Psychometric Investigations in Friedreichs Ataxia

The literature contains few reports of investigations on the mental state of subjects with Friedreich's ataxia. Most are based on clinical findings and lead to differing conclusions. For some authors the neurological disease is associated with mental deficits of varying magnitude, for others the mental state of the patients is accompanied by any deficit. The authors discuss five cases and try to supplement clinical findings with a series of psychometric tests in three areas: intellectual efficiency, higher cortical functions and elements of personality. The clinical and psychometric results are summarized in a grid comprising the main psychopathological symptoms attached to cerebral organic suffering (psycho-organic syndrome). Comparison of the results with medical data confirm that, in Friedreich's ataxia, there are no specific disorders characteristic of a psycho-organic syndrome. Lastly, the authors discuss certain hypotheses (concerning methods used, precocious appearance of symptoms and reactions to the disease) which try to explain the divergence of conclusions reached in the literature for this type of study.