Ein Autopsiefall von olivocerebellarer und Thalamusdegeneration, diffuser Marksklerose und hypertrophischer Neuropathie: Frühkindliche Systemerkrankung?

Abstract

The brain of a 21-year-old man with imbecility and spastic tetraplegia since early childhood showed a diffuse sclerosis of the cerebral white matter and symmetrical degeneration of the thalamus and olivocerebellar system. The spinal nerve roots as well as proximal branches of the spinal nerves were hypertrophic and there was seen a marked fibrosis with some onion-bulb formations of Schwann cells. In spite of a possible relation to an unknown infection at the age of 3 months this case could preferably be regarded as a peculiar form of the combined system degeneration in the early childhood, including the cerebral white matter and peripheral nerves. An uncle of the patient had died of a similar nervous disease at the age of 20 years.

Thalamus Degeneration in Japan

“…Several additional cases of such multisystematic degeneration of the CNS involving the thalamus have been reported [10,11,14,23], and it is well documented that the central nucleus and, less frequently, the anterior and medial nucleus of the thalamus are involved in OPCA [4,6]. The cases of Moriuchi et al [13] could be similar to those cases.…”