Case Communications
Acute Encephalopathy Preceding Shigella Infection
1 2
Raz Somech MD , Yael Leitner MD
1 2
Department of Pediatrics, Pediatric Neurology Unit and
Hospital, Tel Aviv Sourasky Medical Center and Sackler Faculty of Medicine, Tel Aviv University, Israel
Key words: acute encephalopathy,
The term encephalopathy describes a
generalizeddisorder of cerebral function.
The causes of acute encephalopathies in
children are infectious, toxic and is-
chemic. Acute encephalopathy due to
shigellosis is a well-known condition that
usually presents with the classical symp-
toms of fever, toxicity, predominant
intestinal signs, or metabolic derange-
ment accompanied by brain edema. We
report a patient who presented in an
acute encephalopathic state caused by
shigellosis, which was only later manifest
by mild intestinal signs and fever.
Patient Description
A 3 year old girl with an unremarkable
previous medical history was admitted
for evaluation of persistent somnolence
during the preceding 2 days. She had no
other symptoms, and diarrhea, fever,
head trauma and use of possibly culp-
able medication were ruled out. No one
in her immediate surroundings was ill.
Upon arrival at the pediatric emer-
gency department she was in an ob-
viously somnolent state with diffuse
symetric hypotonia. She responded
poorly to commands. Deep tendon
reflexes were normal, pyramidal signs
were negative, and there was no nuchal
rigidity. The rest of the physical exam-
ination was normal. She was diagnosed
as having acute encephalopathy and
further evaluation was undertaken ac-
cordingly.
The laboratory data were as follows:
leukocytes 2.1x106/ml with 78% neutro-
phils, and normal arterial blood gas,
serum glucose, electrolytes, blood urea
nitrogen, serum creatinine, and liver
function tests. The levels of ammonia,
384 R. Somech et al.
1,3
and Zvi Spirer MD
3
Leon Alkalay Chair in Pediatric Immunology, Dana Children's
Shigella, children, diarrhea, stool culture
IMAJ 2001;3:384±385
blood pH and C-reactive protein were infection whose altered conscious-
also normal and a toxic screening of ness preceded mild intestinal symptoms
sonnei
urine was negative. Computed tomogra- and fever. After isolation of
phy of the brain was normal, while from her stool, we attributed this
Shigella
electroencephalogram demonstrated a finding to her abnormal neurological
sonnei
mild asymmetry of the background state. Intoxication and other potential
activity, with greater amplitude at the infectious agents were ruled out.
occipital region on the right both during Shigellosis is a common infectious
the sleep and awake recording. A lumbar disease, especially in underdeveloped
puncture was performed and showed a countries. Its clinical manifestations
high opening pressure (45 cm H O). vary, and include watery or loose stool,
Analysis of the cerebrospinal fluid 2was crampy-like abdominal pain, high tem-
within normal limits (glucose 58 mg/dl, perature, and toxic appearance. Dysen-
protein 10 mg/dl, no leukocytes and one tery results from colonic invasion and
erythrocyte). injury, and the diarrhea becomes bloody
Treatment with ceftriaxone, acyclovir and mucoid [1]. Complications of shi-
and dexametasone was initiated until gellosis include intestinal as well as
negative results were achieved for cere- systemic symptoms [2]. Definitive diag-
brospinal fluid culture, blood culture, nosis is established by isolating the
and herpes antigen. Other serological organisms from stool specimens or rectal
tests for cytomegalovirus, enterovirus, swab [3].
influenza A and B, , Neurological findings are among the
, and Epstein-Barr virus
Mycoplasma Barto-
were negative. One day after admission
nella henselae
most common extra-intestinal manifes-
she had an episode of fever accompanied tations of shigellosis and can occur in as
by mucus diarrhea, and stool cultures many as 45% of hospitalized patients [3].
were taken. On the following day her These may accompany or even precede
mental status was vastly improved: she the development of gastrointestinal signs
was alert, the neurological examination and the condition may be mistaken for a
was normal, the fever had resolved and primary neurological illness [4]. Head-
there were no other symptoms. ache, nuchal rigidity, confusion, memory
was cultured from her stool. loss, lethargy, hallucinations, or delirium
Shigella
Having ruled out any other cause that
sonnei
may manifest as acute encephalopathy in
could explain her condition, we reached shigellosis. The duration of acute ence-
a diagnosis of acute encephalopathy due phalopathy due to shigellosis can last
to infection. from 12 hours to 12 days, and complete
Shigella sonnei
recovery with no residual neurological
Comment deficit is achieved in almost all patients
[5]. Avital et al. [6] reported that
Acute encephalopathy is a life-threaten- neurological manifestations preceded
ing condition that necessitates a careful the gastrointestinal symptoms in one-
evaluation to determine its cause. We fourth of hospitalized patients with
report a child with confirmed Shigella gastroenteritis but in most cases
Shigella
IMAJ . Vol 3 . May 2001
 Case Communications

