
Dominant Spinopontine Atrophy
F. Boller, MD
Neurobehavior Unit Cleveland VA Hosp Cleveland, OH 44106
J. M. Segarra, MD
Dept of Neuropathology Boston VA Hosp Boston, MA 02130
Arch Neurol. 1979;36(4):255.
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To the Editor.—
Pogacar et al reported in the ARCHIVES (35:156, 1978) clinical findings in two members of a family with dominant spinopontine atrophy (DSPA), an entity originally described by us in a study of two other members of the same family.1 On the basis of one postmortem examination, Pogacar et al concluded that DSPA is not clearly distinguishable from olivopontocerebellar atrophy (OPCA). Microscopic examination of the cerebellum and the inferior olivary nuclei of their case undoubtedly showed greater changes than those that occurred in our propositus and in other cases noted in the literature.2.3 The reason for this difference is unclear; however, it is well known that there can be some variation among members of the same family in the pathological features of spinocerebellar atrophies. The description of Pogacar et al suggests cerebellar and olivary lesions considerably less prominent than those found in classical OPCA, in which
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