Hypotheses on physiopathogenic mechanisms of fecal incontinence in Wolfram syndrome.

Type 1 Wolfram syndrome is a rare, neurodegenerative pathology characterized by the association of insulin-dependent diabetes and optic atrophy. Other symptoms include diabetes insipidus, hearing loss and neurological damage. Fecal incontinence was recently recognized as a new symptom. Its pathophysiological mechanism remains unknown. We plan to collect data on the severity of the syndrome at the onset of fecal incontinence, the frequency of signs suggestive of pons volume reduction, and the frequency of urinary disturbances. Based on this information, we will investigate the possible location of the neurological involvement causing this symptom.