Prior reports in the gynecology and sexual medicine literature have described persistent genital arousal disorder as a syndrome of unprovoked, excessive, and frequently unremitting sexual arousal that causes distress and interferes with quality of life.1–3 The typical symptoms previously described for persistent genital arousal disorder are shown in Box 1. However, little attention has been paid to this diagnosis as a result of the lack of a readily identifiable cause and the sexual nature of the disorder.
Box 1 Persistent Genital Arousal Disorder Diagnostic Criteria Cited Here...
- Persistently aroused genitalia
- Arousal is unrelated to desire
- Arousal is triggered by both sexual and nonsexual stimuli
- Symptoms are intrusive and unwelcome
- Arousal remains despite orgasm or requires multiple orgasms to diminish
We encountered a group of patients with persistent genital arousal disorder in a neurosurgical practice among a larger study cohort undergoing surgical treatment for symptomatic spinal meningeal cysts. The patients with persistent genital arousal disorder were among a group with meningeal cysts in the sacral spinal canal causing sacral nerve root compression, suggesting a causal relationship. In the spine, symptomatic compression of a nerve root produces a corresponding radiculopathy. We therefore further conjectured that persistent genital arousal disorder could be a previously undescribed type of radiculopathy symptom, because it was typically present along with other sacral radiculopathy symptoms such as sacral, perineal, and buttock pain or numbness; bladder, bowel, and sexual dysfunction; and dyspareunia.
If these hypotheses were correct, it seemed possible to alleviate persistent genital arousal disorder symptoms by neurosurgically treating the meningeal cyst causing sacral nerve root compression. The postoperative outcomes relating to persistent genital arousal disorder we observed are herein described.
The most common types of meningeal cysts found in the sacral spinal canal, known as extradural meningeal cysts, are shown in Figure 1.4,5 They differ from intradural meningeal cysts such as arachnoid cysts, which arise inside the spinal sac. Instead, extradural cysts typically arise from the dura of the spinal sac or nerve roots and project outward into the spinal canal. The most prevalent types of extradural meningeal cysts are perineurial (also known as Tarlov) cysts and meningeal diverticula. Also not uncommonly seen are ectatic spinal sac cysts, a distinct type of meningeal cyst, in which the spinal sac does not come to a discreet tapered end in the upper sacrum, but instead balloons out to fill and expand the entire sacral spinal canal. Meningeal diverticula and ectatic spinal sac cysts are typically singular, whereas Tarlov cysts can be singular or multiple. Patients with ectatic spinal sac cysts have a higher incidence of associated connective tissue disorders such as Marfan's or Ehlers-Danlos syndrome.
The mentioned meningeal cysts are all in direct communication with the spinal sac and are filled with cerebrospinal fluid. When present in the sacral spinal canal, they can expand and cause compression of the adjacent S1, S2, S3, and S4 nerve roots. This nerve root compression can in turn produce sacral radiculopathy symptoms. The laterality and type of symptoms produced depend on the location of the cyst in the spinal canal and which nerve roots it is compressing. Magnetic resonance imaging (MRI) is the gold standard for diagnosing spinal meningeal cysts.
MATERIALS AND METHODS
Our practice has identified patients with symptomatic spinal meningeal cysts as part of an ongoing institutional review board-approved prospective surgical outcomes study that began in 2008. Institutional review board approval was granted at Medical City Hospital, Dallas, Texas, and Research Medical Center, Kansas City, Missouri. As part of this study, we first encountered a patient who fit the persistent genital arousal disorder criteria in Box 1 in January of 2009. From that time forward we continued to consecutively accrue a case series of similar patients with persistent genital arousal disorder as a subgroup in the larger cohort. In each case patients had to have the typical symptoms of persistent genital arousal disorder and an MRI that confirmed the presence of one or more meningeal cysts in the spinal canal causing nerve root compression.
The decision to proceed with surgery was based on the correlation of findings in the patient history, physical examination, and imaging studies. All patients were required to have exhausted nonoperative treatment modalities such as pain clinic management, physical therapy, nonsteroidal anti-inflammatory drugs, narcotics, pain modulating drugs, etc.
Preoperative data collection included the age, sex, race, type of meningeal cyst involved, and how long patients had experienced persistent genital arousal disorder. All patients underwent prone position surgery utilizing the operating microscope and intraoperative nerve monitoring. A sacral laminectomy was carried out to expose symptomatic cysts. The surgical strategy for meningeal cyst treatment varied depending on the cyst type involved, but in all cases, the goal of surgery was spinal nerve root decompression. Further information on spinal meningeal cyst surgical techniques is available.4,6,7
Postoperatively, patients were asked to self-report whether their persistent genital arousal disorder was eliminated, significantly improved (ie, changed from previously constant to intermittent only or from intermittent to rare episodes), the same, or worse. This information was obtained in tandem with the outcomes assessments and MRIs for the larger study cohort at 3 months, 6 months, 1 year, and 2 years after surgery. An additional inquiry into the status of their persistent genital arousal disorder was currently obtained from patients beyond 2 years after surgery.
