eMedicine Specialties > Neurology > Neurological Infections

Ramsay Hunt Syndrome: Differential Diagnoses & Workup

Author: Augusto A Miravalle, MD, Fellow, Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School
Contributor Information and Disclosures

Updated: Aug 20, 2009

Differential Diagnoses

Bell Palsy
Persistent Idiopathic Facial Pain
Postherpetic Neuralgia
Temporomandibular Disorders
Trigeminal Neuralgia

Other Problems to Be Considered

Adenocystic carcinoma
Carcinoma of the nasopharynx
External otitis
Otitis media
Referred pain
Dental infection or abscess
Labyrinthitis
Vertigo

Workup

Laboratory Studies

  • The diagnosis of Ramsay Hunt syndrome is usually made without difficulty when the clinical characteristics are present. If necessary, varicella zoster virus (VZV) may be isolated from vesicle fluid and inoculated into susceptible human or monkey cells for identification by serologic means.
  • WBC count, erythrocyte sedimentation rate (ESR), and serum electrolytes are helpful in distinguishing the infectious and inflammatory nature of this syndrome.
  • When CNS complications are suspected (eg, meningitis, meningoencephalitis, myelitis, arteritis [large and small vessel], and ventriculitis), spinal fluid analysis and CNS imaging studies are recommended.
  • Viral studies include the following:
    • VZV isolation in conventional cell culture is considered the definite diagnostic test. However, growing VZV in cell culture can be difficult and is usually too slow to be clinically helpful.  
    • The sensitivity of conventional cell culture is 30-40%, with a specificity of 100%.
    • Other tests, including Tzanck test, electron microscopy, and polymerase chain reaction (PCR) are generally more rapid and sensitive. The sensitivity of conventional PCR technique is estimated to be 60%.
    • VZV has been detected by PCR in the tear fluid of patients with Bell palsy (prevalence, 25-35%).
    • VZV antigen detection by direct immunofluorescence assay (DFA) is also possible, with sensitivity of 90% and specificity close to 99%.5
    • Antibody determinations on paired sera may be helpful in establishing the diagnosis by comparing titers at time of presentation and a few weeks later.

Imaging Studies

  • Structural lesions can be ruled out by CT scan, MRI, or magnetic resonance (MR) angiography.
  • Gadolinium enhancement of the vestibular and facial nerves on MRI has been described in Ramsay Hunt syndrome.
  • Recent advances in clinical MRI images (eg, 3-Tesla MRI, multichannel phased array coil, 3-dimensional fluid-attenuated inversion recovery [FLAIR]) allow the evaluation of subtle alterations at the level of the blood-labyrinthine barrier.6

Other Tests

  • Audiometry usually reveals sensorineural hearing loss.
  • Unilateral caloric weakness may be present on electronystagmography (ENG).
  • Electrodiagnostic methods, such as facial motor nerve conductions studies (electroneurography), electromyography of facial innervated muscles, the blink reflex, and nerve excitability testing, could add information regarding the extent of seventh cranial nerve (CN VII) involvement, as well as prognostic factors.4

Procedures

  • In the setting of a peripheral facial palsy, cerebrospinal fluid (CSF) rarely is analyzed. Although lumbar puncture is not recommended in the diagnosis of this disease, CSF findings can be helpful in confirming the diagnosis. In one study, CSF findings were abnormal in 11% of 239 patients with idiopathic peripheral facial palsy, in 60% of 17 patients with Ramsay Hunt syndrome (abnormal finding was pleocytosis), in 25% of 8 patients with Lyme disease, and in all 8 patients with HIV infection. Thus, if the CSF is abnormal, a specific cause should be sought.
  • Temporary relief of otalgia in geniculate neuralgia may be achieved by applying a local anesthetic or cocaine to the trigger point, if in the external auditory canal.

Histologic Findings

  • The affected ganglia of the cranial nerve roots are swollen and inflamed.
  • The inflammatory reaction is chiefly of a lymphocytic nature, but a few polymorphonuclear leukocytes or plasma cells may also be present.
  • Some of the cells of the ganglia are swollen and others degenerated.

Staging

Several scales have been developed to quantify the degree of facial muscle weakness. Of those, the House-Brackmann scale is most commonly used.4

The House-Brackmann facial neuropathy scale is as follows:

  • 1- Normal
  • 2 - Mild dysfunction (slight weakness only noticeable on close inspection)
  • 3 - Moderate dysfunction (obvious weakness, but not disfiguring differences between both sides)
  • 4 - Moderately severe dysfunction (obvious weakness and disfigurement)
  • 5 - Only barely perceptive motor function
  • 6 - Complete paralysis

More on Ramsay Hunt Syndrome

Overview: Ramsay Hunt Syndrome
Differential Diagnoses & Workup: Ramsay Hunt Syndrome
Treatment & Medication: Ramsay Hunt Syndrome
Follow-up: Ramsay Hunt Syndrome
Multimedia: Ramsay Hunt Syndrome
References
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References

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  18. Pitkaranta A, Piiparinen H, Mannonen L, Vesaluoma M, Vaheri A. Detection of human herpesvirus 6 and varicella-zoster virus in tear fluid of patients with Bell's palsy by PCR. J Clin Microbiol. Jul 2000;38(7):2753-5. [Medline].

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Further Reading

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Keywords

Ramsay Hunt syndrome, geniculate neuralgia, herpes zoster oticus, nervus intermedius neuralgia, herpesvirus 3, varicella-zoster virus, VZV, varicella zoster, tinnitus, deafness, vertigo, nystagmus, ataxia, ipsilateral facial neuropathy, radiculoneuropathy, geniculate ganglionopathy, chickenpox, treatment, diagnosis

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Contributor Information and Disclosures

Author

Augusto A Miravalle, MD, Fellow, Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School
Augusto A Miravalle, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Medical Editor

Marion Priscilla Short, MD, Assistant Professor, Departments of Neurology, Pediatrics, and Pathology, University of Chicago Hospitals and Clinics
Marion Priscilla Short, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuropathologists, American College of Medical Genetics, American Medical Association, and American Society of Human Genetics
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Florian P Thomas, MD, MA, PhD, Drmed, Director, Spinal Cord Injury Unit, St Louis Veterans Affairs Medical Center; Director, National MS Society Multiple Sclerosis Center; Professor, Department of Neurology and Psychiatry, Associate Professor, Institute for Molecular Virology, and Department of Molecular Microbiology and Immunology, St Louis University
Florian P Thomas, MD, MA, PhD, Drmed is a member of the following medical societies: American Academy of Neurology, American Paraplegia Society, and National Multiple Sclerosis Society
Disclosure: Nothing to disclose.

CME Editor

,, Kathy Roarty Placeholder
Disclosure: Nothing to disclose.

 
 
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