Two cases of pharyngolaryngeal zoster advanced to multiple cranial neuropathy




Abstract


Varicella zoster virus (VZV) infection of the head and neck region may present with various symptoms. I present two cases of VZV infection of the pharynx and larynx with multiple cranial nerve neuropathies. Their initial symptoms such as sore throat, odynophagia, and dysphasia were complicated by otalgia, dizziness, hearing loss, or ipsilateral facial nerve paralysis. All of these lesions tended to lateralize to the ipsilateral side and endoscopic findings suggested VZV infections, which were confirmed by serial serologic examinations.



Introduction


Varicella zoster virus (VZV) infection has two distinctive clinical manifestations. There are chicken pox (varicella) caused by primary VZV infection and herpes zoster (shingles) as a result of a reactivation of preexisting VZV in the neurons of the cranial nerve or dorsal root ganglia . Herpes zoster infection is often associated with impaired immunity, and is characterized by unilateral painful herpetic vesicular eruption along with the dermatome . In the head and neck region, the most common presentation of herpes zoster is Ramsay Hunt syndrome (RHS). Patients present with facial nerve palsy, otalgia, and auricular vesicular eruption, with or without auditory or vestibular involvement . VZV infection of the head and neck may present as multiple cranial neuropathies that are not necessarily associated with cutaneous lesions, as presented in cases of RHS. In the absence of related herpes zoster oticus, herpes zoster of the laryngeal region with cranial nerve palsy is not common and is only reported in a few literature . To the best of my knowledge, herpetic eruptions involving the innervated mucosa of both the glossopharyngeal nerve and vagus nerve and associated with or without unilateral facial paralysis, sudden hearing loss, and vestibulopathy have never been reported in the literature in English. Here, I report two such cases and suggest serial serological tests for confirmation of the diagnosis.





Case 1


A 55-year-old woman presented with a 5-day history of sore throat, hoarseness, and dysphagia. She had visited some clinics and received medications for presumed acute pharyngitis associated with oropharyngeal ulcers before being referred to our hospital. The patient described a sore throat with pain radiating to the left ear as the first symptom, followed by a worsening dysphagia and hoarseness within 5 days. All of these symptoms were noted to localize on her left side. On physical examination, there was no evidence of skin rash within the regions of the ear, face, or oral cavity, but laryngoscopy revealed a swelling mucosa with erythematous erosion over the hypopharynx, aryepiglottic fold, arytenoid, and the epiglottis at the left side ( Fig. 1 ). There was also a significant weakness of the left soft palate with a deviation to the right in saying “Ah” and reduced gag reflex implying a possible left glossopharyngeal and vagus nerve palsy ( Fig. 2 ). In protruding, the tongue was deviated to the left side suggesting the left hypoglossal nerve palsy. Furthermore, the left vocal cord was paralyzed and fixed in the paramedian position ( Fig. 3 ), suggesting a possible vagus nerve palsy. Laboratory tests showed a white blood cell count of 4520/mm 3 with 72.4% neutrophils, 13.5% lymphocytes, and 12.8% monocytes. C-reactive protein was significantly increased with a concentration of 5.9 [< 0.3] mg/dL, and an erythrocyte sedimentation rate of 25 [< 20] mm/h.




Fig. 1


Laryngoscopic examination. Laryngoscopy reveals a swelling mucosa with erythematous erosion over the hypopharynx, aryepiglottic fold, arytenoid, and the epiglottis at the left side.



Fig. 2


Physical examination of oral cavity. There is a significant weakness of the left soft palate with an uvular (asterisk) deviation to the right in saying “Ah” and reduced gag reflex implying a possible left glossopharyngeal and vagus nerve palsy.



Fig. 3


Laryngoscopic examination. During respiration (A) and saying “Ah” (B), laryngoscopy reveals that the left vocal cord (asterisk) was paralyzed and fixed in the paramedian position, suggesting a possible vagus nerve palsy.


Neck computed tomography(CT) with enhancement revealed swelling at the left epiglottis and aryepiglottic fold ( Fig. 4 ). However, there was no definite enhanced mass lesion. Laryngoscopic findings, the results of laboratory tests and CT findings might imply multiple cranial nerve neuropathies not by tumorous condition, malignant infiltration or other infection, but by viral infection, especially VZV. I started antiviral therapy by Intravenous acyclovir and corticosteroids, after a viral infection workup consisting of a serologic immunoglobulin (Ig) titer of VZV IgM and IgG were performed. A prednisone regimen of 0.5 mg/kg of body weight was given twice daily, once in the morning and once in the evening. After a full dose daily for 7 days, dose was decreased to half daily for 7 days, then decreased to another half dose daily for 7 days, and then discontinued.




Fig. 4


Axial computed tomography with enhancement of the neck. Axial view shows swelling at the left epiglottis and aryepiglottic fold with several erosions (A, arrows). Even though the pyriform sinus is narrowed by swelling (B, arrowhead), there is no definite enhanced mass lesion.


Initially VZV IgM antibody was not detected, whereas VZV IgG concentrations reached 10.80 [negative < 0.80 index]. Follow up exam revealed VZV IgM concentration was 3.46 [negative < 0.80 index], and VZV IgG concentrations increased to 11.29 [negative < 0.80 index].


Two days after admission, she complained of left facial weakness. Neurological examination revealed left facial palsy with House–Brackmann (HB) grade IV. After a total 10-day course of acyclovir and 21-day course of corticosteroid treatment, the patient had improved odynophagia and healed mucosal lesions of the larynx and pharynx. Six months after discharge, she presented with incomplete recovery of facial palsy with HB grade III and much improved dysphagia and hoarseness, whereas left side vocal palsy remained persistent. In the laryngoscopic glottal closure was compensated but incomplete.

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Aug 25, 2017 | Posted by in OTOLARYNGOLOGY | Comments Off on Two cases of pharyngolaryngeal zoster advanced to multiple cranial neuropathy

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