Physical: The primary physical findings in classic Ramsay Hunt syndrome include peripheral facial nerve paresis with associated rash or herpetic blisters in the distribution of the nervus intermedius. The location of the accompanying rash varies from patient to patient, as does the area innervated by the nervus intermedius. The patient may have associated ipsilateral hearing loss and balance problems. A thorough physical examination must be performed, including neuro-otologic and audiometric assessment.
Causes: Classic Ramsay Hunt syndrome is ascribed to infection of the geniculate ganglion by herpesvirus 3 (VZV).
Differentials: Atypical Facial Pain, Bell Palsy, Postherpetic Neuralgia, Temporomandibular Joint Syndrome, Trigeminal neuralgia.
Lab Studies: WBC count, erythrocyte sedimentation rate (ESR), and serum electrolytes are helpful in distinguishing the infectious and inflammatory nature of this syndrome. VZV, the virus responsible for this syndrome, is the causative agent for chickenpox. This agent can be cultured in human cells, where it tends to remain bound to cells. Serologic tests also can reveal VZV, although prior history of chickenpox may lead to a positive result. The diagnosis of VZV usually is made without difficulty when the characteristic rash is present as well as vesicular eruption. If necessary, VZV may be isolated from vesicle fluid and inoculated into susceptible human or monkey cells for identification by serologic means. Antibody determinations on paired sera may be helpful in establishing the diagnosis by comparing titers at time of presentation and a few weeks later. VZV can be detected by PCR on samples of tear fluid from affected individuals.
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.
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.
Medical Care: Corticosteroids and oral acyclovir are utilized commonly. Vestibular suppressants may be helpful if vestibular symptoms are severe. As with Bell palsy, care must be taken to prevent corneal irritation and injury. Temporary relief of otalgia may be achieved by applying a local anesthetic or cocaine to the trigger point, if in the external auditory canal. Carbamazepine may be helpful, especially in cases of idiopathic geniculate neuralgia.
Drug Category: Corticosteroids -- These agents reduce the inflammation of the cranial nerves and help alleviate the pain and neurologic symptoms.
Drug Name |
Prednisone (Deltasone) -- May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. May be taken during acute inflammatory period (1-2 wk) and then tapered slowly. As an alternative, Dosepaks (ie, several prepackaged tablets with decreasing doses) can be taken. Individualize dose based on response. |
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Adult Dose | 10 mg PO bid |
Pediatric Dose | Not established |
Contraindications | Documented hypersensitivity; fungal, viral, tubercular skin, and connective tissue infections; peptic ulcer disease; hepatic dysfunction |
Interactions | Coadministration with estrogens may decrease prednisone clearance; when used with digoxin, digitalis toxicity secondary to hypokalemia may increase; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics |
Pregnancy | B - Usually safe but benefits must outweigh the risks. |
Precautions | Patients on immunosuppressant doses of corticosteroids should be warned to avoid exposure to chickenpox and measles; drug-induced secondary adrenocortical insufficiency may be minimized by gradual reduction of dose; use cautiously in patients with ocular herpes simplex because of possible corneal perforation; use with caution in nonspecific ulcerative colitis, active or latent peptic ulcer disease, renal insufficiency, hypertension, osteoporosis and myasthenia gravis |
Drug Name |
Acyclovir (Zovirax) -- Patients experience less pain and faster resolution of symptoms when used within 48 h from onset of symptoms. May prevent recurrent outbreaks. |
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Adult Dose | Acute treatment: 800 mg PO q4h (5 times/d) for 7-10 d Chronic suppressive therapy for recurrent disease: 400 mg PO bid for 12 mo |
Pediatric Dose | Not established |
Contraindications | Documented hypersensitivity |
Interactions | Concomitant probenecid or zidovudine prolongs half-life and increases CNS toxicity |
Pregnancy | B - Usually safe but benefits must outweigh the risks. |
Precautions | Caution in renal failure or when using nephrotoxic drugs; possibility of appearance of less sensitive viruses in humans must be kept in mind |
Drug Category: Anticonvulsants -- Mechanism of action of antiepileptics in this syndrome is still unknown. Carbamazepine has been shown to help the neuralgic pain associated with this syndrome, especially in cases of idiopathic geniculate neuralgia.
Drug Name |
Carbamazepine (Tegretol) -- DOC that may reduce polysynaptic responses and block posttetanic potentiation. Adjust dose depending on response to treatment and blood levels. |
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Adult Dose | 400-800 mg PO qd in divided doses (usually tid) |
Pediatric Dose | Not established |
Contraindications | Documented hypersensitivity; history of bone marrow depression; administration of MAOIs within last 14 d |
Interactions | Serum levels may increase significantly within 30 days of danazol coadministration (avoid whenever possible); do not coadminister with MAOIs; cimetidine may increase toxicity, especially if taken in first 4 wk of therapy; carbamazepine may decrease primidone and phenobarbital levels (their coadministration may increase carbamazepine levels) |
Pregnancy | B - Usually safe but benefits must outweigh the risks. |
Precautions | Do not use to relieve minor aches or pains; caution with increased intraocular pressure; obtain baseline CBC and serum iron prior to treatment, during first 2 months, and yearly or every other year thereafter; can cause drowsiness, dizziness, and blurred vision; caution while driving or performing other tasks requiring alertness |
Prognosis: In general, prognosis is good for the resolution of symptoms. However, fewer than 50% of patients have complete recovery of facial function.