Reduced Formation of Hippuric Acid After Oral Benzoic Acid in Friedreich’s Ataxia

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Reduced formation of hippuric acid after oral benzoic acid in Friedreich's ataxia.

We have observed a markedly decreased formation of hippuric acid after benzoic acid load in patients with typical Friedreich's Ataxia compared to normal control subjects. Since there is evidence for normal or even enhanced tauro-conjugation in the bile of patients with this disease, with a decreased G/T ratio, it is unlikely that co-factor or enzyme concentrations are the cause of this defect. ...

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Dysarthria in Friedreichs Ataxia: A Perceptual Analysis

The aims of this study were to: (1) evaluate the perceptual speech dimensions, speech intelligibility and dys arthria severity of a group of individuals diagnosed with Friedreich’s ataxia (FRDA); (2) determine the presence of subgroups within FRDA dysarthria; (3) investigate the relationship between the speech outcome and the clinical factors of disease progression. The study included 38 indivi...

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ژورنال

عنوان ژورنال: Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques

سال: 1982

ISSN: 0317-1671,2057-0155

DOI: 10.1017/s0317167100043997