Reversible posterior leukoencephalopathy syndrome

2. 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,

3. • 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

4. • 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

5. First MRI
Follow up MRI

6. Differential Diagnosis?
Meningitis/Encephalitis?
Stroke/CVT
Hypertensive encephalopathy?
Epilepsy?
Drugs Adverse effects
Something Else?
6

7. FirstMRI
Followup MRI
FirstMRI
Followup 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.

8. • 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

9. FirstMRI
Followup MRI
FirstMRI
Followup 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.

10. First MRI
Follow up MRI

11. Diagnosis…..

12. 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

13. 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

14. 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 .

15. • 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

16. • 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

17. 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.

18. 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.

19. 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.

20. 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.

21. Thank You