Objective: To describe two case reports of patients with movement disorders due to extrapontine myelinolysis.

Background: Central Pontine Myelinolysis (CPM) and Extra pontine myelinolysis (EPM) are osmotic demyelination syndromes (ODS) associated with a rapid correction of hyponatremia. Both entities course with a non-inflamatory degeneration of olygodendrocytes localized in the basis pontis (CPM), whereas EPM involves cerebellum, basal ganglia, thalami, cerebral white matter and frontotemporal cortex. EPM may present with parkinsonism, dystonia, myoclonus, tics or choreoatetosis.

Method: We present the cases of 2 patients who developed acute parkinsonism due to EPM. We also describe the delayed movement disorders presented by one of them, the magnetic resonance imaging (MRI) findings and the treatment received.

Results: Case 1. A 64 year-old woman was admitted to emergency room with akinetic mutism and quadriparesis. Brain MRI revealed CPM and EPM affecting basal ganglia symmetrically. Later during the admission, parkinsonism was noted and she was started on amantadine 100 mg bid. At follow up after 7 months beside parkinsonism, she had truncal chorea and in the lower limbs (left side predominance), right leg dystonia and mild truncal ataxia. Amantadine was increased to 100 mg tid and at last follow up the patient had optimal control of movement disorders, with only slight parkinsonism and intermittent chorea in lower limbs.
Case 2. A 54 year- old woman with acute vomiting and hyponatremia was admitted to a hospital, dehydratation and electrolitic disturbance was corrected and thepatient was discharged. A week later she developed hypophonia, sialorrhea, hypomimia, rigidity in legs and bradykinesia. Brain MRI showed CPM and EPM in the latero dorsal parts of bilateral putamen and in globus pallidus. She was diagnosed with parkinsonism secondary to EPM and was started on biperiden 2 mg/day which was continued for 6 months. The symptomalogy improved gradually and at follow up 8 months later she was almost completely recovered just with mild rigidity in the right leg.

Conclusion: Acute parkinsonism is a common manifestation of EPM, but delayed movement disorders such as chorea, myoclonus, tics and dystonia has been described in patients with EPM. Symptomatic treatment could ameliorate movement disorders produced by EPM.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/parkinsonism-delayed-chorea-and-dystonia-caused-by-extrapontine-myelinolysis/