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Pontocerebellar hypoplasia in a 3-year-old girl (MRI)

Pontocerebellar hypoplasia is a progressive degenerative disease beginning in fetal life, demonstrated in (A) T1-weighted axial views and (B) T1-weighted sagittal and parasagittal views. Not only are the cerebellar hemispheres and vermis small and poorly formed, but also the basis pontis is undeveloped, and the brainstem appears in sagittal view of uniform diameter throughout its length. In the supratentorial compartment, there is minimal ventriculomegaly due to early deep atrophy, and some of the gyri of the cerebral cortex are shrunken and separated by excessively wide sulci, but no primary malformations such as pachygyria or heterotopia are demonstrated. (Contributed by Dr. Harvey Sarnat.)

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Associated Disorders

  • Chiari malformation
  • Chromosomal disorders
  • Congenital disorders of glycosylation
  • Dandy-Walker malformation
  • Fragile X syndrome
  • Global cerebellar hypoplasia
  • Granuloprival cerebellar hypoplasia
  • Holoprosencephaly
  • Joubert syndrome
  • Lissencephaly/pachygyria
  • Macrocerebellum
  • Meckel-Grüber syndrome
  • Meningomyelocele
  • Menkes kinky hair disease
  • Midbrain agenesis
  • Miller-Dieker syndrome
  • Rett syndrome
  • Rhombencephalosynapsis
  • Selective hypoplasia of the cerebellar vermis
  • Septo-optic-pituitary dysplasia
  • Subcortical band heterotopia
  • Total aplasia of the cerebellum
  • Trisomy 13-15
  • Trisomy 16-18
  • VLDLR-associated pontocerebellar hypoplasia
  • Walker-Warburg syndrome