Acute varicella zoster encephalitis without evidence of primary vasculopathy in a case-series of 20 patients

Abstract

Varicella zoster virus (VZV) is a leading cause of acute viral encephalitis but little is known about its clinical, biological and imaging features. Furthermore, the most favourable treatment regimen has not been determined. We studied a prospective cohort of 20 HIV-negative patients presenting with acute VZV encephalitis caused by primary infection or reactivation. VZV was identified in 16 of 20 cases by PCR detection of the DNA in the cerebrospinal fluid. The four remaining cases occurred during or soon after a VZV rash. The median age of the 17 adults was 76 (19-86) years; the three other patients were children (0.5-5 years). Three patients were immunocompromised. Nine adult patients presented with a rash. Eighteen patients presented with fever and an acute encephalitic syndrome: diffuse brain dysfunction, focal neurological signs, seizures and cranial nerve palsies. Three patients presented with either ventricular or subdural haemorrhage, one with myelitis, and one with asymptomatic stenosis of the middle cerebral artery. The imaging was either normal or revealed non-specific abnormalities such as cortical atrophy but no evidence of stroke. All patients were given acyclovir at various dosages and durations but the case fatality rate remained high (15%) and sequelae were frequently observed either at discharge or at follow-up 3 years later.

Pearls & Oy-sters: An unusual case of varicella-zoster virus cerebellitis and vasculopathy

“…6 TIAs and stroke are not uncommon after VZV reactivation in adults even without rash, and vasculopathy is caused by viral infection of the blood vessels. [5][6][7] In our patient, MRA did not document any focal narrowing or stenosis in the large and medium cerebral arteries, suggesting that disease exclusively affected small arteries in this patient. Acute cerebellar ataxia as a result of VZV is extremely rare in adults and it has not been reported following herpes zoster.…”

“…[1][2][3][4][5][6][7][8] 2. Clinical diagnosis of VZV neurologic disorders is confirmed by virologic analysis with the detection of VZV DNA or anti-VZV immunoglobulin G (IgG) antibody in the CSF.…”

A study on viral CNS inflammation beyond herpes encephalitis

“…Patients in our study presented with optic neuritis and cerebellar gait imbalance, another patient developed cervical myelitis, all patients suffered from altered mental state. No patient developed a typical rash, that may be absent in 37-55 % of VZVE patients (De Broucker et al 2012). In contrast to previous reports, when fever was present at initial presentation in 50-90 % (De Broucker et al 2012;Granerod et al 2010), all our patients were afebrile.…”

“…However, there are notes on a few VZV encephalitis cases with CSF WCC up to 1240 Mpt/l and CSF protein of 3900 mg/l showing normal MRI (De Broucker et al 2012).…”

Corticosteroid Treatment in Autoimmune Encephalitis

“…Notably, CNS reactivation may occur in the absence of skin lesions [34]. In children, on the other hand, most cases occur concurrently with chickenpox or in a post-infectious form [22,35]. Detection of antibodies to VZV in the CSF appears to have greater sensitivity than detection of viral DNA [36]; therefore, we recommend that both assays be sent when possible.…”