Ramsay Hunt syndrome type 2

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Ramsay Hunt syndrome type 2
Other namesHerpes zoster oticus
Ramsey Hunt Syndrome.png
Specialty Infectious diseases   OOjs UI icon edit-ltr-progressive.svg

Ramsay Hunt syndrome type 2, commonly referred to simply as Ramsay Hunt syndrome (RHS) and also known as herpes zoster oticus, is inflammation of the geniculate ganglion of the facial nerve as a late consequence of varicella zoster virus (VZV). In regard to the frequency, less than 1% of varicella zoster infections involve the facial nerve and result in RHS. [1] It is traditionally defined as a triad of ipsilateral facial paralysis, otalgia, and vesicles close to the ear and auditory canal. Due to its proximity to the vestibulocochlear nerve, the virus can spread and cause hearing loss, tinnitus (hearing noises that are not caused by outside sounds), and vertigo. It is common for diagnoses to be overlooked or delayed, which can raise the likelihood of long-term consequences. It is more complicated than Bell's palsy. Therapy aims to shorten its overall length, while also providing pain relief and averting any consequences.

Contents

Signs and symptoms

Early symptoms include intense pain in one ear, the jaw on one side or the neck on one side which may precede the acute facial paralysis by a week or more.

Acute symptoms include:

Because the vestibulocochlear nerve is in proximity to the geniculate ganglion, it may also be affected and patients may also experience:[ citation needed ]

The swallow reflex might also be affected.

Involvement of the trigeminal nerve can cause numbness of the face.[ citation needed ]

Pathophysiology

The varicella zoster virus infects people and results in a distributed vesicular rash with fever, known as chickenpox. Respiratory droplets are the main method of virus transmission during the acute stage of the infection. After it subsides, it stays dormant in nerve cells in the body. It may reactivate under conditions of physiological stress or if the immune system is suppressed in any way (for example during an illness or undergoing chemotherapy), resulting in herpes zoster, also known as shingles or Ramsay Hunt syndrome when facial paralysis in involved. If the nerve cells affected lie within the facial nerves, it causes the symptoms described above. [3]

Ramsay Hunt syndrome type 2 is estimated to account for 12% of all facial nerve paralysis. [4] It occurs in both immunocompetent and immunocompromised individuals with immunocompromised patients often having more severe disease presentation. RHS may occur in any age group with cases reported in patients ranging in age from 3 months to 82 years.

The affected ganglion is responsible for the movements of facial muscles, the touch sensation of a part of ear and ear canal, the taste function of the frontal two-thirds of the tongue, and the moisturization of the eyes and the mouth. The syndrome specifically refers to the combination of this entity with weakness of the muscles activated by the facial nerve. In isolation, the latter is called Bell's palsy. [5]

However, as with shingles, the lack of lesions does not definitely exclude the existence of a herpes infection. Even before the eruption of vesicles, varicella zoster virus can be detected from the skin of the ear.

Diagnosis

Ramsay Hunt syndrome type 2 can be diagnosed based on clinical features; however, in ambiguous cases, PCR or direct immunofluorescent assay of vesicular fluid can help with the diagnosis. Laboratory studies such as WBC count, ESR and electrolytes can distinguish infectious versus inflammatory etiologies.[ citation needed ]

Clinical diagnosis

On a physical exam, look for vesicular exanthema on the external auditory canal, concha and or pinna. Dry eyes with possible lower cornea epithelium damage due to incomplete closure of eyelids. It is possible to have Ramsay Hunt syndrome type 2 without an external rash present. This is called "RHS sine herpete" and may occur in up to 30% of patients.[ citation needed ] [6]

Diagnostic procedures

Ramsay Hunt Syndrome type 2 can usually be diagnosed based on clinical features. However, for suspected cases with unclear presentation, varicella zoster virus can be isolated from vesicle fluid. Tear culture PCR can have positive varicella zoster virus. However, 25–35% of patients with Bell's palsy can have false positive varicella zoster virus detected in tears. If central nervous system complications such as meningitis, ventriculitis or meningoencephalitis are suspected, prompt lumbar puncture with spinal fluid analysis and imaging (CT head) are recommended.[ citation needed ]

An MRI with contrast may be ordered if the diagnosis is ambiguous so as to rule out other causes of acute facial paralysis such as a stroke, Lyme disease, multiple sclerosis, cancer or tumors. This test is most commonly ordered if the patient presents atypically with RHS sine herpete.

