Status epilepticus as manifestation of posterior reversible encephalopathy syndrome in a healthy child – anoxia vs hypoxia

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Pediatric neurology 48 (2013) 418e420

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Pediatric neurology

Journal homepage: www.Elsevier .Com/locate/pnu

Case report

Status epilepticus as manifestation of posterior reversible encephalopathy

Syndrome in a healthy child

Chiara mameli MD *, dario dilillo MD, daniele spiri MD, chiara cerini MD, silvia fasan MD,

Gian vincenzo zuccotti MD

Department of paediatrics, L. Sacco hospital, university of milan, milan, italy

Article information

Article history:

Received 4 october 2012

Accepted 31 december 2012




* communications should be addressed to: D

Pediatrics; L. Sacco hospital; university of M

20157 milano, italy.

E-mail address: mameli.Chiara@hsacco.It

0887-8994/$ – see front matter � 2013 elsevier inc.Anoxia vs hypoxia A



Osterior reversible encephalopathy syndrome is a recently described cliniconeuroradio-

Gical syndrome reported in children with several predisposing conditions such as trans-

Plantation, autoimmune, hematological, infectious, renal, and neoplastic diseases or

Administration of chemotherapeutic immunosuppressive drugs. Seizures are one of the most

Frequent manifestations of posterior reversible encephalopathy syndrome; status epilepticus

Has been described more frequently in adults but rarely in children. We report on the case of

A 6-year-old healthy boy who presented status epilepticus as the main manifestation of

Posterior reversible encephalopathy syndrome in the absence of other underlying condi-

anoxia vs hypoxia

Tions. This is the first report of posterior reversible encephalopathy syndrome in a previously

Healthy child. Our case reminds us that pathogenesis of this condition is far from being

Completely understood and may include both genetic and environmental factors. Moreover,

Posterior reversible encephalopathy syndrome should always be suspected by clinicians in

Cases of status epilepticus with a prolonged neurological failure.

� 2013 elsevier inc. All rights reserved.


In 1996 hinchey et al. [1] were the first to describe the

Reversible posterior leukoencephalopathy syndrome that

Was later renamed posterior reversible encephalopathy

Syndrome (PRES) because of the involvement of both the

White and gray matter.Anoxia vs hypoxia

Currently, PRES is defined as a cliniconeuroradio-

Logical entity that is characterized clinically by headache,

Altered consciousness, visual disturbances, and seizures,

And radiologically by transient posterior (parieto-

Occipital) lesions predominantly in the subcortical white

Matter [1]. PRES is not unusual in children affected

By different diseases (e.G., autoimmune, hematological,

Renal, infectious, neoplastic diseases, or after drug

Administration), but the exact pathophysiology remains

Unclear [2].

R. Mameli; department of

Ilan; via G.B. Grassi 74;

Ll rights reserved.

Status epilepticus (SE) is a potential life-threatening

Event and can lead to neurological sequelae as secondary

Epilepsy, cognitive deterioration, behavioral problems, and

anoxia vs hypoxia

Focal neurological deficits [3]. Seizures are frequent mani-

Festations of PRES; however, SE has been described more

Frequently in adults than in children with PRES [2,3].

In this case report, we describe clinical and radiologic

Findings in a child who developed SE as a manifestation of

PRES, with no other underlying condition.

Case report

Our patient was a 6-year-old italian boy born to healthy parents for

Which familiar anamnesis was free of any known neurological or genetic

Diseases. The pregnancy, delivery, and neonatal period were uneventful.

Psychomotor development and growth were regular. Remote patholog-

Ical anamnesis was negative.

The child was admitted to our pediatric department because of the

anoxia vs hypoxia

Onset of a left-sideweakness followedby a status epilepticus characterized

By loss of consciousness, left-side tonic-clonic seizures with secondary

Generalization, gaze deviation to the right, nystagmus, oculoclonic move-

Ments, palpebral myoclonus, and oral automatisms in absence of fever.

