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Ramsay Hunt Syndrome

Updated : February 13, 2024





Background

Ramsay Hunt syndrome, commonly referred to as geniculate ganglion herpes zoster or herpes zoster oticus, is a delayed consequence of varicella-zoster virus infection that causes inflammation of cranial nerve CN VII geniculate ganglion.

Ramsay Hunt syndrome is differentiated by an ipselateral triad of vesicles, facial paralysis, and otalgia on the auricle or in the auditory canal. Other symptoms include changes in taste perception, dryness of the eye, hyperacusis, tearing, dysarthria, and nasal obstruction.

Hearing loss, tinnitus, and vertigo are symptoms of vestibulocochlear nerve involvement, whereas hoarseness or aspiration are symptoms of vagus nerve involvement. Vesicles can form after the start of neurologic symptoms, but the pain is frequently the first to appear.

Epidemiology

Ramsay Hunt syndrome affects both immunocompromised and immunocompetent patients and has an annual incidence of around 5 per 100,000 persons.

This syndrome accounts for around 7% of acute facial paralysis patients, with zoster sine herpete accounting for approximately 10%.  Immunocompromised individuals are more prone to experience a more severe clinical course and a slower recovery.

Ramsay Hunt syndrome may affect anyone, and instances have been observed in people ranging in age from 3 months to 82 years, with persons in their seventh and eighth decades being the most vulnerable.

Anatomy

Pathophysiology

The syndrome initially begins as chickenpox; the virus remains dormant even after the disappearance of exanthem in the dorsal root ganglia and cranial nerves. During times of physiological stress or immunocompromise condition, reactivation of the virus occurs. The virus reactivates along the seventh cranial nerve via the geniculate ganglion in Ramsay Hunt syndrome.

The first symptom is usually discomfort in the ipsilateral ear, followed by facial paralysis and vesicles within 2 to 3 days. Hearing loss, tinnitus, and vertigo can arise from the facial nerve’s closeness to the vestibulocochlear nerve. Vagal nerve stimulation is also likely to be more prevalent. Unless the patient has hoarseness or aspiration symptoms, vocal cord paralysis is usually missed.

Newly regenerated axons terminate many neuromuscular junctions, enhancing dyscoordination and elevating the resting tone of muscles with more connected axons than before the injury. Patients suffering from synkinesis frequently report eye closure with mouth motions and vice versa, as well as discomfort and facial tension.

Etiology

The varicella-zoster virus, a member of the HHV (Human herpesvirus) family, is the causal agent in Ramsay Hunt syndrome. VZV is a virus with double-stranded DNA that is more precisely identified as human alphaherpesvirus 3 (HHV-3).

Once chickenpox resolves, the virus stays inactive in the dorsal root ganglia or cranial nerve. It may reactivate during immunocompromised conditions or physiological stress resulting in herpes zoster. It is known as Ramsay Hunt Syndrome, when facial paralysis is involved and shingles if it occurs anywhere on the body.

While the frequency of chickenpox and shingles has significantly decreased since the VZV vaccine became widely accessible in 1995, Ramsay Hunt syndrome and shingles have been documented in people with no history of chickenpox; however, they received the live attenuated VZV vaccine.

Genetics

Prognostic Factors

Patients who do not regain their premorbid functioning will develop synkinesis. In the longer run, clinically significant flaccid paralysis is relatively rare. When the paralysis is partial, and the patient is young and healthy, most individuals recover within a year and typically within weeks to months.

Approximately 70% of patients will regain House-Brackmann grade I or II functions. Ramsay Hunt syndrome has a poor outcome. The degree of facial paralysis is the most important prognostic factor in Ramsay Hunt syndrome; however, age over 50, more axonal damage on EMG and ENoG diabetes, oropharyngeal lesions, and multiple cranial neuropathies are all related to poor outcomes.

Clinical History

Physical Examination

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

Media Gallary

References

https://www.ncbi.nlm.nih.gov/books/NBK557409/

Ramsay Hunt Syndrome

Updated : February 13, 2024




Ramsay Hunt syndrome, commonly referred to as geniculate ganglion herpes zoster or herpes zoster oticus, is a delayed consequence of varicella-zoster virus infection that causes inflammation of cranial nerve CN VII geniculate ganglion.

Ramsay Hunt syndrome is differentiated by an ipselateral triad of vesicles, facial paralysis, and otalgia on the auricle or in the auditory canal. Other symptoms include changes in taste perception, dryness of the eye, hyperacusis, tearing, dysarthria, and nasal obstruction.

Hearing loss, tinnitus, and vertigo are symptoms of vestibulocochlear nerve involvement, whereas hoarseness or aspiration are symptoms of vagus nerve involvement. Vesicles can form after the start of neurologic symptoms, but the pain is frequently the first to appear.

Ramsay Hunt syndrome affects both immunocompromised and immunocompetent patients and has an annual incidence of around 5 per 100,000 persons.

This syndrome accounts for around 7% of acute facial paralysis patients, with zoster sine herpete accounting for approximately 10%.  Immunocompromised individuals are more prone to experience a more severe clinical course and a slower recovery.

Ramsay Hunt syndrome may affect anyone, and instances have been observed in people ranging in age from 3 months to 82 years, with persons in their seventh and eighth decades being the most vulnerable.

The syndrome initially begins as chickenpox; the virus remains dormant even after the disappearance of exanthem in the dorsal root ganglia and cranial nerves. During times of physiological stress or immunocompromise condition, reactivation of the virus occurs. The virus reactivates along the seventh cranial nerve via the geniculate ganglion in Ramsay Hunt syndrome.

The first symptom is usually discomfort in the ipsilateral ear, followed by facial paralysis and vesicles within 2 to 3 days. Hearing loss, tinnitus, and vertigo can arise from the facial nerve’s closeness to the vestibulocochlear nerve. Vagal nerve stimulation is also likely to be more prevalent. Unless the patient has hoarseness or aspiration symptoms, vocal cord paralysis is usually missed.

Newly regenerated axons terminate many neuromuscular junctions, enhancing dyscoordination and elevating the resting tone of muscles with more connected axons than before the injury. Patients suffering from synkinesis frequently report eye closure with mouth motions and vice versa, as well as discomfort and facial tension.

The varicella-zoster virus, a member of the HHV (Human herpesvirus) family, is the causal agent in Ramsay Hunt syndrome. VZV is a virus with double-stranded DNA that is more precisely identified as human alphaherpesvirus 3 (HHV-3).

Once chickenpox resolves, the virus stays inactive in the dorsal root ganglia or cranial nerve. It may reactivate during immunocompromised conditions or physiological stress resulting in herpes zoster. It is known as Ramsay Hunt Syndrome, when facial paralysis is involved and shingles if it occurs anywhere on the body.

While the frequency of chickenpox and shingles has significantly decreased since the VZV vaccine became widely accessible in 1995, Ramsay Hunt syndrome and shingles have been documented in people with no history of chickenpox; however, they received the live attenuated VZV vaccine.

Patients who do not regain their premorbid functioning will develop synkinesis. In the longer run, clinically significant flaccid paralysis is relatively rare. When the paralysis is partial, and the patient is young and healthy, most individuals recover within a year and typically within weeks to months.

Approximately 70% of patients will regain House-Brackmann grade I or II functions. Ramsay Hunt syndrome has a poor outcome. The degree of facial paralysis is the most important prognostic factor in Ramsay Hunt syndrome; however, age over 50, more axonal damage on EMG and ENoG diabetes, oropharyngeal lesions, and multiple cranial neuropathies are all related to poor outcomes.

https://www.ncbi.nlm.nih.gov/books/NBK557409/