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April 1983

Dominant Spinopontine Atrophy

Author Affiliations

Neuropathology Division Medical Center General Hospital PO Box 8269 Cranston, RI 02920
Department of Neurology Veterans Administration Medical Center Providence, RI 02908

Arch Neurol. 1983;40(4):259-260. doi:10.1001/archneur.1983.04050040089027

To the Editor.  —We recently obtained a computed tomographic (CT) scan from a 32-year-old woman with dominant spinopontine atrophy (DSPA) who was described by Pogacar et al in 1978 (case 2).1 She is the daughter and granddaughter of two members of the family with DSPA originally described by Boller and Segarra.2Interestingly, her CT scan (Figure) demonstrated considerable atrophy of the cerebellum and brain stem. This observation adds another case of rather marked cerebellar atrophy to the one reported by us as case 1.1 As the cerebellum was not available for examination in one of Boller and Segarra's patients (E.W.), the rather marked cerebellar involvement in the original family with DSPA prevails. Also, the CT scan of the propositus was very similar, if not identical, to those observed in patients with olivopontocerebellar atrophy (OPCA).3In their letter to the editor, Boller