Varicella zoster encephalitis in an immunocompetent patient.

Encephalitis is inflammation of the brain parenchyma, most often caused by viruses. Historically, data have shown herpes simplex virus 1 and 2 to be the most common causes of viral encephalitis, with cases due to varicella zoster virus (VZV) more often seen in older age and immunocompromised states. However, emerging data show VZV as an increasingly common culprit of encephalitis in young, immunocompetent patients. PCR analysis of the cerebrospinal fluid is the most accurate diagnostic modality for viral encephalitis. Appropriate and complete treatment hinges on accurate identification of the cause of encephalitis, underscoring the need for comprehensive testing. We present a case of VZV encephalitis in an immunocompetent male patient in his 40s.

Varicella zoster virus encephalitis: a potentially serious complication during treatment with vedolizumab.

Vedolizumab is a monoclonal antibody that has demonstrated efficacy and a good safety profile in patients with inflammatory bowel disease. Varicella zoster virus encephalitis is a potentially serious complication not previously described with its use, highlighting the importance of vaccination, as well as early diagnosis and treatment of infections in this type of patients.

Clinical and neuroradiologic characteristics in varicella zoster virus reactivation with central nervous system involvement.

OBJECTIVE: To investigate the clinical and magnetic resonance imaging (MRI) characteristics of patients with varicella zoster virus (VZV) reactivation involving the cranial nerves and central nervous system (CNS). METHODS: This is a retrospective, multi-center case-series of 37 patients with VZV infection affecting the cranial nerves and CNS. RESULTS: The median age was 71 years [IQR 51.5-76]; 21 (57%) were men. Cerebrospinal fluid (CSF) was available in 24/37 (65%); median CSF white blood cell count was 11 [IQR 2-23] cells/µL and protein was 45.5 [IQR 34.5-75.5] mg/dL. VZV polymerase chain reaction (PCR) assays were positive in 6/21 (29%) CSF and 8/9 (89%) ocular samples. Clinical involvement included the optic nerve in 12 (32%), other cranial nerves in 20 (54%), brain parenchyma in 12 (32%) and spinal cord or nerve roots in 4 (11%). Twenty-seven/28 immunocompetent patients' MRIs were available for review (96%). Of the 27, 18 had T1 postcontrast fat saturated sequences without motion artifact to evaluate for cranial nerve enhancement and optic perineuritis (OPN). Eight/18 (44%) demonstrated OPN. All 8 experienced vision loss: 3 optic neuritis, 1 acute retinal necrosis, and 3 CNS vasculitis with 1 central and 1 branch retinal artery occlusion and 1 uveitis. Diplopic patients had cranial nerve and cavernous sinus enhancement. All immunosuppressed patients were imaged. Seven/9 (88%) had extensive neuraxis involvement, including encephalitis, vasculitis and transverse myelitis; one case had OPN. CONCLUSION: OPN is a frequent manifestation in VZV-associated vision loss among immunocompetent patients. Immunosuppressed patients had greater neuraxis involvement. Optimizing MRI protocols may improve early diagnosis in VZV reactivation.

A case of ischemic stroke secondary to varicella-zoster virus meningoencephalitis.

Varicella-zoster virus (VZV) lurks in cranial nerves and other brain ganglias after infection. Because middle cerebral artery (MCA) receives the ipsilateral trigeminal ganglia afferent innervations, the reactivated VZV infects the adventitia and intima of cerebral artery wall probably through this way and causes vascular inflammation, finally resulting in artery remodeling, vessel occlusion, and ischemia. In fact, there is a growing clinical recognition that there is an association between VZV reactivation and subsequent stroke. Here, we showed a case of ischemic stroke secondary to varicella-zoster virus meningoencephalitis and reviewed the literature to emphasize the importance of VZV-associated vasculopathy.

Cognitive impairment without altered levels of cerebrospinal fluid biomarkers in patients with encephalitis caused by varicella-zoster virus: a pilot study.

