Wolfram Syndrome Guide for Urologists

Management of WS urological often requires involvement of urologists, rehabilitation physicians and neurologists.
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Urge incontinence due to bladder dyssynnergia or neuropathic bladder has been reported in up to 60% of affected people. These features can present during childhood. The pathology is not well understood, but it is thought that there is a loss of upper motor neuron control over the detrusor muscle; in addition, autonomic nervous system dysfunction may lead to altered bladder sensation. Reports from case series show pelviureteric dilatation on ultrasound scans. The part played by cranial diabetes insipidus is unclear.

Baseline investigations

• Ask about urinary symptoms, complete a voiding diary, undertake a clinical examination for evidence of neurological involvement.
• Check renal function (blood electrolytes, urea, creatinine, glomerular filtration rate (GFR))
• Bladder and renal ultrasound 
• Urodynamic testing to include flow rates on emptying the bladder, and residual bladder volume.

Management may involve:

• Advice about double voiding technique ; electrical stimulation; anticholinergic drugs to stabilise the detrusor muscle; botulinum toxin; clean intermittent self-catheterization; permanent indwelling catheter; ileal conduit surgery.

• Screening for UTI
• Urine culture if fever or other symptoms
• Intermittent self-catheterization
• Preliminary assessment of the ability to self-catheterization, taking into account ataxia, low vision or cognitive deficiency (PP-Test)
• Indwelling urinary catheter
• Risk factors for bladder tumors
• Standard management for sexual dysfunction