Ramsay Hunt Syndrome: Difference between revisions
Wendy Walker (talk | contribs) No edit summary |
Wendy Walker (talk | contribs) No edit summary |
||
| Line 1: | Line 1: | ||
<div class="editorbox"> | <div class="editorbox"> | ||
'''Original Editor '''- [http://www.physio-pedia.com/User:Wendy_Walker Wendy Walker] | '''Original Editor '''- [http://www.physio-pedia.com/User:Wendy_Walker Wendy Walker] | ||
'''Lead Editors''' - Wendy Walker | '''Lead Editors''' - Wendy Walker | ||
</div> | </div> | ||
== Introduction<br> == | == Introduction<br> == | ||
Ramsay Hunt syndrome is defined as an acute peripheral [[ | Ramsay Hunt syndrome is defined as an acute peripheral [[Facial Palsy|facial palsy]]/neuropathy associated with erythematous vesicular rash of the skin of the ear canal, auricle (also termed herpes zoster oticus), and/or mucous membrane of the oropharynx<ref>Bhupal HK. Ramsay Hunt syndrome presenting in primary care. Practitioner. 2010 Mar. 254(1727):33-5</ref>. It is commonly asociated with moderate to severe otalgia (earache).<br> | ||
This syndrome is also known as geniculate neuralgia or nervus intermedius neuralgia. | This syndrome is also known as geniculate neuralgia or nervus intermedius neuralgia. | ||
The syndrome was first described in 1907 by Dr James Ramsay Hunt, describing a patient who had otalgia (ear pain) associated with cutaneous and mucosal rashes, which he ascribed to infection of the geniculate ganglion by human herpesvirus 3 (ie, varicella-zoster virus [VZV])<ref>Goldani LZ, Ferreira da Silva LF, Dora JM. Ramsay Hunt syndrome in patients infected with human immunodeficiency virus. Clin Exp Dermatol. 2009 Jun 1</ref>. | The syndrome was first described in 1907 by Dr James Ramsay Hunt, describing a patient who had otalgia (ear pain) associated with cutaneous and mucosal rashes, which he ascribed to infection of the geniculate ganglion by human herpesvirus 3 (ie, varicella-zoster virus [VZV])<ref>Goldani LZ, Ferreira da Silva LF, Dora JM. Ramsay Hunt syndrome in patients infected with human immunodeficiency virus. Clin Exp Dermatol. 2009 Jun 1</ref>. | ||
== Mechanism of Injury / Pathological Process<br> == | == Mechanism of Injury / Pathological Process<br> == | ||
| Line 16: | Line 16: | ||
Ramsay Hunt syndrome is defined as VZV infection of the head and neck that involves the facial nerve, often the seventh cranial nerve (CN VII). Other cranial nerves (CN) might be also involved, including CN VIII, IX, V, and VI (in order of frequency). This infection often causes vesiculation and ulceration of the external ear, as well as ipsilateral facial neuropathy (in CN VII), radiculoneuropathy, or geniculate ganglionopathy. In some cases there are also vesicles on the ipsilateral anterior two thirds of the tongue and soft palate. <br> | Ramsay Hunt syndrome is defined as VZV infection of the head and neck that involves the facial nerve, often the seventh cranial nerve (CN VII). Other cranial nerves (CN) might be also involved, including CN VIII, IX, V, and VI (in order of frequency). This infection often causes vesiculation and ulceration of the external ear, as well as ipsilateral facial neuropathy (in CN VII), radiculoneuropathy, or geniculate ganglionopathy. In some cases there are also vesicles on the ipsilateral anterior two thirds of the tongue and soft palate. <br> | ||
VZV infection causes 2 distinct clinical syndromes. Primary infection, also known as varicella or chickenpox, is a common pediatric erythematous disease characterized by a highly contagious generalized vesicular rash. The annual incidence of varicella infection has significantly declined after the introduction of mass vaccination programs in most countries of the world<ref>Kleinschmidt-DeMasters BK, Gilden DH. The expanding spectrum of herpesvirus infections of the nervous system. Brain Pathol. 2001 Oct. 11(4):440-51</ref>. | VZV infection causes 2 distinct clinical syndromes. Primary infection, also known as varicella or chickenpox, is a common pediatric erythematous disease characterized by a highly contagious generalized vesicular rash. The annual incidence of varicella infection has significantly declined after the introduction of mass vaccination programs in most countries of the world<ref>Kleinschmidt-DeMasters BK, Gilden DH. The expanding spectrum of herpesvirus infections of the nervous system. Brain Pathol. 2001 Oct. 11(4):440-51</ref>. | ||