Varicella-zoster virus (VZV) is one of the most common agents causing viral infections of the central nervous system (CNS). VZV encephalitis is associated with severe neurological sequelae, despite antiviral treatment. Cognitive impairment has been reported and VZV has been associated with dementia. Our aim was to investigate the cognitive impairment and cerebrospinal fluid biomarkers in a follow-up study of patients with VZV encephalitis. Thirteen patients with VZV encephalitis, diagnosed by detection of VZV DNA in cerebrospinal fluid (CSF) by PCR and concomitant symptoms of encephalitis, were included. Neuropsychological assessment in parallel with a lumbar puncture to obtain CSF was performed 1.5-7 years after acute disease. The CSF biomarkers neurofilament light chain (NFL), S100B, glial fibrillary acidic protein (GFAP), amyloid-ß (Aß) 40 and Aß42, total tau (t-tau) and phosphorylated tau (p-tau) were analysed and compared to controls (n = 24). Cognitive impairment was shown in the domains of executive functions and speed/attention and to a minor degree in the domains of learning/memory and language, indicated by a significantly poorer performance on seven neuropsychological test variables. No convincing evidence of alterations in concentrations of biomarkers in the CSF were shown. Our results indicate that patients with VZV encephalitis suffer from cognitive impairment long time after acute disease. Importantly, these impairments do not seem to be accompanied by biomarker evidence of ongoing neuronal or astrocytic injury/activation or induction of dementia-related brain pathologies by the infection.

Mania presenting as a VZV encephalitis in the context of HIV.

Acute encephalitis can be life-threatening, especially in the immunocompromised population. Viruses are the main infectious agents, with varicella zoster virus (VZV) a common cause. Neuropsychiatric symptoms are well documented, but it is rare for mania to be the only symptom on presentation. Here, we report a case of hypomania in a 31-year-old white British heterosexual man who following investigation was found to be HIV positive and subsequently diagnosed with VZV encephalitis. To date, we are unaware of any similarly reported cases. It is important to raise awareness of atypical HIV presentations to improve clinical outcomes for patients.

Clinical spectrum and prognosis of neurological complications of reactivated varicella-zoster infection: the role of immunosuppression.

Immunosuppressed patients are at higher risk for developing herpes zoster (HZ), and neurological complications are frequent in them. However, the influence of immunosuppression (IS) on the severity and prognosis of neurological complications of varicella-zoster virus (VZV) reactivation is unknown. We studied retrospectively patients with neurological complications due to VZV reactivation who attended our hospital between 2004 and 2019. We aimed to assess the clinical spectrum, potential prognostic factors, and the influence of the immune status on the severity of neurological symptoms. A total of 98 patients were included (40% had IS). Fifty-five patients (56%) had cranial neuropathies which included Ramsay-Hunt syndrome (36 patients) and cranial multineuritis (23 patients). Twenty-one patients developed encephalitis (21%). Other diagnosis included radiculopathies, meningitis, vasculitis, or myelitis (15, 10, 6, and 4 patients, respectively). Mortality was low (3%). At follow-up, 24% of patients had persistent symptoms although these were usually mild. IS was associated with severity (defined as a modified Rankin scale greater than 2) (odds ratio, 4.23; 95% confidence interval, 1.74-10.27), but not with prognosis. Shorter latency between HZ and neurologic symptoms was the only factor associated with an unfavorable course (death or sequelae) (odds ratio, 0.82; 95% confidence interval, 0.71-0.95). In conclusion, the clinical spectrum of neurological complications in VZV reactivation is wide. Mortality was low and sequelae were mild. The presence of IS may play a role on the severity of neurological symptoms, and a shorter time between HZ and the onset of neurological symptoms appears to be a negative prognostic factor.

Determinants of neurological syndromes caused by varicella zoster virus (VZV).

