Purpose of Study XXY syndrome (Klinefelter) and XXYY syndrome share a similar physical phenotype but differ in their behavioral phenotypes. Previous studies on XXY males describe academic difficulties and attentional problems, while reports of males with XXYY syndrome describe impulsivity, aggression, and anxiety. Despite multiple reports describing inattention, impulsivity, and behavioral problems in these groups, a study investigating the diagnosis of attention-deficit hyperactivity disorder (ADHD) and oppositional defiant disorder (ODD) in XXY and XXYY patients has not yet been described. The purpose of this study is to (1) compare rates and subtypes of ADHD and ODD in XXY and XXYY syndromes and (2) to describe response of ADHD symptoms to psychopharmacologic treatment in these 2 groups.
Methods Parents and teachers of males 5 to 25 years with XXY (n = 15) or XXYY (n = 24) completed the SNAP-IV rating scale that contains the DSM-IV criteria for ADHD and ODD. Clinical history and review of medication effectiveness were obtained by interview and record review.
Results 33% of males (5/15) with XXY syndrome met criteria for ADHD. 80% met criteria for ADHD-Inattentive subtype while 20% met criteria for the Combined subtype. 2 of 15 males with XXY (13%) met criteria for ODD, and both also had ADHD. In comparison, 70% (17/24) with XXYY syndrome met criteria for ADHD. 50% of XXYY males with ADHD had the Combined subtype, while the other 50% had the Inattentive subtype. 11 of 24 XXYY male children (46%) also met diagnostic criteria for ODD. Clinical history was consistent with SNAP-IV responses in all patients. Psychopharmacologic treatment of ADHD with stimulants was effective in 65% of patients, although XXYY patients were more likely to need additional medications.
Conclusions ADHD is an important clinical consideration in patients with both XXY and XXYY syndrome. Rates of ADHD of 33% in XXY and 70% in XXYY patients are considerably higher than the 3-10% reported in the general population and have not been previously reported in these patients. Males with XXY are more likely to have symptoms of inattention while XXYY males are more likely to have the combined subtype of ADHD. Males with XXYY are more likely to have ODD than XXY males. Stimulant treatment for ADHD symptoms is effective in both groups, and specific psychopharmacologic interventions will be discussed.
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