Dl. Yao et al., CONCENTRIC SCLEROSIS (BALO) - MORPHOMETRIC AND IN-SITU HYBRIDIZATION STUDY OF LESIONS IN 6 PATIENTS, Annals of neurology, 35(1), 1994, pp. 18-30
Brain tissues from 6 patients with concentric sclerosis (Balo) were ex
amined by in situ hybridization, immunocytochemistry, morphometry, and
histological methods. The patients were 24 to 48 years old and had pr
ogressive cerebral symptoms and signs that lasted 15 to 100 days. Larg
e demyelinative lesions, most frequent in the frontal white matter, co
ntained alternating bands of demyelinated and partly myelinated white
matter that were arranged in concentric or mosaic patterns. In the are
as of demyelination, axons were relatively well preserved and there we
re perivascular inflammatory infiltrates. In 2 specimens, lesions cont
ained regions with the characteristic appearance of actively demyelina
ting multiple sclerosis plaques. Oligodendroglial densities were highe
st in normal-appearing white matter, lower in partially myelinated are
as, and lowest in demyelinated areas, which also contained many hypert
rophic astrocytes closely associated with oligodendroglia. Messenger R
NA levels for myelin-related proteins followed the same pattern; they
were lowest in demyelinated areas, higher in partially myelinated area
s, and highest in normal-appearing white matter beyond lesion margins.
Our findings suggest that concentric sclerosis is a variant of multip
le sclerosis, that oligodendroglial loss is important in the pathogene
sis of demyelination, and that partially myelinated areas probably rep
resent stages of ongoing myelin breakdown rather than remyelination of
previously demyelinated areas.