POSTERIOR REVERSIBLE ENCEPHALOPATHY SYNDROME

1. Chemotherapy Induced
PRES(Posterior Reversible
Encephalopathy Syndrome)
Dr.Kavinkumar.R
DNB Resident
Dept of Neurology

2. Past History
• 47 / Female
• K/C/O advanced carcinoma esophagus
• S/P esophageal stenting (SEMS)
• On Chemotherapy (Paclitaxel,5-FU,Cisplatin) - for past 6
months
• Last chemotherapy on 29/6/2019 evening - only Paclitaxel
• No other co-morbidities

3. Presenting complaints
H/O loss of vision since 30/6/2019
Bilateral
Painless
Sudden onset
H/O on&off headache , vomiting ,giddiness for past 6
months - only during chemotherapy

4. Examination
• BP : 120/80 mmHg
• PR : 140 /min
• RR : 18 / min
• SPO2 : 98% on RA
• Poorly built & nourished
• Afebrile
• No pallor/ictreus/cyanosis

5. • CVS - S1,S2 +
• RS - NVBS +
• PA - soft

6. CNS
• Conscious
• Oriented to time/place/person
• B/L Visual acuity & colour vision - not possible
• No counting fingers close to eyes
• Pupils - 3 mm & Reacting to light
• EOM - full
• No signs of other cranial nerve involvement
• Motor & Sensory system - normal
• No cerebellar signs
• Fundus - Normal

7. Investigations
• TC - 2900
• Hb,Platelet, RFT,LFT - Normal
• ECG - sinus tachycardia

8. MRI Brain
• B/L asymmetric bright signals in parieto occipital lobe
• Mild diffusion restriction & gyral edema
• Effacement of ventricular system and sulci
• No evidence of SOL/Hemorrhage/Infarct
• More in favour of PRES

10. Treatment
• Medical oncology opinion
• Inj. Mannitol 300 mg iv TDS
• Inj. G-CSF 300 mcg SC
• Supportive measures
• Patient regained vision in both eyes in 2 days

11. DISCUSSION

12. PRES - also known as
1. Reversible Posterior Leukoencephalopathy Syndrome
(RPLS)
2. Reversible posterior cerebral edema syndrome
3. Posterior leukoencephalopathy syndrome
4. Hyperperfusion encephalopathy
5. Brain capillary leak syndrome

13. • Clinical radiographic syndrome
• Heterogenous etiologies
• 1st case in 1996
• Mc in females , Mean age 39 - 47 years

14. Etiologies/Risk factors
1. Hypertension - 75%
2. Renal Disorders - AKI,CKD, TTP, HUS, Lupus nephritis
3. Immunosuppressive therapy - Cyclosporine, Tacrolimus,
Interferon
4. Cytotoxic drugs - Platinum (cisplatin , carboplatin)based
regimens ,anti VEGF agents -
Bevacizumab,Pazopanib,sorafenib,Sunitinib
5. Autoimmune disorders - SLE,IBD,PAN

15. Paclitaxel in PRES
• Paclitaxel - peripheral neuropathy(sensory predominant)
• PRES - very rare
• Few case reports - available
• Increased incidence - in combination chemotherapy
• In our case - Possible etiology - Paclitaxel

16. Pathogenesis
• Hypertensive patients - disordered cerebral autoregulation
( if BP > 180/120 mmHg)
• Non - Hypertensive patients - Endothelial dysfunction
Capillary leak and BBB disruption
Axonal swelling and vasogenic edema ,
Protein extravasation ,Fibrinoid necrosis

17. • Posterior circulation - more commonly involved
Not well known
Higher adrenergic innervation in anterior circulation
arterioles - protects from hyperperfusion and edema
• White matter > Cortex
Cortex - tightly packed cells - resists edema

18. Clinical Manifestations
• Headache
constant
nonlocalised
moderate to severe
non responsive to analgesics
• Altered consciousness
somnolence - confusion and agitation - stupor - coma

19. • Visual disturbances
hemianopia
visual neglect
auras
visual hallucinations
cortical blindness
• Seizures
Focal with 2ry GTCS
Status epilepticus

20. Fundus
• Normal (Mc)
• Papilledema
• Flame shaped
hemorrhages
• Exudates

21. MRI
• White matter edema ( T2 hyperintensity)
Posterior Cerebral Hemispheres
Cerebellum
Brainstem
• Rarely involves frontal lobe (Superior frontal gyrus) ,
Cortex , Basal ganglia

22. DD- B/L PCA infarct
MRI finding PRES B/L PCA
infarct
Calcarine and
paramedian lobes
Spared Involved
Cortical gray matter Mostly spared Involved
DWI Hypo or iso intense
signal
Hyper intense signal
ADC Hyper intense signal Hypo intense signal

23. Other DD
1. RCVS - Reversible cerebral vasoconstriction syndrome
2. Eclampsia
3. Hypertensive encephalopathy
4. Autoimmune encephalitis
5. CVT
6. ADEM
7. Acute toxic leuco encephalopathy

24. Treatment
• Hypertension management - i.v drugs
• Stop the offending drug
• Anti epileptics for seizures
• Anti edema meaures
• Treat the underlying cause
• Steroids - contraindicated

25. Complications of PRES
• Cerebral ischemia
• Cerebral Hemorrhage
• Relapse (<10%)

26. PRES - A misnomer??
• Not always reversible (15% irreversible neurological
deficit or death)
• Not always confined to white matter
• Not always confined to posterior regions of brain

27. References
• Hinchey J, Chaves C, Appignani B, et al. A reversible
posterior leukoencephalopathy syndrome. N Engl J Med
1996; 334:494.
• Kamiya-Matsuoka C, Paker AM, Chi L, et al. Posterior
reversible encephalopathy syndrome in cancer patients: a
single institution retrospective study. J Neurooncol
2016;128:75.
• Liman TG, Bohner G, Heuschmann PU, et al. The clinical
and radiological spectrum of posterior reversible
encephalopathy syndrome: the retrospective Berlin PRES
study. J Neurol 2012; 259:155.