Advance Publication by J-STAGE

Japanese Journal of Infectious Diseases

Acute flaccid paralysis as the initial manifestation of Japanese

encephalitis: a case report

Qiuyan Shen, Yan Li, Haitao Lu, Pingping Ning, Hongyan Huang,Quanzhen Zhao, and

Yanming Xu

Received: August 27, 2019. Accepted: March 16, 2020.

Published online: April 30, 2020.
DOI: 10.7883/yoken.JJID.2019.332

Advance Publication articles have been accepted by JJID but have not been copyedited
or formatted for publication.
Acute flaccid paralysis as the initial manifestation of Japanese encephalitis: a case report

Qiuyan Shen1, Yan Li2, Haitao Lu3, Pingping Ning4,Hongyan Huang5,Quanzhen Zhao6, Yanming

Xu7 *

1234567 The Department of Neurology, West China Hospital, Sichuan University, Chengdu,

China

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* Corresponding author: Yanming Xu

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Department of Neurology, West China Hospital, Sichuan University, Chengdu, Sichuan

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Province, 610041, P.R. China. Email: neuroxym999@163.com. Tel: +86 2885422716. Fax: +86

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2885423550.

Running head: Acute flaccid paralysis in Japanese encephalitis

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Keywords: Japanese encephalitis; Japanese encephalitis virus; acute flaccid paralysis; muscle
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wasting.
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Summary

Japanese encephalitis (JE) is an inflammation of the central nervous system resulting in

clinical disease, with symptoms ranging broadly in severity from mild febrile illness to acute

meningomyeloencephalitis. JE has been associated with a variety of neurological

abnormalities such as altered sensorium, seizures, focal neurological deficit, and acute

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flaccid paralysis (AFP). However, AFP has never been reported as the initial manifestation of

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JE. Here we present a case of AFP as the initial manifestation of JE in a Chinese patient. A

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30-year-old Chinese man was admitted to West China Hospital of Sichuan University after

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experiencing AFP in the right upper limb followed by hyperpyrexia and unconsciousness.

Assay of cerebrospinal fluid from lumbar puncture revealed high levels of proteins and anti-
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Japanese encephalitis virus IgM antibodies. Acyclovir (used by intravenous drip) was
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administered. However, the weakness persisted and more extensive muscle wasting from

the proximal to distal right upper limb occurred during seven months. This case reports
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highlights that JE needs to be added to the differential diagnosis of AFP initiating in an adult
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in JE endemic seasons and areas.

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Introduction

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 Japanese encephalitis (JE) is caused by the JE virus (JEV), a flavivirus with five known

genotypes that is transmitted between water birds and/or pigs by vector mosquitoes.

Humans are dead-end hosts of the virus, and the infection manifests as an inflammation of

the central nervous system. The virus has been detected across a wide area covering

eastern and southern Asia, northern Australia, and Papua New Guinea and is responsible for

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approximately 68,000 clinical cases annually (1,2).

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JE virus infections in humans are mostly asymptomatic, and fewer than 1% of infections

result in clinical disease, with severity ranging broadly from mild febrile illness to acute

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meningomyeloencephalitis (3). JE can manifest as undifferentiated febrile illness or may
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present with rigors, coryza or diarrhea followed by the onset of clouding of consciousness,

seizures or vomiting after a few days (4,5). A wide range of neurological abnormalities have
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also been reported in 20-60% of patients with JE, such as altered sensorium, seizures, focal
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neurological deficit, acute flaccid paralysis (AFP) (possibly due to anterior horn cell
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involvement), transient Parkinsonian features, and dystonia (limb, axial, orofacial) (6). JE is
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commonly diagnosed by testing for anti-JEV IgM by in the cerebrospinal fluid, which can be

detected within one week after disease onset (7).

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Here, we present the first case of a patient with AFP as the initial manifestation of JE.
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Case report

A 30 year old Chinese man, who had no JEV vaccination history, was admitted to West

China Hospital of Sichuan University after experiencing 8 days of right upper limb weakness

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followed by hyperpyrexia and unconsciousness. The patient reported experiencing general

fatigue starting 8 days prior to admission at the hospital. After this fatigue began, the distal

muscle in the right upper limb suddenly became weak, and this weakness extended to the

proximal muscle within three days. Then body temperature peaked at 40 °C, followed by

continuous unconsciousness without seizures or incontinence (Glasgow Coma Scale 4/15

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score). At 5 days prior to admission at the hospital, the patient was admitted to another

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hospital, where he received supportive treatment for 5 days. During this period, he

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recovered consciousness and his temperature remained below 37.3 °C, but his weakness

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progressively worsened. He was transferred to West China Hospital of Sichuan University for

further testing and treatment.

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Physical examination at West China Hospital of Sichuan University revealed grade-2
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strength in the right upper limb and grade-5 strength in other limbs (muscle strength
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recording 0-5 grades), hypoactive tendon jerk in the upper limbs and slightly hyperactive
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tendon jerk in the lower limbs, reduced muscle tone in the right upper limb and mildly
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increased muscle tone in the lower limbs, positive nuchal rigidity and Kernig sign. The

routine blood test showed high number of white blood cell (12×10^9/L,4-10×10^9/L) and
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high level of neutrophil ratio (90%). The remaining laboratory test results as liver and kidney
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function, electrolyte, coagulation function, thyroid function, tumor markers, immune

function and so on were normal. Anti-JEV IgM antibody was detected by enzyme-linked

immunosorbent assay (ELISA) (used in commercial kit) in serum and in cerebrospinal fluid

from lumbar puncture. The tests of cerebrospinal fluid showed high number of cells (100×

10^6/L) and high levels of proteins (0.94g/L) and the remaining examined projects such as
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the test result of chloride, glucose and smear for fungi/bacteria/mycobacterium and so on

were normal. Electromyography indicated neurogenic damage in the upper limbs,

predominantly in the right upper limb, and probable injury of the anterior horn (right C5-T1,

left C5-6). No significant alterations were observed in magnetic resonance imaging of the

brain or the cervical spinal or water imaging of the right brachial plexus. These results led to

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a diagnosis of JE with myelitis. Acyclovir (used by intravenous drip), rehabilitative and

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supportive therapies were administered for 12 days. However, the weakness persisted. At

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follow-up 7 months after discharge, the patient showed similar muscle weakness as at

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discharge but more extensive muscle wasting from the proximal to distal right upper limb.
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To our knowledge, this is the first case report of a JE patient with AFP as the initial

clinical manifestation. Previous studies suggest that one third of JE patients show
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generalized or focal weakness without atrophy involving primarily the upper motor neuron
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units, and that one quarter of patients show muscle wasting involving hands, feet, and
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flexors of the neck (8-10). One study suggested that the anterior horn cell was involved to
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varying degrees in many JE patients, which contributes to focal or general weakness and

muscle wasting (11). In our patient, JEV infection manifested with AFP in the right limb,
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which was consistent with previous reports that JE patients could present symptoms of focal
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weakness and muscle wasting.

There were some studies reported that AFP presented in JE patients, but never as the

initial manifestation (12-14). This case highlights that JE, as well as Guillain Barré syndrome,

myasthenia, and acute transverse myelitis, needs to be added to the differential diagnosis of

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AFP initiating in an adult. JE needs to be added to the disease list for differential diagnosis

for AFP.

Acknowledgments

This work was supported by the Platform Construction Project of the Sichuan Provincial

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Department of Science and Technology, China (19PTDJ0042).

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Conflict of interest: none

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