Four cases of infantile neuroaxonal dystrophy (a familial progressive disorder of the central nervous system) are presented with clinical and pathological findings. The clinical picture is characterized by a fairly normal development of the children up to about one year of age when a standstill and regression of motor and psychic development became apparent. Some children develop a marked generalized hypotonia, others a spasticity. Optic nerve atrophy is usually another early clinical sign. Pathologically a marked cerebellar atrophy and the occurrence of so-called ''speroid bodies'' are characteristic; the latter are signs of neuroaxonal degeneration. One of the patients presented here showed extrapyramidal signs which so far have not been seen in infantile neuroaxonal dystrophy.

Keywords:
Infantile neuroaxonal dystrophy, Hypotonia, Spasticity, Optic nerve atrophy, Extrapyramidal signs
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© 1970 S. Karger AG, Basel
1970
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