Early diagnosis and treatment of Ramsay Hunt Syndrome

K. Deepan, C. M. Santhosham

MEM-Resident, Kauvery Hospital, Cantonment, Trichy, Tamil Nadu

Critical Care Specialist, Kauvery Hospital, Cantonment, Trichy, Tamil Nadu

Background

Peripheral facial palsy indicative of lower motor neuron lesion is most frequently caused by idiopathic Bells palsy but also result from infections, Cholesteatoma, trauma, automatic disorders.

While the prognosis for Bells palsy is generally favourable the outcome for other causes depends on the underlying condition. Identifying the etiology is essential for effective treatment and improved outcomes.

RHS is rare complication of varicella zoster virus reactivation, presents with ipsilateral facial paralysis, earpain, vesicular rash. Early recogination is crucial for prompt treatment and optimal outcomes.

Case Presentation

A 62 years old male, with nil comorbidity presented to the emergency department with a one-week history of ear discomfort in left side which progressed to facial asymmetry, characterised by swaying to the right side facial palsy.

Sudden onset of giddiness, inability to walk since morning, swaying to left side while walking. Inability to fully close on his right side eye after symptoms onset. On the day prior to admission, he had worsening ear pain with block, noticed fluid filled blisters on left ear.

No H/O Fever, URTI. No past history of the symptoms. He reported no h/o of immune compromised condition recent vaccinations or travel.

On Examination

On receiving in ER, GCS-E4V5M6, EOM-Full, power-5/5, Gait-swaying to left side. Vitals signs were stable, Systemic examination was unremarkable expect grouped vesicles on his left auricle, extending to the external auditory canal. He exhibited left peripheral facial palsy

Routine investigation normal. MRI Brain and MRA was normal. He had left ear pain, with block, subtle Right LMN facial palsy, gaze disturbance and left herpes zoster oticus, The House- Brockimann system score was grade-II are found to have Ramsay Hunt Syndrome.

Management

He received IV acyclovir 500mg every 8th hourly and steroids as Wysolone 40mg OD. During his hospital stay his Facial palsy showed significant clinical improvement and hence discharged.

Upon discharge he was prescribed with oral antivirals (Val acyclovir 1gm every 8hrly, steroids Wysolone 40mg OD for 2 weeks) with follow up instructions charge.

Discussion

Ramsay Hunt Syndrome is a significant otologic complication VZV reactivation. The latent virus reactivation in the geniculate ganglion and may spread to the 8th cranial nerve, can also involve multiple cranial nerve V, IX, X.

Triad of symptoms as ipsilateral facial paralysis, ear pain, vesicles in auditory canal other symptoms including altered taste perception, tongue lesions, hearing abnormalities, lacrimation, vertigo, nystagmus.

Idiopathic causes for more than half of peripheral facial palsy with other etiologies including viral infection, diabetes, Lyme disease, Sarcoidosis. Among viral cause- HSV is most common pathogen followed by herpes zoster.

James Ramsay Hunt an American neurologist first described three distinct syndromes with HZO.

Several grading systems are used to evaluate severity of facial palsy including House – Brackmann, Sunnybrook. House-Brackmann system is most preferred due to quick, standardized evaluation.

Diagnosis of RHS is primarily based on clinical presentation but in uncertain cases, lab test such as PCR test, viral culture can confirm the diagnosis using CSF.

Treatment includes symptoms control, antiviral agents Glucorticoids. RHS has a high rate of complete recovery with up to 70.4% of patients regarding facial nerve. Particularly with early medical treatment.

Complication

Other than the presenting symptoms of pain, rash, facial paralysis, dysgeusia, hearing loss, tinnitus, vertigo, hoarseness, dysarthria, and others mentioned above, short-term complications of Ramsay Hunt syndrome include corneal abrasion and exposure keratopathy, depression and social anxiety, and transmission of chickenpox to unvaccinated or immunocompromised close contacts. While long-term flaccid paralysis is unlikely, the development of synkinesis is very common. Other long-term complications include postherpetic neuralgia, scarring from the vesicles, and persistent depression and/or social anxiety due to loss of facial function.

Conclusion

Early recognitions and treatment RHS are essential to prevent long term complication. In this case, 62years old male received early diagnosis and antiviral therapy leading to significant symptoms improvement healthcare providers should consider RHS in patients with facial palsy especially when accompanied by ear pain, vesicular rash to ensure prompt and effective treatment.

Reference

[1]    Kim SJ, Lee HY. Acute Peripheral Facial Palsy: Recent Guidelines and a Systematic Review of the Literature. J Korean Med Sci. 2020 Aug 3;35(30):e245. https://doi.org/10.3346/jkms.2020.35.e245

[2]    Adour KK. Otological complications of herpes zoster. Ann Neurol. 1994;35 Suppl:S62-4.https://doi.org/10.1002/ana.410350718

[3]    Goswami Y, Gaurkar SS. Ramsay Hunt Syndrome: An Introduction, Signs and Symptoms, and Treatment. Cureus. 2023 Jan;15(1):e33688.

[4]    Mat Lazim N, Ismail H, Abdul Halim S, Nik Othman NA, Haron A. Comparison of 3 Grading Systems (House-Brackmann, Sunnybrook, Sydney) for the Assessment of Facial Nerve Paralysis and Prediction of Neural Recovery. Medeni Med J. 2023 Jun 20;38(2):111-9.

[5]    Monsanto RD, Bittencourt AG, Bobato Neto NJ, Beilke SC, Lorenzetti FT, Salomone R. Treatment and Prognosis of Facial Palsy on Ramsay Hunt Syndrome: Results Based on a Review of the Literature. Int Arch Otorhinolaryngol. 2016 Oct;20(4):394-400. https://doi.org/10.1055/s-0036-1584267.

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