Category: Rare Genetic and Metabolic Diseases
Objective: Although rapid osmolar correction, mainly in the setting of hyponatremia is the main risk factor of osmotic demyelination syndrome, the pathogenesis of the syndrome is not fully elucidated. Due to the variety of its presentations, osmotic demyelination syndrome is not initially suspected in most cases. As findings may not be evident for up to two weeks, repeated imaging is needed to diagnose the syndrome.
Background: Osmotic demyelination syndrome (ODS) is caused by partial destruction of myelin sheath at the basis pontis (central pontine myelinolysis, CPM) or outside the pons (extrapontine myelinolysis, EPM), although the mechanism of ODS is not fully understood, the hyponatremia is considered to be the main pathophysiological process. Extrapontine myelinolysis is even rare compared to pontine myelinolysis. ODS has a peak incidence in adults aged 30 to 60 years with male predominance. In some cases, extrapontine involvement leads to various movement disorders such as parkinsonism, tremor, or dystonia, which may even mask pyramidal features leading to diagnostic dilemma
Method: Here we present a 56 years old male patient of no medical history was admitted to our hospital complaining of disturbed conscious level. Neurological examination revealed bilateral asymmetrical rigidity with coarse irregular tremors of 4 limbs being more in upper limbs. We noticed that before admission patient suffered of hyponatremia and received 200 cc of hypertonic saline in a short duration of time with rapid correction of hyponatremia. That raised the possibility of Osmotic Demyelination Syndrome (ODS)
Results: Pt received supportive treatment and symptomatic treatment and showed marked improvement
Conclusion: osmotic demyelination syndrome although rare is a potentially treatable condition with outcome largely depending on early diagnosis and management. there is a lack of definitive clinical management of extrapontine myelinolysis due to lack of clinical trials for adequate diagnosis and management of extrapontine myelinolysis.
To cite this abstract in AMA style:
N. Sarhan. Acute-onset parkinsonism secondary to bilateral extrapontine myelinolysis of the basal ganglia: case report [abstract]. Mov Disord. 2023; 38 (suppl 1). https://www.mdsabstracts.org/abstract/acute-onset-parkinsonism-secondary-to-bilateral-extrapontine-myelinolysis-of-the-basal-ganglia-case-report/. Accessed November 21, 2024.« Back to 2023 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/acute-onset-parkinsonism-secondary-to-bilateral-extrapontine-myelinolysis-of-the-basal-ganglia-case-report/