Related Experiment Video

Updated: Aug 15, 2026

A Primary Neuron Culture System for the Study of Herpes Simplex Virus Latency and Reactivation
12:22

A Primary Neuron Culture System for the Study of Herpes Simplex Virus Latency and Reactivation

Published on: April 2, 2012

Human herpesvirus-6 (HHV-6)-associated necrotizing encephalitis in Griscelli's syndrome

M Wagner1, J Müller-Berghaus, R Schroeder

  • 1Department of Pathology, University of Cologne Medical School, Germany.

Journal of Medical Virology
|November 20, 1997
PubMed

Insights

This study details a pediatric case of Griscelli's syndrome (GS) with severe central nervous system (CNS) damage. Human herpesvirus-6 (HHV-6) was identified as a key factor in the virus-associated CNS lesions.

Area of Science:

  • Neurology
  • Virology
  • Pediatrics

Background:

  • Griscelli's syndrome (GS) is a rare genetic disorder.
  • GS is characterized by pigmentary dilution, neurological deficits, and immunodeficiency.
  • Virus-associated central nervous system (CNS) lesions are a severe complication.

Observation:

  • A pediatric patient with GS presented with recurrent infections and meningitis.
  • The patient exhibited progressive signs of increased intracranial pressure, leading to death at 32 weeks.
  • Autopsy revealed CNS necroses and predominantly perivascular CD8 positive lymphohistiocytic infiltrates.

Findings:

  • Human herpesvirus-6 (HHV-6) genome and specific late antigen (H-AR 3) were detected in neurons, oligodendrocytes, and astrocytes.
  • The presence of HHV-6 was strictly associated with CNS lesions.
  • Other viruses and mycobacteria were not detected, suggesting a specific role for HHV-6.

Implications:

  • Virus-induced CNS damage in GS may not require active viral replication.
  • Immune reactions triggered by viral antigen expression could cause significant neurological damage.
  • This highlights the complex interplay between viral presence and host immune response in GS pathogenesis.

More Related Videos

Detection of the Genome and Transcripts of a Persistent DNA Virus in Neuronal Tissues by Fluorescent In situ Hybridization Combined with Immunostaining13:22

Detection of the Genome and Transcripts of a Persistent DNA Virus in Neuronal Tissues by Fluorescent In situ Hybridization Combined with Immunostaining

Published on: January 23, 2014

Differentiation of the SH-SY5Y Human Neuroblastoma Cell Line08:07

Differentiation of the SH-SY5Y Human Neuroblastoma Cell Line

Published on: February 17, 2016

Related Concept Videos

Herpes01:28

Herpes

Herpes simplex type 1 (HSV‑1) is a widespread pathogen responsible for orolabial lesions. It is an enveloped, double-stranded DNA (dsDNA) virus belonging to the family Herpesviridae. Once the virus infects a host cell, its double‑stranded DNA genome is delivered into the nucleus, where a coordinated cascade of immediate‑early, early, and late gene expression directs viral DNA replication, structural protein synthesis, and virion assembly. After primary infection of epithelial cells, HSV-1...
Arboviral Encephalitis01:25

Arboviral Encephalitis

Arboviral encephalitis refers to brain inflammation caused by arthropod-borne viruses, particularly those transmitted through mosquito vectors. Among these, West Nile virus (WNV), a member of the Flaviviridae family, is a significant public health concern. WNV is an enveloped, positive-sense, single-stranded RNA virus. Human infection typically begins when an infected mosquito introduces the virus into the dermis during feeding. The primary transmission cycle involves birds as amplifying hosts...
Cytomegalovirus Disease01:27

Cytomegalovirus Disease

Cytomegalovirus (CMV) disease is caused by human cytomegalovirus, a double-stranded DNA virus of the Herpesviridae family. While primary CMV infection is often asymptomatic in immunocompetent individuals, the virus can cause severe disease in neonates and immunocompromised patients. CMV is the most common cause of congenital viral infection in the United States, and a major pathogen in solid organ and hematopoietic stem cell transplant recipients.CMV is transmitted via bodily fluids, sexual...
Genital Herpes01:23

Genital Herpes

Genital herpes is a sexually transmitted infection primarily caused by herpes simplex virus type 2 (HSV-2), though herpes simplex virus type 1 (HSV-1) is increasingly implicated in genital infections, particularly among younger populations. Transmission occurs mainly through sexual contact, with asymptomatic viral shedding serving as a major route of spread. This characteristic makes HSV-2 difficult to control at a population level, as individuals may unknowingly transmit the virus even in the...
Encephalitis l: Introduction01:19

Encephalitis l: Introduction

Encephalitis is inflammation of the brain parenchyma, most often due to infections or autoimmune processes. It presents with neuropsychiatric features such as fever, altered mental status, behavioral changes, cognitive dysfunction, seizures, focal deficits, and sometimes autonomic instability. In some cases, the meninges are also involved, resulting in meningoencephalitis.Infectious CausesInfectious encephalitis is most commonly viral but can also result from bacterial, fungal, or parasitic...
Encephalitis ll: Pathophysiology01:26

Encephalitis ll: Pathophysiology

Encephalitis is inflammation of the brain parenchyma caused by direct viral invasion or immune-mediated mechanisms triggered by infections or tumors. Both processes lead to neuronal injury, disrupted neurotransmission, and diverse neurological symptoms, often with overlapping clinical and pathological features.Autoimmune EncephalitisIn autoimmune encephalitis, antibodies target neuronal antigens on cell surfaces, synapses, or within neurons. A key example is anti-NMDAR encephalitis, which can...