Long term neurocognitive improvement after "late" right hemispherectomy: case report and review of the literature.

OBJECTIVE: To study the long-term neurocognitive changes of a right-handed girl with intractable epilepsy after late right hemispherectomy and compare them with data in the literature. METHOD: The girl was affected by an epileptic encephalopathy associated with right fronto-temporo-parietal polymicrogyria; she was submitted to right hemispherectomy at the age of 5 and examined with cognitive and neuropsychological tests at the age of 17 years. The girl took advantage of neurocognitive rehabilitation for several years; she is currently seizure-free and off therapy. RESULTS: At the end of the follow-up, the full-scale IQ is stable and within the normal range (p = 88). As the discrepancy between verbal IQ (pp = 120) and performance IQ (pp = 71) is significantly high, the girl was subjected to neurocognitive evaluation with the following results: verbal problem solving, verbal short- and long-term memory, and executive functions are within normal range. The most fragile functional areas are visual and spatial reasoning, verbal working memory, short-term visuospatial memory, visual attention, and processing speed, all > 2 SD. The spatial tests, such as coding, matrix reasoning, picture concepts, and arithmetic reasoning (which are favored by other functions such as associative memory and learning ability), are less severely impaired. CONCLUSIONS: These findings show that good conceptual skills and verbal reasoning can compensate for some deficits in visual-perceptual and visuospatial functions.

Normal language in abnormal brains.

There is little doubt that, in the adult, specific brain lesions cause specific language deficits. Yet, brain localizations of linguistic functions are made problematic by several reported cases of normal language in spite of major brain anomalies, mostly, but not exclusively, occurring early in life. The signal cases are hydrocephaly, spina bifida and hemispherectomy. These cases are discussed and possible solutions are suggested: namely a vast redundancy of neurons and/or the role of microtubules as neuron-internal processors and key factors in signaling and guiding the growth and reconfiguration of the brain.

Parental experiences before and long-term after their children's hemispherotomy - A population-based qualitative study.

Severe childhood epilepsy has an impact on the whole family. For selected children, hemispherotomy is the treatment of choice. The aim of this study was to explore parents' experiences before and after hemispherotomy as reported at a long-term follow-up and their view on received information and support. This was a population-based qualitative descriptive study, using qualitative content analysis of interviews. Seven to eighteen years after hemispherotomy, parents of twenty-one operated children were interviewed about the family life situation, expectations before surgery, and support and information before and after surgery. Before surgery, the theme 'Living in a chaotic bubble' illuminates how parents felt: the family lived in isolation, they felt both dissatisfaction and satisfaction about support and information, and they experienced that surgery was a question about life or death. After surgery, the theme 'Hovering between success and disaster' illuminates how parents hovered between happiness if the surgery was successful and sadness about e.g., complications and behavior problems. They experienced both excellent and poor support, in hospital and at rehabilitation. Regardless of all concerns, parents were satisfied that the child had received an operation. The hemispherotomies were successful and generated a better life situation. However, in order to cope, families need support and information throughout the whole process, from the onset of epilepsy and for a long time after surgery. If the child has behavior problems, an assessment should be made before surgery in order for the families to get adequate support. The specialist team needs to be involved as early as possible and follow the families for several years after surgery. Focus should be on the whole family, if needed including grandparents; family-centered care might be relevant for this patient group.

A long-term patient perspective after hemispherotomy--a population based study.

PURPOSE: Explore the long-term life situation for Swedish hemispherotomy patients reporting not only seizure outcome but also patients' perspectives on function, quality of life (QoL) and satisfaction with the surgery. METHODS: This population based study uses prospectively collected data from the Swedish National Epilepsy Surgery Register. An independent researcher interviewed patients or parents, using two patient oriented questionnaires. RESULTS: Twenty-nine patients underwent hemispherotomy in Sweden after 1995 and had a five- or ten-year follow-up. At the 2-year follow-up 55% (16/29) were seizure-free since surgery, and 11/29 (38%) were seizure-free at the long term follow up. Twenty-six (90%) participated in this study. Median time to interview was 13.5 years; 9/26 (35%) were seizure-free then; 23% were off antiepileptic medication. In those not seizure-free, seizures were considered mild or moderate; 11% attended mainstream school and 3/12 adults lived independently. Most parents both of seizure-free and non seizure-free patients reported QoL and general health to be very good/good; 73% were satisfied/very satisfied with the hemispherotomy. CONCLUSION: In this series there were more long-term recurrences than previously reported. This might be related to the lower level of function of this cohort and higher percentage of developmental aetiologies compared to other series. However, most hemispherotomy patients have a good QoL in the long run and feel that the operation was worthwhile, even when it did not stop seizures. The majority had persisting impairments. Proxies were not very concerned about seizures, indicating that reduction in seizure frequency and/or severity may be an important gain with hemispherotomy.

Functional consequences of hemispherectomy.

Using the International Classification of Functioning Disability and Health (ICF) (WHO, 2001), impairments, activities and social participation are reported in 12 children (mean age at surgery 5.9 years) who were investigated before and three times over a 2-year period after hemispherectomy. Impairments were assessed (i) in terms of seizure frequency (Engel classification) and seizure severity (HASS) and (ii) with respect to muscle strength (MRC), range of motion (JAM score) and muscle tone (modified Ashworth scale). Activities were assessed in terms of gross motor functioning (GMFM) and self-care, mobility and social function (PEDI). Participation was assessed in terms of epilepsy-related restrictions and quantified by means of the Hague Restrictions in Childhood Epilepsy Scale (HARCES). Nine out of 12 children could be classified as free of seizures (Engel class I), and in the remaining three seizure frequency was Engel class III. HASS scores showed maximum improvement in 10 out of 12 children and near-maximum improvement in the two remaining children. Muscle strength and muscle tone on the side of the body contralateral to the hemispherectomy, which were already decreased preoperatively, decreased even further in the first 6 months after surgery, but returned to the presurgical baseline thereafter, except for the distal part of the arm. Range of motion was abnormal prior to operation and remained so after operation. Mean GMFM increase was 20% after 2 years (95% confidence interval 10-33); all five dimensions improved statistically significantly (P < 0.05). Mean PEDI increase was more than 20 scale points (95% confidence interval 10-35); again, all domains improved significantly (P < 0.05). In nearly all children, HARCES scores had normalized 2 years after surgery. In conclusion, decrease of seizure frequency and severity widens the scope of motor and social functioning, which overrides the effects of remaining motor impairments.

Perception of dichotic chords by normal and commisurotomized human subjects.

A perceptual suppression of an ipsilateral by a concurrent contralateral auditory signal occurs in commissurotomized subjects and probably in normal subjects. This suppression of the ipsilateral signal depends on the nature of the auditory stimuli. For dichotic speech sounds the suppression of the ipsilateral signal is overwhelming; for dichotically presented pure tones it is not present. For dichotically presented pure tones, unlike speech signals, a subcortical central pitch processor determines the contribution to the perceptual experience of the right and left ear signals in both normal as well as commissurotomized subjects.