Varicella zoster virus (VZV) is a pathogenic human herpes virus which causes varicella as a primary infection, following which it becomes latent in peripheral autonomic, sensory, and cranial nerve ganglionic neurons from where it may reactivate after decades to cause herpes zoster. VZV reactivation may also cause a wide spectrum of neurological syndromes, in particular, acute encephalitis and vasculopathy. While there is potentially a large number of coding viral mutations that might predispose certain individuals to VZV infections, in practice, a variety of host factors are the main determinants of VZV infection, both disseminated and specifically affecting the nervous system. Host factors include increasing age with diminished cell-mediated immunity to VZV, several primary immunodeficiency syndromes, secondary immunodeficiency syndromes, and drug-induced immunosuppression. In some cases, the molecular immunological basis underlying the increased risk of VZV infections has been defined, in particular, the role of POL III mutations, but in other cases, the mechanisms have yet to be determined. The role of immunization in immunosuppressed individuals as well as its possible efficacy in preventing both generalized and CNS-specific infections will require further investigation to clarify in such patients.

Indolent varicella encephalitis with vasculopathy in an immunocompromised patient.

A 68-year-old female with B-cell non-Hodgkin's lymphoma presented to us with sequential blindness followed by hemiparesis. Four months earlier, the patient had developed chicken pox that was treated with intravenous acyclovir. An MRI brain showed multiple cerebral infarcts and beaded appearance of her intracranial vasculature. PET-CT showed hypermetabolism in the right frontal lobe and pons suggestive of encephalitis. Cerebral spinal fluid examination showed 15 cells and varicella zoster vasculopathy (VZVV) polymerase chain reaction was positive. A final diagnosis of indolent VZVV vasculopathy and encephalitis in an immunocompromised individual was made. This case highlights the slow and indolent progression of varicella central nervous system involvement.

Acute renal failure related to high doses of acyclovir (15 mg/kg/8 h) during treatment of varicella zoster virus encephalitis.

Varicella zoster virus (VZV) is less susceptible than herpes simplex virus to acyclovir. The optimal acyclovir regimen during VZV encephalitis remains unknown. We report two cases of acute renal failure after an increase in acyclovir dosage from 10 mg to 15 mg/kg/8 h during the treatment of VZV encephalitis according to French guidelines.

A case of varicella zoster encephalitis with glossopharyngeal and vagus nerve injury as primary manifestation combined with medulla lesion.

Varicella zoster virus (VZV) can invade the brainstem or brain via the glossopharyngeal, vagus , or facial nerve, resulting in brainstem inflammation or encephalitis. We report the case of a 66-year-old male patient with a primary manifestation of medulla injury of the glossopharyngeal and vagus nerves, combined with a medulla lesion, who was misdiagnosed with lateral medullary syndrome. Facial nerve injury and earache subsequently occurred and human herpes virus 3 (VZV) was detected by second-generation sequencing of the cerebrospinal fluid. The final diagnosis was varicella zoster encephalitis, which improved after antiviral therapy.

Ramsay Hunt syndrome with pontine lesion: A case report.

RATIONALE: The coexistence of Ramsay Hunt syndrome (RHS) and varicella-zoster virus (VZV) encephalitis is rare. A patient who developed RHS after being infected with VZV, along with a pontine lesion, is reported in the present study. PATIENT CONCERNS: A 41-year-old male patient presented with his mouth askew for 7 days, and dizziness, accompanied by hearing loss for 3 days. DIAGNOSES: The patient was initially diagnosed with RHS. Brainstem encephalitis was confirmed by lumbar puncture and cerebrospinal fluid. Brain magnetic resonance imaging (MRI) and diffusion-weighted imaging (DWI) revealed how VZV entered the intracranial space along the vestibulocochlear nerve and facial nerve in the acute period. INTERVENTIONS: Intravenous acyclovir, IV, immunoglobulins (IVIg) and methylprednisolone were administered. OUTCOMES: The herpes was cleared up and left facial nerve palsy was improved, but hearing loss in the left ear did not improve. LESSONS: An MRI was necessary for some VZV infections limited to the cranial nerve, although there was no evidence of brain stem injury. DWI provided evidence, showing how VZV entered the brain in the early stage. This allowed the doctor to judge the necessity of a lumbar puncture.

