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Neurologic Manifestations and Outcome of West Nile Virus Infection

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Neurologic Manifestations and Outcome of West Nile Virus Infection BRIEF REPORT Neurologic Manifestations and Outcome of West Nile Virus Infection James J. Sejvar, MD Context The neurologic manifestations, laboratory findings, and outcome of patients Maryam B. Haddad, MSN, MPH, FNP with West Nile virus (WNV) infection have not been prospectively characterized. Bruce C. Tierney, MD Objective To describe prospectively the clinical and laboratory features and long- term outcome of patients with neurologic manifestations of WNV infection. Grant L. Campbell, MD, PhD Design, Setting, and Participants From August 1 to September 2, 2002, a com- Anthony A. Marfin, MD, MPH munity-based, prospective case series was conducted in St Tammany Parish, La. Stan- Jay A. Van Gerpen, MD dardized clinical data were collected on patients with suspected WNV infection. Con- firmed WNV-seropositive patients were reassessed at 8 months. Aaron Fleischauer, PhD Main Outcome Measures Clinical, neurologic, and laboratory features at initial A. Arturo Leis, MD presentation, and long-term neurologic outcome. Dobrivoje S. Stokic, MD Results Sixteen (37%) of 39 suspected cases had antibodies against WNV; 5 had men- Lyle R. Petersen, MD, MPH ingitis, 8 had encephalitis, and 3 had poliomyelitis-like acute flaccid paralysis. Movement disorders, including tremor (15 [94%]), myoclonus (5 [31%]), and parkinsonism (11 [69%]), OST HUMAN INFECTIONS were common among WNV-seropositive patients. One patient died. At 8-month follow- with West Nile virus up, fatigue, headache, and myalgias were persistent symptoms; gait and movement dis- (WNV) are subclinical or orders persisted in 6 patients. Patients with WNV meningitis or encephalitis had favor- able outcomes, although patients with acute flaccid paralysis did not recover limb strength. manifest as a mild fe- Mbrile illness, but a small proportion of Conclusions Movement disorders, including tremor, myoclonus, and parkinson- patients (Ͻ1%) develop acute neuro- ism, may be present during acute illness with WNV infection. Some patients with WNV 1-4 infection and meningitis or encephalitis ultimately may have good long-term out- logic illness. Although recent WNV come, although an irreversible poliomyelitis-like syndrome may result. outbreaks have been associated with se- JAMA. 2003;290:511-515 www.jama.com vere neurologic disease,1-5 retrospec- tive studies have failed to identify clini- cal features that distinguish WNV from count; or cerebrospinal fluid [CSF] pleo- electrophysiologic findings were re- other viral encephalitides.1,5-9 The US cytosis) along with clinical evidence of corded and updated 1 week following outbreak of WNV in 200210 presented meningitis, encephalitis, or acute focal initial assessment, during repeat neu- an opportunity to assess neurologic weakness (BOX). Infection with WNV rologic evaluation. manifestations, laboratory and neuro- was confirmed if WNV-specific antibod- Approximately 8 months later (March diagnostic findings, and outcome as- ies were detected in acute-phase serum 15-April 4, 2003), patients with con- sociated with WNV infection.1,7,11 or CSF samples by IgM antibody- firmed WNV infection were reexam- capture enzyme-linked immunosor- ined. The Centers for Disease Control METHODS bent assay (MAC-ELISA)12 and were and Prevention institutional review From August 1 to September 2, 2002, confirmed by plaque-reduction neutral- 13 Author Affiliations: Division of Viral and Rickettsial patients from St Tammany Parish, La, ization assay. Diseases (Dr Sejvar) and Division of Vector-Borne In- with suspected WNV infection were Eligible enrollees were assessed on fectious Diseases (Drs Campbell, Marfin, and Petersen), National Center for Infectious Diseases, and Epi- identified through state-based surveil- presentation to medical care. Patients demic Intelligence Service, Epidemiology Program Of- lance at local hospitals and regional medi- were approached under the auspices of fice (Drs Tierney and Fleischauer and Ms Haddad), Cen- a public health event; oral consent was ters for Disease Control and Prevention, Atlanta, Ga; cal centers. Suspected WNV infection was Department of Neurology, Ochsner Clinic, New Or- defined as illness with evidence of an obtained. Standardized case histories leans, La (Dr Van Gerpen); Center for Neuroscience and initial symptoms and signs were and Neurological Recovery, Methodist Rehabilita- acute infectious process (eg, tempera- tion Center, Jackson, Miss (Drs Leis and Stokic). ture Ն39°C; elevated white blood cell collected. One neurologist (J.J.S.) ex- Corresponding Author and Reprints: James J. Sejvar, amined each patient; a second neurolo- MD, Division of Viral and Rickettsial Diseases, Na- gist verified findings for 7 