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Osmotic demyelination syndrome with two-phase movement disorders: case report
Y C Chuang1, C S Chang, S P Hsu
1Department of Neurology, Chang Gung Memorial Hospital, Kaohsiung, Taiwan, R.O.C.
Changgeng Yi Xue Za Zhi
|March 13, 1999
Summary
Osmotic demyelination syndrome (ODS) can cause movement disorders, even when brain lesions resolve. This case highlights a two-phase movement disorder in ODS, responding to medication.
Area of Science:
- Neurology
- Neuroscience
- Radiology
Background:
- Osmotic demyelination syndrome (ODS) is a rare neurological disorder characterized by demyelination, primarily in the pons.
- It is often associated with rapid correction of electrolyte imbalances, particularly hyponatremia, in malnourished or alcoholic patients.
- Movement disorders are infrequently reported complications of ODS.
Observation:
- This case study details a 22-year-old female patient who developed ODS following correction of severe hyponatremia.
- The patient presented with a unique two-phase neurological disorder: initial acute dystonia followed by generalized rigidity and tremors.
- Initial MRI revealed demyelination in the pons, thalami, and basal ganglia, with partial resolution in the second phase.
Findings:
- The patient exhibited a distinct two-phase movement disorder, with dystonia followed by rigidity and tremors.
- Neuropathological findings on MRI showed demyelinating lesions in key brain areas.
- The clinical progression of the movement disorder did not directly correlate with the resolution of demyelinating lesions on imaging.
Implications:
- This case expands the understanding of potential neurological manifestations of ODS, emphasizing movement disorders.
- The delayed, biphasic nature of the movement disorder and its dissociation from imaging resolution are noteworthy.
- Pharmacological interventions, including propranolol and trihexyphenidyl, showed efficacy in managing the movement disorder during the second phase.