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Ramsay-Hunt Syndrome Accompanied by Dysphagia: A Videofluoroscopic Swallowing Study

Kim, Tae Uk MD; Kim, Seo Yeon MD; Kim, Jae Il MD, PhD; Lee, Seong Jae MD, PhD

American Journal of Physical Medicine & Rehabilitation: June 2013 - Volume 92 - Issue 6 - p 547–550
doi: 10.1097/PHM.0b013e318269ed5f
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From the Department of Rehabilitation Medicine (TUK, SYK, SJL) and Department of Neurology (JIK), Dankook University College of Medicine, Cheonan, Republic of Korea.

All correspondence and requests for reprints should be addressed to: Seong Jae Lee, MD, PhD, Department of Rehabilitation Medicine, Dankook University Hospital, San 16-5 Anseo-dong, Dongnam-gu Cheonan, Chungnam 330-715, Republic of Korea.

The present research was conducted by the research fund of Dankook University in 2010.

Financial disclosure statements have been obtained, and no conflicts of interest have been reported by the authors or by any individuals in control of the content of this article.

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Ramsay-Hunt syndrome is caused by reactivation of varicella zoster virus. It consists of facial nerve palsy, otalgia, and erythematous vesicular rash on the auricle and external auditory canal. Vesicular eruption may also develop on the pinna and retroauricular area. Since it was first reported by Ramsay-Hunt in 1907,1 atypical symptoms have also been reported, such as involvement of the other cranial nerves2,3 or dysphagia.4 Dysphagia has been reported occasionally in patients with Ramsay-Hunt syndrome, but the details of swallowing difficulty, including abnormalities in videofluoroscopic swallowing study (VFSS), have not been described. A few reports presented the findings of VFSS,5–7 but the description was insufficient. No report provided a clinical course of dysphagia or follow-up data of VFSS. The authors experienced and hereby report a case of Ramsay-Hunt syndrome accompanied by dysphagia, with the detailed results of VFSS. In this report, the course of dysphagia and VFSS were fully followed until resolution. To the authors’ knowledge, follow-up data of VFSS have never been published for patients with Ramsay-Hunt syndrome. This is an unprecedented report of sequential VFSS of dysphagia in Ramsay-Hunt syndrome.

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A 73-yr-old female patient was admitted to the department of internal medicine because of dysphagia that had developed 3 days before admission. She also complained of left-sided facial weakness, headache, otalgia, and dizziness. Her medical history and family history were unremarkable. Detailed physical examination revealed erythematous vesicles scattered on the left face and auricle. Movement of the tongue was restricted, gag reflex was hyporeflexic, and laryngeal elevation was decreased when swallowing saliva. Auditory examination showed normal hearing function, and laryngoscopy demonstrated normal vocal cord movement. No evidence of brain pathology was found on magnetic resonance imaging. Temporal bone computed tomography revealed inflammatory changes in the left middle ear. Two weeks after admission, perforation of the tympanic membrane was detected.

She was referred to the department of rehabilitation medicine for evaluation and management of swallowing difficulty. On the seventh day of admission, a VFSS was carried out according to the modified Logemann method.8 Parameters representing oropharyngeal swallowing function were analyzed while swallowing thin and thick barium liquid (Table 1). Oral bolus formation was inadequate, triggering of pharyngeal swallowing was delayed, and laryngeal elevation was reduced. The oral transit time, pharyngeal delay time, and pharyngeal transit time were prolonged, suggesting that oropharyngeal motility was impaired (Table 2). Massive bolus residue was found in both pyriform sinuses and epiglottic vallecula after swallowing and was aspirated with repeated swallowing attempts (Figs. 1A, B).







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Acyclovir (1050 mg/day) was administered intravenously for 7 days, and prednisolone (35 mg/day) was administered orally for 15 days, beginning on the day of admission. At the same time, swallowing training was conducted by an occupational therapist according to the results of the VFSS. Exercise and electrical stimulation of oral and pharyngeal muscles were emphasized in swallowing training, which included other techniques. On the tenth day of admission, gag reflex and laryngeal elevation became almost normal, but tongue movement was still limited. The patient was transferred to the department of rehabilitation medicine for intensive swallowing training, and oral feeding was tried with a soft blended diet.

The VFSS was repeated in the eighth week of admission (Table 2). Supraglottic penetration still existed, but aspiration was no longer seen. Oral transit time improved to reference range, although pharyngeal delay time and pharyngeal transit time were still prolonged. The amount of residue in the vallecula and pyriform sinuses decreased significantly. She was discharged and the hospital maintained oral feeding with a soft blended diet. At discharge, her facial palsy had gradually improved, the otalgia disappeared, and the eschar on the left side of the face and auricle was improved. Six months after onset, VFSS showed no penetration or aspiration, and the amount of pharyngeal residue was minimal (Table 2). Her diet was changed to a normal regular diet.

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Ramsay-Hunt syndrome is caused by varicella zoster virus and leads to facial weakness, Bell palsy, hearing loss, a painful rash on the eardrum, and vertigo.1,5 The diagnosis can usually be made clinically by the signs of facial weakness and vesicular rash. Treatment with antiviral medications, such as acyclovir or famciclovir, is recommended for 7 to 10 days, along with a strong anti-inflammatory drug such as prednisolone.9,10 Steroids have been used for a strong anti-inflammatory and antiedematous effect in the treatment of Ramsay-Hunt syndrome.11 The antiviral agent acyclovir, which is currently used for the treatment of Ramsay-Hunt syndrome, may prevent disease progression and improve prognosis by inhibiting the proliferation and spread of the herpes zoster virus.12 Ramsay-Hunt syndrome can be accompanied by a number of cranial nerve palsies, including facial, auditory, and trigeminal nerves.3,10 Rarely, involvement of the cranial nerves (IV, IX, XI, and XII) may result in hearing disturbance, tinnitus, dizziness, or hoarseness.3,13 Some authors reported cases of Ramsay-Hunt syndrome that invaded multiple cranial nerves (V, VII, IX, and X), resulting in unilateral vocal cord palsy and facial hypoesthesia.14 Although it is not clear whether the medical treatment is effective in the treatment of atypical cranial nerve dysfunction and dysphagia, the present case indicates that dysphagia, along with other clinical symptoms, resolved after treatment with antiviral and steroid medications.

