Tremor and Other Hyperkinetic Movements · 2014 · 23 citations · 0 references
MedicineNeurologyNeuroscienceMovement DisordersNeurodegenerationNeuropathologyBrain ImagingCongenital Brain MalformationsNeurodegenerative DiseasesNeurodegenerative DisordersNeurophysiology Of Movement DisordersTremor DisordersCavernous MalformationsFunctional ImprovementHolmes TremorInferior Olivary NucleusMagnetic ResonanceT2-weighted ImagingTremor PathophysiologyZona Incerta
<strong>Background:</strong> Hypertrophic olivary degeneration (HOD) is a rare phenomenon, probably related to transsynaptic degeneration of the inferior olivary nucleus. It usually occurs as a response to primary injury of dento-rubro-olivary pathways. <strong>Case report:</strong> A young man developed Holmes’ tremor 7 months after a cavernous malformation bleed in the midbrain. Typical findings of HOD were observed in the magnetic resonance images: bilateral and asymmetric hypertrophy of the olivary nucleus with slight hypersignal in T2-weighted images. Because of the striking disability related to drug-resistant tremor, the patient underwent stereotactic thalamotomy (nucleus ventralis intermedius of the thalamus/zona incerta) with pronounced functional improvement over time. <strong>Discussion:</strong> Disruption of circuits in the Guillain–Mollaret triangle classically results in palatal myoclonus, however midbrain (Holmes’) tremor can also occur, as we now describe.