Cognitive Characteristics Associated with Noonan Syndrome: Two Case Reportsy

Troyer, Angela K.; Joschko, Michael

doi:10.1080/09297049708400643

Cited by 9 publications

(14 citation statements)

References 21 publications

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“…Research focusing on aspects of memory in NS children is very scarce, and the few publications available are based on results of only 1 or 2 tests. Nevertheless, the findings are consistent, pointing out slight problems in working memory and memorizing, but not in storage and retrieval functions per se [19,24,28]. Attention problems have been put forward as the underlying cause.…”

Section: Neuropsychological Profile In Ns Childrensupporting

confidence: 51%

“…Several authors have suggested that attention problems can be regarded as a key dysfunction in NS, having reached this conclusion mainly on the basis of parental evaluations [19,24,26,30]. Inattention, in NS literature mostly described as a lack of selective or sustained attention, is mentioned frequently, while divided attention is generally thought to be intact.…”

Section: Neuropsychological Profile In Ns Childrenmentioning

confidence: 99%

“…Shaw et al [22] consider feeding problems as an early marker of not only delayed language development, but also long-term educational achievement. A few case-reports have been written about NS children with specific spelling and reading impairments [23,24,25], and, in the larger cohort of Wood et al [26], 43% of the children showed a language impairment. In adulthood, when language is sufficiently learned, problems in this area seem to resolve.…”

Section: Neuropsychological Profile In Ns Childrenmentioning

confidence: 99%

“…Immaturity, a lack of interaction with peers, a diminished insight in social situations and problems in verbalizing and explaining experiences have been mentioned with respect to this subject [19,20,24,26,30]. …”

Section: Neuropsychological Profile In Ns Childrenmentioning

confidence: 99%

“…Mostly, they are associated with musculoskeletal changes like hyperextensibility and hypotonia, but a correlation with abnormalities of the cerebellum has also been suggested [24]. The few studies addressing the cognitive components of motor functioning indeed revealed below average achievement on motor coordination tasks, and a developmental coordination disorder has been suggested in half of the children [20,24,28]. When studying the results in more detail, impaired motor responses (the handling aspects per se) seem to be more explanatory than dysfunctions in visual perception or planning.…”

Section: Neuropsychological Profile In Ns Childrenmentioning

confidence: 99%

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Neuropsychological and Behavioral Aspects of Noonan Syndrome

et al. 2009

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The current paper introduces concise neuropsychological assessment as an essential tool for studying the contribution of cognition and behavior in the expression of genetic syndromes, like Noonan syndrome (NS). Cognitive and behavioral findings in NS show intelligence scores across a wide range, with a mildly lowered average level. Language and motor development are often delayed, but no longer dysfunctional in adulthood. Continuing mild problems in selective and sustained attention are noted, as well as suboptimal organization skills and compromised abilities to structure complex information. These problems seem to culminate in learning difficulties, requiring attention for special needs in education. It seems that a complex of psychosocial immaturity, alexithymia and amenable traits is typical of NS patients. Consequently, psychopathology or psychological problems in leading a self-serving life may often remain underreported. This is why the authors advocate the integration of the domain of social cognition and personality in NS assessment.

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Section: Neuropsychological Profile In Ns Childrensupporting

confidence: 51%

Section: Neuropsychological Profile In Ns Childrenmentioning

confidence: 99%

Section: Neuropsychological Profile In Ns Childrenmentioning

confidence: 99%

Section: Neuropsychological Profile In Ns Childrenmentioning

confidence: 99%

Section: Neuropsychological Profile In Ns Childrenmentioning

confidence: 99%

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Neuropsychological and Behavioral Aspects of Noonan Syndrome

et al. 2009

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References

2010

Neuropsychological Assessment and Intervention for Childhood and Adolescent Disorders

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Alexithymia, emotion perception, and social assertiveness in adult women with Noonan and Turner syndromes

Roelofs

Wingbermühle

Freriks

et al. 2015

American J of Med Genetics Pt A

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Noonan syndrome (NS) and Turner syndrome (TS) are associated with cognitive problems and difficulties in affective information processing. While both phenotypes include short stature, facial dysmorphisms, and a webbed neck, genetic etiology and neuropsychological phenotype differ significantly. The present study examines putative differences in affective information processing and social assertiveness between adult women with NS and TS. Twenty-six women with NS, 40 women with TS, and 40 female controls were matched on age and intelligence, and subsequently compared on (1) alexithymia, measured by the Bermond-Vorst Alexithymia Questionnaire, (2) emotion perception, evaluated by the Emotion Recognition Task, and (3) social assertiveness and social discomfort, assessed by the Scale for Interpersonal Behavior. Women with TS showed higher levels of alexithymia than women with NS and controls (P-values < 0.001), whereas women with NS had more trouble recognizing angry facial expressions in comparison with controls (P = 0.01). No significant group differences were found for the frequency of social assertiveness and the level of social discomfort. Women with NS and TS demonstrated different patterns of impairment in affective information processing, in terms of alexithymia and emotion perception. The present findings suggest neuropsychological phenotyping to be helpful for the diagnosis of specific cognitive-affective deficits in genetic syndromes, for the enhancement of genetic counseling, and for the development of personalized treatment plans.

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Cognitive Characteristics Associated with Noonan Syndrome: Two Case Reportsy

Cited by 9 publications

References 21 publications

Neuropsychological and Behavioral Aspects of Noonan Syndrome

Neuropsychological and Behavioral Aspects of Noonan Syndrome

References

Alexithymia, emotion perception, and social assertiveness in adult women with Noonan and Turner syndromes

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