Diagnosis and Treatment of Persistent Genital Arousal Disorder

November 2017

Case of the Month Diagnosis and Treatment of Persistent Genital Arousal Disorder NYU Case of the Month, November 2017 Seth D. Cohen, MD Department of Urology, NYU Langone Medical Center, New York, NY [Rev Urol. 2017;19(4):265–267 doi: 10.3909/riu0784] © 2018 MedReviews , LLC ® A woman in her early 40s presented with hyperarousal and bladder discomfort lasting for approximately 2.5 years. She had a horseback riding accident 3 years prior in which the horse bounced in the air and she landed on the hard saddle. The next morning, she noticed a sizable perineal hematoma that was confirmed with computed tomography imaging. However, no evidence of pelvic fracture or spinal cord defect was seen. The onset of her symptoms was temporally related to the pelvic trauma. The perineal pain and hematoma resolved within a few months, and she became sexually intimate again with her boyfriend. A few days after having intercourse, she developed a urinary tract infection and was placed on antibiotics. She began to have new pelvic symptoms, which she described as feelings of pressure and discomfort in the vagina and the bladder, with the additional symptom of arousal. These symptoms persisted daily despite additional antibiotics and anti-inflammatory agents. These symptoms have had a significant negative impact on her life, including causing poor sleep and making her unable to exercise because exercise triggers these persistent unwanted arousal episodes, which are accompanied by uncomfortable urinary urgency and pelvic pressure. She avoids sexual activity because she is afraid the discomfort will get worse. She has tried behavioral modifications to abate the arousal symptoms, including but not limited to masturbation, but nothing has alleviated the symptoms. The patient’s past medical history is significant for anxiety, irritable bowel syndrome, human papillomavirus infection, hypothyroidism, and an ovarian cyst (which, according to the patient, is resolved). Over the past 2.5 years, the patient has seen more than 20 doctors for this issue and has undergone extensive urologic workup and treatment. Procedure The patient underwent two rounds of urodynamic testing, the results of which were normal, and revealed no overactive bladder or urgency. After five cystoscopy procedures, no inflammation, Hunner ulcers, or any mucosal inflammation was found. The patient showed no evidence of improvement after three bladder instillations. Lifestyle Changes The patient tried a diet indicated to alleviate interstitial cystitis, a Paleo diet, and other diets, all without relief. Vol. 19 No. 4 • 2017 • Reviews in Urology • 265 4170007_10_RiU0784_V4_rev04.indd 265 1/25/18 5:15 PM Diagnosis and Treatment of Persistent Genital Arousal Disorder continued Other Interventions The patient underwent pelvic floor physical therapy and was told she has an extremely high-tone pelvic floor. She had mild yet transient improvement. Acupuncture yielded some reduction in anxiety but no improvement in her arousal and urgency symptoms. Medications Current medications include the following: (1) escitalopram, 20 mg/d, which has improved her overall anxiety but not improved her arousal and urgency symptoms; pregabalin (started 2 months ago)—she is currently taking 300 mg/d, titrating up toward 600 mg/d—which has provided mild improvement in symptoms. She discontinued gabapentin because it provided no improvement in her symptoms. Current Therapies The patient’s current treatments include functional medicine, acupuncture, physical therapy, and psychiatry. Evaluation A focused physical examination revealed a clitoral hood with no phimosis; no lesions were present on the labia majora or minora. No lesions were present on the interlabial sulci, and the vulvar vestibule showed normal, healthy mucosa. No prolapse was evident in the urethra; mild erythema was present on the periurethral glands, with pain upon palpation with cotton swab testing. Examination of the pelvic floor muscles revealed moderate discomfort, high tonicity, with uncontrolled contractions in the pubococcygeus, iliococcygeus, and puborectalis muscles bilaterally. The patient had