The mental status of patients with Friedreich's ataxia

In the clinical literature, the majority of patients with Friedreich's ataxia are described as having signs of intellectual decline and serious psychiatric symptomatology. Recent studies contradict this clinical picture, but indicate some discrete mental status changes, such as slowing of information processing speed not related to motor abnormality, in a more strictly defined Friedreich's population. This study describes the mental status changes in a sample of 38 patients seen at the University of California at Los Angeles Neuropsychiatric Hospital, Ataxia Clinic. The sample was defined using strict clinical and biochemical criteria. Only one of the 38 patients showed evidence of intellectual deterioration. Ninety-two percent of the patients experienced some affective difficulty, however, ranging from major depression to normal grief. Three patients have reached their mid-forties (one is 64 years of age) without any serious mental status changes. These findings point out the importance of nurses' expecting these patients to function normally in the cognitive domain. Implications related to specific nursing interventions are discussed.