Hypertrophic olivary degeneration

Case contributed by Dr Bruno Di Muzio

Presentation

Old cerebellar ICH on the right. New palatal myoclonus.

Patient Data

Age: 61
Gender: Male
Annotated image

Rounded T2/FLAIR hyperintensity in the left inferior pons. This is consistent with hypertrophic olivary degeneration related to a contralateral dentate nucleus lesion.

MRI

MRI Brain

Haemosiderin staining centred on the right dentate nucleus is dimensions are 10 x 12 mm and T2 weighted sequence, with blooming on the susceptibility weighted imaging. On T1 sequences this corresponds to a central region of signal dropout. Intrinsic T1 hyperintensity but no pathological contrast enhancement. No adjacent developmental venous anomaly is identified. Two further susceptibility foci are identified, one in the right thalamus and the other in left temporal white matter.

9 mm rounded T2 hyperintensity in the left inferior pole of, without enhancement. This is consistent with hypertrophic olivary degeneration related to a contralateral dentate nucleus lesion.

The remainder of the brain is normal for age, with a few scattered white matter foci of T2 hyperintensity compatible with mild changes of chronic small vessel ischaemia. No hydrocephalus.

T2 hypointense and T1 hyperintense ovoid mass in the midline to left pituitary, between anterior and posterior pituitary has dimensions of 5.7 x 7 x 13 mm.

MR angiogram demonstrates foetal supply of the left posterior cerebral artery and asymmetrically large calibre right A2 and A3 segments of the anterior cerebral artery.

No intracranial aneurysm or evidence of vascular malformation.

Conclusion:

Left hypertrophic olivary degeneration secondary to previous bleed in the right dentate nucleus.

The site of the previous haemorrhage does not have a clear underlying popcorn lesion of an underlying cavernoma.

Based on location, the previous dentate nucleus haemorrhage and right thalamic microbleed could have hypertensive aetiology, but the susceptibility focus in left temporal white matter would be an atypical location for hypertensive haemorrhage, and could represent a tiny cavernoma.

No AVM identified.

Probable Rathke's cleft cyst.

Case Discussion

Hypertrophic olivary degeneration is a rare condition characterised by unique pattern of trans-synaptic degeneration. It is caused by a lesion in the triangle of Guillain and Mollaret, resulting in hypertrophy of the inferior olivary nucleus. In this case the previous haemorrhagic lesion in the right dentate nucleus caused the interruption that led to hypertrophic olivary degeneration.  

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Case information

rID: 38438
Case created: 20th Jul 2015
Last edited: 18th May 2017
Tag: rmh
Inclusion in quiz mode: Included

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