PMID- 19804889
OWN - NLM
STAT- MEDLINE
DCOM- 20100212
LR  - 20211203
IS  - 1878-5883 (Electronic)
IS  - 0022-510X (Linking)
VI  - 287
IP  - 1-2
DP  - 2009 Dec 15
TI  - Activation of microglia/macrophages expressing phosphorylated S6 ribosomal 
      protein in a case of hemimegalencephaly with progressive calcification and 
      atrophy.
PG  - 52-9
LID - 10.1016/j.jns.2009.09.010 [doi]
AB  - A 3-year-old boy with right hemimegalencephaly (HME) showed massive calcification 
      in the subcortical white matter and progressive atrophy of the affected 
      hemisphere. Hemispherotomy was successful in amelioration of the patient's 
      intractable epilepsy, and a surgical specimen from the epileptic focus was 
      examined pathologically. Disarrangement of cortical layers along with dysmorphic 
      appearance of neurons, balloon cells in the cortex and white matter, bi-layered 
      calcifications in the superficial cortical layer and subcortical white matter, 
      heterotopic neurons in the white matter, and diffuse astrogliosis were noted. 
      Perivascular clustering of alpha-B-crystallin positive balloon cells was 
      occasionally observed in the area of calcification. A diffuse increase was 
      observed in the number of CD68-positive microglia/macrophages, particularly in 
      perivascular and peri-calcification areas. These cells were often located within 
      the calcification foci, which implicates their participation in the calcification 
      process. Phosphorylated S6 ribosomal protein (P-S6) was expressed in large-sized 
      neurons and numerous balloon cells, as well as in CD68-positive cells. In 
      contrast, phosphorylated mammalian target of rapamycin (mTOR) was expressed in a 
      small percentage of astrocytes, and phosphorylated p70S6 kinase was rarely 
      identified in perivascular cells. These findings suggest that inflammatory 
      processes have contributed to the pathogenesis of progressive calcification and 
      atrophy in the megalencephalic hemisphere in this patient. Dissociation of 
      expression of mTOR cascade components is common to other reported cases of HME, 
      but P-S6 expression in microglia/macrophages has not been recognized. The 
      cellular mechanism and significance of P-S6-specific activation of the mTOR 
      cascade in HME, particularly in the inflammatory cell lineage, should be explored 
      further.
FAU - Nonoda, Yutaka
AU  - Nonoda Y
AD  - Department of Child Neurology, National Center Hospital of Neurology and 
      Psychiatry, National Center of Neurology and Psychiatry, 4-1-1 Ogawahigashi-cho, 
      Kodaira, Tokyo 187-8551, Japan.
FAU - Saito, Yoshiaki
AU  - Saito Y
FAU - Itoh, Masayuki
AU  - Itoh M
FAU - Nakagawa, Eiji
AU  - Nakagawa E
FAU - Sugai, Kenji
AU  - Sugai K
FAU - Takahashi, Akio
AU  - Takahashi A
FAU - Otsuki, Taisuke
AU  - Otsuki T
FAU - Saito, Yuko
AU  - Saito Y
FAU - Arima, Kunimasa
AU  - Arima K
FAU - Mizuguchi, Masashi
AU  - Mizuguchi M
FAU - Goto, Yu-ichi
AU  - Goto Y
FAU - Sasaki, Masayuki
AU  - Sasaki M
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20091004
PL  - Netherlands
TA  - J Neurol Sci
JT  - Journal of the neurological sciences
JID - 0375403
RN  - 0 (Biomarkers)
RN  - 0 (Intracellular Signaling Peptides and Proteins)
RN  - 0 (Ribosomal Protein S6)
RN  - EC 2.7.1.1 (MTOR protein, human)
RN  - EC 2.7.11.1 (Protein Serine-Threonine Kinases)
RN  - EC 2.7.11.1 (TOR Serine-Threonine Kinases)
SB  - IM
MH  - Atrophy/metabolism/pathology/physiopathology
MH  - Biomarkers/analysis/metabolism
MH  - Brain/*metabolism/*pathology/physiopathology
MH  - Calcinosis/metabolism/pathology/physiopathology
MH  - Cell Movement/physiology
MH  - Child, Preschool
MH  - Encephalitis/metabolism/pathology/physiopathology
MH  - Gliosis/metabolism/pathology/physiopathology
MH  - Humans
MH  - Intracellular Signaling Peptides and Proteins/metabolism
MH  - Macrophages/metabolism
MH  - Male
MH  - Malformations of Cortical Development/*metabolism/*pathology/physiopathology
MH  - Microglia/*metabolism
MH  - Phosphorylation
MH  - Protein Serine-Threonine Kinases/metabolism
MH  - Ribosomal Protein S6/analysis/genetics/*metabolism
MH  - Signal Transduction/physiology
MH  - TOR Serine-Threonine Kinases
EDAT- 2009/10/07 06:00
MHDA- 2010/02/13 06:00
CRDT- 2009/10/07 06:00
PHST- 2009/01/17 00:00 [received]
PHST- 2009/08/27 00:00 [revised]
PHST- 2009/09/10 00:00 [accepted]
PHST- 2009/10/07 06:00 [entrez]
PHST- 2009/10/07 06:00 [pubmed]
PHST- 2010/02/13 06:00 [medline]
AID - S0022-510X(09)00852-1 [pii]
AID - 10.1016/j.jns.2009.09.010 [doi]
PST - ppublish
SO  - J Neurol Sci. 2009 Dec 15;287(1-2):52-9. doi: 10.1016/j.jns.2009.09.010. Epub 
      2009 Oct 4.