was accompanied with high fever. En-
cephalopathy as the only neurological
complication was reported in 22% of
those patients [6]. It is important to
emphasize that the patient we describe
minant neurological symptoms dis- 2. Bennish ML. Potentially lethal complications
guised the condition as being a of shigellosis.
4):S319±24.
Rev Infect Di s 1991;13(Suppl
primary central nervous system disease.
A neurological symptom preceding the 3. Ozuah PO. Shigella update.
development of gastrointestinal signs in 1998;19:100.
Pediatr Rev

here had no fever during her encephalo- shigellosis is rare, especially when the 4. Ferrera PC, Jeanjaquet MS, Mayer DM.
pathic state. ensuing gastrointestinal signs are mild Shigella-induced encephalopathy in an adult.
1996;14:173±5.
The pathogenesis of the encephalo- and even episodic and no fever is Am J Emerg Med

pathy in shigellosis remains unknown. It
may be attributed to shiga toxin
(although some species usually
do not produce the toxin), or to other
Shigella
documented. We believe that all the
symptoms of this patient were pro-
duced by the same etiological agent,
, and by its toxins. We
5. Kavaliotis J, Karyda S, Konstantoula T,
Kansouzidou A, Tsagaropoulou H. Shigellosis
of childhood in northern Greece: epidemiolo-
gical, clinical and laboratory data of hospita-

lized patients during the period 1971-96.

toxins yet to be classified. Fever or recommend that stool culture be con-
Shigella sonnei
2000;32:207±11.
Scand

metabolic abnormalities can explain the sidered in every patient with an other-
J Infect Dis

6. Avital A, Maayan C, Goitein KJ. Incidence of

encephalopathy in some cases. wise unexplained encephalopathic state, convulsions and encephalopathy in childhood
A particularly lethal toxic encephalo- despite the absence of intestinal symp- shigella infections. 1982;11:645±8.
pathy of shigellosis known as Ekiri (in toms or fever.
Clin Pediatr

7. Goren A, Freier S, Passswell JH. Lethal toxic

Japanese ``epidemic dysentery'') is re- encephalopathy due to childhood shigellosis
portedly no longer a public health We thank Esther Eshkol for in a developed country. 1992;
problem [2]. However, Goren et al. [7] 89:1189±93.
Pediatrics

editorial assistance.
Acknowledgment.

reported this as the cause of death in all

patients with shigellosis consistent with Dr. S. Spirer, Chairman,
toxic encephalopathy. References Dana children's Hospital, Tel Aviv Sourasky
Correspondence:

The noteworthy aspect of our report 1. Plotz FB, Arets HGM, Fleer A, Gemke RJBJ. Medical Center, 6 Weizmann St., Tel Aviv
is the clinical presentation of a patient Lethal encephalopathy complicating childhood 64239, Israel. Phone: (972-3) 697-4721, Fax:
suffering from shigellosis whose predo- shigellosis 1999;158:550±2.
. Eur J Pediatr (972-3) 697-533, email: spirer@post.tau.ac.il

Capsule
Bacterial antibiotic factories

Drugdiscovery has largely been a process of screening natural
products from microorganisms and plants, identifying a
promising lead, and then tinkering with the chemical
structure in the laboratory to improve pharmacokinetics
and diminish side effects. Recently, there have been efforts to
shift the tinkering stage back into the natural producers in
order to co-opt the unparalleled skill with which enzymes
achieve stereospecificity. Pfeifer et al. have taken an
alternative approach by transferring the polyketide biosyn-
thetic genes from the producer of erythromycin, Sacchar-
opolyspora erythraea, to the bacterial workhorse, Escherichia
coli. This strain of E. coli yields the core of erythromycin in
quantities comparable to that of the parent.
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Capsule
Bitter-taste receptors

A large family of bitter-taste receptors has recently been among different bitter stimuli. These results are in agreement
characterized and cloned. The expression of multiple mes- with earlier psychophysical data and the results from taste-
senger RNAs of members of this family in individual cells nerve recording, and indicate that there is much heterogeneity
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taste and that single gustatory cells respond broadly to bitter
compounds. However, Caicedo and Roper show that, in the
majority of cases, individual taste cells can discriminate Science 2001;291:1557
IMAJ . Vol 3 . May 2001 Acute Encephalopathy Preceding Shigella Infection 385