Baló’s concentric sclerosis associated with primary human herpesvirus 6 infection

Background: Baló’s concentric sclerosis (BCS) is a demyelinating disorder believed to be a rare variant of multiple sclerosis (MS). Human herpesvirus 6 (HHV-6) is a highly neurotropic virus causing severe central nervous system (CNS) infections predominantly following reactivation of latent HHV-6 in...

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Bibliographic Details
Published in:Journal of neurology, neurosurgery and psychiatry neurosurgery and psychiatry, 2005-12, Vol.76 (12), p.1723-1725
Main Authors: Pohl, D, Rostasy, K, Krone, B, Hanefeld, F
Format: Article
Language:English
Subjects:
Adolescent
Antibodies
Baló’s concentric sclerosis
BCS
Biological and medical sciences
central nervous system
CMV
CNS
Cytomegalovirus
Diagnostic tests
Diffuse Cerebral Sclerosis of Schilder - drug therapy
Diffuse Cerebral Sclerosis of Schilder - etiology
Diffuse Cerebral Sclerosis of Schilder - virology
Disease
DNA, Bacterial - analysis
EBV
Encephalitis
Encephalomyelitis
Epstein-Barr virus
Female
Guillain-Barre syndrome
herpes simplex virus
Herpes viruses
Herpesvirus 6, Human - genetics
Herpesvirus 6, Human - pathogenicity
HHV-6
HIV
HSV
Human herpesvirus 6
Human immunodeficiency virus
Human viral diseases
Humans
IFA
immune fluorescence assay
Immunoglobulin G - analysis
Immunoglobulins
Immunoglobulins - therapeutic use
Infections
Infectious diseases
iv immunoglobulins
IVIG
Learning disabilities
Measles
Medical sciences
multiple sclerosis
Multiple sclerosis and variants. Guillain barré syndrome and other inflammatory polyneuropathies. Leukoencephalitis
Neurology
neurovirology
Pathogenesis
Polymerase Chain Reaction
Roseolovirus Infections - complications
Serology
Short Report
TBE
tick borne encephalitis
Treatment Outcome
varicella zoster virus
Viral diseases
Viral diseases of the lymphoid tissue and the blood. Aids
VZV
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Summary:Background: Baló’s concentric sclerosis (BCS) is a demyelinating disorder believed to be a rare variant of multiple sclerosis (MS). Human herpesvirus 6 (HHV-6) is a highly neurotropic virus causing severe central nervous system (CNS) infections predominantly following reactivation of latent HHV-6 in immunocompromised individuals. Primary infection with HHV-6 usually occurs in early childhood manifesting as exanthema subitum. The clinical spectrum of primary infection in adolescents or adults has not yet been evaluated. Case report: A previously healthy 13 year old girl developed acute hemianopsia and anomia 5 days after an episode of fever and malaise of unknown origin. Cerebral MRI revealed three white matter lesions, one with ring-like contrast enhancement. Lumbar puncture showed mononuclear pleocytosis of 30 cells/µl, oligoclonal IgG, and a normal protein level. Follow up cerebral MRI scans revealed lamellar concentric hemispheric lesions characteristic of BCS. The first neurological symptoms of the patient coincided with primary HHV-6 CNS infection, diagnosed by a positive PCR test of the CSF together with seroconversion. Response to antiviral and corticosteroid treatment was only temporary, but immunoglobulin treatment has so far been followed by clinical stability for 30 months. Conclusions: To our knowledge, this is the first report both of an association between HHV-6 and BCS and of immunoglobulin treatment of BCS. A late primary infection with HHV-6 might be associated with BCS. Further studies in patients with this rare disease are needed to confirm this association and to evaluate the efficacy of antiviral and immunoglobulin treatment.
ISSN:0022-3050
1468-330X
DOI:10.1136/jnnp.2004.062331