Morphometric analysis of the prefrontal cortex in Huntington's disease.
We performed a morphometric analysis of cresyl violet-stained sections from the dorsolateral prefrontal cortex of 81 patients with Huntington's disease (HD) (grades 2, 3, and 4) and 23 age-matched normal controls. We counted large pyramidal neurons, small neurons, astrocytes, oligodendroglia, and microglia under the guidance of a specifically predefined set of morphologic criteria for each cell type and recorded the thickness of each cortical layer. Our results demonstrate a selective and progressive loss of a subset of the large pyramidal neurons in cortical layers III, V, and VI of HD patients, and a decrease in the thickness of the respective cortical laminae. A genetically determined, cell-autonomous degeneration of cortical neurons could constitute the primary pathologic process. However, the loss of only a fraction of pyramidal cells suggest a parallel, or an alternative, possibility of a retrograde degeneration of cortical neurons that project solely, or principally, to the site of primary degeneration in caudate nuclei.
Sotrel
A
A
Department of Developmental Neurobiology, Eunice K. Shriver Center for Mental Retardation, Waltham, MA 02254.
Paskevich
P A
PA
Kiely
D K
DK
Bird
E D
ED
Williams
R S
RS
Myers
R H
RH
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001253:Astrocytes / Q000473:pathology; D002452:Cell Count; D005625:Frontal Lobe / Q000473:pathology*; D006801:Humans; D006816:Huntington Disease / Q000473:pathology*; D008875:Middle Aged; D009474:Neurons / Q000473:pathology; D009836:Oligodendroglia / Q000473:pathology