, most of the children showed subnormal vision when measured with standard optotypes. CONCLUSION: Clinicians should remain optimistic about the potential for some visual development in children affected by cerebral palsy. However, great caution should be exercised in extrapolating information from early grating acuity measurements. Regular assessments with the acuity card procedure are necessary in order to gain an insight into the natural course of visual development in children affected by cerebral palsy.

Cerebral visual impairment in cerebral palsy: relation to structural abnormalities of the cerebrum.

In patients with cerebral palsy (CP), cerebral visual impairment (CVI) is frequently found in addition to ophthalmological disorders. Lesions in the visual areas are found in CT scans of CP patients with CVI. The aim of the present study was to determine the prevalence of these specific findings in CP patients with CVI. CT scans of 49 cerebral palsy patients were studied; CVI was diagnosed in 36 patients; in 13 patients, visual acuity was normal. In 8 patients, comparison with an MRI scan was possible (6 with CVI, 2 with normal acuity). The CT scans were scored according to the criteria used by Van Nieuwenhuizen (1987): normal, abnormalities of the white matter adjacent to the posterior horns of the lateral ventricles, abnormalities of the white matter located under the visual cortex, abnormalities of the visual cortex and abnormalities elsewhere. Abnormalities in the visual areas were found in 15% of the normal acuity group and in 53% of the CVI group. In 17 of the 19 CVI patients with abnormalities in the visual areas, the lesions were located in the white matter surrounding the posterior horns (89%). MRI imaging revealed the same abnormalities as the CT scans in 6 patients, but in one patient the abnormality was seen in more detail and in one patient the lesion in the occipital area was seen only on MRI. MRI examination seems to detect at least as many, but in some cases even more specific lesions in CVI patients compared to CT scanning, but the numbers were too small to allow any definitive conclusions to be drawn.

Impact of cerebral visual impairment on the everyday life of cerebral palsied children.

A group of 43 patients suffering from cerebral palsy and cerebral visual impairment was compared with a group of 24 cerebral palsy patients with normal visual acuity, with regard to a functional level in daily life. Four categories were considered: communication, emotional contact, self-care and intelligence. Cerebral visual impairment-cerebral palsy patients scored significantly lower in all categories than cerebral palsy patients with normal visual acuity. In the cerebral visual impairment-cerebral palsy group, there was a higher frequency of tetraplegia compared with the group with normal acuity, but even after this predominance had been excluded, a significant difference remained. When dealing with cerebral palsy patients, occurrence of cerebral visual impairment must be taken into account. By initiating intervention programmes, one can prevent extra handicaps due to the consequences of diminished visual acuity.

Identical morphology of the rolandic spike-and-wave complex in different clinical entities.

The morphology of the rolandic spike, the trough between the rolandic spike and the following slow wave, and of the slow wave itself was quantitatively studied in 43 children, classified into five clinical groups: (a) functional with epilepsy benign focal epilepsy of childhood with centrotemporal spikes (BECT) with oropharyngeal seizures or (b) BECT with unilateral or generalized seizures or (c) functional without epilepsy, and (d) organic with or (e) without epilepsy. The morphologic features of the rolandic spike-and-wave complex were identical in the five clinical categories. Thus, a quantitative description of the rolandic spike-and-wave complex can be given that is valid for the 43 children of the present study, although they represent a heterogeneity of associated clinical syndromes. The rolandic spike appeared to be not a spike but a sharp wave with a mean duration of 88 ms. In contrast to the opinion of several investigators, the morphology of the rolandic spike does not provide a clue to its "epileptogenicity" or to the presence or absence of an organic cerebral lesion in the individual child. In clinical practice, additional information (background activity of the EEG, computed tomography (CT) scan, or magnetic resonance imaging (MRI) of the brain) is needed to determine the significance of rolandic spikes occurring in the EEG of a child with respect to the probability of a cerebral lesion and the prognosis in relation to epileptic seizures.

Rolandic spikes in the inter-ictal EEG of children: contribution to diagnosis, classification and prognosis of epilepsy.

The clinical correlates of Rolandic spikes were studied in 47 children to determine the significance of this EEG finding to the diagnosis and classification of epilepsy. The children were classified into 'functional' and 'organic' groups, with and without epilepsy. Children with epilepsy were further subdivided into those with Rulandic and those with non-Rulandic seizures. In children without neurological abnormalities, the EEG finding of Rolandic spikes plays a decisive role in the diagnosis of an epileptic syndrome as benign focal epilepsy of childhood with centro-temporal spikes (BECT), a diagnosis with an excellent prognosis. Neurological and neuroradiological examinations of the 'functional' group revealed that the Rolandic spike may occur as a true 'functional' spike. The frequency of a family history of epilepsy among neurologically normal children with Rolandic spikes suggests, in addition to the inheritance of BECT and the EEG trait, the existence of a hereditary susceptibility to epilepsy.

Grating-acuity in children. Normal values of visual acuity in children up to 13 years as assessed by the acuity card procedure.

The acuity card procedure proved to be a useful method for assessing visual acuity in children. Normal values of visual acuity measured by this method had already been assessed in children up to four years. To enable application of the test in older children this study obtained values in a group of 396 normal children, aged three months to 13 years. The mean curve as well as the 10th centile was calculated in different age groups. A considerable variation of acuity values causing a 'dip' in the 10th centile was found in the ages 18 to 24 months. A smaller 'dip' was found in the ages 48 to 52 months. This has to be attributed to behavioural properties connected with age. The results obtained in the group of children under four years of age corresponded fairly well with other studies. In schoolchildren a fair agreement could also be found between the data obtained using the acuity card procedure and the data obtained with the Landolt-C rings. Obtaining normal values for the acuity card procedure in children covering a wide age range facilitates recognition of visual handicap in children who are difficult to assess.

The prevalence of cerebral visual disturbance in children with cerebral palsy.

Assessment of visual acuity using the visual acuity card procedure in 164 children with cerebral palsy revealed low visual acuity in 71 per cent. Results of ophthalmological examination were available for 74 of these patients, but could not explain adequately the low visual acuity of 36 of the 43 patients (84 per cent) assessed by both the acuity card procedure and other techniques. There is a high probability that cerebral visual disturbance is present in these patients. Awareness of visual disability when compiling a programme of visual and neurodevelopmental stimulation for children with cerebral palsy is essential.