MORPHOLOGICAL SUBSTRATES OF INFANTILE SPASMS - STUDIES BASED ON SURGICALLY RESECTED CEREBRAL TISSUE

被引:110
作者
VINTERS, HV
FISHER, RS
CORNFORD, ME
MAH, V
SECOR, DL
DEROSA, MJ
COMAIR, YG
PEACOCK, WJ
SHIELDS, WD
机构
[1] UNIV CALIF LOS ANGELES,MED CTR,DEPT PATHOL,LOS ANGELES,CA 90024
[2] UNIV CALIF LOS ANGELES,MED CTR,DEPT ANAT & CELL BIOL,LOS ANGELES,CA 90024
[3] UNIV CALIF LOS ANGELES,MED CTR,DEPT SURG NEUROSURG,LOS ANGELES,CA 90024
[4] UNIV CALIF LOS ANGELES,MED CTR,DEPT PEDIAT,LOS ANGELES,CA 90024
[5] UNIV CALIF LOS ANGELES,MED CTR,DEPT NEUROL,LOS ANGELES,CA 90024
[6] UNIV CALIF LOS ANGELES,MED CTR,BRAIN RES INST,LOS ANGELES,CA 90024
[7] UNIV CALIF LOS ANGELES,MED CTR,MENTAL RETARDAT RES CTR,LOS ANGELES,CA 90024
关键词
INFANTILE SPASMS; BRAIN; MALFORMATIONS; DYSPLASIA; CORTICAL; HEMISPHERECTOMY; EPILEPSY; IMMUNOCYTOCHEMISTRY; HAMARTOMAS; CNS;
D O I
10.1007/BF00316556
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Extensive surgical resections of neocortical cerebral tissue (including hemispherectomies) from 13 infants and children with infantile spasms showed that 12 of 13 specimens contained either malformative and dysplastic lesions of the cortex and white matter (sometimes with associated hamartomatous proliferation of globular cells), or destructive lesions possibly acquired as a result of anoxic-ischemic injury, or a combination of the two. In brain tissue from 4 patients, coarse neuronal cytoplasmic fibrils resembling neurofibrillary tangles were seen in areas of dysplastic brain on silver-stained (Bielschowsky technique) sections. Immunohistochemical (immunoperoxidase) study of cortical lesions containing globular cells employing primary antibodies to glial fibrillary acidic protein and synaptophysin as markers of astrocytic and neuronal differentiation, respectively, revealed that many cells showed astrocytic and/or neuronal features, suggesting the local proliferation of primitve or multipotential neuroectodermal cells as one substrate for this seizure disorder. Morphological abnormalities of a severe degree and wide extent in the resected tissue (e.g., in one patient with hemimegalencephaly) often showed features to suggest that they may represent variants of tuberous sclerosis. These most likely result from abnormal movement and/or local proliferation of neuroectodermal precursors that have migrated from the germinal matrix to the cortical mantle. Cellular, molecular and neurophysiological study of these abnormalities is likely to yield information about basic molecular mechanisms of brain malformation and injury important in the pathogenesis of infantile spasms and other forms of focal or generalized epilepsy.
引用
收藏
页码:8 / 17
页数:10
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