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      Ramsay Hunt Syndrome Leading to the Brainstem and Cerebellar Involvement: A Case Report of a Rare Co-occurrence

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          Abstract

          Dear Sir, A 55-year-old gentleman working as a farmer presented to the emergency department with sudden onset of left ear pain with erythematous rashes, which were initially vesicular and then gradually scabbed. On the 3rd day of rash, the patient developed bilateral facial paresis, flaccid dysarthria, dysphagia, and on the following day, he developed gait ataxia. He denied fever, headache, nuchal rigidity, altered consciousness level, vertigo, weakness, or other neurological complaints. He was not diabetic or hypertensive. He was a nonalcoholic and a nonsmoker. Plain computerized tomography (CT brain) done at another center was interpreted as normal. His cerebrospinal fluid analysis showed six cells with normal proteins and glucose. Qualitative polymerase chain reaction (PCR) for varicella zoster virus (VZV) in cerebrospinal fluid (CSF) was positive. Magnetic resonance imaging (MRI) showed T2 and fluid attenuation inversion recovery (FLAIR) hyperintense signal in the left dorsal medulla and pons [Figure 1], traceable to the intrparenchymal course of the left facial nerve, which was mildly enhancing on postgadolinium contrast. Susceptibility-weighted imaging (SWI) images did not reveal any blooming. Axial diffusion-weighted images revealed no restriction in the lesion. The angiogram was normal. Figure 1 On admission, brain magnetic resonance imaging showed T2 hyperintense signal in the left paramedian aspect of the dorsal medulla and pons traceable to the intraparenchymal course of the left facial nerve (a-c), with mild enhancement seen in the course of the cisternal and meatal segments (d). The lesion was T1 hypointense (e) with normal angiogram on time-of-flight sequence (f). It did not reveal diffusion restriction (g and h) or blooming on susceptibility-weighted sequence (i) He was initiated on intravenous methylprednisolone 1 g/day for 5 days and intravenous acyclovir at 30 mg/kg/day in three divided doses for 7 days followed by tablet valacyclovir 1 g twice daily for 7 days. Five days after the treatment, his gait ataxia improved with mild improvement in speech. His routine blood parameters, including complete blood count, renal function tests, liver function tests, sodium, and potassium, were normal. Serum B12 levels, folate levels, and homocysteine levels were normal. His human immunodeficiency virus (HIV) by enzyme-linked immunosorbent assay (ELISA), hepatitis B surface antigen (HBsAg), hepatitis C virus (HCV), and venereal disease research laboratory (VDRL) were nonreactive, and glycated hemoglobin (HBA1C) was 4.9%. Anti-nuclear antibody (ANA) and anti-neutrophil cytoplasmic antibody (ANCA) profiles were negative. The absolute CD4 count was 52 cells/μL. Pure tone audiometry showed a mild sensorineural hearing loss on the left side. DISCUSSION James Ramsay Hunt had first described this eponymous syndrome in 1907. Ramsay Hunt had hypothesized that Gasserian, geniculate, petrous, accessory, jugular, plexiform, and second and third cervical dorsal root ganglia comprised a chain of contiguous ganglia.[1] This could explain the associated tinnitus, hearing loss, nausea, vomiting, vertigo, and nystagmus found in some cases. A retrospective study of 102 patients with Ramsay Hunt Syndrome (RHS) done in 1988 showed that only 10% of the patients achieved complete recovery.[2] Varicella-Zoster vasculopathy (VZV) is a distinct complication of zoster infection leading to infarction or hemorrhage with MR angiogram abnormalities. Pietersen had reported 2,500 cases of peripheral facial nerve palsies, and in this study, a complete recovery was achieved only in 21% of the herpes zoster oticus cases.[3] Predisposing factors for reactivation include diabetes mellitus, spinal anesthesia, malignancies, and conditions associated with immune suppression such as lymphoma, steroid therapy, immunosuppressive agents, and acquired immunodeficiency syndrome (AIDS). However, none of these were evident in our patient. We describe an immunocompromised patient who developed brainstem and cerebellar involvement following RHS. Our patient presented with vesicular rash and left ear pain, following which he developed bilateral facial palsy, flaccid dysarthria, and dysphagia, which suggested brainstem involvement. He then developed gait ataxia, which suggested cerebellar involvement. Acute cerebellar involvement following VZV infection is well known in children, however, rare in adults. The MRI features, CSF analysis, and pure tone audiometry also correlated with the clinical picture. After 5 days of intravenous pulse methylprednisolone and intravenous acyclovir, the patient showed significant improvement in gait ataxia and mild improvement in dysphagia. Timely initiation of steroids and antiviral agents is the key to recovery.[4] However, dysarthria and bilateral facial paresis did not improve. The prognosis of facial palsy in RHS remains poor.[5] The recovery in this patient can be attributed to the pharmacological intervention or the natural course of the disease. The neurological illness being due to axonal spread of the virus, rather than a vasculopathy is supported by the fact that the course of the disease was progressive, rather than multiple transient ischemic attacks, with no evidence of diffusion restriction or angiogram abnormality on magnetic resonance imaging, and the T2 hyperintensities were not respecting strict vascular territories. A similar observation was noted by Hu et al. in 2003.[6] The CSF VZV PCR is sensitive as well as a specific biomarker for zoster infection with neurological symptoms.[7] It has been postulated that mutations in genes participating in the toll-like receptor 3 (TLR3) pathway may predispose to encephalitis caused by VZV.[8] Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

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          Bell's Palsy: The Spontaneous Course of 2,500 Peripheral Facial Nerve Palsies of Different Etiologies

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            Ramsay Hunt syndrome.

