Emerging Cases of Powassan Virus Encephalitis in New England: Clinical Presentation, Imaging, and Review of the Literature

Piantadosi, Anne; Rubin, Daniel B.; McQuillen, Daniel P.; Hsu, Liangge; Lederer, P; Ashbaugh, Cameron D.; Duffalo, Chad; Duncan, Robert A.; Thon, Jesse M.; Bhattacharyya, Shamik; Basgoz, Nesli; Feske, Steven K.; Lyons, Jennifer L.

doi:10.1093/cid/civ1005

Cited by 125 publications

(167 citation statements)

References 23 publications

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“…Cases in the United States are typically reported in the northeastern and midwestern states. In recent years, the incidence of human infection due to POWV appears to be rising (Hinten et al 2008, Ebel et al 2010, Piantadosi et al 2016). From 1958 through 1998, only 27 human POWV cases were reported from eastern Canada and the northeastern United States (Gholam et al 1999); whereas from 2003 to present day, 85 human cases of Powassan encephalitis have been documented in the United States alone (USGS Disease maps: ).…”

Section: Introductionmentioning

confidence: 99%

Powassan Virus: An Emerging Arbovirus of Public Health Concern in North America

Hermance

Thangamani

2017

Vector-Borne and Zoonotic Diseases

157

166

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Powassan virus (POWV, Flaviviridae) is the only North American member of the tick-borne encephalitis serogroup of flaviviruses. It is transmitted to small- and medium-sized mammals by Ixodes scapularis, Ixodes cookei, and several other Ixodes tick species. Humans become infected with POWV during spillover transmission from the natural transmission cycles. In humans, POWV is the causative agent of a severe neuroinvasive illness with 50% of survivors displaying long-term neurological sequelae. POWV was recognized as a human pathogen in 1958 when a young boy died of severe encephalitis in Powassan, Ontario, and POWV was isolated from the brain autopsy of this case. Two distinct genetic lineages of POWV are now recognized: POWV (lineage I) and deer tick virus (lineage II). Since the index case in 1958, over 100 human cases of POWV have been reported, with an apparent rise in disease incidence in the past 16 years. This recent increase in cases may represent a true emergence of POWV in regions where the tick vector species are prevalent, or it could represent an increase in POWV surveillance and diagnosis. In the past 5 years, both basic and applied research for POWV disease has intensified, including phylogenetic studies, field surveillance, case studies, and animal model development. This review provides an overview of POWV, including the epidemiology, transmission, clinical disease, and diagnosis of POWV infection. Recent research developments and future priorities with regard to the disease are emphasized.

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Section: Introductionmentioning

confidence: 99%

Powassan Virus: An Emerging Arbovirus of Public Health Concern in North America

Hermance

Thangamani

2017

Vector-Borne and Zoonotic Diseases

157

166

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“…WNV and POWV can cause severe neuropathology and death in humans ( 16, 17 ). The enzootic cycle of WNV is between Culex species of mosquitos and birds, with humans being incidental dead-end hosts.…”

Section: Introductionmentioning

confidence: 99%

Zika virus–related neurotropic flaviviruses infect human placental explants and cause fetal demise in mice

et al. 2018

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Although Zika virus (ZIKV) infection in pregnant women can cause placental damage, intrauterine growth restriction, microcephaly, and fetal demise, these disease manifestations only became apparent in the context of a large epidemic in the Americas. We hypothesized that ZIKV is not unique among arboviruses in its ability to cause congenital infection. To evaluate this, we tested the capacity of four emerging arboviruses (West Nile virus, WNV; Powassan virus, POWV; chikungunya virus, CHIKV; and Mayaro virus, MAYV) from related (flavivirus) and unrelated (alphavirus) genera to infect the placenta and fetus in immunocompetent, wild-type mice. Whereas all four viruses caused placental infection, only infection with the neurotropic flaviviruses (WNV and POWV) resulted in fetal demise. WNV and POWV also replicated efficiently in second-trimester human maternal (decidua) and fetal (chorionic villi and fetal membrane) explants whereas CHIKV and MAYV replicated less efficiently. In mice, RNA in situ hybridization and histopathological analysis revealed that WNV infected the placenta and fetal central nervous system, causing injury to the developing brain. In comparison, CHIKV and MAYV did not cause substantive placental or fetal damage despite evidence of vertical transmission. Based on the susceptibility of human maternal and fetal tissue explants and pathogenesis experiments in immunocompetent mice, other emerging neurotropic flaviviruses may share with ZIKV the capacity for transplacental transmission, and subsequent infection and injury to the developing fetus.

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“…POWV infection typically presents with an initial febrile illness that can progress to encephalitis or encephalomyelitis after an incubation period of 1-5 weeks. 6,7 Prior case reports have described both flaccid and spastic paralysis in adults with POWV. [6][7][8] Around 10% of infections are fatal, with over 50% of patients having permanent neurologic deficits, including hemiplegia, ophthalmoplegia, headaches, and memory loss.…”

Section: Discussionmentioning

confidence: 99%

“…6,7 Prior case reports have described both flaccid and spastic paralysis in adults with POWV. [6][7][8] Around 10% of infections are fatal, with over 50% of patients having permanent neurologic deficits, including hemiplegia, ophthalmoplegia, headaches, and memory loss. 6 CSF analysis typically shows a neutrophil or lymphocyte pleocytosis with elevated protein.…”

Section: Discussionmentioning

confidence: 99%

“…MRI findings commonly demonstrate T2 hyperintensities (with or without enhancement) in the brainstem, cerebellum, deep gray structures, and cortex, though normal brain and spine imaging have been seen in some cases. 7 Postmortem pathologic evaluation of patients with POWV infection has revealed neuronal destruction in multiple areas of the CNS, including the cortex, basal ganglia, thalami, brainstem, spinal cord, anterior horn cells, and cerebellum, which may explain the differing patterns of neurologic deficits that have been reported. 6 It is reasonable to assume that our patient's age and history of immunosuppression made him more susceptible to profound disease sequelae, given that these 2 characteristics are associated with increased severity of other flaviviridae infections, such as WNV.…”

Section: Discussionmentioning

confidence: 99%

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Clinical Reasoning: A man with rapidly progressive weakness and respiratory failure

Murphy

Balu

et al. 2018

Neurology

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A 70-year-old man presented in late summer with rapidly progressive weakness and respiratory failure. He was in his usual state of health until 1 week prior to presentation, when he developed malaise, headache, and fevers, followed by right leg weakness, prompting admission to a local hospital. Over the next 3 days, his weakness progressed to involve his right arm, then his extraocular, facial, and bulbar muscles, followed by his left arm and leg. On the fourth day of hospitalization (2 weeks after the onset of constitutional symptoms), he was intubated due to an inability to clear oral secretions and was transferred to our institution.Upon arrival, the patient had a temperature of 102.3°, intact mental status, normal pupillary responses, restricted horizontal and vertical gaze, downbeating nystagmus on downward gaze, weakness of his bilateral upper and lower facial muscles, and reduced muscle tone. He had 0/5 strength in all extremities, save trace left finger movement, intact sensation without a sensory level, trace reflexes, and absent Babinski signs.

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Emerging Cases of Powassan Virus Encephalitis in New England: Clinical Presentation, Imaging, and Review of the Literature

Cited by 125 publications

References 23 publications

Powassan Virus: An Emerging Arbovirus of Public Health Concern in North America

Powassan Virus: An Emerging Arbovirus of Public Health Concern in North America

Zika virus–related neurotropic flaviviruses infect human placental explants and cause fetal demise in mice

Clinical Reasoning: A man with rapidly progressive weakness and respiratory failure

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