Prevention

Shingles is prevented by immunizing against the causal virus, varicella zoster, using a zoster vaccine. Vaccination is recommended for adults 50 and older. Two versions of the vaccine are available, the live attenuated Zostavax (now discontinued in the US, essentially a larger-dose chickenpox vaccine) and the protein subunit Shingrix. [7]

Treatment

Treatments for Ramsay Hunt syndrome type 2 are used to reduce further damage caused by the viral infection. These medications will not reverse any damage that has already occurred at the time that they are prescribed.[ citation needed ] Initial treatment with a corticosteroid such as prednisone and an antiviral drug such as acyclovir, valacyclovir or famciclovir for 5 to 7 days is standard; however, some studies have shown later damage to the facial nerve and recommend 21 days of antivirals. [4] [2] Studies indicate that treatment started within 72 hours of the onset of facial paralysis improves the chances of the patient experiencing significant recovery. [8] Chances of recovery appear to decrease when treatment is delayed. Delay of treatment may result in permanent facial nerve paralysis. However, some studies demonstrate that even when steroids are started promptly, only 22% of all patients achieve full recovery of facial paralysis. [9] Treatment apparently has no effect on the recovery of hearing loss.

Meclizine, benzodiazepines such as diazepam, and vestibular therapy are sometimes used to treat the vertigo.

During the acute recovery phase, the eye on the affected side of the face may not blink completely or at all and may not close tightly or at all when sleeping. If the eye is dry or feels irritated, this is a strong indication that the eye is not properly blinking or closing completely. Using artificial tears every 5 to 20 minutes while awake and protecting the eye while asleep are very important to maintaining the health of the eye. While asleep, applying overnight eye gel and using sensitive skin medical tape or an eye patch to keep the eye closed or using a moisture chamber can protect the eye. Taking these precautions is extremely important to preserve the health and functionality of the eye and prevent corneal abrasions and corneal ulcers. [2]

Nerve pain associated with Ramsay Hunt Syndrome may be extreme and centered in the ear, neck, cheek, jaw and face. This nerve pain may not respond well to standard pain treatments including NSAIDS and opioids. Medication specifically for nerve pain such as tricyclic antidepressants and gabapentin have been shown to be effective for the neuropathic pain and post-herpetic neuralgia common with RHS. [2]

Physical therapy, excessive movement or electrical stimulation practiced during the first year of recovery greatly increase the chances of long term complications, including hyperactive muscles and synkinesis, both of which are permanent. [ citation needed ] The most common form of synkinesis for Ramsay Hunt Syndrome patients involves the eye being connected to the mouth (i.e. blinking while speaking, tearing while eating) and chin dimpling (chin dimples forming when speaking). Many forms of synkinesis can be managed with use of medical Botox administered by a qualified doctor. [10]

Prognosis

Overall between 30% and 70% of Ramsay Hunt syndrome type 2 patients recover most functionality depending on early diagnosis and treatment with chances of recovery dropping to 50% if treatment is delayed beyond 72 hours. [2]

Once the active infection has been cleared with antivirals, the facial nerves will begin to regrow at approximately 1mm per day. The recovery process for Ramsay Hunt syndrome is significantly longer than Bell's palsy. On average, Ramsay Hunt syndrome patients begin to see their symptoms resolve between 5 and 12 months post diagnosis and can expect to see continued resolution of symptoms for up to 2 years post diagnosis. Occasionally, patients may experience minor improvements beyond 2 years. The order in which symptoms resolve is highly individual. Although most patients will experience some recovery; complete recoveries with no lingering symptoms are in the minority. The main factors affecting the overall prognosis are the severity of symptoms at onset, the age and general health of the patient and the timing of initial treatments

Common long term effects include:

Less common long term effects include:

Some patients report an increased sensitivity to barometric pressure with changes in weather patterns causing pain on the affected side of the face.

