A Rare Case Of Bilateral hypertrophic olivary degeneration

Abstract

Hypertrophic olivary degeneration is a result of a primary lesion damaging the dentorubro- olivary pathway. Causes include primary lesions in contralateral dentate nucleus or ipsilateral central tegmental tract, focal brain stem insults  leading to dentatorubral-olivary pathway  disruption. Ischemia, demyelination, Hemorrhage,   cerebrovascular malformation, or diffuse axonal injury following severe head trauma are most common causes for this  degeneration. It is a transynaptic form of degeneration and unique, causing hypertrophy rather than atrophy of the inferior olivary nucleus. Progressive ataxia and palatal tremor(PAPT) is a rare distinct clinical entity characterized by symptomatic   palatal tremor associated with progressive ataxia.Only few cases have been described in literature, and MRI findings show, in most cases, cerebellar atrophy and unilateral inferior olivary hypertrophy . We report a case where MRI study at 1.5T  demonstrated marked bilateral olivary complex hyperintensity and hypertrophy and marked cerebellar atrophy.