Síndrome de Shy Drager, Repercusiones Urológicas

Background

Shy-Drager syndrome, also known as multiple system atrophy, is a neurodegenerative entity of autoimmune etiology. Clinical symptoms encompass Parkinsonism and cerebellar and autonomous symptomatology, including urinary and sexual alterations and cortical dysfunction of the pyramidal pathway. There is little drug response. Incidence is similar by sex, disease onset is in adulthood, and it is a progressive pathology with a poor prognosis.

Case 1: We present herein the case of a 39-year-old woman that presented with signs of neurocardiogenic syncope and dysautonomia 5 years prior to her present hospital admission. That evaluation protocol showed data of sinus bradycardia, treated with a pacemaker, as well as gastric intolerance and urinary retention. Due to the insidious progression of her illness, the clinical data, and auxiliary diagnostic studies, the diagnosis of multiple system dysautonomia, or Shy-Drager syndrome, was made. In relation to urology, the patient presented with recurrent urinary infections and urinary retention on numerous occasions. A urodynamic study was performed, but was inconclusive due to a dysautonomic event during the study. In the filling cystomanometry, storage was 700 cc, but the patient was unable to micturate. Given the above, long-term management with clean intermittent catheterization was decided upon, but it could not be achieved due to another dysautonomic event, and therefore a transurethral catheter was left in place. The patient sought an alternative, because she wanted to continue to have an active sex life and the urethral catheter caused episodes of intense dyspareunia and dysautonomia. Thus, the joint decision was made to perform cystostomy, which the patient retains today.

Aim

To report on the urologic implications and management in a patient with Shy-Drager syndrome.

Material and methods

The case record of a patient diagnosed with Shy-Drager syndrome was employed and she was treated for urologic complications at the Hospital Ángeles Pedregal.

Results

Following medical-surgical management, the patient was released to her home 3 days after her hospital admission due to clinical improvement. Currently she is being monitored as an outpatient.

Discussion

Shy-Drager syndrome was first described in 1960 by Milton Shy and Glenn Drager. It is classically described as the presence of orthostatic hypotension, anhidrosis, and cerebellar dysfunction and/or Parkinsonism of varying magnitude. Alterations in bladder voiding and erectile dysfunction are the predominant urologic disorders. In the case presented herein, the patient had been previously treated for cardiovascular problems and she is currently receiving satisfactory management for urologic complications.

Conclusions

Urologic symptoms have been observed in 60% of the patients that present with this pathology. Unfortunately, there is no effective therapy, and so the aim is to control the symptomatology that is mainly related to thermal and cardiovascular aspects of dysautonomia. The quality of life of these patients is poor and outcome is bleak. The management of complications is relatively complex and the results are not very satisfactory. These patients generally end up with sphincteric incompetence and so the goal is to facilitate urinary storage.