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Risperidone Treatment for Psychosis in End-Stage Friedreich’s Ataxia

To the Editor: Friedreich’s ataxia is an inherited autosomal-recessive condition with childhood onset of progressive ataxia of gait and limbs and absent deep tendon reflexes and extensor plantar responses (1). Despite reports of cognitive decline and psychotic symptoms in end-stage Friedreich’s ataxia (2–5), MEDLINE and PsychINFO searches revealed no reports on the treatment or incidence of the psychosis that occasionally complicates the final stages of this illness. We describe a patient with Friedreich’s ataxia who developed psychosis in the end stage of his illness and responded to risperidone treatment.

Mr. A was a 36-year-old Caucasian man who had had Friedreich’s ataxia since age 5. Dysphoria, decreased energy, poor concentration, and anhedonia had evolved over several months. A previous 2-month trial of fluoxetine, 20 mg/day, resulted in no improvement. Mr. A was in a wheelchair and had recently lost the ability to perform daily functions, such as maintaining hygiene and caring for his daughter. His most recent decrease in function made him entirely dependent on care provided by his mother. Mr. A’s family history was notable for an older brother who also had Friedreich’s ataxia and died at the age of 34 from the illness.

His mother reported a gradual onset of unusual behaviors that Mr. A minimized and rationalized as part of his sensory deterioration. At home, he kept the radio on to drown out frightening sounds that he (but no one else) heard. He developed paranoia and multiple conspiracy theories, e.g., believing his ex-wife had thrown a puppy out of the window, even when presented with evidence to the contrary. He developed visual hallucinations that included seeing an aide walk into the room and then vanish.

We diagnosed Mr. A with psychotic disorder not otherwise specified and started him on a regimen of 1 mg of risperidone at night, increased to 1 mg b.i.d. after 1 week. His dysphoria improved, the delusions about his caregivers and his ex-wife resolved, and his visual hallucinations diminished. He experienced no extrapyramidal symptoms or other side effects. He became more observant and more insightful into his health status and was able to address many end-of-life issues as his thought organization improved. He died from progressive cardiac complications of Friedreich’s ataxia 6 weeks after starting risperidone treatment.

Risperidone was an effective and well-tolerated treatment for psychosis in our patient with end-stage Friedreich’s ataxia.

REFERENCES

1. Adams RD, Victor M, Roper AH: Principles of Neurology, 6th ed. New York, McGraw Hill, 1997, pp 1081–1084
2. Barbeau A: Friedreich’s ataxia 1976: an overview. Can J Neurol Sci 1976; 3:389–397[Medline]
3. Durr A, Cossee M, Agid Y, Campuzano V, Mignard C, Penet C, Mandel JL, Brice A, Koenig M: Clinical and genetic abnormalities in patients with Friedreich’s ataxia. N Engl J Med 1996; 335:1169–1175
4. Hart RP, Kwentus JA, Leshner RT, Frazier R: Information processing speed in Friedreich’s ataxia. Ann Neurol 1985; 17:612–614[Medline]
5. Johnson WG: Friedreich’s ataxia. Clin Neurosci 1995; 3:33–38[Medline]

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