The Condition That Isn't As Fun As It Sounds ...

We are tackling the diagnosis of Persistent Genital Arousal Disorder/ Genito-Pelvic Dysesthesias. A panel from The International Society for the Study of Women’s Sexual Health (ISSWSH) congregated in 2019 to discuss the latest research and put forth the consensus paper we’ll review today. Here are the things we’ll discuss:

Afterwards, we’ll review how pelvic health providers help patients in ways the article didn’t discuss.

Overwhelmed by biased news? Cut through the clutter and get straight facts with your daily 1440 digest. From politics to sports, join millions who start their day informed.

Sign up now!

Goldstein et al (2021) published a consensus paper for The International Society for the Study of Women’s Sexual Health (ISSWSH) on the epidemiology, contributing factors, diagnosis, and treatment of Persistent Genital Arousal Disorder / Genito-Pelvic Dysesthesia (PGAD/GPD) in cis-gendered women.

Leiblum and Nathan first described the condition in 2001 and called it “Persistent Sexual Arousal Syndrome”. The name changed in 2006 to “Persistent Genital Arousal Disorder” (PGAD) after determining the problem was genital arousal, not sexual arousal. At the 2019 PGAD consensus meeting, genito-pelvic dysesthesia (GPD) was added to the name to include those experiencing concomitant lower extremity dysesthesia.

Epidemiology studies reported PGAD occurs in approximately 0.6% to 3% of the world population. The average age of primary onset is 18, while the average age of acquired onset is 37. 

A complex combination of biopsychosocial factors contribute to the development and maintenance of PGAD. These include:

Other relevant psychosocial factors include lack of clinician awareness of PGAD, the shame and embarrassment associated with PGAD, and the limited treatment options.

Women with PGAD are more likely to have a history of previous sexual abuse than (46.7-52.6%) women without a history. Many women with PGAD/GPD report that stress (33.98%), anxiety (29.13%), and loss (13.59%) initially triggered their symptoms.  Suicidal ideation was present in 54% of women with PGAD - more than double of the control group without the condition.

Early in the development of PGAD/GPD, symptoms may begin as increased genital awareness before progressing to the development of dysesthesia symptoms with possible orgasm/ejaculations. Neuropathy affecting the nerve or nerve root can lead to the nerve hyperfunctioning (creating symptoms of arousal, pain, and/or itch) or hypofunctioning (creating symptoms of genital anesthesia, anorgasmia, and/or anejaculation). Other patients reported a spontaneous onset of symptoms accompanied by pain before the onset of PGAD/GPD symptoms.

Discuss standard symptom questions (such as onset, location of symptoms, aggravating and easing factors), and don’t forget to ask about the following:

Clinicians also need to assess patient cognition and emotion state and their impact the patient’s symptoms. With higher incidence of suicide in this population, it is imperative that clinicians ask about the patient’s social support network and explicitly ask about suicidal ideation, intent, and plans.

Triggers may originate in one or more of 5 regions:

Click here to see the flowsheet

The authors recommend that physical examination beginning with a detailed assessment of regions one and two.

If the contributors cannot be identified, then the patient is considered to have an idiopathic form of PGAD.

Areas involved: Clitoris, vulva, vestibule, vagina, urethra, bladder, perineum, and perianal areas.

Areas to examine: Urethra, urethral meatus, trigone, bladder 

Patients with GPAD/GPD may complain about the sensation of genital engorgement. While there will be no signs of arousal of physical exam, there may be unilateral changes in sensation. 

Anesthesia testing - A topical compounded anesthetic is applied affected structure. Any clinically significant improvement in symptoms indicates end organ as the source. If symptoms persist, look to regions 1-4.

Genital quantitative sensory testing - Sensory testing of the pudendal nerve dermatomes including clitoris (dorsal nerve), vestibule (perineal nerve), and perianal region (inferior hemorrhoidal nerve) with various stimuli targeting different nerve fiber types.

Non-genital sacral dermatome screening – Patient lies prone and vibration perception is tested at gluteal dermatomes(S1-4), posterior thigh dermatomes (S1-2), and posterior calf dermatomes (S1-2).

