Abstract Details

Title Central and Extra Pontine Myelinolysis: More Than What Meets the Eye

Topic Neurotoxicology

Presentation(s) P06 - (-)

Poster/Presentation
Number 198

Objective

Background BACKGROUND: Central Pontine Myelinolysis (CPM) is a rare osmotic demyelinating disorder characterized by myelin loss in the central pons. Lesions can also occur outside the pons, i.e. Extra Pontine Myelinolysis (EPM). We describe a patient who along with classic central pontine lesion had extensive myelinolysis of subependymal areas of the ventricles and spinal cord, that posed as a diagnostic uncertainty. We are unaware of similar published cases of pathologically proven EPM involving these locations.

Design/Methods DESIGN/METHODS: Case report and review of literature.

Results RESULTS: A 74-year old man presented with subacute onset of vision changes, dysarthria and progressive legs weakness. He was previously managed at another institution for vomiting, dehydration and severe hyponatremia. On examination he could only perceive hand movements with both eyes; was profoundly paraparetic (1-2/ 5 on MRC), diffusely hyporeflexic and had a sensory level at T10. Cranio-spinal MRI revealed T2 hyperintensity in the central pons, periventricular areas and central spinal cord (T2-T11), which were mildly enhancing on contrast studies. He had an exhaustive workup for nutritional, inflammatory, immunologic, infectious, and malignant etiology, all of which were normal. He was empirically treated with IV steroids but with no clinical improvement. Similarly, a repeat MRI remained unchanged. He eventually succumbed to pneumonia while in a nursing home. An autopsy revealed a triangular, well-demarcated pontine lesion, devoid of myelin and with no inflammation, pathognomonic of CPM. Other destructive areas involving lateral geniculate bodies, subependymal areas of the ventricles and central gray matter of the spinal cord shared similar features to the pons making these highly suggestive of EPM.

Conclusions CONCLUSIONS: With modern neuroimaging, clinicians should be aware of atypical radiological findings and suspect 'Extra Pontine Myelinolysis' in the context of known risk factors for osmotic demyelination; especially in presence of a central pontine lesion.

Authors/Disclosures
Sajish Jacob, MBBS (Lone star Neurology) PRESENTER	No disclosure on file
Shirley Ong, MD (Ohio State University Wexner Medical Center)	No disclosure on file
Betul B. Gundogdu, MD (UCSD)	Dr. Gundogdu has nothing to disclose.
Salah G. Keyrouz, MD (WUSM)	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file

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