Sclerotic Microgyria or Ulegyria

Grossly, in sclerotic microgyria or ulegyria (Figs. 14.4 through 14.7), the brain is small, and the affected convolutions are severely atrophic and sclerotic. They extend from a few convolutions or one or several lobes to the entire cortex of one or both hemispheres. Any region of the cortex may be involved, but preferentially the frontal and parietal lobes, medial occipital convolutions, and hippocampus. On transverse sections, the affected convolutions display a mushroom shape due to more severe destruction of their walls than of their crests. Histologi-cally, the cortex shows multiple patchy, but more often laminar, neuronal losses involving one or several laminae or the entire width of the cortex. Remaining dead neurons are encrusted with iron and calcium (ferrugination).

figure 14.4

Bilateral symmetrical sclerotic microgyria in a 2.5-year-old mentally retarded, epileptic, and quadriplegic boy. He had been delivered 2 weeks premature and labor was complicated by premature separation of the placenta. The dorsal aspects of the cerebral hemispheres show sclerotic atrophy of the central convolutions.

figure 14.4

Bilateral symmetrical sclerotic microgyria in a 2.5-year-old mentally retarded, epileptic, and quadriplegic boy. He had been delivered 2 weeks premature and labor was complicated by premature separation of the placenta. The dorsal aspects of the cerebral hemispheres show sclerotic atrophy of the central convolutions.

figure 14.5

Multifocal sclerotic microgyria. A 5-year-old boy, severely retarded, quadriplegic, and epileptic was born cyanotic and did not cry for hours. The mother was hypertensive and hem-orrhaged during pregnancy, heavily before delivery. Macrosection of the cerebral hemispheres shows multifocal cortical necrosis and diffuse white matter atrophy (Weil stain). Note the mushroom-shaped atrophy of the convolutions.

figure 14.5

Multifocal sclerotic microgyria. A 5-year-old boy, severely retarded, quadriplegic, and epileptic was born cyanotic and did not cry for hours. The mother was hypertensive and hem-orrhaged during pregnancy, heavily before delivery. Macrosection of the cerebral hemispheres shows multifocal cortical necrosis and diffuse white matter atrophy (Weil stain). Note the mushroom-shaped atrophy of the convolutions.

figure 14.6

Histology of sclerotic microgyria. A. The necrosis is confined chiefly to the walls and depths of convolutions, giving a mushroom shape. B. Multifocal neuronal losses. C. Multilaminar neuronal losses, D. Necrosis of the entire width of the cortex (cresyl violet). E. Dense astrofibrosis in subcortical white matter (Holzer stain).

figure 14.6

Histology of sclerotic microgyria. A. The necrosis is confined chiefly to the walls and depths of convolutions, giving a mushroom shape. B. Multifocal neuronal losses. C. Multilaminar neuronal losses, D. Necrosis of the entire width of the cortex (cresyl violet). E. Dense astrofibrosis in subcortical white matter (Holzer stain).

figure 14.7

Porencephaly with sclerotic microgyria. An 8-year-old severely retarded epileptic girl was a twin, delivered second. Transverse section shows a large porencephalic cyst in one hemisphere and sclerotic atrophic convolutions in the opposite hemisphere.

figure 14.7

Porencephaly with sclerotic microgyria. An 8-year-old severely retarded epileptic girl was a twin, delivered second. Transverse section shows a large porencephalic cyst in one hemisphere and sclerotic atrophic convolutions in the opposite hemisphere.

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