On the general ward there is a 49 yo female patient with rapidly progressive encephalopathy, starting with vertigo and predominant ataxia, developing bilateral Babinski’s and cognitive decline over three weeks. There are slight FLAIR hyperintensities in the mesial temporal lobe on the left, diffuse slowing, triphasic waves and later epileptiform discharges in the EEG, lymphocytic pleocytosis and a 3-class-reaction with bands in the CSF. Blood works show some antibodies (ANA 1:400, TPO, PCA) and nothing fancy otherwise. Malignancy search (FDG-PET, CT trunk) is more or less normal. Finally, there is no improvement on steroids 1g x now 3 days. Quite remarkably, she has the skin changes of Neurofibromatosis (cafe au lait, neurofibromas) and a huge DVA in the right frontal lobe – all things we could not yet connect to the current disease.
We discuss the differential, using the wonderful article
- Flanagan et al. Autoimmune Encephalopathy. Sem Neurol 2011 (available via medscape)