Dr.Azad A Haleem AL.MezoriDCH, FIBMS

Lecturer University Of Duhok

Faculty of Medical ScienceSchool Of Medicine-Pediatrics Department

2015azad82d@gmail.com

Reversible posterior leukoencephalopathy

syndrome

Case Summary Thirteen years old boy ……… presented to district ?Accident and Emergency department

with headache, vomiting low grade fever and sweating for three days

they diagnosed him as a case of meningitis and treated by empirical therapy; Antibiotics.

• After two days of admission the patient developed GTC seizures lasting > 20 minutes with tongue biting and incontinence and then impaired consciousness.

• He received Diazepam 5 mg IV twice as he was still convulsing. • His RBS was 100 mg/dl.• After his seizures stopped, he was transferred to Heevi

pediatric teaching hospital in Duhok/Kurdistan,

• Heevi pediatric teaching hospital complete history taken and general and systemic examination done for the patient which revealed ;

• unconscious, confusion and disoriented ,afebrile with sweating,

• Blood pressure was 210/110 mmgh?• chest; harsh vesicular breathing with good air entry. • Heart: Audible S1 & S2with systolic murmur in the

apex. • Abdomen: liver and spleen just palpable. • CNS: unconscious with no focal neurological deficit.

Case Summary

• CBC, ABGs, RFTs, LFTs and Brain CTScan: within normal limits except slightly high creatinine,

• blood sugar was still 120 mg/dl. • He had two short convulsions in next 2 hrs. – • he was loaded with Phenytoin and

Antihypertensive IV with monitoring of Blood pressure,

• after 24 hours the patient regain consciousness with controlled blood pressure and continue with antihypertensive Amlodipine tab.

• MRI of brain ?? Done for the patient ?Case Summary

First MRI

Follow up MRI

Differential Diagnosis?

Meningitis/Encephalitis?Stroke/CVTHypertensive encephalopathy?Epilepsy?Drugs Adverse effectsSomething Else?

6

First MRI

Follow up MRI

First MRI

Follow up MRI

BRAIN MRI on 27th of October 2014: Multiple hyperintense FLAIR/T2WI and hypointense T1WI lesions are seen involving the occipital, parietal, temporal and frontal lobes. The lesions are mainly subcortical in distribution. Few areas of cortical involvement are seen in occipital lobes. No enhancement is seen within the lesions. The radiological picture returned completely to normal on a follow up scan acquired at 5th of February 2015.

• On further enquiry…after three days of hospital admission investigations done for the patient to know the cause of hypertension focusing on renal, Suprarenal, Endocrine and cardiac causes all where normal.

• After one week of admission the patient become totally normal on Amlodepine and discharged home .

• A follow up MRI of the brain was totally normal. • Now the patient is totally normal with controlled

blood pressure by Amlodepine tab and on follow up.

Case Summary

First MRI

Follow up MRI

First MRI

Follow up MRI

BRAIN MRI on 27th of October 2014: Multiple hyperintense FLAIR/T2WI and hypointense T1WI lesions are seen involving the occipital, parietal, temporal and frontal lobes. The lesions are mainly subcortical in distribution. Few areas of cortical involvement are seen in occipital lobes. No enhancement is seen within the lesions. The radiological picture returned completely to normal on a follow up scan acquired at 5th of February 2015.

First MRI

Follow up MRI

REVERSIBLE POSTERIOR LEUKOENCEPHALOPATHY SYNDROME

?Diagnosis…..

Reversible posterior leukoencephalopathy syndrome

• (RPLS) is a clinical radiographic syndrome of heterogeneous etiologies that are grouped together because of similar findings on neuroimaging studies.

• It is also often referred to as:• Posterior reversible encephalopathy syndrome (PRES) • Reversible posterior cerebral edema syndrome.• Posterior leukoencephalopathy syndrome.• Hyperperfusion encephalopathy, • Brain capillary leak syndrome

Clinically

• Reversible posterior leukoencephalopathy syndrome is characterized clinically by:

• headache and vomiting, • abnormalities of mental status & visual

perception, • seizures, • characteristic radiologic findings, • PRES is typically reversible once the cause is

removed

Pathophysiology• The underlying pathophysiology is still not well

understood. • The two main hypotheses contradict each other. • One involves impaired cerebral autoregulation

responsible for an increase in cerebral blood flow (CBF), • whereas the other involves endothelial dysfunction with

cerebral hypoperfusion. • Under both hypotheses, the result of the cerebral blood

perfusion abnormalities is bloodbrain barrier dysfunction with cerebral vasogenic edema .

• PRES related to hypertension might be due to:• sudden elevation of blood pressure causing

disruption of the autoregulatory mechanisms of the central nervous system vasculature, leading to development of areas of vasoconstriction and vasodilatation, breakdown of the blood-brain barrier, and focal transudation of fluid and petechial hemorrhages.

Pathophysiology

• The preferential involvement of the parietal and occipital lobes is hypothesized to be related to the less dense sympathetic nervous system innervation of the posterior cerebral circulation.

• In a recent study, proton magnetic resonance spectroscopy of two patients with reversible posterior leukoencephalopathy syndrome showed diffuse metabolic abnormalities (increases in both choline and creatine and reduced N-acetylaspartate) in regions of the brain both with and without abnormal MRI appearance.( These mechanism may have a role?)

Pathophysiology

Associations• Hypertension• Toxic Agents• Infection/Sepsis/Septic Shock• Autoimmune Disease• Hemolytic-uremic syndrome• Malignancies• Vasculitis • others like; sickle cell disease , Guillain-Barr´e syndrome ,

hypomagnesemia , hypercalcemia , tumor lysis syndrome, porphyria , pheochromocytoma and Cushing syndrome.

Neuroimaging• The lesions of posterior leukoencephalopathy are best

visualised with magnetic resonance (MR) imaging. • T2 weighted MR images, at the height of symptoms,

characteristically show:• diffuse hyperintensity selectively involving the

parieto-occipital white matter. • Occasionally the lesions also involve the grey matter. • Computed tomography can also be used satisfactorily

to detect hypodense lesions of posterior leukoencephalopathy.

Management

• Identification and early diagnosis, • Stopping early the offending

agents/medications,• Management of the root cause, • Management of the co-morbidities, Seizure

and BP control.

Prognosis

• The MRI scan is useful in Follow up and prognosis.

• If the condition is caught on time it is reversible

• but, if infarction has occurred, there will be irreversible damage.

• Delay in diagnosis gives a worse prognosis.• Recurrence can occur but is unusual.

Thank You