Hypertrophic olivary degeneration

Case contributed by Dr Nick Tarasov

Presentation

History of pontine hemorrhage in 10 months prior. Now she presents with all limb weakness, palatal myoclonus, gait disturbance (delivered to the study on wheelchair).

Patient Data

Age: 65
Gender: Female
MRI

MRI Brain

There are post hemorrhagic infarct changes in left pons/superior cerebellar peduncle/midbrain and the in the right caudate nucleus as well. The inferior olivary nucleus is larger and with increased T2 signal intensity bilaterally, but more evident on the left. 

Case Discussion

This is a rare case of hypertrophic olivary degeneration (HOD) in a patient with previous history of pontine hemorrhagic infarct. 

HOD is a rare condition characterized by a unique pattern of trans-synaptic degeneration caused by damage dentato-rubro-olivary pathway. This disease process is different from all other types of neuronal degeneration in that the main pathophysiology is hypertrophy of neurons. 

Differential diagnosis:

  • infarction
  • neoplasms: primary or secondary
  • demyelination
  • infections
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Case information

rID: 39090
Published: 19th Aug 2015
Last edited: 16th Jul 2018
Inclusion in quiz mode: Included

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