patients. tional Center for Infectious Diseases, Centers for Dis- See also p 524 and Patient Page. ease Control and Prevention, 1600 Clifton Rd, MS Laboratory results, neuroimaging and A-39, Atlanta, GA 30333 (e-mail: [email protected]). ©2003 American Medical Association. All rights reserved. (Reprinted) JAMA, July 23/30, 2003—Vol 290, No. 4 511 NEUROLOGIC MANIFESTATIONS OF WEST NILE VIRUS INFECTION of the 15 WNV-seropositive patients Box. Diagnostic Criteria with headache described it as frontal/ West Nile Meningitis retro-orbital, and 5 of the 16 WNV- A. Clinical signs of meningeal inflammation, including nuchal rigidity, Kernig or seropositive patients reported a rash. Brudzinski sign, or photophobia or phonophobia Fifteen WNV-seropositive patients re- B. Additional evidence of acute infection, including 1 or more of the following: ported “shakiness” or “twitching,” with fever (Ͼ38°C) or hypothermia (Ͻ35°C); cerebrospinal fluid pleocytosis (Ն5 5 describing it as notable in the evening leukocytes/mm3); peripheral leukocyte count Ͼ10000/mm3; neuroimaging find- prior to sleep. Among patients with ings consistent with acute meningeal inflammation WNE, the most common complaints West Nile Encephalitis were behavioral or personality changes, A. Encephalopathy (depressed or altered level of consciousness, lethargy, or per- manifested as irritability, confusion, or sonality change lasting Ն24 hours) disorientation. Two patients with WNM B. Additional evidence of central nervous system inflammation, including 2 or more and 1 patient with WNE reported dif- of the following: fever (Ն38°C) or hypothermia (Յ35°C); cerebrospinal fluid ficulty with balance and gait. Five pa- pleocytosis (Ն5 leukocytes/mm3); peripheral leukocyte count Ͼ10000/mm3; tients reported weakness, which was fo- neuroimaging findings consistent with acute inflammation (with or without cal in the 3 patients developing AFP and involvement of the meninges) or acute demyelination; presence of focal neu- was generalized in 2 patients. rologic deficit; meningismus (as defined in A); electroencephalography find- The 8 patients with WNE had a mean ings consistent with encephalitis; seizures, either new onset or exacerbation of previously controlled admission Glasgow Coma Scale score of 11 (range, 4T [intubated] to 15). A Acute Flaccid Paralysis median of 3 days passed between symp- A. Acute onset of limb weakness with marked progression over 48 hours tom onset and changes in mental sta- B. At least 2 of the following: asymmetry to weakness; areflexia/hyporeflexia of tus. Cranial nerve and bulbar abnor- affected limb(s); absence of pain, paresthesia, or numbness in affected limb(s); cerebrospinal fluid pleocytosis (Ն5 leukocytes/mm3) and elevated protein lev- malities were observed in several patients els (Ն45 mg/dL); electrodiagnostic studies consistent with an anterior horn cell with WNE. Results of formal strength process; spinal cord magnetic resonance imaging documenting abnormal in- testing displayed mild-to-moderate dif- creased signal in the anterior gray matter fuse weakness in 4 patients and focal weakness in the 3 patients with AFP. New sensory abnormalities were not board approved the follow-up proto- tients, 5 were classified as having West observed. Four patients with WNE and col. Using a standardized question- Nile meningitis (WNM), 8 as having 1 patient with WNM displayed abnor- naire, patients were queried about symp- West Nile encephalitis (WNE), and 3 mal hyperreflexia; the 3 patients with toms, functional status, and ability to as having acute flaccid paralysis (AFP) AFP all had areflexia or hyporeflexia of perform daily activities. The neuro- (TABLE). One patient classified with the affected limbs. logic assessment was repeated (J.J.S.). AFP also had encephalitis. Informa- Dyskinesias (ie, movements includ- Exact Wilcoxon rank-sum test was tion regarding the initial presentation ing tremor, myoclonus, and features of used for comparison of medians. Statis- of 3 patients with AFP had been re- parkinsonism) were observed in 15 of the tical analyses were performed using SAS, ported previously.14,15 Patients with 16 WNV-seropositive patients (Table). version 8.1 (SAS Institute, Cary, NC). WNM (median age, 35 years) were Tremor was observed in 15 patients; 9 younger than those with WNE (me- had onset of tremor after day 5 of ill- RESULTS dian, 70 years) (P=.003). One patient ness. Tremor in all 15 patients was static Clinical Features with severe WNE had systemic lupus or kinetic, asymmetric, and involved the Of 39 patients evaluated, WNV infec- erythematosis and was treated with cor- upper extremities. Two patients addi- tion was confirmed in 16 patients. Dis- ticosteroids. No other WNV-seroposi- tionally displayed intentional move- charge diagnoses of the 23 patients tive patient had a clear condition indi- ment dysmetria. Myoclonus was
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