Severe Dystonia as a Manifestation of West Nile Neuroinvasive Disease

Objective: We describe a unique case of severe generalized dystonia in a teenage patient affected with West Nile encephalitis (WNE). Background: Viral encephalitides can result in a variety of movement disorders. WNE has been associated with tremor, myoclonus, and parkinsonism. Dystonia has been reported in several cases of the related flavivirus Japanese encephalitis virus, but to date there have been no reports of dystonia described in association with West Nile virus (WNV). Design/Methods: An 18 year-old previously healthy male presented with unresponsiveness after a five day history of fever, malaise, sore throat, and diarrhea. On arrival to the emergency center, he had a GCS of 9 and required intubation. CSF was significant for WBC of 220 and protein of 160. WNV IgM was markedly elevated in the CSF, and MRI showed symmetric T2/FLAIR hyperintensities of the bilateral basal ganglia and thalami. The patient was extubated after nine days and initially had some improvement in his mental status, but then declined and developed severe, generalized dystonic posturing that has remained persistent for months following the initial presentation. Results: After the patient’s encephalopathy and posturing worsened, repeat MRI showed stable involvement of the deep gray structures, but with new cortical involvement of the frontal and temporal lobes. A comprehensive infectious and autoimmune workup was repeated and WNV IgM remained positive whereas all other studies were negative. The patient received multiple medications to attempt to alleviate the dystonia including benzodiazepines, carbidopa-levodopa, trihexyphenidyl, and enteral baclofen, without significant improvement. Conclusions: This case demonstrates that severe dystonia can be a complication in WNE, and may prove difficult to manage with oral medications. Additionally, practitioners should be aware that WNE can follow a relapsing course after initial clinical improvement. Disclosure: Dr. Herring has nothing to disclose. Dr. Savitt has nothing to disclose. Dr. Parnes has nothing to disclose.