Abdominal pain was reported during the preceding 3 days and an acute

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anoxia vs hypoxia



C. Mameli et al. / pediatric neurology 48 (2013) 418e420 419

Gastroenteritis occurred during theweek prior to the admission. Therewas

No history of blurring of vision, drug intake, and head injury. On admission

Hewas afebrilewith a heart rate of 126beats perminute, respiratory rate of

30breaths perminute, bloodpressure of 139-112mmhg in the right upper

Arm (95th percentile for age and sex). Physical examination was normal

And no meningeal signs were present. Intravenous diazepamwas initially

Administered unsuccessfully; status epilepticus was stopped by intrave-

Nous infusionofphenobarbital, subsequentlyadministeredorally.Anoxia vs hypoxia after the

Resolution of seizures, hypertension resolved spontaneously; neurological

Examination revealed hypotonia of the left side and absence of focal

Neurological deficits. Osteotendinous reflexes were depressed on the left

Side, while a normal flexion plantar reflex was present bilaterally. Blood

Analyses, including complete blood count, inflammatorymarkers, glucose,

Serum electrolytes, and calcium, and liver and kidney function were

Normal. A lumbar puncture was also performed, and cerebrospinal fluid

Analysis showed normal cell count, glucose, and protein levels; cerebro-

Spinal fluid culture and polymerase chain reaction for enterovirus were

Negative. Blood culture, fecal culture, andparasitological stool examination

anoxia vs hypoxia

Were negative. Magnetic resonance imaging with fluid-attenuated inver-

Sion recovery sequence was performed and showed bilateral asymmetric

Regions of low hyperintensity in thewhite and graymatter of the posterior

Parietal and occipital lobes, affecting the right cerebral hemisphere more

Than the left side (fig). The electroencephalogram exhibited an encepha-

Lopathic pattern of posterior lobeswith no specific epileptic abnormalities.

Electrocardiogram, echocardiogram, and 24-hour blood pressure moni-

Toring were normal.

The patient completely recovered within the following 6 days, and at

Discharge therewere no residual neurological deficits. The phenobarbital

Dose was gradually decreased and the medication was stopped 2 weeks

anoxia vs hypoxia

After the discharge.

A neurologic, radiological, and electroencephalographic follow-up

Evaluation was performed 3 months later: the neurological examina-

Tion was normal and a complete resolution of neuroradiologic and

Electroencephalogram anomalies was observed. Blood pressure was

Always normal. Additionally, our patient did not develop epilepsy or any

Medical condition during the following 12 months.


PRES is a recently described cliniconeuroradiological

Entity with typical radiological findings in the posterior

Figure. Brain magnetic resonance image; T2-weighted sequence. Asym-

Metric regions of hyperintensity in the white and gray matter of the

Posterior parietal and occipital lobes (right more than left).Anoxia vs hypoxia

Regions of the cerebral hemispheres, certainly well

Defined in adults. There are several predisposing condi-

Tions for developing PRES, such as organ and bone

Marrow transplantation, use of chemotherapeutic immu-

Nosuppressive drugs, sepsis, shock, and with autoimmune

Diseases, such as systemic lupus erythematosus, takayasu

Arteritis, cancer, renal diseases, continuous ambulatory

Peritoneal dialysis, guillain-barré syndrome, and hema-

Tologic diseases, including sickle cell anemia and

Thrombotic thrombocytopenic purpura, hemolytic-uremic

Syndrome, henoch-schönlein purpura, and intravenous

Immunoglobulin administration [4-8]. Recently siebert

Et al. [9] showed that affected children suffered most

Frequently from renal and hemato-oncologic diseases and

anoxia vs hypoxia

All received medications that have been shown to be

Directly associated with PRES.

Pathophysiology of PRES is not completely understood,

And several factors are thought to be involved. A rapid rise

In blood pressure is proposed as the key event to cause

Breakdown in autoregulation of cerebral blood flow, which

Leads to bloodebrain barrier dysfunction and leakage of

Fluid into the brain parenchyma, detected as a vasogenic

Edema in neuroimaging studies [10]. The relative paucity of

Sympathetic innervation in the posterior brain results in an

Increased susceptibility to hyperperfusion and vasogenic

Edema during acute blood pressure elevations [11].

Although it is believed that severe hypertension plays a key

anoxia vs hypoxia

Role in pathogenesis of PRES, this hypothesis does not

Explain over 20% of cases of PRES that occur in the absence

Of hypertension [2].