History and notable cases

The syndrome is named for James Ramsay Hunt, the neurologist who first described it. [11] [12]

In 2013, Olivia Chow was diagnosed with the condition. [13]

On June 10, 2022, Canadian singer Justin Bieber announced in a video posted on Instagram that he had been diagnosed with the condition. [14] He has since entered treatment, and recovered successfully. [15]

On May 18, 2023, the New York Times reported that United States Senator Dianne Feinstein had developed the condition as a side effect of a shingles infection. [16]


Related Research Articles

<span class="mw-page-title-main">Bell's palsy</span> Facial paralysis resulting from dysfunction in the cranial nerve VII (facial nerve)

Bell's palsy is a type of facial paralysis that results in a temporary inability to control the facial muscles on the affected side of the face. In most cases, the weakness is temporary and significantly improves over weeks. Symptoms can vary from mild to severe. They may include muscle twitching, weakness, or total loss of the ability to move one or, in rare cases, both sides of the face. Other symptoms include drooping of the eyebrow, a change in taste, and pain around the ear. Typically symptoms come on over 48 hours. Bell's palsy can trigger an increased sensitivity to sound known as hyperacusis.

<span class="mw-page-title-main">Varicella zoster virus</span> Herpes virus that causes chickenpox and shingles

Varicella zoster virus (VZV), also known as human herpesvirus 3 or Human alphaherpesvirus 3 (taxonomically), is one of nine known herpes viruses that can infect humans. It causes chickenpox (varicella) commonly affecting children and young adults, and shingles in adults but rarely in children. As a late complication of VZV infection, Ramsay Hunt syndrome type 2 may develop in rare cases. VZV infections are species-specific to humans. The virus can survive in external environments for a few hours.

<span class="mw-page-title-main">Shingles</span> Viral disease caused by the varicella zoster virus

Shingles, also known as herpes zoster or zona, is a viral disease characterized by a painful skin rash with blisters in a localized area. Typically the rash occurs in a single, wide mark either on the left or right side of the body or face. Two to four days before the rash occurs there may be tingling or local pain in the area. Other common symptoms are fever, headache, and tiredness. The rash usually heals within two to four weeks, but some people develop ongoing nerve pain which can last for months or years, a condition called postherpetic neuralgia (PHN). In those with poor immune function the rash may occur widely. If the rash involves the eye, vision loss may occur.

Postherpetic neuralgia (PHN) is neuropathic pain that occurs due to damage to a peripheral nerve caused by the reactivation of the varicella zoster virus. PHN is defined as pain in a dermatomal distribution that lasts for at least 90 days after an outbreak of herpes zoster. Several types of pain may occur with PHN including continuous burning pain, episodes of severe shooting or electric-like pain, and a heightened sensitivity to gentle touch which would not otherwise cause pain or to painful stimuli. Abnormal sensations and itching may also occur.

<span class="mw-page-title-main">Facial nerve paralysis</span> Loss of motor function in the facial muscles

Facial nerve paralysis is a common problem that involves the paralysis of any structures innervated by the facial nerve. The pathway of the facial nerve is long and relatively convoluted, so there are a number of causes that may result in facial nerve paralysis. The most common is Bell's palsy, a disease of unknown cause that may only be diagnosed by exclusion of identifiable serious causes.

<span class="mw-page-title-main">Valaciclovir</span> Antiviral medication

Valaciclovir, also spelled valacyclovir, is an antiviral medication used to treat outbreaks of herpes simplex or herpes zoster (shingles). It is also used to prevent cytomegalovirus following a kidney transplant in high risk cases. It is taken by mouth.

<span class="mw-page-title-main">Neuritis</span> Inflammation of a nerve or generally any part of the nervous system

Neuritis, from the Greek νεῦρον), is inflammation of a nerve or the general inflammation of the peripheral nervous system. Inflammation, and frequently concomitant demyelination, cause impaired transmission of neural signals and leads to aberrant nerve function. Neuritis is often conflated with neuropathy, a broad term describing any disease process which affects the peripheral nervous system. However, neuropathies may be due to either inflammatory or non-inflammatory causes, and the term encompasses any form of damage, degeneration, or dysfunction, while neuritis refers specifically to the inflammatory process.

<span class="mw-page-title-main">Herpetic gingivostomatitis</span> Inflammation of the mouth, lips, and gums, often due to herpes infection

Gingivostomatitis is a combination of gingivitis and stomatitis, or an inflammation of the oral mucosa and gingiva. Herpetic gingivostomatitis is often the initial presentation during the first ("primary") herpes simplex infection. It is of greater severity than herpes labialis which is often the subsequent presentations. Primary herpetic gingivostomatitis is the most common viral infection of the mouth.

