Congenital apnea with medullary and olivary hypoplasia: a pathologic study with computer reconstructions.
We report a three-month-old infant with congenital central apnea who was ventilator-dependent throughout his brief life. At autopsy the most significant findings were localized to the medulla and included severe tegmental necrosis involving respiratory-related sites and olivary hypoplasia. Golgi impregnations of the cerebellum demonstrated several Purkinje cells with changes consistent with experimental olivary ablation. The majority of Purkinje cells, however, were normal; this observation suggests that in an early and subtotal olivary lesion, the incomplete complement of olivary neurons maintains sufficient collateral axonal branches to compensate for decreased cell number. Computer graphics enabled us to dissect the components of the complex medullary pathology and examine them individually and in selected combinations in three dimensions. Computer reconstruction aided the identification and dating of a malformative lesion (first trimester) from a later (second/third trimester), superimposed destructive lesion. This report suggests that the synthesis of complex morphologic data in human neuropathology into meaningful three-dimensional visual displays by computer reconstruction facilitates their comprehension. Computer reconstruction is especially valuable in the elucidation of 3-D topographic relationships in functionally and architecturally complex regions such as the brain stem.
Division of Neuroscience, Children's Hospital, Boston, MA 02115.
H C
HC
Kinney
J J
JJ
Filiano
J E
JE
Brazy
P C
PC
Burger
R L
RL
Sidman
D001049:Apnea / Q000151:congenital* / Q000473:pathology; D002531:Cerebellum / Q000002:abnormalities; D003196:Computer Graphics; D006801:Humans; D007223:Infant; D008297:Male; D008526:Medulla Oblongata / Q000002:abnormalities*; D009336:Necrosis; D009847:Olivary Nucleus / Q000002:abnormalities*; D011689:Purkinje Cells / Q000473:pathology