CEREBELLAR HYPOPLASIA ASSOCIATED WITH SYSTEMIC DEGENERATION IN EARLY LIFE

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Abstract
One infant with ponto-neocerebellar hypoplasia showed the usual developmental anomalies of the griseum pontis, arcuate nuclei, and inferior olivary nuclei along with involvement of perihypoglossal and midline groups of nuclei in the medulla. Severe degeneration was present in the dentate nuclei and transneuronal degeneration was present in the ventrolateral nuclei of the thalamus, the mammillary bodies and the antero-ventral thalamic nuclei. The 2nd case was complicated by absence of the corpus callosum and pyramidal tracts. There was mild developmental arrest and atrophy of the dentate nuclei. The corpora Luysii showed recent and severe degeneration associated with gliosis and neuronal shrinkage of the internal pallidal segments.