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]
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Conflicts of interest
There are no conflicts of interest.