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 Brain

Modality: MRI

There are post haemorrhagic 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 haemorrhagic infarct. 

HOD is a rare condition characterised 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
Case created: 19th Aug 2015
Last edited: 9th Mar 2016
Inclusion in quiz mode: Included

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