New observations of urological dysfunction occurring in Alström Syndrome

Two definite, and possibly three more patients with Alström Syndrome have manifested a progressive bladder/urethral inco-ordination. This has resulted in initial infrequency of passing urine and eventually retention of urine.

Unlike the typical paralyzed bladder which can occur in multiple sclerosis, strokes or paraplegia, the Alström Syndrome patients DO NOT fare well with indwelling urinary catheters. This is because of the incoordination of the bladder - so called detrusor/urethral dys-synergia. This continues to cause severe spasmodic pain and leakage of acid urine despite catheterisation. Two Alström Syndrome patients in the UK have benefited from an ileal conduit. The relationship between renal failure and the urological problems is as yet unclear.