A 18-year young man, firstly complained muscle weakness of bilateral lower limbs, followed by fever, dizziness and muscle weakness of the neck and bilateral upper limbs. At 5th day of illness onset (on October 3,2018), he felt dyspnea without convulsion and impaired consciousness and was referred to the emergency room of our hospital. He abruptly presented deteriorate respiratory distress and adopted invasive ventilator in emergency room of our hospital on October 3,2018. The brain magnetic resonance imaging (MRI) was normal (Fig. 1C).Blood tests revealed elevated white blood cell counts (11.79 x 109/L) and neutrophils count (9.56 x 109/L). Positive anti-JEV immunoglobulin(Ig) M antibody(EEB-IgM, Enzyme immunoassay test kit, Shanghai B&C Biological Technology Company) was detected in serum. Unexpectedly, high level anti-JEV IgG antibody (JE detect ™IgG, ELISA, InBios) was also be detected in serum. The cerebrospinal fluid (CSF) showed elevated the leukocyte counts (49×106/L, lymphocytic pleocytosis) and protein level (814 mg/L, normal range:100–600mg/L), while anti-JEV IgM for CSF sample was undetected. He was from a rural area in Liaoning province of China where JEV is epidemic in the summer of 2018. He has a definite history of twice JE vaccination in childhood scheduled at eight-months old and at two years old respectively. He adopted methylprednisone (500mg per day) followed by reduced dosage treatment.
Ten days later, his condition partly improved. He refused to continue invasive ventilator though he still had respiratory disturbance with elevated PaCO2 58.0mmHg and decreased PaO2 72.0mmHg (at intranasal oxygen therapy of 10L/min). Despite clear consciousness, proper cognition and functional bladder or bowel, he had markedly disturbances in coughing, articulation and swallowing with disappeared pharyngeal reflex and disabled soft palate movement, which indicated the injured cranial nerves(bulbar paralysis). He could only use pectoral type breathing without any thoracic breathing, which suggested the respiratory myoparalysis. We examined weakened muscle power in left upper limb (grade 4/5), right upper limb (grade 2/5) and bilateral lower limb (grade 2/5), which were already better than before. His muscle tone of four extremities was decreased. Deep tendon reflexes were decreased without pathological reflex and sensations disturbance.
Auscultation showed purring sound in bilateral lungs. Lung computed tomography (CT) indicated consolidation in the lower lobe of the right lung (Fig. 1A). Blood test showed negative antibodies for Epstein-Barre virus, herpes simplex virus, varicella-zoster virus, cytomegalovirus and human immunodeficiency virus. At 24th day of illness onset, both repeated brain MRI and spine MRI showed normal (Fig. 1D, 1E). He did not accept lumbar puncture again. The detection for anti-gangliosides antibodies was unavailable in our hospital. At 27th day of illness onset, electrophysiology examination showed abnormal discoveries including prolonged latency of H reflex in bilateral tibial nerves, declined amplitude of evoked velocity in right median nerve and slowed motor conduction in the right tibial nerve, while the sensory nerves conduction were normal.JEV infection-associated GBS was considered, which matched with Brighton criteria level 1. He declined intravenous immunoglobulin and mechanical ventilator. Despite the application of broad-spectrum antibiotics and sputum clearance with bronchoscopy, he developed progressive dyspnea, deteriorated carbon dioxide retention and respiratory acidosis (PaCO2 120 mmHg; PH 7.185). Lung CT indicated aggravated consolidation in the lower lobe of bilateral lung, mainly in the right lung (Fig. 1B). As a result of refusing mechanical ventilator again and tracheotomy, the patient died on the 36th day of illness onset.
We made a literature review of a total of 85 cases of JEV-associated AFP reported previously (Table 1), 73 cases were considered as GBS. There were 4 cases with reported a history of previous JE vaccination.
Table 1
Clinical features in patients with acute flaccid paralysis caused by Japanese encephalitis virus
Case numbers
|
Reference number/
year of publication
|
Age(yr)
|
Gender
(M/F)
|
Area of report
|
Clinical
presentations
|
Encephalitis
|
Brain MRI
|
JE vaccination
|
Treatment
|
Outcome
D/S/N
|
21
|
[4]/1994
|
6–58
|
18/3
|
India
|
GBS
|
No
|
NM
|
NM
|
GC or MV
|
4/15/2
|
1
|
[5]/2014
|
23
|
1/0
|
China
|
GBS
|
No
|
Normal
|
No
|
IG + GC
|
S
|
1
|
[13]/2015
|
14
|
1/0
|
India
|
GBS
|
Yes
|
Abnormal
|
NM
|
IG + MV
|
S
|
1
|
[3]/2007
|
22
|
1/0
|
Taiwan
|
AFP
|
No
|
Normal
|
Yes
|
GC + MV
|
S
|
12
|
[12]/1998
|
3–15
|
9/3
|
Vietnam
|
AFP
including GBS(1)
|
Yes(4 )
|
NM
|
NM
|
No MV
|
S
|
1
|
[14]/2021
|
43
|
0/1
|
China
|
GBS
|
Yes
|
Abnormal
|
NM
|
IG + GC + MV + PAIA
|
S
|
47
|
[8]/2020
|
*59 (24–63)
|
26/21
|
China
|
GBS
|
39
|
NM
|
Yes(2)
|
IG(28) + GC(47) + MV(44)
|
S
|
1
|
PR
|
18
|
1/0
|
China
|
GBS
|
No
|
Normal
|
Yes
|
GC + MV
|
D
|
GBS,Guillain-Barre syndrome; AFP, acute flaccid paralysis |
NM, not mentioned; PR, present report |
D,died;S,survived; N,no follow up |
IG,immunoglobulins; GC,glucocorticoid; MV,mechanical ventilator; PAIA,protein A immunoadsorption |
*Median age (IQR) |