Objective: To report a rare case of a patient with severe tremors related to central pontine myelinolysis (CPM), and effective treatment of this patient’s tremors with deep brain stimulation (DBS).
Background: Movement disorders, such as tremor, have been reported as sequelae of CPM (1). DBS reports for Holmes’ and other lesional tremors are scarce and highly heterogeneous (2), yet DBS can be a very effective treatment for these uncommon but highly disabling types of tremor.
Method: A now 58 year old female with a history of long-standing alcohol abuse and cirrhosis had a hospitalization in March of 2011 where she developed CPM. In November 2011, she developed a tremor, which became progressively worse. She says that the tremor was worst with action, such as signing her name, eating, and taking things out of the microwave. She used to be very coordinated and do a lot of crafts, so this was very debilitating to her. She also developed gait instability. She tried multiple medications including propranolol, carbidopa/levodopa, as well as primidone which did not help. A MRI brain showed a lobular cross-shaped T2 hyperintense focus in the pons and cerebellar atrophy. She was started on clonazepam for a diagnosis of a cerebellar outflow tremor related to CPM.
Results: She was referred to the Yale Movement Disorders clinic for further evaluation and was deemed to be a good candidate for DBS for her right dominant side. She subsequently underwent left stereotactic placement of a deep brain stimulator electrode within the ventralis intermedius nucleus (VIM) of the thalamus. Post-op, she noticed a 10-15% improvement in symptoms due to lesional effect, and then marked improvement of 85% after initial programming. Current voltage is 2.9V, pulse width 90 us, and frequency 130 Hz. Her handwriting is much better, she is able to drink from a full glass without spilling, and she has resumed doing her crafts once again.
Conclusion: This case serves as one of the few examples of a movement disorder developing after CPM, and is the only known example of tremor related to CPM treated with DBS. In this case, a cerebellar tremor was related to cerebellar atrophy and extension of myelinolysis to the middle cerebellar peduncles. Traditionally, the VIM of the thalamus has been considered the main target for such medically intractable tremors (3) as in this case where marked improvement of tremor was achieved with DBS in the VIM.
References: 1. Maraganore DM, Folger WN, Swanson JW, Ahlskog JE. Movement disorders as sequelae of central pontine myelinolysis: report of three cases. Mov Disord. 1992;7(2):142-8. doi: 10.1002/mds.870070208. PMID: 1584236. 2. Mendonça MD, Meira B, Fernandes M, Barbosa R, Bugalho P. Deep brain stimulation for lesion-related tremors: A systematic review and meta-analysis. Parkinsonism Relat Disord. 2018 Feb;47:8-14. doi: 10.1016/j.parkreldis.2017.12.014. Epub 2017 Dec 13. PMID: 29249682. 3. Ramirez-Zamora A, Okun MS. Deep brain stimulation for the treatment of uncommon tremor syndromes. Expert Rev Neurother. 2016 Aug;16(8):983-97. doi: 10.1080/14737175.2016.1194756. Epub 2016 Jun 9. PMID: 27228280; PMCID: PMC4975099.
To cite this abstract in AMA style:D. Machado, A. Kelly. Central Pontine Myelinolysis Related Tremor Effectively Treated With Deep Brain Stimulation [abstract]. Mov Disord. 2021; 36 (suppl 1). https://www.mdsabstracts.org/abstract/central-pontine-myelinolysis-related-tremor-effectively-treated-with-deep-brain-stimulation/. Accessed December 5, 2023.
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