7
Hypertrophic
olivary degeneration (HOD):
an unusual way to degenerate
Drs. Fabrizio
Venturi, Donatella Tampieri, Roland Brassard & Denis Melanson
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In
the CNS the degeneration of an anatomical structure is usually characterized
by neuronal loss replaced by proliferation of glial elements. This
change is reflected in the appearance on MRI: a loss of volume associated
with a hypersignal on long TR sequences. There is, however, a case
in which the degeneration is accompanied by hypertrophy: the degeneration
of the inferior olivary nucleus, described for the first time in
1887 by Oppenheim from anatomic specimens. Not seen on CT scan due
to artefacts caused by bony structures, the hypertrophic olivary
degeneration (HOD) is more recently detected in vivo by MRI and
is characterized by an hypersignal of the olive of the medulla oblongata
on PD/T2 images with a variable enlargement of the olive itself.
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HOD
is considered a trans-synaptic degeneration because it occurs following
loss of neuronal input to a cell, in this case the neurons of the
inferior olivary nucleus. HOD occurs when a lesion, usually a haemorrhage,
causes an interruption of the Guillain-Mollaret triangle (Fig1).
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Fig1 |
The
Guillain-Mollaret triangle is a triangular circuit connecting the
dentate nucleus of the cerebellum of one side with the red nucleus
and the inferior olivary nucleus of the other side, via the superior
cerebellar peduncle and the central tegmental tract (CTT) (Figs. 2,
3).
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Fig 2
Anatomical oblique section along the superior cerebellar peduncles.
The path of the cerebello-rubral tract from dentate nucleus to
the contralateral red nucleus is shown.
Fig 3 |
Anatomical, axial section of the
brainstem, at the level of the pons, showing the location of the
central tegmental tracts. |
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