Background: Androgen insensitivity syndrome (AIS) is a disorder of sex development characterized by variable defects in virilization of individuals with 46,XY karyotype. It is caused by mutations in the X chromosome androgen receptor gene, which, depending on their specific location, result in complete or partial peripheral androgen resistance.
Objective: This case report highlights a possible increased liability of patients with AIS to drug-induced extrapyramidal symptoms (EPS).
Case summary: A 28-year-old patient with partial AIS was admitted to the hospital because of paranoid ideation. At puberty onset, she had undergone bilateral orchiectomy; estrogen replacement therapy was prescribed but stopped 2 months later against medical advice. During her hospitalization, severe EPS manifested following initiation of risperidone 2 mg/d. She was later switched to sertindole 12 mg/d with a satisfactory response and no motor side effects.
Conclusions: Patients with AIS may have an increased susceptibility to drug-induced EPS, which may be caused by striatal dysfunction that is possibly associated with resistance to androgens during critical periods of early brain differentiation or direct effects of androgen receptor gene mutations on nigrostriatal function and development. Clinicians should cautiously treat psychosis in patients with AIS, preferably with antipsychotic compounds that have a low risk of EPS.
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