Genitourinary Dysfunction in Multiple System Atrophy: Clinical Features and Treatment in 62 Cases

TLDR

Multiple system atrophy is a disorder of progressive neuronal atrophy in CNS regions that regulate urogenital function. The study stresses the need to recognize this disorder and to implement effective, nonoperative treatments. The authors describe the neuro‑urological features of 62 patients with MSA. All 62 patients showed abnormal sphincter EMG; impotence occurred in 96 % of men (first symptom in 37 %), urinary dysfunction stemmed from detrusor hyperreflexia and sphincter weakness, leading to misdiagnosis and unnecessary surgeries in 43 % of men and 57 % of women (half of whom had surgery), with poor surgical outcomes but marked improvement (82 %) after intermittent catheterization, anticholinergic medication, and desmopressin spray.

Abstract

Multiple system atrophy is a disorder characterized by progressive neuronal atrophy at certain sites of the central nervous system, several of which are important in the control of urogenital function. The neuro-urological features of 62 patients with this condition are described. All patients had abnormal urethral or anal sphincter electromyography when individual motor unit analysis was performed, a finding diagnostic of the condition in the appropriate clinical setting. Impotence occurred in 96% of the men and was the first symptom alone in 37%. Urinary symptoms resulted from a combination of detrusor hyperreflexia and urethral sphincter weakness followed by failure of detrusor contraction. In men these symptoms simulated those of outflow obstruction so that 43% underwent prostatic or bladder neck surgery before the correct diagnosis was made. Stress incontinence occurred in 57% of the women and of these half had undergone surgery. The results of surgery in both sexes were poor. Treatment with intermittent catheterization, anticholinergic medication and desmopressin spray markedly improved continence in 82%. The importance of recognizing this disorder and introducing effective, nonoperative treatments is stressed.