As summarized in Table 1, we identified 11 patients with persistent genital arousal disorder out of a cohort of 1,045 with surgically treated symptomatic spinal meningeal cysts. All 11 were female, aged 33–69 years old, and mean age of 46 years. All patients had a meningeal cyst in the sacral spinal canal causing sacral nerve root compression. The length of time patients had experienced persistent genital arousal disorder preoperatively ranged from 1 to 41 years. All 11 patients also had one or more accompanying symptoms typical of sacral radiculopathy.
The type of meningeal cyst encountered varied (Figs. 2 and 3). Tarlov cysts were found in eight patients, two had a meningeal diverticulum, and one had an ectatic spinal sac cyst. The patient with the ectatic spinal sac cyst also carried the diagnosis of Marfan's syndrome. None of the other patients had a connective tissue disorder.
In the patients with Tarlov cysts, the number of cysts treated in each case ranged from two to five with a mean of three cysts per patient. Each Tarlov cyst arises from a single spinal nerve root, and the most common nerve roots that were Tarlov cysts were S2 and S3. The nerve roots that were most often compressed by a Tarlov cyst were S2, S3, and S4. The follow-up periods at the time of this report ranged from 2 to 72 months with a mean of 23 months. One patient was lost to follow-up 5 years after surgery as a result of death attributable to unrelated causes.
Postoperatively, seven (64%) patients reported complete elimination of their persistent genital arousal disorder, three (27%) said their symptoms were significantly better than before surgery, and one (9%) stated that their symptoms were unchanged. None reported that their persistent genital arousal disorder was worse. In other words, 91% of the patients stated that their persistent genital arousal disorder symptoms were significantly better or eliminated after surgery. In two patients a preoperative diagnostic nerve block at the site of their intrasacral cysts transiently improved their symptoms, lending further support to the need for cyst surgery. Both patients had Tarlov cysts. After surgery, one reported their persistent genital arousal disorder was gone, and one said it was significantly better than before surgery.
Postoperative MRIs revealed no recurrent or residual intrasacral meningeal cysts in any of the patients at any time point with good sacral nerve root decompression in all cases. There were no surgical complications requiring rehospitalization or reoperation.
In this case series of 11 women with persistent genital arousal disorder and sacral nerve root compression by a meningeal cyst, 91% had elimination or improvement of persistent genital arousal disorder after surgical decompression of the cyst. This supports a causal role for such cysts in some cases of persistent genital arousal disorder. As yet, it is unknown what the incidence of persistent genital arousal disorder in individuals with sacral nerve compression from meningeal cysts nor what the incidence of these cysts is in women with persistent genital arousal disorder.
The incidence of spinal meningeal cysts in the general public is unclear. One study of 500 patients undergoing a lumbar MRI for back pain identified one or more spinal meningeal cysts in 5%.8 In approximately 1% of these, the cyst was felt to be the source of the symptoms. In our unpublished cohort of 1,045 surgically treated patients with symptomatic spinal meningeal cysts of various types, 553 surgeries were for Tarlov cysts, and among these, 90% of patients were female and only 10% male with an average age of 50 years.
A prior publication in 2012 also identified that several patients with persistent genital arousal disorder had Tarlov cysts.9 We found that eight of our patients with persistent genital arousal disorder had Tarlov cysts. However, it is clear that Tarlov cysts are not the only type of meningeal cyst found in the sacrum that can cause persistent genital arousal disorder. In this series of 11 patients with persistent genital arousal disorder, we also found other types of sacral meningeal cysts, including meningeal diverticula and an ectatic spinal sac cyst. The commonality they all shared was that the meningeal cysts were causing sacral nerve root compression. It therefore seems that the presence of sacral nerve root compression is more important than the particular type of meningeal cyst involved.
From a neurological perspective, it seems logical that sacral nerve root compression by a meningeal cyst could be causally related to persistent genital arousal disorder. Anatomically speaking, the dermatomes of the lower sacral nerve roots cover the perineal area and they play a major role in sexual function. In our series we found that persistent genital arousal disorder was present along with other sacral radiculopathy symptoms in all 11 patients, implying that persistent genital arousal disorder is also a form of sacral radiculopathy. Additionally, in our larger cohort we have found patients with other sexual dysfunctions. These dysfunctions are typically related to the loss of perineal sensation resulting in loss of sexual pleasure or intolerable skin hypersensitivity resulting from paresthesias. In the context of these findings, it seems possible that persistent genital arousal disorder is one of several forms of sexual dysfunction potentially caused by sacral nerve root compression.
Based on our series and that of Komisaruk and Lee,9 we recommend lumbar MRI with and without contrast and with attention to the sacral region in patients with persistent genital arousal disorder, particularly when accompanied by other sacral radiculopathy symptoms.
There is an association between sacral nerve root compression by spinal meningeal cysts and persistent genital arousal disorder and it is reasonable to conclude that it is causal. The presence of sacral nerve root compression seemed to be more important than the particular type of meningeal cyst involved. In our series, persistent genital arousal disorder typically presented along with other sacral radiculopathy symptoms. Surgical treatment of the sacral meningeal cysts resulted in significant improvement or elimination of persistent genital arousal disorder in 11 of 12 patients. We recommend that patients with persistent genital arousal disorder undergo MRI to rule out the presence of intrasacral meningeal cysts.
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© 2015 by The American College of Obstetricians and Gynecologists. Published by Wolters Kluwer Health, Inc. All rights reserved.
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