Ramsay Hunt Syndrome

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Continuing Education Activity

Ramsay Hunt syndrome, also known as herpes zoster oticus, is a late complication of varicella-zoster virus infection that results in inflammation of the geniculate ganglion of cranial nerve VII. Ramsay Hunt is a clinical diagnosis and classically is described as a triad of ipsilateral facial paralysis, otalgia, and vesicles near the ear and auditory canal. Diagnosis is often missed or delayed, which can lead to an increased incidence of long-term complications. The condition is self-limiting, but treatment is targeted at decreasing the total duration of the illness as well as providing analgesia and preventing the complications that can occur. This activity reviews the role of the interprofessional team in the diagnosis and treatment of Ramsay Hunt syndrome.

Objectives:

  • Identify the clinical signs and symptoms of Ramsay Hunt syndrome.
  • Review the evaluation of patients with suspected Ramsay Hunt syndrome.
  • Outline the management options available for Ramsay Hunt syndrome.
  • Discuss interprofessional team strategies for improving care coordination and communication to improve patient outcomes for Ramsay Hunt syndrome.

Introduction

Ramsay Hunt syndrome, also known as herpes zoster oticus or geniculate ganglion herpes zoster, is a late complication of varicella-zoster virus (VZV) infection, resulting in inflammation of the geniculate ganglion of cranial nerve CVII.[1] The syndrome is named for James Ramsay Hunt (1872-1937), an American neurologist and army officer in World War I who described three different syndromes, the most famous of which is the second, which is discussed herein as "Ramsay Hunt syndrome."[2] Early stages of VZV infection cause fever and diffuse vesicular rash, a condition that is commonly referred to as chickenpox. After the initial infection, the virus will often remain dormant in the body. Subsequent reactivation of the virus causes a "zoster" or "herpes zoster" phenomenon. This syndrome consists of pain and a vesicular rash along the involved nerve's distribution, typically corresponding to a single dermatome. The distribution and associated symptoms depend on the nerve involved. Less than 1% of zoster cases involve the facial nerve and result in Ramsay Hunt syndrome.[3] 

Ramsay Hunt syndrome is characterized by a classic triad of ipsilateral facial paralysis, otalgia, and vesicles in the auditory canal or on the auricle.[4] Additional symptoms include a change in taste sensation, dry eye, tearing, hyperacusis, nasal obstruction, and dysarthria. Hearing loss, tinnitus, and vertigo can be seen with the involvement of the vestibulocochlear nerve, and hoarseness or aspiration may indicate involvement of the vagus nerve. Vesicles may develop after the onset of neurologic symptoms, but the first presenting symptom is usually pain.[5] The vesicles typically appear on the auricle but can be seen along the affected side of the face, scalp, palate, and tongue. In some cases, no vesicles are present, and the patient's chief complaints are severe pain and facial palsy; this variant is known as zoster sine herpete and can be very difficult to distinguish clinically from Bell palsy.

Etiology

The causative agent in Ramsay Hunt syndrome is the varicella-zoster virus, a member of the human herpesvirus family. More specifically, it is part of the alphaherpesvirinae subfamily, along with herpes simplex viruses 1 and 2 (HHV-1 and HHV-2). VZV is a double-stranded DNA containing a virus, more technically known as human alphaherpesvirus 3 (HHV-3).[6] Once the clinical VZV infection, chickenpox, has cleared, the virus remains latent in cranial nerve or dorsal root ganglia and may subsequently reactivate in times of physiological stress or immunocompromise, leading to herpes zoster, known as "shingles" anywhere on the body or "Ramsay Hunt syndrome" when facial paralysis is involved.[1] While the incidence of chickenpox and shingles have decreased dramatically since VZV vaccination became widely available in 1995, shingles and Ramsay Hunt syndrome have nevertheless been reported in patients who have never contracted chickenpox but who have been vaccinated with live attenuated VZV.[7][8][9]

Epidemiology

Ramsay Hunt syndrome affects both immunocompetent and immunocompromised patients and has an incidence of about 5 per 100,000 people per year; in contrast, the incidence of Bell palsy is much higher, at about 15-30 per 100,000 people per year.[10][11] Ramsay Hunt syndrome accounts for roughly 7% of acute facial paralysis cases, with zoster sine herpete comprising approximately 10% of those.[12][13] Immunocompromised patients are likely to have a more severe disease process and less complete recovery. Ramsay Hunt syndrome can present in anyone, and there are cases reported in patients ranging from 3 months of age to 82 years, although patients in their 7th and 8th decades are most susceptible.[14] Factors that increase the risk of herpes zoster will increase the incidence of Ramsay Hunt syndrome, including stress, chemotherapy, immunocompromise, infection, malnutrition, and others.