Moyamoya syndrome as a manifestation of varicella-associated cerebral vasculopathy-case report and review of literature.

BACKGROUND: Varicella-associated cerebral vasculopathy (VACV) is a serious complication of Varicella zoster virus (VZV) infection. VACV has protean manifestations, with varying clinical, radiological features and prognosis. CASE DESCRIPTION: Moyamoya syndrome (MMS) with VACV is reported in few cases in the past. All the patients were in paediatric age group, presenting with multiple episodes of transient ischemic attacks (TIAs) and infarct. Our case was a 10-year-old Indian girl with ischemic stroke due to VACV who was treated with intravenous acyclovir. She presented 11 months later with multiple episodes of TIAs. Her angiogram showed bilateral moyamoya vasculature. Acetazolamide challenge study revealed areas of hypoperfusion. Previously reported such cases had been treated medically with steroids and antiplatelets. Most of these patients had resolution of motor symptoms after long follow-up; however, this period was marred by recurrent symptoms. Our patient underwent cerebral revascularisation procedure, following which her TIAs resolved, there was improvement in her limb power and, according to her parents, her performance in school has improved at 2-year follow-up. CONCLUSION: MMS can be a manifestation of VACV and should be suspected in paediatric patient of non-east Asian population. These patients require treatment with intravenous acyclovir to inactivate the virus. Those with TIAs should undergo cerebral revascularisation procedures. Medical management should be reserved for patients with adequate collaterals.

Varicella-Zoster Virus in Cerebrospinal Fluid at Relapses of Multiple Sclerosis is Infective in Vitro.

BACKGROUND: We have reported the presence of varicella-zoster virus (VZV) DNA and viral particles in the cerebrospinal fluid (CSF) of multiple sclerosis (MS) patients during exacerbation. It is not known whether these viruses are infective. AIM: To determine whether the VZV found in CSF of MS patients in exacerbation phase are infective. METHODS: VZV found in CSF of MS patients was quantified by qPCR. Vero E6 cell cultures were incubated with CSF of five MS cases positive for VZV DNA, containing herpes-like viral particles. Propagated virus harvested from these cultures were used to infect new VeroE6 cells. Localization of an immediate-early and a late structural VZV proteins was monitored by confocal microscopy after 72 h. CSF from five non-inflammatory neurological (NIN) patients were used as controls. RESULTS: A cytopathic effect was found in cultured cells inoculated with CSF from MS patients. Both, structural VZV glycoprotein (gB) and immediate-early VZV protein (IE62) were detected in Vero E6 cultures inoculated with samples from all five MS cases. CSF from control patients produced no effect on Vero E6 cells. CONCLUSION: When present in the CSF at relapses of MS, VZV is infective under in vitro conditions.

Encephalitis associated with Zika virus infection and reactivation of the varicella-zoster virus in a Brazilian child.

We report a case of encephalitis associated with Zika virus infection and reactivation of varicella-zoster virus in the central nervous system of a Brazilian child. This case raises the possibility that reactivation of the latent varicella-zoster virus may be a mechanism of neurological impairment induced by acquired Zika virus infection.

Varicella zoster virus cerebral aneurysmal vasculopathy presenting in a newly-diagnosed HIV-positive patient.

We report the case of a newly-diagnosed HIV-positive patient with varicella zoster virus aneurysmal vasculopathy confirmed on intrathecal antibody testing, despite a negative Cerebrospinal fluid (CSF) Varicella Zoster Virus (VZV) Polymerase Chain Reaction (PCR). This highlights the importance of prompt treatment with antiviral and steroid therapy in the presence of clinical or radiological suspicion whilst awaiting further confirmatory testing.