intact sensation bilaterally. Deep bilateral palpation of the Alcock (pudendal) canal demonstrated a pudendal nerve trigger of the patient’s symptoms, confirming the diagnosis of persistent genital arousal disorder (PGAD) with a pudendal trigger. the patient reported a continued reduction of her PGAD symptoms .50%. She was offered intravaginal onabotulinumtoxinA injection at that time; however, she was pleased with the level of resolution of symptoms. She is scheduled for a 3-month follow-up for reassessment and additional injections, if warranted. Management Discussion Because of the pudendal nerve trigger, treatment for PGAD was offered. The high-tone pelvic floor dysfunction was secondary to an irritated and misfiring pudendal nerve. The patient was offered a bilateral pudendal nerve block followed by subsequent intravaginal onabotulinumtoxinA injections if the pelvic floor dysfunction responded to the pudendal nerve block. Procedure: Bilateral Pudendal Nerve Block The patient was brought to the procedure suite and placed in the lithotomy position; 0.25% lidocaine, 10 mL, was injected into the right and left Alcock canal to achieve a full bilateral pudendal nerve block.1 The patient tolerated the procedure well without complications. After the procedure, the patient was encouraged to walk outside for 30 minutes to test whether her symptoms had abated. After 30 minutes, the patient returned and was re-evaluated in the lithotomy position, including palpation of the Alcock canal. Her symptoms were reduced by more than 50%. The decision was made to complete the block with the addition of the corticosteroid triamcinolone. A total of 50 mg was injected, 25 mg into the right and 25 mg into the left Alcock canal; 2 weeks later, PGAD is a condition characterized by symptoms of physiologic (typically, genital) sexual arousal in the absence of perceived subjective sexual arousal. The physiologic arousal can last for hours or days, or it can be constant, and it does not typically remit after orgasm. Leiblum and Nathan2 first described PGAD in the scientific literature in a series of five case studies of women with varied experiences and diverse backgrounds. The authors described the symptoms as distressing, intrusive, unwanted, and sometimes painful. PGAD often causes significant distress and is associated with feelings of shame and isolation, and often with suicidal ideation. Although many etiologic hypotheses have been proposed, the cause or causes of PGAD remain unknown. It is likely not attributable to a single cause or biopsychosocial factor, and there may be subgroups of women with PGAD who develop the condition through a combination of factors. These hypothesized etiologies include central and peripheral dysregulation, vascular changes, meningeal cysts (most commonly, Tarlov cysts), and pharmacologic and psychologic explanations.3 Little is known about the frequency of occurrence or the validity of each hypothesized etiology because the majority of this work is 266 • Vol. 19 No. 4 • 2017 • Reviews in Urology 4170007_10_RiU0784_V4_rev04.indd 266 1/25/18 5:15 PM Diagnosis and Treatment of Persistent Genital Arousal Disorder in the form of individual case re­­ ports; in most cases, assessments (eg, physical, neurologic, cardiovascular, endocrine) yielded no abnormal findings. In the present study, the patient’s PGAD presumably arose from pelvic trauma; the pudendal trigger was identified and targeted and effective therapy was initiated.4 It is important to understand that these patients, similar to patients with interstitial cystitis, are never cured with one or two treatments. References 1. 2. 3. 4. Romanzi L. Techniques of pudendal nerve block. J Sex Med. 2010;7:1716-1719. Leiblum S, Nathan SG. Persistent sexual arousal syndrome in women: a not uncommon but little recognized complaint. Sex Relation Ther. 2002;17:191-198. Leiblum SR, Nathan SG. Persistent sexual arousal syndrome: a newly discovered pattern of female sexuality. J Sex Marital Ther. 2001;27:365-380. Rea W, Curran N. Abdominopelvic pain syndromes. Contin Educ Anaesth Crit Care Pain. 2015;15:38-43. Vol. 19 No. 4 • 2017 • Reviews in Urology • 267 4170007_10_RiU0784_V4_rev04.indd 267 1/25/18 5:15 PM