Objective:

To discuss the clinical manifestations and management of bilateral hypertrophic olivary degeneration (HOD) with associated Holmes tremor.

Background:

HOD is a rare neurological condition characterized by enlargement of both inferior olivary nucleius due to lesions that disrupt the dentato-rubro-olivary pathway (Triangle of Guillain-Mollaret). HOD can present as a variety of symptoms including palatal tremor, Holmes tremor, oculopalatal tremor, nystagmus, and cerebellar ataxia.  The etiologies of HOD include ischemic or hemorrhagic stroke, vascular malformations, trauma, neoplasm, and surgical intervention.

Design/Methods:

NA

Results:

Patient is a 56-year-old male who developed episodic dizziness followed by  tinnitus, dysarthria, gait instability, nausea, and vomiting. He was found to have a left beating nystagmus and subsequently diagnosed with peripheral vertigo.  CT angiography revealed stenosis at the origin of the right vertebral artery and left V1 vertebral artery occlusion with distal reconstitution. MRI brain then revealed abnormal FLAIR signal and mild hypertrophy of the bilateral interior olivary nuclei and multiple small remote infarcts in the bilateral cerebellar hemispheres and left superior cerebellar peduncle. He was referred to our institution for evaluation.    Patient was found to have mildly impaired  horizontal saccades, palatal myoclonus contributing to hoarseness of voice, mild bradykinesia of the left upper extremity, bilateral upper extremity tremor, and difficulty with tandem gait. The etiology of stroke was most likely thought to be large vessel disease with artery to artery embolization. He was treated with antiplatelet, lipid lowering medication, and propranolol for tremors.

Conclusions:

Ischemia is a known cause of hypertrophic olivary degeneration, and a proportion of patients will go on to develop Holmes tremor. Determining the etiology of hypertrophic olivary degeneration is critical for effective risk stratification and secondary stroke prevention in this case.  Optimal management requires a multidisciplinary approach involving movement disorder specialist.