Pathophysiology

Initial infection with the varicella-zoster virus causes a disseminated vesicular rash with fever, known as chickenpox. During the acute phase of this infection, the virus is spread by respiratory droplets. After the viremia and exanthem have resolved, the virus can remain dormant in cranial nerves and dorsal root ganglia. During times of physiological stress or immunocompromise, reactivation of the virus within the distribution of the nerve in which it has been dormant can occur. The rash is infectious, and the virus is shed from the vesicles; VZV can also be cultured from tears and saliva or identified on polymerase chain reaction (PCR) testing.

During an episode of zoster, vesicular rashes tend to appear within a single dermatome. In Ramsay Hunt syndrome, the virus reactivates along the seventh cranial nerve via the geniculate ganglion. According to Coulson et al., The initial presenting symptom is typically pain in the ipsilateral ear (55% of patients), with facial paralysis and vesicles appearing within 2 to 3 days. In 23% of patients, facial paralysis is the presenting symptom, and in only 2%, vesicles appear first. While 86% of their patients reported that the rash occurred only on the auricle, 7% only had vesicles in the oral cavity, and 8% had them in both locations.[5] Rashes have also been reported on the scalp and the cheek.

The proximity of the facial nerve to the vestibulocochlear nerve can result in hearing loss, tinnitus, and vertigo. Sensorineural hearing loss was present in 43% of patients in Coulson's series, imbalance or vertigo in 51%, and tinnitus in 20%.[5] Vagal nerve involvement is also probably more common than it appears to be. Unless the patient is symptomatic with hoarseness or aspiration, vocal cord paralysis is not usually noted because it requires mirror or fiberoptic laryngoscopy to discover.[15][16] Less frequently, other cranial nerves can be involved as well, including the trigeminal, glossopharyngeal, and hypoglossal, although cranial polyneuropathy is more likely to present in immunocompromised patients, such as those with diabetes mellitus or human immunodeficiency virus infection.[17][18][19][15]

The facial paralysis resulting from Ramsay Hunt syndrome has a worse prognosis than that seen in Bell's palsy, with only 70% regaining normal or near-normal facial function compared with over 90% in Bell palsy.[20][21] After Bell palsy, the rate of synkinesis development is roughly 16%, but it is closer to 40% after Ramsay Hunt syndrome.[12][22] The neuritis associated with Ramsay Hunt syndrome appears to be more severe than that of Bell's palsy, given that more than twice as many patients present with complete hemifacial paralysis with Ramsay Hunt syndrome as in Bell palsy.[5][12] 

When patients fail to recover premorbid function, synkinesis is a common sequela, in which aberrant reinnervation connects axons with neuromuscular junctions different from the ones to which they were connected prior to the inflammation. Severe neuritis can cause an injury similar to a traumatic one, wherein Wallerian degeneration of facial nerve axons occurs along with varying degrees of damage to the internal architecture of the nerve. When the endoneurium, perineurium, or epineurium are disrupted (as in Sunderland class III-V injuries), the axons no longer have a physical structure in place to guide reinnervation and may ultimately connect with the incorrect muscle or end-organ. In many cases, newly regenerated axons will also terminate on multiple neuromuscular junctions, which has the effect of further increasing dyscoordination as well as raising the resting tone of the muscles that subsequently have more associated axons than they did before the injury. Patients with synkinesis often complain of eye closure with mouth movements and vice versa, as well as facial tension and pain. Because the facial nerve supplies parasympathetic fibers to the lacrimal glands, patients may also develop gustatory lacrimation, or Bogorad syndrome, due to aberrant reinnervation; this causes tearing with eating. All of these issues, as well as the difficulties associated with acute facial palsy, can have a serious negative impact on patient quality of life.[23]

