Abstract • In five generations of Anglo-Saxon family W, 28 members were affected with dominant hereditary ataxia. In 1969, two members of this family were reported as prototypes of dominant spinopontine atrophy. We discuss two additional members of the original family, with one postmortem examination, including ultrastructual study. In contrast to previously accepted clinical generalizations, we found abolished tendon reflexes and flexion contractures of the lower extremities in patient 1 and onset of illness at the age of 18 years, palatal myoclonus, and optic atrophy in patient 2. Dementia was observed in both patients. Unlike in previous pathological reports, our patient 1 showed definite involvement of the cerebellum and mild degeneration of the inferior olivary nuclei. We conclude, therefore, that clinical and pathological distinction between dominant spinopontine atrophy and olivopontocerebellar atrophy is not clear and raises the question of the justification for regarding dominant spinopontine atrophy as a nosological entity. References 1. Boller F, Segarra JM: Spino-pontine degeneration . Eur Neurol 2:356-373, 1969.Crossref 2. Taniguchi R, Konigsmark BW: Dominant spino-pontine atrophy . Brain 94:349-358, 1971.Crossref 3. Schut JW, Haymaker W: Hereditary ataxia: Pathological study of five cases of common ancestry . J Neuropathol Exp Neurol 1:183-213, 1951. 4. Scherer HJ: Extrapyramidale Störungen bei der Olivopontocerebellaren Atrophie . Z Gestamte Neurol Psychiatr 145:406-409, 1933.Crossref 5. Petito CK, Hart MN, Porro RS, et al: Ultrastructural studies of olivopontocerebellar atrophy . J Neuropathol Exp Neurol 32:503-522, 1973.Crossref 6. Landis D, Rosenberg RN, Landis SC, et al: Olivopontocerebellar degeneration . Arch Neurol 31:295-307, 1974.Crossref 7. Takahashi K, Agari M, Nakamura H: Intraaxonal corpora amylacea in ventral and lateral horns of the spinal cord . Acta Neuropathol 31:151-158, 1975.Crossref
Archives of Neurology – American Medical Association
Published: Mar 1, 1978
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