PMID- 11315201
OWN - NLM
STAT- MEDLINE
DCOM- 20010920
LR  - 20080116
IS  - 0174-304X (Print)
IS  - 0174-304X (Linking)
VI  - 32
IP  - 1
DP  - 2001 Feb
TI  - Fulminant course in a case of diffuse myelinoclastic encephalitis-- a case 
      report.
PG  - 41-4
AB  - 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.
FAU - Poppe, M
AU  - Poppe M
AD  - Children's Hospital, Technical University Dresden, Germany. 
      poppe@rcs.urz.tu-dresden.de
FAU - Bruck, W
AU  - Bruck W
FAU - Hahn, G
AU  - Hahn G
FAU - Weissbrich, B
AU  - Weissbrich B
FAU - Heubner, G
AU  - Heubner G
FAU - Goebel, H H
AU  - Goebel HH
FAU - Todt, H
AU  - Todt H
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - Germany
TA  - Neuropediatrics
JT  - Neuropediatrics
JID - 8101187
RN  - 0 (DNA, Viral)
SB  - IM
MH  - Biopsy
MH  - Brain/pathology
MH  - Child
MH  - DNA, Viral/analysis
MH  - Demyelinating Diseases/diagnosis/pathology
MH  - Diffuse Cerebral Sclerosis of Schilder/*diagnosis/pathology
MH  - Disease Progression
MH  - Follow-Up Studies
MH  - Herpesvirus 6, Human/genetics/isolation & purification
MH  - Humans
MH  - Intracranial Hypertension/diagnosis/pathology
MH  - Male
EDAT- 2001/04/21 10:00
MHDA- 2001/09/21 10:01
CRDT- 2001/04/21 10:00
PHST- 2001/04/21 10:00 [pubmed]
PHST- 2001/09/21 10:01 [medline]
PHST- 2001/04/21 10:00 [entrez]
AID - 10.1055/s-2001-12214 [doi]
PST - ppublish
SO  - Neuropediatrics. 2001 Feb;32(1):41-4. doi: 10.1055/s-2001-12214.