Ramsay Hunt Syndrome is an infection of the facial nerve accompanied by a rash, some of the signs and symptoms of which include pain and weakness in the facial muscles. Ramsay Hunt Syndrome was first described in detail by an American doctor named James Ramsay Hunt in 1907.
1. Definition of Ramsay Hunt syndrome
Ramsay Hunt syndrome is an infection of the facial nerve with a rash, some of the signs and symptoms of which include pain and weakness in the facial muscles.
In the general population, Ramsay Hunt syndrome is relatively uncommon. However, it is often seen in high-risk subjects such as the elderly, weak, immunocompromised due to cancer, diabetes, radiation therapy, immunosuppressive drugs, stress, AIDS, etc.
3. Etiology and pathophysiology
The culprit that causes Ramsay Hunt syndrome is the varicella-zoster virus (VZV). In humans, there are two clinical variants associated with this virus, chickenpox and shingles. After causing chickenpox, this virus lies inactive in the nerve ganglia, waiting for the body’s immune system to weaken the virus will reactivate to cause shingles. Herpes zoster virus reactivation in the knee ganglia causes damage to the VII nerve, clinically manifesting as Ramsay Hunt syndrome.
The onset of Ramsay Hunt syndrome can be scary. The symptoms can make some people fear they are having a stroke. But, often treatment is effective for Ramsay Hunt syndrome. Sometimes, Ramsay Hunt can lead to facial muscle weakness and hearing loss. Treatment can reduce the risk of complications.
Manifestations of the disease are quite diverse, two typical and common symptoms of this syndrome are:
Blisters on an erythematous base in the inner ear, outer ear canal, or around the ear. When it breaks, it oozes fluid and can become infected. Blisters can also be seen on the nasopharynx, and the anterior 2/3 of the tongue following the distribution of the VII . nerve
– Peripheral paralysis VII on the same side of the lesion, may appear before, after or at the same time as the appearance of vesicles. The degree of paralysis is graded according to House – Brackmann
Classification of facial paralysis according to House – Brackmann
Grade I: Normal with normal facial movements without synergism.
Grade II: Mild deformity: good forehead function, mild synergism, mild weakness, slight facial asymmetry when observed close to the patient
Grade III: Moderate facial weakness, weak forehead function, eyes closed on exertion, asymmetry. There is the Bell phenomenon.
Grade IV: Moderate to severe facial weakness: increased synergy, no forehead activity, eyes closed.
Grade V: Severe facial paralysis, face asymmetry when thinking, only a few tones left, eyes cannot close.
Grade VI: Total facial paralysis, no tone.
Other symptoms may also be present:
– Dizziness may be accompanied by nausea, vomiting
– Tinnitus, ear pain
Hearing loss in the ipsilateral ear occurs in 50% of cases, often aggravated by the presence of vertigo.
– Paraphrasing disorder
– Dry eyes
– Change of taste
– Neck lymph nodes
– Encephalitis, meningoencephalitis
Bacterial superinfection is more likely to occur when the blisters are broken
Facial paralysis, deafness or hearing loss: Most cases of facial paralysis and hearing loss are temporary, but some cases of facial paralysis and hearing loss are permanent.
Blindness: Decreased tear secretion by the lacrimal gland and inadequate eye closure can lead to retinal damage that causes blindness if not treated early and properly.
Nerve pain: The virus damages nerve VII, causing it to send false sensory signals to the brain that cause pain. Instead, the information becomes confusing and exaggerated, causing pain that can linger long after the signs and symptoms of Ramsay Hunt syndrome have subsided.
7. Diagnosis of Ramsay Hunt Syndrome
Most cases are diagnosed based on history and physical examination. However, the diagnosis is sometimes difficult because the symptoms of the disease are non-specific, do not appear simultaneously, and sometimes only a few symptoms lead to missed or misdiagnosed. Blood tests and imaging, and electromyography to help rule out or assess the extent of the disease
Virus isolation has 100% specificity, but is rarely used because of its low sensitivity of 30-40%, time consuming and difficult to culture.
PCR of exudate in lesions with a sensitivity of 60%
VZV antigen with immunofluorescence has 90% sensitivity and 99% specificity.
Early detection and treatment is essential to reduce the complications of the disease. Many studies show that, with proper initiation of treatment in the first 3 days of the disease, the recovery rate is up to 70%, after this time, the effectiveness of treatment will decrease. Treatment includes treating the cause and symptoms of the disease and restoring function. Treat the cause with acyclovir 800mg x 5 times/day x 7 days. Symptomatic treatment with corticosteroids, NSAIDs, paracetamol codeine, carbamazepine, antiepileptic drugs for pain relief, treatment of dizziness with antihistamines, diazepam. Artificial tears, eye shielding, and Botox injections into the upper eyelid are necessary when the eyes are not closed to avoid damage to the cornea.
There is no way to prevent Ramsay Hunt syndrome when the varicella-zoster virus is in the body. However, the chickenpox virus vaccine (Varivax) can reduce the risk of getting the virus. This vaccine is a routine childhood immunization, given between 12 months and 18 months of age. This vaccine is also recommended for older children and adults who have never had chickenpox. The chickenpox virus vaccine prevents chickenpox for most people. If chickenpox develops after vaccination, it is usually less serious.
The varicella vaccine (Varivax) can reduce the risk of getting this virus. The US Food and Drug Administration (FDA) has approved Zostavax vaccine to help prevent shingles, Ramsay Hunt syndrome and VZV-related diseases in people over 50 years of age. This vaccine is given as a single injection, preferably in the upper arm. The most common side effects are redness, pain, and itching at the injection site.
References: Vinmed, dieutri.vn