Korean J Radiol.  2013 Apr;14(2):316-320. 10.3348/kjr.2013.14.2.316.

Bilateral Hypertrophic Olivary Degeneration in Wilson Disease

• Otto J
• Guenther P
• Hoffmann K

• KMID: 1482793
• DOI: http://doi.org/10.3348/kjr.2013.14.2.316

Abstract

Hypertrophic olivary degeneration resulting from lesions of the dento-rubro-olivary pathway, also called Guillain-Mollaret-triangle, has been described previously in a number of cases. Reports about bilateral hypertrophic olivary degeneration of the inferior olivary nuclei are very limited, and the magnetic resonance imaging findings of hypertrophic olivary degeneration in Wilson disease have not yet been described to the best of our knowledge. Herein, we present the first report of bilateral hypertrophic olivary degeneration diagnosed by magnetic resonance imaging in a patient suffering from Wilson disease.

Keyword

Hypertrophic olivary degeneration; Wilson disease; Magnetic resonance imaging

MeSH Terms

Diagnosis, Differential
Hepatolenticular Degeneration/*pathology
Humans
Hypertrophy/pathology
Magnetic Resonance Imaging/*methods
Male
Nerve Degeneration/*pathology
Olivary Nucleus/*pathology
Young Adult

Figure

• 

  Fig. 1 Bilateral hypertrophic olivary degeneration in Wilson disease (WD). 23-year-old man suffering from WD with initial MRI findings. A. Transverse T2WI (TR/TE: 6000/96 ms, FA = 120°) at level of medulla oblongata without signs of hypertrophy or increased signal intensity of inferior olivary nuclei on both sides. B. Transverse T2WI at level of pons: symmetrical hyperintensity of pontine tegmentum. C. Midsagittal T2WI: hyperintense midbrain with atrophy of cerebellum. D. Transverse SWI (TR/TE: 28/20 ms, FA = 15°) at level of basal ganglia shows low signal intensity of heads of caudate nuclei and putamina. SWI = susceptibility weighted image, T2WI = T2-weighted image MRI findings of same patient one year later. E. Transverse T2WI (TR/TE: 6000/96 ms, FA = 120°) at level of medulla oblongata: bilateral hyperintense and hypertrophic inferior olivary nuclei (arrows). F. Transverse T2WI at level of pons: symmetrical hyperintensity and atrophy of medial cerebellar peduncles and pons. G. Parasagittal T2WI: hyperintense midbrain, pons, medulla; atrophy of superior cerebellar peduncle (arrow) and cerebellum. H. Transverse SWI (TR/TE: 28/20 ms, FA = 15°) at level of basal ganglia: considerable volume and ongoing signal loss in heads of caudate nuclei and putamina. SWI = susceptibility weighted image, T2WI = T2-weighted image

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