We report on a 10-year old previously healthy boy who exhibited a fulminant and nearly
monophasic clinical course of demyelinating encephalitis with relapsing intracranial
hypertension syndrome. Histologic examination of a diagnostic brain biopsy revealed
an inflammatory demyelinating process with perivascular T lymphocytic infiltration
and axonal damage reminiscent of multiple sclerosis-like lesions. In the brain, the
DNA of human Herpes virus 6 (HHV6) was detectable. Eleven months after the initial
symptoms and on maintainance with oral steroids, MRI showed demyelination of both
hemispheres as well as demyelination of the brain stem and Wallerian degeneration.
The boy exhibited a severe neurologic defect syndrome. The clinical and radiological
course is unusual because of the asymmetric progression of the encephalitis and the
extensive confluent lesions without demarcated border or enhancement of the rim after
injection of gadolinium. The clinical course showed no definite steroid response.
The pathogenetic relevance of HHV6 remains elusive. Although single patients with
HHV6-associated encephalomyelitis have been reported, HHV6 DNA is occasionally detected
in brains of healthy individuals.
Key words
Myelinoclastic diffuse encephalitis - Schilder disease - HHV6 - Demyelination - Steroids
- ADEM
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Dr. Maja Poppe
Klinik und Poliklinik für Kinderheilkunde der TU Dresden
Fetscherstrasse 74
01307 Dresden
Germany
eMail: Poppe@rcs.urz.tu-dresden.de