Polyneuritis Cranialis Caused by Varicella Zoster Virus in the Absence of Rash

Abstract

A 21-year-old man presented with acute hoarseness, pharyngeal pain, and dysphagia on December 24, 2008. He was able to drink water only and his condition did not improve; therefore, he was admitted to our hospital on January 5, 2009. On admission, his body temperature was 38.5°C, and he had mild headache and tenderness of the left external auditory meatus. There was no evidence of a skin rash around his ears or face, and no rash was observed in the oral cavity. No lymphadenopathy or hepatosplenomegaly was observed. On neurologic examination, there was paralysis of the left soft palate and the constrictor muscles of the pharynx and hypoesthesia of the left soft palate and pharynx. The remainder of the neurologic examination was normal. Meningeal signs, long tract signs, and autonomic dysfunction were not observed. These findings were consistent with acute isolated left hemilateral glossopharyngeal and vagus nerve palsy.CSF examination revealed a cell count of 218/mm 3 (mononuclear cells: polynuclear cells was 216:2), and a protein concentration of 56 mg/dL. Antibody titers against varicella zoster virus (VZV) were elevated in both the serum (enzyme immunoassay [EIA]: immunoglobulin G [IgG], 78.6; immunoglobulin M [IgM], 3.78; normal value IgG Ͻ2.0, IgM Ͻ0.8) and CSF (EIA: IgG, 0.57; IgM, 1.39). We performed PCR to identify VZV-DNA in the CSF and pharyngeal scraping throat exudates, using methods described previously. 1 VZV-DNA was detected in both CSF and throat exudates.Cranial MRI with gadolinium enhancement was performed on the day of admission. On plain T1weighted images and T2-weighted images, there was an isointense nodular lesion near the left jugular foramen. The T1-weighted image with contrast showed that this nodular lesion was enhanced (figure). Additional enhancement was observed at the surface of the brainstem. The neurologic findings, presence of antibodies against VZV, and PCR-positive reaction against VZV-DNA supported a diagnosis of polyneuritis cranialis due to VZV, in the absence of a rash. The patient was treated with IV acyclovir (750 mg/day) for 14 days. His symptoms were immediately improved and no residual signs were observed. The cell count, protein concentration, and anti-VZV antibodies in the CSF were decreased after the treatment (cell count, 9/mm 3 ; protein, 53 mg/dL; and EIA: IgG, 2.77; IgM, 0.82). Discussion.Our patient showed only unilateral lower cranial neuritis (glossopharyngeus and vagus nerve) without herpetic lesions. The diagnosis of lower cranial neuropathy was based on the elevation of VZV-IgM antibodies and a PCR-positive reaction against VZV-DNA in the CSF.A noteworthy feature of this patient was the lack of cutaneous herpetic manifestation through the course of the disease. A wide spectrum of neurologic disorders that include polyneuritis cranialis, aseptic meningitis, encephalitis, acute polyneuritis, and myelitis are associated with VZV infection without skin lesions. 2-4 This condition has been termed zoster sine herpete. 5,6 We diagnosed our patient with a lower cra...

Varicella Zoster Virus Meningoencephalitis Presenting with Elsberg Syndrome without a Rash in an Immunocompetent Patient

“…The dermatological clinical manifestations of VZV range from primary infection with chickenpox to reactivated infection with shingles. VZV reactivation usually presents with a skin rash, although cases of VZV infection without skin rashes (zoster sine herpete) associated with neurological diseases have been reported (3,4).…”

Varicella-zoster

“…Importantly, multiple forms of trigeminal (Easton, 1970; Hevner et al, 2003; Yamada et al, 2003) and facial (Morgan and Nathwani, 1992; Murakami et al, 1998; Terada et al, 1998) distribution zoster as well as polyneuritis cranialis due to VZV (Osaki et al, 1995; Murata et al, 2010) may occur in the absence of rash.…”

Complications of Varicella Zoster Virus Reactivation

“…The blood supply of cranial nerves III, IV, V1 and VI comes from the carotid circulation, while V 2 , V 3 and VII, IX, X, XI and XII are supplied by the external carotid circulation [15]. It is important to recognize that multiple forms of trigeminal- [16, 17] and facial- [18] distribution zoster as well as polyneuritis cranialis due to VZV [19, 20] may occur in the absence of rash.…”