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Nervenheilkunde 2011; 30(08): 563-568
DOI: 10.1055/s-0038-1628395
Ulmer Heft
Schattauer GmbH

Das Noonan-Syndrom

Ein psychiatrischer Kolibri?Noonan syndromeA psychiatric curiosity?
H. Graf
1   Klinik für Psychiatrie und Psychotherapie III, Universitätsklinik Ulm
,
D. Brummer
1   Klinik für Psychiatrie und Psychotherapie III, Universitätsklinik Ulm
,
A. Spiegel
1   Klinik für Psychiatrie und Psychotherapie III, Universitätsklinik Ulm
,
B. Abler
1   Klinik für Psychiatrie und Psychotherapie III, Universitätsklinik Ulm
› Institutsangaben
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Publikationsverlauf

Eingegangen am: 20. April 2011

angenommen am: 04. Mai 2011

Publikationsdatum:
23. Januar 2018 (online)

    Lizenzen und Reprints

Zusammenfassung

Das Noonan-Syndrom (NS) ist eine genetisch bedingte Entwicklungsstörung, die durch Kleinwuchs, kardiale Fehlbildungen sowie charakteristische Gesichtsdysmorphien gekennzeichnet ist. Darüber hinaus fallen Patienten häufig durch psychiatrisch-psychologisch relevante Symptome auf, die nur selten die diagnostischen Kriterien einer psychischen Störung nach der ICD-10 voll erfüllen. Trotz der hohen Inzidenz dieser Erkrankung ist das NS in der Erwachsenenpsychiatrie weitgehend unbekannt, stellt jedoch bei Patienten mit Dysmorphien unterschiedlichen Ausmaßes mit psychiatrischen Symptomen eine wichtige differenzialdiagnostische Überlegung dar. Dieser Artikel gibt eine Übersicht über die psychiatrischen und über die für die Diagnose zielführenden somatischen Symptome sowie das diagnostische Vorgehen bei der Verdachtsdiagnose des NS.

Summary

Noonan syndrome (NS) is a genetic and clinically variable developmental disorder characterized by short stature, cardiac defects and typical facial dysmorphism. In addition, patients with NS often show psychiatric and psychological symptoms which do not meet diagnostic criteria for a specific psychiatric disorder. Despite of the high incidence of the syndrome, the NS is largely unknown in adult psychiatry although it represents an important differential diagnostic consideration in patients with varying degrees of dysmorphism and psychiatric symptoms. This article gives an overview of somatic and psychiatric symptoms and the diagnostic approach to the diagnosis of NS.

Schlüsselwörter

Noonan-Syndrom - psychiatrische Aspekte - Alexithymie

Keywords

Noonan-Syndrome - psychiatric aspects - alexithymia
 

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