Friedreich’s Ataxia Patients Show Mental Stability Despite Physical Decline, Study Shows

Patients with Friedreich’s ataxia (FA) show notable mental health stability despite physical decline compared to patients with other neurodegenerative disorders. These findings are reported in a three-year longitudinal study of FA patients, using self-reporting questionnaire scales.

In the study, “A longitudinal study of the SF-36 version 2 in Friedreich ataxia,” published in Acta neurologica scandinavica, researchers followed the physical and mental health of 122 Australian patients with Friedreich’s ataxia over three years. A self-reported quality of life questionnaire called 36 item Short-Form Health Survey version 2 (SF-36V2) was used.

The scale comprises 36 questions in eight sections and has been extensively studied. Also, the Friedreich Ataxia Rating Scale (FARS) was used to connect the physical and mental health scores with FA clinical signs.

The results showed a gradual decline of physical health over the three years and was found to be linked with disease duration, age at review, and FARS score. However, the mental component showed no connection with any Friedreich’s ataxia disease parameter.

These findings suggest that despite physical decline, people with Friedreich’s ataxia have a relatively stable mental well-being. There was no difference in mental health points compared to the general population, which is consistent with former studies of FA patients.

Although SF-36V2 has been investigated in earlier FA studies, this is the first time it is used in a long-term study. No difference in the physical or mental state was seen after one and two years of the study, indicating this scale is not sensitive in measuring short-term changes in this disease. This may be due to the slow disease progression of Friedreich’s ataxia. A limitation of the study was that only 37 patients completed the final questionnaire.

Friedreich’s ataxia is a rare inherited disorder characterized by progressive damage to the nervous system. Other neurodegenerative disorders like Parkinson’s disease (PD) and multiple sclerosis (MS) are well known to show a high rate of a second psychiatric condition.

In Parkinson’s and MS, roughly 50 percent of the patients develop depression, which is 10 times more than in the general population. Studies have revealed that psychosocial factors and degree of disability are not the main cause of depression in those conditions. Instead, it seems to be a consequence of disease-related nerve changes and damage in the brain.

The results of this study showed that, despite a physical decline over the three years of follow-up, Friedreich’s ataxia patients remained relatively mentally stable. This could be due to study limitations or that the affected nerve cell networks in FA are not involved in mood regulation to the same extent as in Parkinson’s and MS. Additional studies are needed in this area.