[Loss of spinal anterior horn cells in X-linked recessive bulbospinal muscular atrophy--a morphometric study of lower motoneuron loss].
A morphometric analysis was performed to study three-dimensional distribution of the anterior horn cells in the L1 segmens from four cases of X-linked bulbospinal muscular atrophy (X-BSMA) and three age-matched controls. At autopsy, the L4 spinal segment was removed and fixed in 4% paraformaldehyde in PBS at pH 7.4, then embedded in paraffin. Serial sections of 10 microns in thickness from the rostral end of L4 segment were obtained. Every tenth section was stained with Klüver-Barrera method. The anterior spinal horn in this study was designated as the gray matter anterior to the line from the central spinal canal perpendicular to the ventral spinal fissure. The diameter of the remaining neurons with obvious nucleolus in the anterior horn was measured with TGZ-3 particle size analyzer (Zeiss) on the 205 time-magnified picture, and their location was schematically plotted on a montage of the ventral horn. Neuronal loss was more marked in X-BSMA than in amyotrophic lateral sclerosis (ALS) and Shy-Drager syndrome (SDS). In X-BSMA, the loss was most prominent in cells with large-size, and in those located in the lateral and medial vental nuclei (lamina IX after Rexed's classification). Small or intermediate cells located in the area of inner-medial portion of the ventral horn (lamina VII & VIII after Rexed's classification) were also significantly depleted. In some cases, the loss of small neuron was more marked than SDS.(ABSTRACT TRUNCATED AT 250 WORDS)
Terao
S
S
Sobue
G
G
Takeda
A
A
Mitsuma
T
T
Takahashi
A
A
D000368:Aged; D000870:Anterior Horn Cells / Q000473:pathology*; D005808:Genes, Recessive; D008040:Genetic Linkage; D006801:Humans; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000473:pathology*; D009134:Muscular Atrophy, Spinal / Q000235:genetics / Q000473:pathology*; D013116:Spinal Cord / Q000473:pathology*; D014960:X Chromosome