Idiopathic bilateral hypertrophic olivary degeneration

Description

A 55-year-old man presented with involuntary movements of the tongue and soft palate associated with unsteadiness in walking for a period of 3 months. The patient did not complain of ear clicking. Examination revealed palatal myoclonus, tongue fasciculation and ataxic gait. MRI of the brain showed increased T2 and Fluid Attenuation Inversion recovery (FLAIR) signal intensities in the bilateral inferior olivary complex without diffusion restriction (figure 1). Midbrain, pons, and cerebral and cerebellar parenchyma were normal (figure 2). Imaging features were consistent with bilateral hypertrophic olivary degeneration. Hypertrophic olivary degeneration is a unique type of trans-synaptic neuronal degeneration caused by damage to the dentatorubral pathway or the triangle of Guillain and Mollaret (figure 3). Focal lesions like infarction, haemorrhage, demyelination  and trauma involving this neuronal pathway lead to interruption, and following sequential pathological changes are described (table 1).1

Figure 1

Coronal T2 (A), axial T2 (B),...

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