Reports clinical and histological findings of a case of infantile spino-cerebellar degeneration. Beginning psychomotor development was normal. Parents first noted little movement of the upper right limb and inability to maintain a sitting position at 1 yr. Slow progressive psychomotor deterioration was associated with flaccid paralysis and muscle atrophy of the upper limbs. Death occurred at age 7. Histopathology showed cerebellar atrophy, grave atrophy of the anterior horn, of Clarke's column, of the nuclei of the X and XII cranial nerves, a degeneration of the pyramidal and spino-cerebellar tracts, and of the dentate nucleus and substantia nigra. Granular atrophy of the cerebellum can have a genetic basis and can also be caused by exogeneous chemical and physical agents and viral infections when they occur in a determinate period of embryonic development. The noxa, whether genetic or exogeneous, when intervening in the prenatal or immediate postnatal period, at the time of greatest proliferative activity and maturation of the granule cells, causes a destruction of this cellular population and alters intercellular relations which are at this time establishing themselves. (English summary) (35 ref) (PsycLIT Database Copyright 1980 American Psychological Assn, all rights reserved)
Clinical-pathological study of a case of infantile spino-cerebellar degeneration
RIZZUTO, Nicolo';
1976-01-01
Abstract
Reports clinical and histological findings of a case of infantile spino-cerebellar degeneration. Beginning psychomotor development was normal. Parents first noted little movement of the upper right limb and inability to maintain a sitting position at 1 yr. Slow progressive psychomotor deterioration was associated with flaccid paralysis and muscle atrophy of the upper limbs. Death occurred at age 7. Histopathology showed cerebellar atrophy, grave atrophy of the anterior horn, of Clarke's column, of the nuclei of the X and XII cranial nerves, a degeneration of the pyramidal and spino-cerebellar tracts, and of the dentate nucleus and substantia nigra. Granular atrophy of the cerebellum can have a genetic basis and can also be caused by exogeneous chemical and physical agents and viral infections when they occur in a determinate period of embryonic development. The noxa, whether genetic or exogeneous, when intervening in the prenatal or immediate postnatal period, at the time of greatest proliferative activity and maturation of the granule cells, causes a destruction of this cellular population and alters intercellular relations which are at this time establishing themselves. (English summary) (35 ref) (PsycLIT Database Copyright 1980 American Psychological Assn, all rights reserved)
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