Introduction: Huntington's Disease, that is caused by CAG trinucleotide repeat expansion in the IT15 gene located on the short arm of chromosome 4, is an autosomal dominant, progressive neurodegenerative disorder characterized by motor, cognitive, and psychopathological symptoms (1). The disease is accompanied by a variety of psychiatric disorders and its incidence rates range from 33 to 76%. Although depression is the most common accompanying psychiatric disorder, anxiety, obsessive-compulsive disorder, irritability and manic and psychotic symptoms may be associated with Huntington's Disease (2). In this case report, we discuss the effectiveness of aripiprazole on the motor and psychiatric symptoms of a patient with Huntington's disease, who had psychotic symptoms.
Case: Two years ago, psychotic symptoms developed in addition to existing neurological symptoms in a fifty-year-old patient who was known to have had Huntington's disease for twenty years. After one month of 30 mg/day aripiprazole treatment for a diagnosis of 'Psychotic Disorder Due to a General Medical Condition', a significant decline was observed in the motor and psychotic symptoms that had been present at the beginning of the disease.
Discussion: Although the pathogenesis of the Huntington's disease has not been exactly solved, the glutamatergic and dopaminergic systems with striatal neurodegeneration are thought to be responsible for the clinical signs of the disease (3). By the consideration of this process it is conceivable that aripiprazole, which is used in the treatment of psychosis and chorea, may be a well-tolerated agent for its effects on the negative and positive symptoms with low metabolic and extrapyramidal side effects.
References:
1. Walker FO: Huntington's disease. Lancet 2007; 369:218–228
2. van Duijn E, Kingma EM, van der Mast RC: Psychopathology in verified Huntington's disease gene carriers. J Neuropsychiatry Clin Neurosci 2007, 19:441-8.
3. Paoletti P, Vila I, Rife´ M, et al: Dopaminergic and glutamatergic signaling crosstalk in Huntington's disease neurodegeneration: the role of p25/ cyclin-dependent kinase 5. J Neurosci 2008; 28:1090–1101