[Torpid degeneration of the lateral pallidum with Bielschowsky bodies. Light and electronmicroscopic findings (author's transl)].
In a 60-year man with a very slowly progressive motor disturbance probably of torsion systonic or choreoathetotic type which started in childhood, we found an isolated degeneration of the exterior pallidum with intraneuronal deposition of Bielschowsky bodies and only slight deficit of neurons. The Bielschowsky bodies show the same structure as corpora amylacea and Lafora bodies. They might also be the result of a cellular disturbance mainly concerning the carbohydrate metabolism. In this case they are localised only in the neurons of the exterior part of the pallidum--in contrast to the organogenitically otherwise deriving (from the nucl. entopeduncularis) intact neurons of the pallidum internum--and they induce, after an excessive accumulation of the metabolites, not till extremely late loss of neurons. This circumstance explains the very torpid progress of this disease.
D001264:Athetosis / Q000473:pathology; D001927:Brain Diseases / Q000473:pathology*; D002819:Chorea / Q000473:pathology; D005917:Globus Pallidus / Q000473:pathology; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D009069:Movement Disorders / Q000473:pathology; D009474:Neurons / Q000473:pathology; D013997:Time Factors; D014102:Torsion Abnormality
Torpide verlaufende Degeneration des äusseren Pallidumgliedes mit Bielschowsky-Körperchen. Licht- und elektronenmikroskopische Befunde