Bulbocavernosus reflex latency testing

Underlying pathology of the vulva, vestibule, vagina, urethra, and/or bladder associated with PGD should be treated. Nerve blocks and botox injections also may be indicated.

PGAD symptoms associated with cliterodynia may have clitoral adhesions between the precipice and the glans. Adhesions can be released in physician’s office under local anesthesia. There is no data to support a cliterodectomy as a treatment method for PGAD – in fact, dysesthesia symptoms often persist or become exacerbated. 

Areas involved: Pelvic Girdle and extra pelvic region muscles and connective tissue (such as the spine, abdomen, and hips).

Areas to examine: Pelvic floor (Look at the ability to contract, relax, lengthen; all layers of muscle and connective tissue; PERF). Overactivity can also be measured with SEMG or transperineal ultrasound (by looking at the anorectal angle). The pudendal nerve is often blamed for chronic pelvic pain disorders since it includes both sensory and motor components. Pudendal neuropathy may result from trauma, entrapment, neuroma or compression.

Additional contributors:  Vascular pathologies (pelvic congestion syndrome and pelvic arteriorvenous malformation), damage to abdominal wall somatic afferent nerves (such as iliohypogastric nerve, ilioinguinal nerve, and genitofemoral nerve) from laparoscopic/robotic surgery, or damage to nerves during radical hysterectomy (such as the pelvic nerves, hypogastric nerve, and vagus nerve).

Pelvic floor therapy improves pelvic floor tension/overactivity and addresses pudendal neuropathy though manual therapy, neuromuscular re-education, education, therapeutic exercise. Discourage kinesiophobia and hypervigilant behavior that may perpetuate symptoms. Encourage movement that reduces symptoms and other self-management tools (like TENS unit). Pain neuroscience education is beneficial here.

Interventions impacting nerve function, such as pudendal nerve blocks, can be used for testing and can provide relief. Neurolysis may be used to address abdominal wall nerve involvement. 

Other interventions may target vascular causes. These should be performed by vascular interventionists.

Areas involved / to examine: Lumbar spine, Sacroiliac joint, Sacral nerve roots

Other potential contributors: Temporary iatrogenic radiculopathy following sacral nerve stimulation, Tarlov cysts/ meningeal diverticulum, history of connective tissue disorders (like Ehlers-Danlos Syndrome), a history of physical trauma to the area, lumbar/lumbosacral pathology.

A note on cysts: Tarlov cysts typically occur at the dorsal root ganglia at S2-3 near the internal surface of the perineurium where the dura matter transitions to weaker perineurium. A meningeal diverticulum can also form proximal to the dorsal root ganglia and nerve roots. These cysts, which are indistinguishable from each other, irritate the nerve causing pain and anesthesia of the clitoris, vagina, or urethra as well as dysesthesias and pain down the buttock, the back of the leg, and the side of the foot. 

MRI (T2 weighted imaging) will visualize of a Tarlov cyst or meningeal diverticulum that irritates nerve root fibers.

Neurogenital quantitative sensory testing (discussed in region 5) when lumbosacral radiculopathy is suspected. 

Nerve blocks performed at the hypothesized source by a pain management physician. Significant clinical improvements following injections in the lumbar spine may indicate the origin of symptoms. These patients may benefit from surgery.

Pelvic health rehab interventions should assess lumbar spine mobility, sacroiliac joint

Areas involved: Lateral and ventral spinothalamic tracts, spinoreticular tracts, spinohypothalamic tracts of the spinal cord (the ascending pathways from the genitals to the brain that transmit pain and other sensations). 

Areas to examine: Cervical and thoracic spine (looking for typical pathology – annular tears, herniations, stenosis, facet problems, synovial cysts). Inflammation of the neural pathway from the brain stem to the spinal cord generating intense sensory activity.

Potential Causes: Trauma to the area. Beginning or ending SSRI/SNRI medications. These medications act on neural pathways descend from the brainstem and connect with second order opioid interneurons that modulate incoming painful stimuli at the level of the spinal cord via the pain gate mechanism.