The occurrence of PRES in our patient with no clinically

Evident disease is certainly an interest finding, and it

Reminds us that more complicated mechanisms underlie

The development of this syndrome. Acute gastroenteritis

Was the only relevant anamnestic piece of data temporarily

Associated with the onset of the disease, nevertheless, it

May be only coincidental. An infectious-associated PRES

Triggered by an unidentified gastrointestinal virus is

A fascinating hypothesis; although there was insufficient

Evidence, this association may be considered. Moreover, the

Occurrence of PRES in a healthy child could raise the

anoxia vs hypoxia

Unexplored hypothesis that host genetic variation plays

A role in determining the susceptibility to the syndrome.

The possibility that yet unidentified factors (genetic and

Environmental) may predispose to the development of

PRES in healthy children represents an intriguing field that

Needs further investigation.

PRES in children occurs with a wide radiological and

Clinical spectrum. The most frequent clinical symptoms

Include headache, visual disturbances, focal neurological

Deficits, altered level of consciousness, and seizures [12]. In

Childrenwith PRES, multiple seizures have been reported to

Be the most frequent presentation (in over 59% of cases); in

Adults, generalized seizures and status epilepticus seem to

anoxia vs hypoxia

Occur even more commonly (87% of cases) [2,12].

Although its underlying pathophysiology is still

Unknown and is likely multifactorial, this discrepancy in

Clinical pattern in patients with PRES may be due to

Differences in autoregulatory capacity of cerebral arterioles

Between adults and children or to greater susceptibility of

The bloodebrain barrier to hypertensive insults in elderly

Patients. However, recently cordelli et al. [3] showed that in

C. Mameli et al. / pediatric neurology 48 (2013) 418e420420

Childrenwith PRES, status epilepticus is more frequent than

Previously reported and is often the main manifestation

After hematopoietic stem cell transplantation.

The imaging studies typically demonstrate symmetric

anoxia vs hypoxia

Vasogenic edema with different characteristic patterns, not

Influenced by the different predisposing risk factors.

Watershed distribution is the most frequently observed

Pattern, representing a distribution between lateral and

Medial cerebral arterial branches. As reported in our

Patient, parietal and occipital edematous lesions were

Encountered in most children affected, even if frontal

Lesions have been recently found to occur as frequently as

Parietal-occipital lesions [9]. The mechanism responsible

For the imaging appearance still remains unclear and

Controversial [14,15]. Clinical outcome is excellent, with

A complete recovery within a few days, while the magnetic

Resonance imaging abnormalities tend to resolve much

anoxia vs hypoxia

More slowly. Although cerebral lesions are generally known

To be fully reversible, persistent lesions such as infarction or

Hemorrhages are well recognized in children [9]. Recur-

Rence is infrequent, although trigger factors for PRES were

Repeatedly experienced by the patients.

Therapy consists of removal of any offending agents,

When present, and treatment of any associated disorders is

Essential to prevent progression to irreversible brain injury

Or death. The effectiveness of antiepileptic medications in

Treatment of seizures related to PRES has been well docu-

Mented; however, how long the treatment should be

Continued is still controversial, especially in children. Some

Reports showed that antiepileptic medications have been

anoxia vs hypoxia

Frequently discontinued after resolution of symptoms

Without any recurrent seizures [13].


This case highlights that the pathogenetic mechanisms

Of PRES are far from being completely understood, and

Geneeenvironment interactions could play a key role in the

Development of disease. Moreover our case reminds us of

The importance to suspect PRES in a status epilepticus

Without any other underlying cause, also in the pediatric

Population. Although the clinical picture of presmay not be

Specific, imaging studies (usually magnetic resonance)

Should be promptly considered in patients with SE, espe-

Cially in those with hypertension, because it leads to the

Correct diagnosis in most cases. Prompt recognition of PRES

anoxia vs hypoxia

Is essential to rationalize diagnostic approach (other

Investigations are not generally helpful except to rule out

Differential diagnoses) and to plan for adequate follow-up.


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anoxia vs hypoxia


Status epilepticus as manifestation of posterior reversible encephalopathy syndrome in a healthy child


Case report