Résumé
Between 1985and 1987, 31 patients with sporadic olivopontocerebellar atrophy (SOPCA) and 3 patients with familial olivopontocerebellar atrophy (FOPCA) were examined in the Neurologic Clinic of Yongdong Severance Hospital. The incidence of the disease among our neurology clinic patients was 0.9% and 3.4% of those patients were admitted. Seventeen of them were men and seventeen women, and their ages of onset ranged from 16 to 75 years (mean, 48.2 years). In comparison with SOPCA, the disease began earlier in FOPCA (mean age, 51.0 VS 19.3 years), but there were no other differences in clinical feature of the disease. Four patients had parkinsonism, one dementia, and one ophthalmoplegia. None presented spinal involvement or abnormal movements. Eight had a coexisting disease; 3, ch(03)nic alcoholism; 2, hypertension; 2, diabetes mellitus; and 1, malignant neoplasm.
Sujets)
Adolescent , Adulte , Sujet âgé , Femelle , Humains , Mâle , Diagnostic différentiel , Corée , Adulte d'âge moyen , Atrophies olivo-ponto-cérébelleuses/diagnostic , Dégénérescences spinocérébelleuses/diagnosticRésumé
Se presentan 11 pacientes portadores de una atrofia olivo ponto cerebelosa, describiéndose en detalle la sintomatología neurológica y su evolución. Las diversas formas sindromáticas se analizan en relación a los hallazgos de la Tomografía Axial Computada de cerebro, apreciándose que todos nuestros casos tienen evidencia tomográfica de atrofia del vermis cerebeloso, hemisferios cerebelosos y protuberancia, independientemente de la forma sindromática que presentan. Finalmente se comenta la relación de este cuadro con el Síndrome de Shy-Drager y la degeneración striato-nigral.