Current terminology[ edit ] The current terminology and diagnostic criteria for the disease were established at a conference of experts and set forth in a position paper. It is sometimes termed striatonigral degeneration, parkinsonian variant. It is sometimes termed sporadic olivopontocerebellar atrophy. Supervision[ edit ] Ongoing care from a neurologist specializing in movement disorders is recommended,[ by whom? A recent trial reported that only 1.
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Actualmente, es vigilada de forma ambulatoria. El manejo de las complicaciones es relativamente complicado y con resultados poco satisfactorios. Estos pacientes generalmente derivan en una incompetencia esfinteriana, por lo que la meta frecuentemente consiste en facilitar el almacenamiento urinario. AbstractBackgroundShy-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 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 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.
AimTo report on the urologic implications and management in a patient with Shy-Drager syndrome. ResultsFollowing 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. 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.
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. Year:
Síndrome de Shy-Drager: causas, síntomas, tratamiento, pronóstico, esperanza de vida
Atrofia de múltiplos sistemas
Atrofia multisistémica. Síndrome Shy-Drager