A 29-year-old patient with FA (compound heterozygous for the [GAA] expansion [800 repeats] and a mutation of the ATG start codon, Met1Ile), who was a former chess tournament champion, presented to our department with a 1-week history of irritability, dysphoria, perseverations, derailment, paranoid delusions (fear of being poisoned or castrated by his family), auditory hallucinations (men whispering to his ear), and somatic sensations (feeling of being raped). He had become suspicious, and at times hostile, refusing to take his medication. Four days before symptom onset, he had been treated with IV amiodarone in an emergency department because of an episode of paroxysmal tachyarrhythmia, presumably resulting from dilated cardiomyopathy. The wheelchair-bound patient presented with a classic FA phenotype, in particular, severe visual impairment resulting from optic atrophy, saccadic hypermetria, square-wave jerks, saccadic pursuit, dysarthria, tetraparesis with distal amyotrophy, absent tendon reflexes, bilateral Babinski signs, and abolished joint position and vibration sense distally.
Thyroid function was normal. He was diagnosed with a psychotic episode and admitted. Aripiprazole (5 mg) was commenced. At that time, the patient also received aspirin, metoprolol, insulin, and idebenone. Symptoms subsided within 4 days, and he was discharged. One month later, he was readmitted because his initial symptoms had recurred. He was then taking 30 mg of aripiprazole daily, albeit irregularly. He was aggressive and refused insulin treatment, causing hyperglycemia (310 mg/dL) and metabolic acidosis. Risperidone (2 mg mane) was introduced. Three days later, his introspection, affect, psychotic symptoms, and thought disorder had improved. Considering the difficulties of daily administration and lack of compliance, paliperidone palmitate (PP) was introduced (first intramuscular dose: 150 mg) and risperidone stopped. Initially, the patient became somnolent, but then improved over the course of the subsequent days with remission of psychiatric symptoms for approximately 2 months. Over the ensuing period, however, recurrent relapse of psychotic symptoms led to repeated emergency admissions. Risperidone (2 mg mane) was reintroduced, parallel to PP. Though psychotic symptoms improved, the patient was severely sedated for most of the day. He did not present to the clinic again, but we were informed that he had become lethargic, apathetic, and refused food and beverages. He died a few weeks later. The patient's family refused an autopsy, and the exact cause of death remains unclear.