Histopathology

Microscopic evaluation with the Tzanck smear technique can be performed on the fluid obtained from the vesicles. This should reveal multinucleated giant cells using a Giemsa stain, methylene blue, or Wright's stain. The sensitivity of the test is low, but it has a high specificity when pre-test clinical suspicion is high. Distinguishing between herpes simplex, varicella-zoster, and cytomegalovirus infections can be challenging because they are all herpesviruses; the Tzanck smear is also used to identify pemphigus vulgaris, leprosy, and leishmaniasis.[24]

History and Physical

Ramsay Hunt syndrome is diagnosed on a clinical basis, as laboratory testing is often slow or impractical. Affected patients typically present with the classic triad of ipsilateral facial paralysis, otalgia, and vesicles on the auricle; however, patients who present early in the course of the disease may have pain only or lack either facial paralysis or a rash. The painful, erythematous vesicular rash can involve the auricle, auditory canal, scalp, cheek, tongue, or palate.[4] Often there is a prodrome with pain, fever, and fatigue for 1 to 3 days, followed by the onset of facial paralysis and exanthem.[5] The facial palsy typically takes 1 to 3 days to reach its nadir, with more rapid development often resulting in more severe paralysis. The rash generally begins as erythematous papules and then progresses to frank vesicles, which subsequently rupture and crust over within 1 to 7 days. The timing of the rash relative to facial paralysis is variable, but it may appear after the palsy.[14] 

Lesions can persist for 2 to 3 weeks, leaving erythematous macular scars.[14] While pain is the most common initial symptom, patients will often complain of myriad other symptoms during the acute phase of the syndrome: hyperacusis, dysgeusia, nasal obstruction, epiphora, xerophthalmia, drooling, dysarthria, smile asymmetry, vertigo, tinnitus, hearing loss, hoarseness, dysphagia, and facial numbness. This list reflects the broad range of functions controlled by the cranial nerves potentially affected in Ramsay Hunt syndrome: V, VII, VIII, IX, X, XII.[4] The sine qua non of Ramsay Hunt syndrome, however, are unilateral hemifacial paralysis and severe pain. When vesicles are absent, but severe pain precedes the acute facial palsy, patients are diagnosed with zoster sine herpete, which accounts for approximately 10% of Ramsay Hunt syndrome presentations, and can be challenging to differentiate from Bell's palsy, which also often presents with otalgia, though typically not severe.[13] Patients should also be asked about emotional status, as depression commonly accompanies facial palsy in some demographic groups, particularly young women.[23]

Generally, the most apparent feature on the physical examination will be unilateral hemifacial palsy, which will be complete in close to 50% of patients.[5] Facial analysis may reveal the absence of transverse forehead rhytides, brow ptosis, lagophthalmos, ectropion, effacement of the nasolabial fold, the collapse of the nasal valve, malposition of the oral commissure inferiorly, and absence of platysmal banding on the affected side. The philtrum and nasal base will also likely shift away from the paralyzed side. Examination of a facial paralysis patient should be performed in three distinct passes. The first should look for asymmetry at rest, evaluating the upper third of the face, followed by the middle, and then the lower face and neck. The second should assess each of the major extratemporal branches of the facial nerve in sequence: frontal by brow elevation, zygomatic by gentle and forceful eye closure, buccal by smiling, marginal mandibular by depression of the lower lip, and cervical by platysmal contraction. Evaluating each of these movements also makes grading and documentation using the House-Brackmann scale straightforward. In the House-Brackmann system, a grade I represents normal facial function, grade II slight asymmetry with movement but complete eye closure with gentle effort, grade III more asymmetry with movement but complete eye closure with full effort only, grade IV still greater asymmetry with movement and inability to close the eye completely; grade V has gross resting asymmetry and inability to close the eye, and grade VI demonstrates no movement at all.[25] Numerous other clinical facial nerve grading systems are available, but the House-Brackmann scale remains the most widely employed due to its ease of use.[26][27][28] 

The third pass of the facial examination should repeat the same movements as the second pass, but the examiner should look for involuntary movements in other areas of the affected hemiface. These synkinetic movements, commonly seen as twitching of the mouth during eye closure or winking during a smile, indicate aberrant regeneration and do not typically appear until 4-6 months after the onset of the paralysis; they are more common in patients with more severe paralysis initially.[12] For documentation of longitudinal follow-up, it is helpful to take photographs and videos of patients performing the maneuvers of the facial nerve exam.[29]

