T. Hanihara et al., HYPERTROPHY OF THE INFERIOR OLIVARY NUCLEUS IN PATIENTS WITH PROGRESSIVE SUPRANUCLEAR PALSY, European neurology, 39(2), 1998, pp. 97-102
Hypertrophic changes in the inferior olivary nuclei have been occasion
ally described in patients with progressive supranuclear palsy (PSP).
To elucidate the incidence of olivary hypertrophy, we investigated mor
phologically the olives and their associated pathways in 20 autopsied
cases: 11 cases of PSP, 3 cases of Machado-Joseph disease and 6 cases
of dentatorubropallidoluysian atrophy (DRPLA) as control diseases that
usually exhibited lesions of the cerebellofugal pathway. Olivary hype
rtrophy was observed in 5 of 11 PSP cases, but not in the other diseas
es, except for 1 case of DRPLA with an old infarct in the dentate nucl
eus. In the olivopetal pathway, grumose degeneration of the dentate nu
cleus and mild neuronal loss in the red nucleus were observed in all p
atients with PSP and in the control subjects. Atrophy and fibrillary g
liosis of the tegmentum of the pens, including that of the bilateral c
entral tegmental tracts, were observed in all patients with PSP, more
severe in the cases with hypertrophic neurons in the olives. We specul
ate that a lesion that involves the central tegmental tracts may play
a major role in inducing hypertrophy of the olives in patients with PS
P.