Case Report: A case of neurogenic bladder in the setting of Behçet's disease after an initial diagnosis of multiple sclerosis

Alexander W Pastuszak,Timothy B Boone

doi:10.12688/f1000research.6261.1

Abstract

Behçet’s disease (BD) is an autoimmune vasculitis with an unclear etiology presenting with a classic triad of symptoms including oral and genital ulcers as well as iridocyclitis. A subset of BD patients exhibit neurological symptoms including psychiatric disturbances, balance problems, and voiding dysfunction, and the symptoms of BD can mimic other neurological diseases, including multiple sclerosis (MS). Differentiating between potential diagnoses is challenging due to the lack of specific tests for these disorders and the overlap between clinical symptoms and radiological findings. We describe the case of a 52 year old woman initially diagnosed with and treated for MS. From the urologic standpoint, she was treated for neurogenic detrusor overactivity with detrusor-sphincter-dyssynergia utilizing ileocecal augmentation cystoplasty with a continent stoma for intermittent catheterization. The patient was later diagnosed with BD in light of additional clinical findings.

Highlights

Behçet’s disease (BD) was first described by the Turkish dermatologist Hulusi Behçet in 1937 as a triad of clinical symptoms consisting of recurrent uveitis and aphthous ulcers of the mouth and genitalia[1]
Micturitional disturbances have been found in 5–67% of patients with neurologic BD and these have been linked to pontine lesions affecting the pontine micturition center (PMC), pontine urine storage center (PUSC), and the nucleus reticularis pontis oralis (PoO)[1,3,5]
The patient was diagnosed with multiple sclerosis (MS) on the basis of imaging findings as well as clinical symptoms that were consistent with the diagnosis, including central nervous system (CNS) plaques in a distribution typically associated with MS, cranial nerve palsy, balance difficulties, and upper extremity tremors

Summary

Background

Behçet’s disease (BD) was first described by the Turkish dermatologist Hulusi Behçet in 1937 as a triad of clinical symptoms consisting of recurrent uveitis and aphthous ulcers of the mouth and genitalia[1]. The patient underwent ileocecal augmentation cystoplasty with continent stoma in February 2004 without complications[18] Her post-operative course was unremarkable and on subsequent office visits, she was doing well with much improved bladder symptoms consisting primarily of infrequent bladder spasms and occasional urinary tract infections with no urethral incontinence. A larger study evaluating 24 subjects with neurological BD demonstrated a 50% incidence of detrusor overactivity and 12.5% incidence of detrusor-sphincter-dyssynergia[3] These patients generally had high post-void residuals, early first sensation, and low-normal bladder capacities, which mimic the findings in our patient. Given the incomplete response to oral medications in our patient, as well as the desire to be free of an indwelling catheter in the setting of limited dexterity, ileocecal augmentation cystoplasty with a continent stoma was performed with a notable decrease in urgency symptoms and increase in bladder capacity postoperatively. In agreement with these studies, we found our patient to have no uninhibited contractions as well as improved bladder compliance and increased bladder capacity after augmentation cystoplasty

Findings

Conclusions

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