Rapid Reversal of Locked-in Syndrome from Acute Neuroinvasive West Nile Virus with High-dose Steroids
Sumayya Mohammed1, Ai Ohno1, Lisa Sovory2
1California University of Science and Medicine, 2S’napse Neurology
Objective:

To describe a case of a middle-aged male with acute-onset and rapidly progressive encephalopathy manifesting with confusion, stupor, and bulbar signs. 

Background:

There have been recurring West Nile Virus (WNV) epidemics in the United States, but only about 1% of cases present as a neuroinvasive disease. There are anecdotal cases of successful treatments for neuroinvasive WNV beyond supportive care. However, evidence is still considered limited for routine use. 

Design/Methods:
NA
Results:

A 61-year-old male with a history of hypertension and diabetes presented to the emergency department with 4 days of fever, chills, vomiting, severe abdominal pain, and Glasgow coma scale (GCS) 15. He was septic with tachycardia, fever, total white blood cell count of 12,000/mm3, and a urinary tract infection. Despite appropriate antibiotic therapy, the patient became progressively confused and somnolent on day 3. On day 5 patient was akinetic with mutism, tremors, and cogwheel rigidity. By day 8 the patient appeared “locked-in” necessitating respiratory support. Uremic encephalopathy was the leading consideration given BUN 54 mg/dL and creatinine 4.57 mg/dL. There was no improvement after 4 sessions of hemodialysis. Serial Head CT and MRI of the brain were unremarkable. EEG on day 8 showed nonspecific slowing and evolved to continuous, 1 hz GPDs on day 15. Patient was treated empirically with vancomycin, acyclovir, ampicillin, and ceftriaxone prior to return of CSF studies without improvement. On day 16, CSF WNV IgM and IgG titers returned 7.98 and 1.66 respectively.  Methylprednisolone 1g IV was started empirically on day 16 with a marked improvement in mental status.  GCS improved to 15, the patient was following commands, and moving all 4 extremities after 2 of 5 planned doses. He was extubated on day 19.

Conclusions:

Rapid reversal of mental status following treatment with corticosteroids suggests its potential benefit in the treatment of neuroinvasive WNV infection.

10.1212/WNL.0000000000202469