After chickenpox, VZV remain latent in neurons of cranial nerve and dorsal root ganglia. Subsequent reactivation of latent VZV can result in localized vesicular rash, known as herpes zoster. VZV infection or reactivation involving the geniculate ganglion of CN VII within the temporal bone is the main pathophysiological mechanism of Ramsay Hunt syndrome | After chickenpox, VZV remain latent in neurons of cranial nerve and dorsal root ganglia. Subsequent reactivation of latent VZV can result in localized vesicular rash, known as herpes zoster. VZV infection or reactivation involving the geniculate ganglion of CN VII within the temporal bone is the main pathophysiological mechanism of Ramsay Hunt syndrome | ||
[[Image:PP RHS.png|right|300px]] | [[Image:PP RHS.png|right|300px]] | ||
== Clinical Presentation == | == Clinical Presentation == | ||
| Line 28: | Line 28: | ||
Patients usually present with paroxysmal pain deep within the ear. The pain often radiates outward into the pinna of the ear and may be associated with a more constant, diffuse, and dull background pain. The onset of pain usually precedes the rash by several hours and even days. | Patients usually present with paroxysmal pain deep within the ear. The pain often radiates outward into the pinna of the ear and may be associated with a more constant, diffuse, and dull background pain. The onset of pain usually precedes the rash by several hours and even days. | ||
Classic Ramsay Hunt syndrome can be associated with the following: | Classic Ramsay Hunt syndrome can be associated with the following: | ||
Vesicular rash of the ear or mouth (as many as 80% of cases)<br>The rash might precede the onset of facial paresis/palsy (involvement of the seventh cranial nerve [CN VII])<br>Ipsilateral lower motor neuron facial palsy (CN VII)<br>Vertigo and ipsilateral hearing loss (CN VII)<br>Tinnitus<br>Otalgia<br>Headaches<br>Dysarthria<br>Gait ataxia<br>Fever | Vesicular rash of the ear or mouth (as many as 80% of cases)<br>The rash might precede the onset of facial paresis/palsy (involvement of the seventh cranial nerve [CN VII])<br>Ipsilateral lower motor neuron facial palsy (CN VII)<br>Vertigo and ipsilateral hearing loss (CN VII)<br>Tinnitus<br>Otalgia<br>Headaches<br>Dysarthria<br>Gait ataxia<br>Fever | ||
== Diagnostic Procedures == | == Diagnostic Procedures == | ||
| Line 40: | Line 40: | ||
In particular, VZV antigen detection by direct immunofluorescence assay (DFA) is used, which has sensitivity of 90% and specificity close to 99%<ref>Coffin SE, Hodinka RL. Utility of direct immunofluorescence and virus culture for detection of varicella-zoster virus in skin lesions. J Clin Microbiol. 1995 Oct. 33(10):2792-5</ref>. | In particular, VZV antigen detection by direct immunofluorescence assay (DFA) is used, which has sensitivity of 90% and specificity close to 99%<ref>Coffin SE, Hodinka RL. Utility of direct immunofluorescence and virus culture for detection of varicella-zoster virus in skin lesions. J Clin Microbiol. 1995 Oct. 33(10):2792-5</ref>. | ||
Structural lesions (such as [[ | Structural lesions (such as [[Acoustic Neuroma|Acoustic Neuroma]]) can be ruled out by MRI or CT scan.<br> | ||
== Outcome Measures == | == Outcome Measures == | ||
Revision as of 23:58, 7 December 2015
Original Editor - Wendy Walker
Lead Editors - Wendy Walker
Introduction
[edit | edit source]
Ramsay Hunt syndrome is defined as an acute peripheral facial palsy/neuropathy associated with erythematous vesicular rash of the skin of the ear canal, auricle (also termed herpes zoster oticus), and/or mucous membrane of the oropharynx[1]. It is commonly asociated with moderate to severe otalgia (earache).
This syndrome is also known as geniculate neuralgia or nervus intermedius neuralgia.