<span class="mw-page-title-main">Progressive outer retinal necrosis</span> Medical condition

Progressive outer retinal necrosis (PORN) syndrome is a form of chorioretinitis, an infection in the retina, the back of the eye. The disease is most commonly caused by the varicella zoster virus and is found almost exclusively in patients with HIV/AIDS. Progressive outer retinal necrosis is the second most common opportunistic retinal infection in North America among people with AIDS. The reason this disease process is considered opportunistic is precisely because it only presents in patients with AIDS, a disease that attacks and weakens the immune system, making space for other infections, like Varicella zoster virus (VZV) and Herpes simplex virus (HSV), to attack the body.

<span class="mw-page-title-main">Tolosa–Hunt syndrome</span> Medical condition

Tolosa–Hunt syndrome is a rare disorder characterized by severe and unilateral headaches with orbital pain, along with weakness and paralysis (ophthalmoplegia) of certain eye muscles.

Geniculate ganglionitis or geniculate neuralgia (GN), also called nervus intermedius neuralgia, Ramsay Hunt syndrome, or Hunt's neuralgia, is a rare disorder characterized by severe paroxysmal neuralgic pain deep in the ear, that may spread to the ear canal, outer ear, mastoid or eye regions. GN may also occur in combination with trigeminal or glossopharyngeal neuralgia.

Synkinesis is a neurological symptom in which a voluntary muscle movement causes the simultaneous involuntary contraction of other muscles. An example might be smiling inducing an involuntary contraction of the eye muscles, causing a person to squint when smiling. Facial and extraocular muscles are affected most often; in rare cases, a person's hands might perform mirror movements.

Three different neurological syndromes carry the name of Ramsay Hunt syndrome. Their only connection is that they were all first documented by the famous neurologist James Ramsay Hunt (1872–1937).

<span class="mw-page-title-main">Cranial nerve disease</span> Impaired functioning of one of the twelve cranial nerves

Cranial nerve disease is an impaired functioning of one of the twelve cranial nerves. Although it could theoretically be considered a mononeuropathy, it is not considered as such under MeSH.

<span class="mw-page-title-main">Mollaret's meningitis</span> Medical condition

Mollaret's meningitis is a recurrent or chronic inflammation of the protective membranes covering the brain and spinal cord, known collectively as the meninges. Since Mollaret's meningitis is a recurrent, benign (non-cancerous), aseptic meningitis, it is also referred to as benign recurrent lymphocytic meningitis. It was named for Pierre Mollaret, the French neurologist who first described it in 1944.

<span class="mw-page-title-main">Chickenpox</span> Human viral disease

Chickenpox, also known as varicella, is a highly contagious, vaccine-preventable disease caused by the initial infection with varicella zoster virus (VZV), a member of the herpesvirus family. The disease results in a characteristic skin rash that forms small, itchy blisters, which eventually scab over. It usually starts on the chest, back, and face. It then spreads to the rest of the body. The rash and other symptoms, such as fever, tiredness, and headaches, usually last five to seven days. Complications may occasionally include pneumonia, inflammation of the brain, and bacterial skin infections. The disease is usually more severe in adults than in children.

<span class="mw-page-title-main">Herpes</span> Viral disease caused by herpes simplex viruses

Herpes simplex, often known simply as herpes, is a viral infection caused by the herpes simplex virus. Herpes infections are categorized by the area of the body that is infected. The two major types of herpes are oral herpes and genital herpes, though other forms also exist.

<span class="mw-page-title-main">Herpes zoster ophthalmicus</span> Shingles in the human eye

Herpes zoster ophthalmicus (HZO), also known as ophthalmic zoster, is shingles involving the eye or the surrounding area. Common signs include a rash of the forehead with swelling of the eyelid. There may also be eye pain and redness, inflammation of the conjunctiva, cornea or uvea, and sensitivity to light. Fever and tingling of the skin and allodynia near the eye may precede the rash. Complications may include visual impairment, increased pressure within the eye, chronic pain, and stroke.

Facial nerve decompression is a type of nerve decompression surgery where abnormal compression on the facial nerve is relieved.

Preherpetic neuralgia is a form of nerve pain (neuralgia) specifically associated with a Shingles viral infection. This nerve pain often precedes visible indications of a Shingles infection and consequently can be a key early indicator of a need to begin preventative anti-viral drug therapy. Pain associated with Shingles can be extremely difficult to treat whereas the source is related to the virus attacking the nervous system itself. Pain symptoms can last months or years beyond any outward sign of viral infection and can be quite severe. The combination of extreme pain severity and longevity can contribute to chronic depression and even suicide.