Patients with lagophthalmos should have their Bell's phenomenon evaluated, as this is an important corneal protection mechanism. The Bell's phenomenon is the upward rotation of the globe when the eyelids close; it is often lost as patients age. Patients who complain of eye pain or a foreign body sensation should undergo a slit-lamp examination to assess for corneal abrasions or exposure keratopathy, which are very common in cases of facial paralysis. Assessment of corneal sensation is also important because even though corneal anesthesia is rare in Ramsay Hunt syndrome, patients will be predisposed to ocular injury if the eye does not close properly and foreign bodies cannot be felt. Patients with hearing or balance complaints should undergo audiometry and potentially vestibular testing, and patients who note hoarseness or dysphagia should undergo fiberoptic laryngoscopy to evaluate the vocal cords. All patients should receive a thorough cranial nerve examination and inspection of the oral cavity, scalp, external ears, and auditory canals to look for a vesicular eruption. The nose and eyes are not typically involved with vesicles in Ramsay Hunt syndrome, but this can be seen in other forms of zoster. Vesicles near the tip of the nose, Hutchinson's sign, can be associated with ocular lesions.

Evaluation

Because the diagnosis of Ramsay Hunt syndrome is made based on the clinical history and physical examination, routine testing is not indicated.[14] The presence of rash, pain, and facial droop is pathognomonic for the condition. A Tzanck smear can be performed on fluid obtained from the vesicles, and PCR analysis of tears, saliva, or fluid from the vesicles is available in some academic settings, but these modalities are rarely necessary. Magnetic resonance imaging (MRI) should demonstrate inflammation near the geniculate ganglion of the affected facial nerve, but computed tomography will not generally contribute to the diagnosis, nor is the MRI strictly necessary. Audiometry, vestibular testing, and flexible fiberoptic laryngoscopy may help determine the extent of cranial nerve involvement. When the cause of acute facial paralysis is unclear clinically, imaging and serological studies may be helpful, but most cases of Ramsay Hunt syndrome are readily apparent.[13] 

When available, electrodiagnostic testing such as electroneuronography (ENoG) and electromyography (EMG) may provide useful prognostic information by quantifying the extent of nerve damage more precisely than is possible with a physical examination alone, predominantly in the case of House-Brackmann grade VI paralysis.[30][31]

Treatment / Management

Herpes zoster is generally self-limiting in nature. Therefore, the main goals of treatment are to decrease the incidence of late complications, including spastic facial paralysis and postherpetic neuralgia. Multiple studies have shown a significant decrease in long-term complications with the use of oral antivirals and steroids.[20][5] It is unclear, however, whether these medications decrease the length or severity of acute symptoms. Acyclovir, valacyclovir, and famciclovir have all been studied and found effective. Acyclovir, 500 mg five times a day, is usually the most affordable option. Valacyclovir, 1000 mg three times a day, is easier for most patients to take and appears to be more effective, at least in Bell's palsy.[32] Another option is famciclovir, 500 mg three times a day, which also appears to be more effective than acyclovir.[33]

Antiviral treatment is usually administered for 7 to 10 days; however, some studies have reported prolonged or delayed degeneration of facial nerve axons up to 21 days after paralysis onset, and therefore recommend continuing antiviral therapy for 21 days.[1][5] High-dose corticosteroids are often administered as well, such as prednisone 60 mg daily, also for 21 days, followed by a taper to prevent acute adrenal insufficiency. Corticosteroids may produce multiple side effects, and patients should be counseled about the possibility of irritability, insomnia, gastroesophageal reflux, and hyperglycemia.[8] Brittle diabetic patients may have difficulty tolerating steroids in these high doses and should adjust their hypoglycemic medications appropriately. In some cases, temporary use of insulin may be necessary rather than forgoing the steroids because the combination of glucocorticoids and antiviral drugs has been shown to be more effective than antivirals alone.[20] Treatment should be started as soon as possible, but initiating therapy as late as one week after onset is still helpful for patients who present in a delayed fashion.

