Progressive supranuclear palsy: neuronal and glial cytoskeletal pathology in the higher order processing autonomic nuclei of the lower brainstem.
The medial and lateral parabrachial nuclei (MPB, LPB), the gigantocellular reticular nucleus (GI), the raphes magnus (RMG) and raphes obscurus nuclei (ROB), as well as the intermediate reticular zone (IRZ) represent pivotal subordinate brainstem centres, all of which control autonomic functions. In this study, we investigated the occurrence and severity of the neuronal and glial cytoskeletal pathology in these six brainstem nuclei from 17 individuals with clinically diagnosed and neuropathologically confirmed progressive supranuclear palsy (PSP). The association between the severity of the pathology and the duration of the disease was investigated by means of correlation analysis. The brainstem nuclei in all of the PSP cases were affected by the neuronal cytoskeletal pathology, with the IRZ and GI regularly showing severe involvement, the MPB, RMG, and ROB marked involvement, and the LPB mild involvement. In the six nuclear greys studied, glial cells undergo alterations of their cytoskeleton on an irregular basis, whereby diseased oligodendrocytes predominantly presented as coiled bodies and affected astrocytes as thorn-shaped astrocytes. In all six nuclei, the severity of the neuronal or glial cytoskeletal pathology showed no correlation with the duration of PSP. In view of their functional role, the neuronal pathology in the nuclei studied offers a possible explanation for the autonomic dysfunctions that eventually develop in the course of PSP.
Rüb
U
U
Department of Clinical Neuroanatomy, Johann Wolfgang Goethe University, Frankfurt, Main, Germany. u.rueb@em.uni-frankfurt.de
Del Tredici
K
K
Schultz
C
C
de Vos
R A I
RA
Jansen Steur
E N H
EN
Arai
K
K
Braak
H
H
D000368:Aged; D000369:Aged, 80 and over; D000544:Alzheimer Disease / Q000473:pathology; D001341:Autonomic Nervous System / Q000473:pathology; D002467:Cell Nucleus / Q000473:pathology; D003599:Cytoskeleton / Q000473:pathology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009457:Neuroglia / Q000473:pathology*; D009474:Neurons / Q000473:pathology*; D011903:Raphe Nuclei / Q000473:pathology*; D012154:Reticular Formation / Q000473:pathology*; D013494:Supranuclear Palsy, Progressive / Q000473:pathology*