MRI imaging of the cervical and/or thoracic spine.

Authors did not list clear recommendations for pelvic rehab interventions. Things a pelvic health rehab provider might look at and address are cervical spine and thoracic spine mobility, dural mobility / nerve mobility, diaphragm mobility, postural muscle / periscapular muscle strength, and breathing strategy.

Areas involved: Paracentral gyrus, insula (posterior insular gyrus), cingulate cortex, amygdala, hippocampus, medial preoptic area, ventral tegmental area, and the mesolimbic/mesostriatal systems.

Potential causes: Organic brain pathology (such as TBIs, epileptic seizures, arteriovenous malformations, aneurysm, or other space occupying lesions). Exposure or withdrawal from SSRIs, SNRIs, and other CNS active medications due to their ability to act on the “pain-gate” system of the spinal cord. Discontinuation or reintroduction of the medication correlates with improvement of symptoms.

Trazadone is also associated with priapism (persistent genital engorgement) due to its role as an alpha-1 adrenergic antagonist. It also affects histamine transport in the brain which would increase dopamine-mediated processing of genito-pelvic dysesthesias and intensified sexual arousal.

MRI, EEG, and magnetic EEG.

While there no approved pharmacologic interventions for GPAD/GPD, there are some off-label options:

Psychological factors play a role in perpetuating the vicious cycle of symptoms. The spontaneous onset of symptoms often increased anxiety and sympathetic nervous system drive. With an upregulated nervous system, there is an increase in arousal sensation and increased focus on these sensations by the patient.

When a patient perceives their sensations as threatening, they demonstrate avoidance behaviors of activities causing genital arousal. These behaviors contribute to negative emotional states and behaviors (ex: depression, anxiety, interference work and social life) that fuel the unwanted genital sensations and perpetuate the cycle.

These patients benefit most when addressing the aforementioned psychological concerns in conjunction treatment.

This looks like:

Source: Irwin Goldstein, Barry R. Komisaruk, Caroline F. Pukall, Noel N. Kim, Andrew T. Goldstein, Sue W. Goldstein, Rose Hartzell-Cushanick, Susan Kellogg-Spadt, Choll W. Kim, Robyn A. Jackowich, Sharon J. Parish, April Patterson, Kenneth M. Peters, James G. Pfaus, International Society for the Study of Women’s Sexual Health (ISSWSH) Review of Epidemiology and Pathophysiology, and a Consensus Nomenclature and Process of Care for the Management of Persistent Genital Arousal Disorder/Genito-Pelvic Dysesthesia (PGAD/GPD), The Journal of Sexual Medicine, Volume 18, Issue 4, April 2021, Pages 665–697, https://doi.org/10.1016/j.jsxm.2021.01.172

This article targeted the entire medical community - not just rehab professionals. It suggests that pelvic rehab providers impart the most benefit to these patients when the source of pain occurs in region two (pelvis and perineum); however, other medical providers often refer patients to rehab when they have no ideas on what to offer their patients.

Listening to the patient’s story and the physical exam (both pelvic health and orthopedic focused) determines the starting point in management algorithm. Refer them to the appropriate provider, if necessary, but also give them something actionable so the patient doesn’t feel “brushed off”. This can look like coaching the patient on what to emphasize when talking to other providers so the other provider (hopefully) listens, encouraging documentation of symptoms (aggravating factors vs easing factors), or encourage behaviors to prevent catastrophization or hypervigilent behaviors.

Reassure the patient by tying their symptoms into the typical presentation. This validates the patient’s experience - something they don’t often experience - and facilitates patient buy in.

Look at the region before and after your hypothesized region. In orthopedic rehab, you address the area proximal/superior and distal/inferior to the affected area - this should be no different here. If the patient presents with region two symptoms, look at the the structures of regions one and three. Was phimosis ruled out? Does the patient have limitations in upper lumbar mobility and a poor breathing strategy? Is neural tension present?

If this email helped you in any way, please pass it along to colleagues who would benefit from this information (or from future emails).

You can let me know what you think by replying to this email, answering this google survey, or responding to the poll below.

Have a great week 😃