RESUMO
Very few pediatric cases of arachnoid cyst of ponto-cerebellar angle are described in the literature. Only 4 are described with hearing loss. It is a pathology which poses especially a problem of early diagnosis. In this paper we describe the management of a 16-year-old patient with an arachnoid cyst of the cerebellopontine angle with an isolated auditory deficit that was treated surgically. The follow up was marked by a Full recovery of hearing after surgical treatment. Arachnoid cyst of the cerebellopontine angle is rare in the pediatric population. early surgical management help to increase the chances of recovery.
RESUMO
We report on a cross-syndrome comparison of hand, foot, eye and ear laterality in three groups of individuals with different genetic disorders (trisomy 21, del7q11.23, and del22q11.2) to test the relationship between atypical laterality and intellectual disability. These groups were compared to a group of typically developing persons. Hand, foot, eye and ear laterality was assessed using item tasks, conducted twice, and Bishop's card-reaching test. Ordering of the mean IQ score for each of the three groups was as follows: trisomy 21 < del7q11.23 < del22q11.2. We observed the same ordering as for IQ, particularly in mixed handedness, degree of laterality, hand and foot consistency. The existence of a cognitive threshold, below which lateral preference is atypical, advocates for a causal link between cognition and laterality in those with low IQ although unknown other factors underlying both could determine this association.
Assuntos
Deleção Cromossômica , Cromossomos Humanos Par 7/genética , Transtornos Cognitivos/genética , Síndrome de DiGeorge/genética , Síndrome de Down/genética , Lateralidade Funcional/genética , Síndrome de Williams/genética , Adolescente , Adulto , Criança , Cromossomos Humanos Par 22/genética , Transtornos Cognitivos/diagnóstico , Síndrome de Down/diagnóstico , Feminino , Humanos , Inteligência/genética , Estudos Longitudinais , Masculino , Testes Neuropsicológicos , Síndrome de Williams/diagnóstico , Adulto JovemRESUMO
The first aim of the study was to compare developmental trends in the degree of laterality (independent of direction) observed in two handedness tasks. The second aim was to assess family resemblance in the degree of laterality using the same two tasks. The sample was comprised of 186 left-handers and 302 right-handers aged from 6 to 66. Some of the sample were members of the same families. Bishop's card-reaching task was used to assess hand preference, and Annett's peg-moving task to assess manual performance. For the card-reaching task, children aged 7 to 10/11 recorded more midline crossings than the other age groups (both younger and older). No general age-related trend was observed for the Annett pegboard. For the card-reaching task, family resemblance was very low and not significant. The degree of laterality, assessed with the peg-moving task, showed a small but significant resemblance in father-offspring pairs (sons and daughters). Putative involvement of a maternally suppressed gene on chromosome 2p12 and of the androgen receptor was discussed.
Assuntos
Lateralidade Funcional/genética , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Cromossomos Humanos Par 2/genética , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Receptores Androgênicos/genética , Análise e Desempenho de TarefasRESUMO
Persons with trisomy 21 (T21) and Williams-Beuren syndrome (WBS) have different brain abnormalities which may affect manual laterality. We assessed 45 persons with T21 and 34 with WBS (mean age 13) and 81 typically developing children (TD). Manual laterality was assessed with a fifteen-item task administered two times, and Bishop's card-reaching task. We found more left-handers in the T21 group compared to the other two groups. Inconsistent laterality was higher in the two groups with genetic diseases than in the TD group. For Bishop's test, both T21 and WBS participants were less right-oriented than the TD group. They displayed different response patterns in midline crossing when reaching for the cards, but did not display more midline crossing inhibition than the TD group. Is atypical handedness linked to specific genetic syndromes and, more specifically for persons with T21, to the trisomy of some of the genes?
Assuntos
Síndrome de Down/fisiopatologia , Síndrome de Down/psicologia , Lateralidade Funcional , Inteligência , Síndrome de Williams/psicologia , Envelhecimento , França , Humanos , Itália , Atividade Motora , Estados Unidos , Síndrome de Williams/genética , Síndrome de Williams/fisiopatologiaRESUMO
The aim of the study was the validation of Bishop's reaching card test (Bishop et al., 1996) in a French population. Annett's peg-moving test (1985) and the Purdue pegboard (Tiffin, 1948) were also used to assess manual performance. Subgroups of left- and right-handers were established according to manual preference as measured by Annett's questionnaire (1985). A sample of 260 adults was tested, including 158 self-professed right-handers, 77 self-professed left-handers, and 25 self-professed ambidextrous people. The three manual performance tests and the questionnaire presented good metric qualities (homogeneity, test-retest reliability). However the constitution of subgroups of laterality (in particular left-handers) as a function of manual preference could not be validated by classic tasks, or by Bishop's test, which is supposed to combine features of both preference and performance. Moreover the link between performance and preference was weak. Both evaluations should be used to produce a complete pattern of laterality for each participant.
