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

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.

Update on the neurology of Parkinson's disease

“…There are a number of other neurodegenerative conditions which may present with PD-like features, that is parkinsonism, Multiple System Atrophy (MSA) being the one most likely to be seen by Urologists, because urinary incontinence is often a premonitory symptom [Beck et al, 1994]. MSA (formerly known in its advanced stages as Shy Drager syndrome) is characterised by a variable combination of parkinsonism, cerebellar ataxia and autonomic failure [Wenning et al, 1994].…”

“…Approximately 50% of patients are initially misdiagnosed as having PD [Quinn, 1995] and since urinary symptoms occur so early [Beck et al, 1994;Sakakibara et al, 2000] the commonest presentation to a Urologist will be of a patient of either gender (mean age 52 years) usually complaining of urinary urgency and urgency incontinence [Beck et al, 1994] or much less commonly, urinary retention. The patient may at this stage have subtle symptoms of other neurological involvements with a somewhat expressionless face, impaired movements and an early akinetic rigid syndrome, or a cerebellar syndrome with unsteadiness and dysarthria.…”

“…Antimuscarinics often provide considerable relief from symptoms of an overactive bladder and a raised post-micturition residual volume can be successfully managed by intermittent self-catheterisation at a stage of the disease when the patient is otherwise only moderately disabled [Beck et al, 1994]. Desmopressin at night may improve nocturnal frequency, a symptom that can be a particular problem if there is postural hypotension with low standing blood pressures during the day.…”

“…Why ED either precedes or accompanies urinary incontinence in men [Beck et al, 1994;Kirchhof et al, 2003] is unknown. Studies that have looked at the sequence of clinical symptoms as well as laboratory investigations showed that ED is not secondary to orthostatic hypotension [Kirchhof et al, 2003]; and the suggestion that it is due to a central dopamine failure is an attractive hypothesis.…”

Erectile and urinary dysfunction may be the presenting features in patients with multiple system atrophy: a retrospective study

“…SpEMG was performed according to the method described in Beck et al 24 and Palace et al 20 using a concentric needle electrode inserted into the subcutaneous layer of the external anal sphincter about 2 cm from the external anal orifice. The mean duration of 10 motor units was calculated and values greater than 10.0 ms were considered abnormal.…”