The syndrome was first described in 1907 by Dr James Ramsay Hunt, describing a patient who had otalgia (ear pain) associated with cutaneous and mucosal rashes, which he ascribed to infection of the geniculate ganglion by human herpesvirus 3 (ie, varicella-zoster virus [VZV])[2].
Mechanism of Injury / Pathological Process
[edit | edit source]
Ramsay Hunt syndrome is defined as VZV infection of the head and neck that involves the facial nerve, often the seventh cranial nerve (CN VII). Other cranial nerves (CN) might be also involved, including CN VIII, IX, V, and VI (in order of frequency). This infection often causes vesiculation and ulceration of the external ear, as well as ipsilateral facial neuropathy (in CN VII), radiculoneuropathy, or geniculate ganglionopathy. In some cases there are also vesicles on the ipsilateral anterior two thirds of the tongue and soft palate.
VZV infection causes 2 distinct clinical syndromes. Primary infection, also known as varicella or chickenpox, is a common pediatric erythematous disease characterized by a highly contagious generalized vesicular rash. The annual incidence of varicella infection has significantly declined after the introduction of mass vaccination programs in most countries of the world[3].
After chickenpox, VZV remain latent in neurons of cranial nerve and dorsal root ganglia. Subsequent reactivation of latent VZV can result in localized vesicular rash, known as herpes zoster. VZV infection or reactivation involving the geniculate ganglion of CN VII within the temporal bone is the main pathophysiological mechanism of Ramsay Hunt syndrome
Clinical Presentation[edit | edit source]
The presenting feature is often pain deep within the ear. A herpetic rash or blisters, which may be on the skin of the ear canal, auricle or both.
Patients usually present with paroxysmal pain deep within the ear. The pain often radiates outward into the pinna of the ear and may be associated with a more constant, diffuse, and dull background pain. The onset of pain usually precedes the rash by several hours and even days.
Classic Ramsay Hunt syndrome can be associated with the following:
Vesicular rash of the ear or mouth (as many as 80% of cases)
The rash might precede the onset of facial paresis/palsy (involvement of the seventh cranial nerve [CN VII])
Ipsilateral lower motor neuron facial palsy (CN VII)
Vertigo and ipsilateral hearing loss (CN VII)
Tinnitus
Otalgia
Headaches
Dysarthria
Gait ataxia
Fever
Diagnostic Procedures[edit | edit source]
In most cases, the diagnosis is made purely on clinical findings: the presence of herpetic blisters in and around the ear, accompanied in most cases by pain in and around the ear and an ipsilateral facial palsy.
On occasions virological studies, both serological and molecular, can be used to confirm the clinical diagnosis.
In particular, VZV antigen detection by direct immunofluorescence assay (DFA) is used, which has sensitivity of 90% and specificity close to 99%[4].
Structural lesions (such as Acoustic Neuroma) can be ruled out by MRI or CT scan.
Outcome Measures[edit | edit source]
add links to outcome measures here (see Outcome Measures Database)
Management / Interventions
[edit | edit source]
add text here relating to management approaches to the condition
Differential Diagnosis
[edit | edit source]
add text here relating to the differential diagnosis of this condition
Key Evidence[edit | edit source]
add text here relating to key evidence with regards to any of the above headings
Resources
[edit | edit source]
add appropriate resources here
Case Studies[edit | edit source]
add links to case studies here (case studies should be added on new pages using the case study template)
Recent Related Research (from Pubmed)[edit | edit source]
Extension:RSS -- Error: Not a valid URL: Feed goes here!!|charset=UTF-8|short|max=10
References[edit | edit source]
References will automatically be added here, see adding references tutorial.
- ↑ Bhupal HK. Ramsay Hunt syndrome presenting in primary care. Practitioner. 2010 Mar. 254(1727):33-5
- ↑ Goldani LZ, Ferreira da Silva LF, Dora JM. Ramsay Hunt syndrome in patients infected with human immunodeficiency virus. Clin Exp Dermatol. 2009 Jun 1
- ↑ Kleinschmidt-DeMasters BK, Gilden DH. The expanding spectrum of herpesvirus infections of the nervous system. Brain Pathol. 2001 Oct. 11(4):440-51
- ↑ Coffin SE, Hodinka RL. Utility of direct immunofluorescence and virus culture for detection of varicella-zoster virus in skin lesions. J Clin Microbiol. 1995 Oct. 33(10):2792-5