References

  1. ↑ Sommer, T.; Karsy, M.; Driscoll, M. J.; Jensen, R. L. (2018). "Varicella-Zoster Virus Infection and Osteomyelitis of the Skull". World Neurosurg. 115: 297–300. doi:10.1016/j.wneu.2018.04.194. PMID   29733991. S2CID   13688872.
  2. 1 2 3 4 5 6 Crouch, Andrew E.; Hohman, Marc H.; Andaloro., Claudio (1 May 2022). "Ramsay Hunt Syndrome". StatPearls. National Institutes of Health - National Library of Medicine. PMID   32491341.
  3. ↑ Sweeney, C.J.; Gilden, D.H. (August 2001). "Ramsay Hunt syndrome". Journal of Neurology, Neurosurgery, and Psychiatry. 71 (2): 149–54. doi:10.1136/jnnp.71.2.149. PMC   1737523 . PMID   11459884.
  4. 1 2 Paiva, A L C; Araujo, J L V; Ferraz, V R; Veiga, J C E (2017). "Facial paralysis due to Ramsay Hunt syndrome - A rare condition". Rev Assoc Med Bras. 63 (4): 301–302. doi: 10.1590/1806-9282.63.04.301 . PMID   28614529. S2CID   37303883.
  5. ↑ Kim, In Sup; Shin, Seung-Ho; Kim, Jinn; Lee, Won-Sang; Lee, Ho-Ki (2007). "Correlation between MRI and Operative Findings in Bell's Palsy and Ramsay Hunt Syndrome". Yonsei Medical Journal. 48 (6): 963–968. doi:10.3349/ymj.2007.48.6.963. PMC   2628199 . PMID   18159587.
  6. ↑ Nishizawa, Toshinori; Ishikawa, Kazuhiro; Matsuo, Takahiro; Higuchi, Naofumi; Ishiguro, Kenji; Mori, Nobuyoshi (March 2021). "Atypical Ramsay Hunt syndrome (zoster sine herpete) with otitis media". Journal of General and Family Medicine. 22 (6): 344–346. doi:10.1002/jgf2.433. PMC   8561112 . PMID   34754713.
  7. ↑ "Shingles (Herpes Zoster) - Get the new shingles vaccine if you are 50 or older". CDC. 2019-07-02. Retrieved 15 June 2022.
  8. ↑ Murakami, Shingo; Hato, Naohito; Horiuchi, Joji; Honda, Nobumitsu; Gyo, Kiyofumi; Yanagihara, Naoaki (1 March 1997). "Treatment of Ramsay Hunt syndrome with acyclovir-prednisone: Significance of early diagnosis and treatment". Annals of Neurology. 41 (3): 353–357. doi:10.1002/ana.410410310. PMID   9066356. S2CID   35050065.
  9. ↑ Finsterer, Josef (2008). "Management of peripheral facial nerve palsy". European Archives of Oto-Rhino-Laryngology. 265 (7): 743–752. doi:10.1007/s00405-008-0646-4. PMC   2440925 . PMID   18368417.
  10. ↑ "Is Botox for Facial Synkinesis Safe and Effective?". www.facialparalysisinstitute.com. Retrieved 15 June 2022.
  11. ↑ synd/2246 at Who Named It?
  12. ↑ "The Ramsay Hunt syndrome". Proceedings of the Royal Society of Medicine. 47 (5): 371–384. May 1954. doi: 10.1177/003591575404700517 . PMC   1918846 . PMID   13167057.
  13. ↑ Connor, Kevin (January 4, 2013). "Olivia Chow diagnosed with Ramsay Hunt syndrome". Toronto Sun. Archived from the original on 2016-03-03. Retrieved March 12, 2014.
  14. ↑ "Justin Bieber's facial paralysis: 'I shed a couple of tears' - Ramsay Hunt sufferer is 'heartbroken' seeing singer with illness". Sky News.
  15. ↑ Guenot, Marianne (16 June 2022). "Justin Bieber is 'getting better every day' from Ramsay Hunt syndrome and expects to recover fully, Hailey Bieber says". news.yahoo.com. Retrieved 20 June 2022.
  16. ↑ Karni, Annie; Hulse, Carl (May 18, 2023). "Feinstein Suffered More Complications From Illness Than Were Publicly Disclosed: Senator Dianne Feinstein, 89, whose recent bout with shingles included contracting encephalitis, is frailer than ever. But she remains unwilling to entertain discussions about leaving the Senate". New York Times . Retrieved May 18, 2023.
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