Session Time: 12:00pm-1:30pm
Location: Exhibit Hall located in Hall B, Level 2
Objective: To present a case of delayed-onset dystonia and parkinsonism secondary to osmotic demyelination syndrome, with dramatic response to pramipexole 3mg per day.
Background: Osmotic demyelination syndrome, otherwise known as pontine myelinolysis, commonly affects extra-pontine areas in the cerebrum (de Souza. Parkinsonism Relat Disord 2013). Striatal involvement often results in movement disorders, which typically have limited response to treatment (de Souza. Parkinsonism Relat Disord 2013).
Methods: We report a 43 year old man with central osmotic demyelination syndrome affecting the pallidum. He developed delayed-onset dystonia and parkinsonism, with significant craniofacial involvement and marked dysarthria and dysphagia. He was followed clinically with serial video documentation, and various pharmacologic treatments were tried including pramipexole.
Results: Despite prior treatment with levodopa 450mg/day, amantadine 200mg/day, and trihexylphenidyl 6mg/day, the patient’s dystonia and parkinsonism did not improve. Pramipexole was started and remarkable improvement was noted at a dose of 3mg per day, particularly in speech. Symptoms worsened when pramipexole was discontinued for severe impulse control disorder. Once his mood was stabilized with lamotrigine, he was restarted on pramipexole with reproducible improvement in speech. Videos at each phase are included.
Conclusions: The explanation for our patient’s particular response to pramipexole at 3mg/day dosing is unclear. Symptom worsening with the discontinuation of pramipexole and the reproducible improvement with its resumption argues against spontaneous fluctuations in the patient’s condition. Levodopa seemed to have no appreciable beneficial affect. It is possible the post-synaptic action of pramipexole accounts for its unique effectiveness in our patient’s case.
To cite this abstract in AMA style:L.E. Katus, S.J. Frucht. Osmotic demyelination syndrome with dystonia and parkinsonism responsive to dopaminergic therapy [abstract]. Mov Disord. 2016; 31 (suppl 2). https://www.mdsabstracts.org/abstract/osmotic-demyelination-syndrome-with-dystonia-and-parkinsonism-responsive-to-dopaminergic-therapy/. Accessed September 21, 2023.
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MDS Abstracts - https://www.mdsabstracts.org/abstract/osmotic-demyelination-syndrome-with-dystonia-and-parkinsonism-responsive-to-dopaminergic-therapy/