RESUMO
We describe a case of Neuromyelitis Optica Spectrum Disorder (NMOSD) mimicking Wernicke's Encephalopathy (WE) to highlight an atypical presentation of NMOSD. A 39-year-old female presented with subacute encephalopathy and progressive ophthalmoplegia. Her MRI revealed T2 hyperintensities involving the mammillary bodies, periaqueductal grey matter, medial thalami, third ventricle, and area postrema. Whole blood thiamine levels were elevated and she did not improve with IV thiamine. CSF was notable for lymphocytic pleocytosis and elevated protein. She tested positive for serum Aquaporin-4 (AQP4) antibody. Subsequent imaging revealed multilevel lesions in the cervical and thoracic spinal cord. Her CSF GFAP antibody also came back positive. She steadily and significantly improved after high-dose IV steroids and plasmapheresis. She later started on chronic rituximab therapy. This represents a unique case of NMOSD presenting with the classical clinical and imaging features of WE, as opposed to the typical presenting symptoms of NMOSD. As such, demyelinating disorders should be considered when there is concern for diencephalic and midline pathologies, particularly without classic WE risk factors. Conversely, clinicians should be aware of secondary nutritional complications arising from severe area postrema syndrome.
RESUMO
Background and Objectives: Parkinson disease (PD) and progressive supranuclear palsy (PSP) are often difficult to differentiate in the clinic. The MR parkinsonism index (MRPI) has been recommended to assist in making this distinction. We aimed to assess the usefulness of this tool in our real-world practice of movement disorders. Methods: We prospectively obtained MRI scans on consecutive patients with movement disorders with a clinical indication for imaging and obtained measures of MRI regions of interest (ROIs) from our neuroradiologists. The authors reviewed all MRI scans and corrected any errors in the original ROI drawings for this analysis. We retrospectively assigned diagnoses using established consensus criteria from progress notes stored in our electronic medical record. We analyzed the data using multinomial logistic regression models and receiver operating curve analysis to determine the predictive accuracy of the MRI ratios. Results: MRI measures and consensus diagnoses were available on 130 patients with PD, 54 with PSP, and 77 diagnosed as other. The out-of-sample prediction error rate of our 5 regression models ranged from 45% to 59%. The average sensitivity and specificity of the 5 models in the testing sample were 53% and 80%, respectively. The positive predictive value of an MRPI ≥13.55 (the published cutoff) in our patients was 79%. Discussion: These results indicate that MRI measures of brain structures were not effective at predicting diagnosis in individual patients. We conclude that the search for a biomarker that can differentiate PSP from PD must continue.