In this case, VFSS revealed more detailed features and noticeable progress until the dysphagia completely resolved. The initial VFSS showed reduced pharyngeal motility, resulting in accumulation of a lot of residue in the vallecula and pyriform sinuses; the residue was eventually aspirated with repeated swallowing of thick and thin liquid. After medical treatment and swallowing training, the VFSS identified improvement of swallowing function. Penetration and aspiration disappeared, and the oropharyngeal motility (represented by oral transit time, pharyngeal delay time, and pharyngeal transit time) recovered to reference range. The patient showed progressive improvement and finally recovered completely.

Most existing reports usually only describe the presence of dysphagia or aspiration symptoms, depending on the subjective complaints of the patients.4,15 The authors found three VFSS reports that related dysphagia with Ramsay-Hunt syndrome5–7; those reports only indicated the presence of penetration or aspiration.6,7 In one report,5 VFSSs were analyzed about the degree of pharyngeal wall contraction, laryngeal elevation, and presence of aspiration. No further details about oral dysfunction or time parameters (such as oral transit time, pharyngeal delay time, and pharyngeal transit time) were presented.

Even in the reports in which VFSS was conducted, the progression of dysphagia was followed only by the patient’s symptomatic improvement.5–7 The present report is valuable in that the dysphagia in Ramsay-Hunt syndrome was analyzed objectively by VFSS and traced until the dysphagia completely resolved. Follow-up VFSS showed serial decrease in residue and aspiration and shortening of oropharyngeal transit time. The present report would provide valuable information about the evaluation and treatment of dysphagia in Ramsay-Hunt syndrome. The authors recommend VFSS as a useful diagnostic tool for dysphagia in Ramsay-Hunt syndrome.

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In a patient with Ramsay-Hunt syndrome accompanied by dysphagia, the authors found and report a decrease in oropharyngeal motility, which resulted in a large amount of residue that was subsequently aspirated with repeated swallowing. The abnormalities were identified by VFSS and have never before been reported. The authors suggest that VFSS provides more concrete evaluation of dysphagia in Ramsay-Hunt syndrome and helps clinicians establish a strategy for proper treatment.

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1. Hunt J: On herpetic inflammations of the geniculate ganglion: A new syndrome & its complication. J Nerv Ment Dis 1907; 34: 73–96
2. Nicola M, Michelangelo M, Gianfranco C, et al.: Ramsay-Hunt syndrome complicated by unilateral multiple cranial nerve palsies. Neurol Sci 2008; 29: 497–8
3. Sugita K, Sato S, Koizuka I: Ramsay Hunt syndrome with cranial polyneuropathy involving cranial nerves VII, VIII, IX, and X. Nihon Jibiinkoka Gakkai Kaiho 2009; 112: 656–9
4. Wang F, Chen X: Case of Ramsay-Hunt syndrome associated with dysphagia. J Dermatol 2011; 38: 1195–7
5. Chitose S, Umeno H, Hamakawa S, et al.: Unilateral associated laryngeal paralysis due to varicella-zoster virus: Virus antibody testing and videofluoroscopic findings. J Laryngol Otol 2008; 122: 170–6
6. Ju S, Han J, Choi I, Lee S, et al.: The correlation between peripheral facial neuropathy and oropharyngeal dysfunction. Ann Rehabil Med 2003; 27: 551–6
7. Shim J, Park J, Kwon B, et al.: Dysphagia in Ramsay Hunt syndrome. Ann Rehabil Med 2011; 35: 738–41
8. Logemann J: Evaluation and Treatment of Swallowing Disorders. 2nd ed. San Diego, CA, College Hill Press, 1998
9. John D, Thomas S, Sharon G: Herpes zoster oticus: Treatment with intravenous acyclovir. Laryngoscope 1988; 98: 776–9
10. Sweeney C, Gilden D: Ramsay Hunt syndrome. J Neurol Neurosurg Psychiatry 2001; 71: 149–54
11. Minoru K, Mutsuo A, Mitsuhiro M, et al.: Acyclovir improves recovery rate of facial nerve palsy in Ramsay Hunt syndrome. Auris Nasus Larynx 2001; 28: 223–6
12. Ryu E, Lee H, Lee S, et al.: Clinical manifestations and prognosis of patients with Ramsay Hunt syndrome. Am J Otolaryngol 2012; 33: 313–8
13. Xanthopoulos J, Noussios G, Papaioannides D, et al.: Ramsay Hunt syndrome presenting as a cranial polyneuropathy. Acta Otorhinolaryngol Belq 2002; 56: 319–23
14. Miyazaki Y, Tajima Y, Sudo K, et al.: A case of Ramsay Hunt syndrome initiated with hoarseness and dysphagia: Consideration on spreading mechanisms of cranial neuropathy. Rinsho Shinkeiqaku 2002; 42: 855–8
15. Coleman C, Fozo M, Rubin A: Ramsay Hunt syndrome with severe dysphagia. J Voice 2012; 26: 27–8
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CME Self-Assessment Exam Answers CME Answers

American Journal of Physical Medicine & Rehabilitation

Vol. 92, No. 6 • June 2013

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CME Article • 2013 Series • Number 5:

T. Ellis, et al.

  1. B
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