Symptomatic management is also critical, particularly for two aspects of Ramsay Hunt syndrome: pain and corneal exposure. Analgesia is often needed with zoster; acetaminophen or ibuprofen and long-acting opioids can all be used. Tricyclic antidepressants and gabapentin are useful for the treatment of neuropathic pain and postherpetic neuralgia. Meclizine and benzodiazepines can be effective for managing acute vertigo as well. Artificial tears throughout the day and ocular lubricant ointment at night are helpful for the prevention of exposure keratopathy, and patients with frank lagophthalmos should be instructed in how to stretch the eyelid and how to tape the eye closed at night in such a way as to avoid scratching the cornea in the process (see videos). Patients with multiple comorbidities, such as diabetes, old age, hypertension, and immunocompromise, may take longer to recover than most, and they may benefit from the placement of an upper eyelid weight to aid eye closure and prevent exposure keratopathy during the recovery period.[34] Eyelid weight placement is particularly indicated in patients with corneal hypesthesia.[13] Further surgical intervention in patients with acute Ramsay Hunt syndrome is not generally necessary. While there is considerable debate regarding the utility of craniotomy for facial nerve decompression in Bell palsy patients, there does not appear to be any literature supporting the practice in cases of Ramsay Hunt syndrome.

In the long term, management of synkinesis can be accomplished with both conservative and surgical approaches.[35] Conservative approaches include massage and physical therapy as well as chemodenervation with botulinum toxin. Surgical management of synkinesis may involve selective neurectomy and/or myomectomy, or even nerve or functional free muscle transfer to improve smile symmetry.[36][37][38][39][40]

Differential Diagnosis

Not many clinical entities produce both a facial rash and paralysis, but other common causes of localized facial rashes include herpes simplex virus outbreaks, Staphylococcal impetigo, and contact dermatitis that may arise from topical agents like neomycin or exposure to an irritant such as poison ivy. Acute facial paralysis is most commonly caused by Bell's palsy, which presents similarly to zoster sine herpete, but other non-traumatic etiologies include Lyme disease, benign skull base tumors, and extratemporal malignancies, autoimmune conditions, otologic disease, other viral infections, neurosyphilis, and stroke.[14][13] While cortical strokes classically spare the forehead, brainstem strokes cause paralysis of the entire hemiface; regardless, an evolving stroke should present with vital sign instability and neurological symptoms other than facial paralysis. Multiple cranial neuropathies can occur in the setting of central nervous system pathology as well as viral infections, including SARS-CoV-2.[41][42]

Prognosis

All patients recover from Ramsay Hunt syndrome; the question is, "to what degree?" Patients who present with House-Brackmann grade III paralysis tend to recover to normal function; patients with House-Brackmann grade IV or V paralysis are more likely to recover to grade II function, and patients with House-Brackmann grade VI function at presentation are more likely to recover to grade III function.[5] Patients who do not recover their premorbid function will almost certainly develop some degree of synkinesis. Clinically significant flaccid paralysis is extremely rare in the long term. Most patients complete their recovery within one year and often within several weeks to a few months when the paralysis is incomplete, and the patient is young and healthy. Overall, roughly 70% of patients will recover to House-Brackmann grade I or II function.[5] In general, the prognosis for Ramsay Hunt syndrome is worse than that for Bell's palsy, which has a lower rate of synkinesis development and failure to return to premorbid function.[43]

The most important prognostic indicator in Ramsay Hunt syndrome is the severity of the facial paralysis, although age over 50 is also associated with worse outcomes, as are greater axonal damage on ENoG and EMG, presence of oropharyngeal lesions, multiple cranial neuropathies, and diabetes.[4][20][22][44] In addition to synkinesis, a common sequela of Ramsay Hunt syndrome is postherpetic neuralgia - pain presents longer than three months after onset - which is more likely to develop in patients older than 50 years and patients who experience facial numbness in the acute period.[45]

Complications

Other than the presenting symptoms of pain, rash, facial paralysis, dysgeusia, hearing loss, tinnitus, vertigo, hoarseness, dysarthria, and others mentioned above, short-term complications of Ramsay Hunt syndrome include corneal abrasion and exposure keratopathy, depression and social anxiety, and transmission of chickenpox to unvaccinated or immunocompromised close contacts. While flaccid paralysis in the long-term is unlikely, the development of synkinesis is very common. Other long-term complications include postherpetic neuralgia, scarring from the vesicles, and persistent depression and/or social anxiety due to loss of facial function.

