[Urinary disorders of Wolfram syndrome. Clinical and urodynamic analysis from 6 observations]. / Les troubles vésico-sphinctériens du syndrome de Wolfram. Analyse clinique et urodynamique à partir de 6 observations.

Wolfram syndrome is a neurodegenerative disorder characterized by childhood onset diabetes mellitus, optic nerve atrophy, diabetes insipidus, hearing impairment, brainstem alteration and commonly bladder and bowel dysfunction. OBJECTIVE: We present here, 6 new cases of urinary dysfunction in this rare disease. RESULTS: All patients had urinary retention with overactive bladder. The urodynamic assessment found overactive detrusor in 3 cases. Five out of six patients performed self-catheterization and were treated with anticholinergics or intradetrusor injection of botulinum toxin. The follow-up at 5 years found an alteration of the upper urinary tracts and a renal failure 3/6. CONCLUSION: Urinary dysfunction is common in Wolfram syndrome, mainly characterized by overactive bladder and urinary retention. The urological risk is major requiring a systelmatic follow-up of these patients. LEVEL OF EVIDENCE: 4.