[Urinary disorders of Wolfram syndrome. Clinical and urodynamic analysis from 6 observations].

UNLABELLED: 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.