Consultations

While Ramsay Hunt syndrome is ideally managed pharmacologically by a primary care provider, specialist consultation with a physician with acute facial paralysis experience may be valuable. An otolaryngologist or facial plastic surgeon may be more familiar with the treatment of Ramsay Hunt syndrome than a general practitioner and is more likely to have easy access to audiometry and flexible fiberoptic laryngoscopy. A neurologist is also helpful in evaluating cranial neuropathies and treatment of chronic neuralgia and may be able to provide electrodiagnostic testing for patients with complete hemifacial paralysis. An ophthalmologist can evaluate the health of the cornea with slit lamp examination and fluorescein dye, and both the ophthalmologist and the otolaryngologist should be capable of placing an eyelid weight, if necessary. An internist or endocrinologist may be required to manage blood glucose levels during prolonged high-dose steroid administration. Lastly, some patients will require consultation with a behavioral health specialist to assist with managing mood symptoms and anxiety that stem from facial dysfunction.

Deterrence and Patient Education

When discussing Ramsay Hunt syndrome with patients, it is essential to emphasize that everyone gets better, but not everyone gets all the way better. Explaining that "not all the way better" may involve synkinesis and/or postherpetic neuralgia is critical. Perhaps even more crucial is impressing upon patients the importance of corneal protection during the period of flaccid paralysis because the temporary nature of the facial palsy does not preclude the possibility of sustaining a permanent ocular injury during that interval. Accordingly, the application of artificial tears throughout the day and ocular lubricant ointment at night, and the use of eyelid stretching and taping can mean the difference between a satisfactory long-term outcome and an unsatisfactory one. It is also important for patients with active vesicles to avoid contact with unvaccinated and immunocompromised individuals, as they can spread the varicella-zoster virus from their lesions. Lastly, administering the shingles vaccine likely prevents Ramsay Hunt syndrome as well, but the vaccine is not 100% effective. It is also important to remember that while most patients will not develop chickenpox or zoster more than once, it has been reported, particularly in immunocompromised individuals.[46]

Enhancing Healthcare Team Outcomes

Ramsay Hunt syndrome affects patients in myriad ways, with pain, paralysis, cochleovestibular symptoms, and behavioral health concerns all occurring commonly in the acute period. In the long term, while most patients do recover the majority of their premorbid function when managed appropriately, pain, facial dysfunction, scarring, and behavioral health concerns may all persist. For this reason, optimal patient outcomes occur when healthcare teams include members with expertise across a broad range of specialties. In the short term, primary care, otolaryngology, neurology, ophthalmology, and psychology/psychiatry are often required. In the long-term, plastic surgery or otolaryngology, pain management, ophthalmology, speech or physical therapy, and psychology/psychiatry may be needed. Patients who develop synkinesis may require regular visits over the course of years and years with a physician or nurse who can administer botulinum toxin injections; it is critical to surround these patients with an experienced interprofessional team early on in the treatment process in order to provide them the care and support they need to maximize their quality of life outcomes.[Level 1] 



(Click Image to Enlarge)
vesicular rash extending to the auricle with facial droop from Ramsay Hunt Syndrome
vesicular rash extending to the auricle with facial droop from Ramsay Hunt Syndrome
Contributed vy Andrew Crouch DO

(Click Image to Enlarge)
vesicular rash extending to the auricle with facial droop from Ramsay Hunt Syndrome
vesicular rash extending to the auricle with facial droop from Ramsay Hunt Syndrome
Contributed by Andrew Crouch DO

(Click Image to Enlarge)
vesicular rash extending to the auricle with facial droop from Ramsay Hunt Syndrome
vesicular rash extending to the auricle with facial droop from Ramsay Hunt Syndrome
Contributed by Andrew Crouch DO

(Click Image to Enlarge)
Ramsay-Hunt Syndrome
Ramsay-Hunt Syndrome
Graphic by Ella Workman

(Click Image to Enlarge)
Vesicles on the left soft palate in Ramsay Hunt syndrome
Vesicles on the left soft palate in Ramsay Hunt syndrome
Contributed by Marc H Hohman, MD, FACS
Article Details

Article Author

Andrew E. Crouch

Article Author

Marc H. Hohman

Article Editor:

Claudio Andaloro

Updated:

5/1/2022 7:33:33 PM

PubMed Link:

Ramsay Hunt Syndrome

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