To the Editor: The frequency of psychiatric disorders in patients with Klinefelter syndrome could be higher than that in the general population (1). Among the personality features reported in men with the XXY karyotype of Klinefelter syndrome are passivity, weak concentration, emotional immaturity, shyness, and hypersensitivity (1). Some cases of Klinefelter syndrome with schizophrenia have been reported (2, 3), although aggressive behaviors in Klinefelter patients with schizophrenia have rarely been described.
Mr. A was a 21-year-old Caucasian man who was initially arrested for criminal violence against persons, including children. He was subsequently admitted to our psychiatric department without his consent. He fulfilled DSM-IV criteria for the paranoid type of schizophrenia, with transient persecutory delusional beliefs and auditory hallucinations. The results of a karyotype confirmed Klinefelter syndrome (karyotype 47, XXY). Throughout his hospitalization, Mr. A exhibited irritability, open hostility, and anger; he made frequent aggressive threats, impulsive physical attacks against individuals and objects, self-directed attacks, and assaults on mental health staff. He made three impulsive suicide attempts (twice trying to set himself on fire). His insight was poor. Mr. A was referred to our forensic psychiatric department four times, for a total duration of 6 years. An EEG showed nonspecific abnormalities localized to the temporal lobes (slow-wave activity). Mr. A never had the substance use disorders frequently associated with such violence.
During Mr. A’s hospitalization, a variety of antipsychotic medications were used, including 150 mg of intramuscular fluphenazine decanoate every 2 weeks, 1000 mg/day of chlorpromazine, and 30 mg/day of haloperidol. Mr. A also received carbamazepine, 800 mg/day, and lorazepam, 5 mg/day, for 2 years. He was found to meet criteria for resistance to conventional antipsychotics; he had at least two periods of 6 weeks of treatment with antipsychotics from at least two chemical classes (chlorpromazine-equivalent doses higher than 1000 mg/day) without significant symptom improvement.
Mr. A’s aggressive behaviors occurred persistently for 9 years and suddenly stopped after he starting olanzapine treatment, 20 mg/day. He showed a significant improvement in conceptual disorganization, hallucinatory behavior, violence, and unusual thoughts. His selective attention and insight were especially improved. To date, Mr. A’s psychiatric symptoms have been controlled for 20 months with olanzapine.
To our knowledge, this is the first report of a violent patient with comorbid Klinefelter syndrome and schizophrenia. Two atypical antipsychotics, clozapine and olanzapine, are thought to have antiaggressive effects (4). This case suggests that olanzapine may be an effective treatment for hostile agitation, threatening, and assaultive violence in schizophrenia patients with Klinefelter syndrome.