Unusual Movement Disorders and Atypical Magnetic Resonance Imaging (MRI) Findings in Patients with West Nile Encephalitis: Case Reports of 2 Patients with Evidence of Clinical and Imaging Resolution with IVIG

Abstract

Case series Patients: Male, 34-year-old • Female, 57-year-old Final Diagnosis: West Nile virus myeloradiculitis • West Nile virus opsoclonus myoclonus ataxia syndrome Symptoms: Confusion • lower extremity weakness • opsoclonus-myoclonus-ataxia Medication: — Clinical Procedure: Lumbar puncture Specialty: Infectious Diseases • Neurology Objective: Unusual clinical course Background: West Nile virus (WNv) is the leading cause of epidemic arbovirus encephalitis in the continental United States. Movement disorders (MDs) have been reported in 20% to 40% of patients with WNv and about 37% of patients with WNv encephalitis have changes on magnetic resonance imaging (MRI). We report 2 unusual cases of neuroinvasive WNv in patients with unusual MDs and unreported MRI findings. Case Reports: In the first case, a 34-year-old man presented with a 1-week history of disinhibition, agitation, opsoclonus-myoclonus and ataxia syndrome (OMAS), tremor, and facial agnosia. Evaluation of his cerebrospinal fluid (CSF) revealed elevated immunoglobulin (Ig)M against WNv, a high level of protein (98 mg/dL), and an elevated white blood cell (WBC) count (134, 37% lymphocytes). An MRI of the brain showed an area of diffusion restriction in the splenium of the corpus callosum . The patient’s MRI findings and OMA improved significantly after 2 treatments with i.v. IG (IVIG). In the second case, a 57-year-old woman presented with fever, headaches, psychosis, and ataxia; she was subsequently intubated for airway protection. Analysis of her CSF showed elevated IgM against WNv, a high level of protein (79 mg/dL), and elevated WBC count (106, 90% lymphocytes). One week after the onset of symptoms, the patient experienced facial dyskinesia. Later, she developed proximal bilateral lower extremity weakness. An MRI of her lumbar spine showed evidence of myeloradiculitis with contrast enhancement of the conus medullaris and ventral nerve roots. After a single treatment with IVIG, she had partial improvement in weakness. Conclusions: MDs and changes on MRI have been reported in patients with neuroinvasive WNv disease. Our patient with OMAS also had transient splenial diffusion restriction on imaging, which, to the best of our knowledge, has not been previously reported with WNv infection. In both patients, treatment with IVIG resulted in improvement in symptoms.

West Nile Virus Meningoencephalitis—A Consideration for Earlier Investigation

“…Cardiac arrhythmias, although uncommon, have been observed particularly in patients diagnosed with West Nile Encephalitis [8]. Furthermore, as there are multiple different neurologic manifestations of neuroinvasive disease, tremors predominantly manifest in the upper extremities of patients rather than the lower extremities [9,15]. As such, these vague findings in our patient may or may not have been associated with neuroinvasive disease, yet they indicate the importance for considering a broad differential that includes WNV.…”