            The strict definition of the Ramsay Hunt syndrome is peripheral facial nerve palsy accompanied by an erythematous vesicular rash on the ear (zoster oticus) or in the mouth. J Ramsay Hunt, who described various clinical presentations of facial paralysis and rash, also recognised other frequent symptoms and signs such as tinnitus, hearing loss, nausea, vomiting, vertigo, and nystagmus. He explained these eighth nerve features by the close proximity of the geniculate ganglion to the vestibulocochlear nerve within the bony facial canal. Hunt's analysis of clinical variations of the syndrome now bearing his name led to his recognition of the general somatic sensory function of the facial nerve and his defining of the geniculate zone of the ear. It is now known that varicella zoster virus (VZV) causes Ramsay Hunt syndrome. Compared with Bell's palsy (facial paralysis without rash), patients with Ramsay Hunt syndrome often have more severe paralysis at onset and are less likely to recover completely. Studies suggest that treatment with prednisone and acyclovir may improve outcome, although a prospective randomised treatment trial remains to be undertaken. In the only prospective study of patients with Ramsay Hunt syndrome, 14% developed vesicles after the onset of facial weakness. Thus, Ramsay Hunt syndrome may initially be indistinguishable from Bell's palsy. Further, Bell's palsy is significantly associated with herpes simplex virus (HSV) infection. In the light of the known safety and effectiveness of antiviral drugs against VZV or HSV, consideration should be given to early treatment of all patients with Ramsay Hunt syndrome or Bell's palsy with a 7-10 day course of famciclovir (500 mg, three times daily) or acyclovir (800 mg, five times daily), as well as oral prednisone (60 mg daily for 3-5 days). Finally, some patients develop peripheral facial paralysis without ear or mouth rash, associated with either a fourfold rise in antibody to VZV or the presence of VZV DNA in auricular skin, blood mononuclear cells, middle ear fluid, or saliva. This indicates that a proportion of patients with "Bell's palsy" have Ramsay Hunt syndrome zoster sine herpete. Treatment of these patients with acyclovir and prednisone within 7 days of onset has been shown to improve the outcome of recovery from facial palsy.
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              TLR3 Mutations in Adult Patients With Herpes Simplex Virus and Varicella-Zoster Virus Encephalitis.

              Defects in genes of the Toll-like receptor 3 (TLR3) pathway are associated with susceptibility to herpes simplex virus type 1 encephalitis (HSE). We analyzed a cohort of 11 adult Italian patients in whom viral encephalitis developed. We detected 2 rare missense mutations in TLR3: 1 in a patient with HSE (p.Leu297Val) and 1 in a patient with varicella-zoster virus encephalitis (p.Leu199Phe). Both mutations are extremely rare in human populations and have pathogenicity scores highly suggestive of a functional effect. Data herein expand the phenotypic spectrum of TLR3 mutations to varicella-zoster virus encephalitis and support the role of TLR3 genetic defects as risk factors for HSE in adults.
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                Author and article information

                Journal
                Ann Indian Acad Neurol
                Ann Indian Acad Neurol
                AIAN
                Annals of Indian Academy of Neurology
                Wolters Kluwer - Medknow (India )
                0972-2327
                1998-3549
                Jan-Feb 2022
                03 June 2021
                : 25
                : 1
                : 159-161
                Affiliations
                [1]Department of Neurology, National Institute of Mental Health and Neurosciences, Hosur Road, Bengaluru, Karnataka, India
                [1 ]Department of Neuroimaging and Interventional Radiology, National Institute of Mental Health and Neurosciences, Hosur Road, Bengaluru, Karnataka, India
                Author notes
                Address for correspondence: Dr. Ravi Yadav, Department of Neurology, National Institute of Mental Health and Neuro Sciences (NIMHANS), Bangalore, Karnataka - 560 029, India. E-mail: docravi20@ 123456yahoo.com
                Article
                AIAN-25-159
                10.4103/aian.AIAN_48_21
                8954324
                b515ee93-c347-481d-bdda-5ea770c698b6
                Copyright: © 2006 - 2021 Annals of Indian Academy of Neurology

                This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.

                History
                : 22 January 2021
                : 29 January 2021
                : 31 January 2021
                Categories
                Letters to the Editor

                Neurology
                Neurology

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