Objective: We present a case of Osmotic Demyelination Syndrome (ODS) in a patient who underwent liver transplant and subsequently developed multiple movement disorders.

Background: ODS is an uncommon neurological disorder caused by damage to the myelin sheath of brain cells. Central pontine myelinolysis is the classic presentation, reflecting the vulnerability of pontine white matter tracts, but extrapontine involvement is common. The exact pathogenesis is unknown, but a common trigger in clinical practice is rapid correction of chronic hyponatremia. Liver transplant recipients are frequently implicated in its development.

Method: A 62-year-old woman with Non-Alcoholic Steatohepatitis (NASH) presented with decompensated cirrhosis and underwent orthotopic liver transplantation. Post-operative day 8 was complicated by encephalopathy and staring spells. Her initial exam was notable for multifocal myoclonus. Over next few days she developed catatonia (unresponsive to benzodiazepines), symmetrical parkinsonism, oculogyric crisis, mild bilateral hand dystonia, echolalia, and bradyphrenia.

Results: On day 1, her sodium level was 120 mmol/L, which peaked at 160 mmol/L by hospital day 12, with a single day jump of 11 mmol following the transplant. EEG revealed generalized slowing, but no epileptiform activity. MRI head demonstrated increased T2 signal in the pons, medial thalami, globus pallidi, and cerebellar tonsils. She was diagnosed with ODS with pontine and extrapontine involvement. When she later manifested the variety of movement disorders described, she was started on carbidopa/levodopa and titrated up to 600 mg/day with remarkable improvement in her neurological exam.

Conclusion: Clinical or radiologic evidence of neurologic damage to due to ODS begins 0.5 to 7 days after osmotic shifts occur but may be delayed by as long as 16 days. Patients generally exhibit a biphasic course that initially manifests as encephalopathy or seizures, followed thereafter by neurological deterioration characterized classically by variable tetraparesis, bulbar palsy, coma or locked-in state. The varied topographical localization of lesions in extrapontine myelinolysis leads to many different clinical symptoms including altered consciousness, emotional lability, ataxia, tremor, myoclonus, akinetic mutism, catatonia, dysautonomia, quadriparesis, and later progression to dystonia, choreoathetosis or parkinsonism.

References: 1.Singh, T. D., J. E. Fugate, and A. A. Rabinstein. “Central Pontine and Extrapontine Myelinolysis: A Systematic Review.” European Journal of Neurology 21, no. 12 (2014): 1443-450. 2.De Souza, Aaron. “Movement Disorders and the Osmotic Demyelination Syndrome.” Parkinsonism and Related Disorders 19, no. 8 (2013): 709-16. 3.Micieli, Andrew, Umberin Najeeb, and William Kingston. “Central Pontine (and Extrapontine) Myelinolysis despite Appropriate Sodium Correction.” Practical Neurology 20, no. 1 (2020): 64-65.

« Back to MDS Virtual Congress 2020

MDS Abstracts - https://www.mdsabstracts.org/abstract/catatonia-parkinsonism-and-dystonia-in-a-patient-with-osmotic-demyelination-syndrome/