art. 1.4267

European Review for Medical and Pharmacological Sciences

2015; 19: 1842-1844

Acute encephalopathy associated with hemolytic
uremic syndrome caused by Escherichia coli
O157: H7 and rotavirus infection
G. IMATAKA1, K. WAKE2, M. SUZUKI3, H. YAMANOUCHI4, O. ARISAKA1
1

Department of Pediatrics, Dokkyo Medical University School of Medicine, Tochigi, Japan
Department of Emergency and Critical Care Medicine, Dokkyo Medical University School ofMedicine, Tochigi, Japan3
Department of Pediatric Neurology, Aichi Children's Health and Medical Center, Aichi, Japan
4
Department of Pediatrics, Saitama Medical University, Saitama, Japan2Abstract. – We reported a case of a 22months child with hemolytic uremic syndrome
associated with encephalopathy. As the cause ofthis case, the involvements of verotoxin 1 and 2
caused by O157: the H7 strain of the enterohemorrhagic Escherichia coli and rotavirus were presumed. We administered brain hypothermic therapy and steroid pulse therapy in the intensive
care unit, but we were not able to save his life
and the child died on the 6th day from the onset.
Key Words:
EHEC, VT, Hemolytic uremic syndrome (HUS), Brain
hypothermic therapy, Steroid pulse therapy.

Introduction
Rotavirus, the primary cause of viral gastroenteritis, causes children to vomit and have white
diarrhea. The basic treatment is rehydration andcorrecting the electrolyte imbalances. Rotavirus
inflammation in children may rarely develop into
rotavirus encephalopathy that is accompanied byseizures and acute encephalopathy1.
However, enterohemorrhagic Escherichia coli
(EHEC) may cause, in addition to high fever,acute stomach aches and blood feces, hemolytic
uremic syndrome (HUS) or acute encephalopathy, and may become a serious condition2 for thepatient. As EHEC produces verotoxin (VT), it iscalled verotoxin-producing Escherichia coli(VTEC).
We had a child patient who developed rotavirus encephalopathy accompanied by HUS encephalopathy caused by O157: H7. As we haveno record of such a case in the past, we report theprogression of the case.
1842

Care Report
The 22-month old male child was transported
to our hospital as he was observed with highfever, vomition, and status epilepticus. His family history and motor developments were normal.
When he arrived at our hospital, he was in cardiopulmonary arrest, and the resuscitative maneuver was immediately conducted in the emergency room. His heartbeat resumed for at least
15 minutes, but no spontaneous breathing wasobserved. The diameters of the pupils of botheyes were 6 mm and fixed, the light reflex waslost. After the commencement of artificial respiration, he responded to pain stimulations withshaking off actions but did not open his eyes.
Head CT revealed a diffuse edema in the brain(Figure 1). An enormous amount of water diarrhea was observed, and the result of the rapid
antigen test for rotavirus was positive. Based on
the clinical course, we diagnosed it as rotavirus
related encephalopathy, and we hospitalized himin ICU and commenced the brain hypothermic
therapy and the steroid pulse therapy. The resultof the blood test was: WBC 14,000/µL, hemoglobin 6.0 g/dL, platelet 0.6×104/µL, BUN 36
mg/dL, Cre 1.8 mg/dL, AST 126 IU/L, CK 202
IU/L, CRP 0.3 mg/dl. Based on the progression,
we diagnosed HUS concurrence, and we administered blood transfusion, anticoagulant therapy,
and intracranial pressure reduction therapy simultaneously. As he developed disseminated intravascular coagulation (DIC) on the second dayof intensive care, we stopped the brain hypothermic therapy after 24 hours. As we checked theelectroencephalogram, it showed flat waves without the base activities (Figure 2). In the fecal bacterial cultivation upon admission, only resident
bacteria were detected, but when we conducted

Corresponding Author: George Imataka, Ph.D; e-mail: geo@dokkyomed.ac.jp

Acute encephalopathy associated with hemolytic uremic syndrome

Discussion

Figure 1. Cephalic CT on the first day on hospitalization.
CT revealed severe form of diffuse cerebral edema.

O-157:7 direct method on the third day of HUS
progression, it indicated positive. As we conducted the verotoxin (VT) quick test by enzyme immunoassay (EIA) method and immune chromatography method, VT1 and VT2 were positivein both tests. The child died on the sixth day ofhospitalization. The infection route of O157: H7
was unknown.

Figure 2. Electroencepharogram on the 2nd day from hospitalization. The patient’s EEG exhibited low-voltage andflat background activity.

The HUS can be diagnosed with hemolytic
anemia, a decrease in platelets and acute disorder of the kidney. The HUS occurs 10% or lessin EHEC. Central nervous system manifestations
accompany EHEC-associated HUS, and 5% orless of the cases leads to death due to acute encephalopathy and perforation of the digestive
tract3. CT/MRI4 and the brain waves5 are effective means to diagnose HUS encephalopathy.
Mild cases of HUS encephalopathy cannot be
detected by CT. However, serious cases present
abnormalities in the splenium of the corpus callosum, and both sides of the optic thalamus andin the nucleus basalis6. The most serious cases
are present edema in the brain and brain hernia6.
On the other hand, the brain waves show abnormalities of base activities even in mild cases ofHUS encephalopathy. Severe cases of HUS encephalopathy present high-amplitude slow
wave5. In this particular case, we observed a severe degree of edema in the brain by CT scan
upon the admission of the patient. As treatmentsof HUS encephalopathy, plasma exchange therapy7, dialysis, anticoagulant therapy8, pressure reduction therapy and other therapies are conducted, but there is no established treatment. We kept
him under intensive care and administered brain
hypothermic therapy 9 and steroid pulse
therapy10, but the child died on the sixth day ofhospitalization.
There are two occurrence mechanisms for
O157-triggered HUS encephalopathy. One occurs as Shiga Toxin of VT1 and VT2 disturbs
protein synthesis on the vascular endothelial11.
The other occurs as TNF-α and IL-1β are produced via lipopolysaccharide (LPS) and induce
inflammatory cytokines such as IL-6 or IL-8 todestroy the renal vascular endothelial and theblood-brain barrier that allows Shiga toxin to affect the brain directly12,13. Abnormalities of theelectrolytes and the circulation balance caused byacute kidney disorder and the TNF can be exacerbating factors as well.
The VT1 and VT2 were found in our case aswell. And the rotavirus antigens in the feces of
the child were positive. Rotavirus is known tocause various central nervous system manifestations. In the case of rotavirus encephalopathy, theviruses may be found in the spinal fluid PCR,
and clarification of the occurrence mechanism ismuch desired1. It is unknown that how much rotavirus affected the HUS encephalopathy in our1843

G. Imataka, K. Wake, M. Suzuki, H. Yamanouchi, O. Arisakacase. However, in the entire body condition, it
was much contributing to making dehydrationand diarrhea serious, and we can assume that it
was an exacerbating factor.

Conclusions
To our best of knowledge, the present case wasthe most severe form of HUS encephalopathy.
–––––––––––––––––-––––
Conflict of Interest

The Authors declare that there are no conflicts of interest.

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