Intoxication with riluzole in Huntington’s disease

Abstract

Huntington’s disease (HD) is associated with a distinct profile of cognitive impairment that includes memory deficits (frontal type) and dysfunctions of various aspects of executive function, indicating a subcortical dementia.1 These behavioral changes can be observed early in the course of the disorder and psychiatric symptoms such as depression, personality changes, and psychotic symptoms are also associated with the syndrome. Suicidal behavior is commonly seen in patients with HD and is the cause of death in nearly 6% of patients.2 There is as yet no therapy that can effectively treat the neurologic pathology underlying HD; treatment is symptomatic. Riluzole, an antiglutamatergic drug, relieves symptoms of chorea and extends survival in patients with ALS and is currently being investigated for therapeutic efficacy in other neurodegenerative disorders, including HD.3,4⇓ Psychiatric symptoms are often treated with antipsychotics. A right-handed, 38-year-old woman was referred to the department of neurology in 1998 because of choreic hyperkinesias. Her medical history included depression since adolescence, which had been treated 2 years previously with psychotherapeutic counseling and since April 1999 with the antidepressant citalopram (10 mg/d). Neurologic examinations and the initial neuropsychological evaluation of the patient revealed a mild cognitive impairment. A synopsis of the clinical results led to the preliminary diagnosis of HD. This diagnosis was confirmed by genetic analysis in May 1999. To relieve chorea, riluzole (50 mg/d twice daily) was prescribed in April 1999. In June 1999, the patient attempted suicide by consuming 2,800 mg of …