posterior reversible leucoencephalopathy
TRANSCRIPT
DR.ANITHA PICU SRMC & RI
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Master K, 12 yr old boy, K/C/O SLE with LUPUS NEPHRITIS since 7 yrs of age.
Admitted in Children’s Hospital with C/o facial puffiness for one month Fever x 10 days Decreased urine output x 10 days On and off headache x 10 days
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Diagnosed as SLE at 7 yrs of age when he had fever 2 weeks, skin rashes and oral ulcers. ANA, dsDNA was positive;
Renal biopsy showed grade IV lupus nephritis.
At varying periods, he has been treated with IV cyclophosphamide, methyl prednisolone, oral steroids, azathioprine, Mycophenolate mofetil. But he did not need Dialysis support.
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He was treated with Broad spectrum Antibiotics; All cultures were negative;
Developed Thrombocytopenia, anaemia with smear showing e/o microangiopathic hemolytic anaemia. Rx with Prednisolone
Developed Hypertension and worsening renal function; Rx with Nicardia, Aldomet.
Child referred to SRMC for Plasmapheresis/ Hemodialysis.
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ANA POSITIVE
dsDNA POSITIVE
C3 33.3
CRP 6mg/dl
Anticardiolipin ab Negative
Troponin Negative
LDH-707 RAISED
ASO Negative
RA Negative
ECG Normal
ECHO Signs of PERICARDIAL EFFUSION
USG Abdomen FREE FLUID +
Renal biopsy LUPUS NEPHRITIS
Bone marrow Not signifigant5
General examination : Afebrile, Anasarca+, Pallor+, no cyanosis, no clubbing, no genaralised lymphadenopathy; No petechiae/ bleeding.
Anthropometry: Weight: 29kg, height: 150 cm
Vitals : HR: 110/min, RR:24/min, BP:130/90 mm hg, Systemic Examination:
CVS/ RS: NADP/A: Soft, Fluid thrill +, Liver 4cm
CNS: Conscious, Alert No focal neurological deficit
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Child continued on oral steroids, eltroxin.I.V antibiotics startedIn view of Hypertension, continued on oral
nifidipine, aldomet, later I.V. lasix, T. Prazocin, Clonidine were added.
Rhematologist Review Prednisolone with dailyMycopenolate Mofetil
with 5 cycles of Plasmapheresis with IV
Immunoglobulin
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Hb 11.2
TC 12450
DC P60L40
PLTS O.80
RETIC 3.2
RFT BUN CREAT
153.2
ESR 43
LIPASE 361
CRP O.2
C3 0.28
ANA POSITIVE
dsDNA POSITIVE
LUPUS ANTI COAGULANT
NEGATIVE
DCT NEGATIVE
LDH 404
FIBRINOGEN 35
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Peripheral smear: microangiopathic haemolytic anaemia
ECHO: EF 52% MILD LVH, Severe PUL HT
C/O recurrent episodes of headache and recurrent episodes of focal seizures involving the right UL and LL; Fully conscious;
In between seziures, no focal neurological deficit;
Was started on I.V Phenytoin, later I.V Phenobarbitone, Sodium valproate
CBG, Serum Sodium, Calcium normal; BP 160/100
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Focal hypo density noted in the left parietal region
In view of raised creatinine, contrast deferred
Suggested diffusion weighted MRI for further evaluation
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Areas of hypo density seen in b/l high parietal and left temporal regions
F/s posterior reversible leucoencephalopathy
MRI venogram-normal
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Seizures settled with Phenytoin, Valparin, and Phenobarbitone;
Started on regular Haemodialysis as renal failure was worsening;
He underwent 7 sessions of plasmapheresis followed by IV Immunoglobulin; following which his microangiopathy improved
Given 2 doses of Inj. Rituximab as lupus was active
Later discharged on oral anticonvulsants and hemodialysis
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Exchange cycles- 5 to 7 cycles Blood Flow - 150ml/hrExchange fluid
900ml of NS4 units of FFPHeparin 3000 units I.V bolus, then
500 units/hr I.V infusion
Post Plasma Exchange - 0.5 gm/kg of I.V immunoglobulin infusion
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Posterior Reversible Encephalopathy Syndrome
Reversible Posterior Cerebral Edema Syndrome
Hyperperfusion Encephalopathy Brain Capillary Leak Syndrome
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Definition - Acute episode of vasogenic edema in the cerebral white matter.
History - First described in 1996 in pregnant women with eclampsia
Incidence – More common in adults than children
Predilection site – Postero-temporal, parietal, occipital, reason unknown
If unrecognised, conversion to irreversible cytotoxic edema may occur
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1. Eclampsia, Hypertension2. Post transplantation3. Immunosupression - Cyclosporin,
Tacrolimus4. Infection, Shock5. Systemic Inflammatory Response
Syndrome6. Autoimmune- SLE, Systemic Sclerosis,
Wegener’s, Polyarteritis nodosa7. Post cancer chemotherapy- Cytarabine,
Cysplatin, Bevacizumab, Interferon alpha8. Antiretroviral therapy
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Hypercalcemia, Hypomagnesemia, Hypocholesterolemia
GBS, HUS I.V Immunoglobulin, Erythropoietin Epherdra overdose, Triple H therapy Tumour Lysis Syndrome Use of stimulant drugs- Caffeine, Cocaine,
Amphetamines Pheochromocytoma
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2 THEORIES OF AETIOLOGY FIRST THEORY
SEVERE HYPERTENSION
FAILED AUTOREGULATION
HYPERPERFUSION
ENDOTHELIAL INJURY
VASOGENIC ODEMA 22
SECOND THEORY
VASOCONSTRICTION AND HYPOPERFUSION
BRAIN ISCHAEMIA
VASOGENIC ODEMA
The latter is more likely23
Moderate to severe hypertension Headache, nausea Altered mental state, lethargy, paresis,
progressing to confusion and coma Convulsion- partial status epilepticus Blurred vision, hemianopia, visual neglect,
cortical blindness, papillodema, haemorrhages, exudates.
Hallucinations
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Vasogenic odema Activated, reactive astrocytes,
scattered macrophages, lymphocytes without inflammation
Ischemia, neuronal damage, laminar necrosis
Demyelination
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CT/MRI IMAGING – -Focal regions of non confluent
hypodensity-Sites: parietal, occipital most
commonly involved, followed by frontal, inferio-temporal, occipital junction, cerebellum
-Lesions confluence, may resemble brain water shed zone
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MRI DIFFUSION WEIGHTED IMAGING (DWI)
- Instrumental in demonstrating that areas of abnormality represents vadogenic odema
CATHETER ANGIOGRAPHY
-String of beads appearance
PROTON MR SPECTROSCOPY-Decreased n-acetyl aspartate:choline-Decreased n-acetyl;creatinine
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1. STROKE2. SINUS THROMBOSIS3. DEMYELINATION4. VASCULITIS5. ENCEPHALITIS
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Reversible Controlling BP Eliminating the use of drugs which are
implicated in causing pres To treat sepsis If odema extensive: hydrocephalus and
brain stem compression may occur
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If it is caught in time, it is completely reversible, but if infarction has occurred then it will become irreversible
Delay in diagnosis gives a worse prognosis
MRI scan is useful Recurrence can occur, but it is unusual
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PRES is more often a radiological diagnosis rather than a clinical diagnosis
The therapeutic implications for separating PRES from stroke or cerebritis are important. If diagnosed in time , it’s completely reversible
We propose that PRES should be considered in the differential diagnosis in SLE patients with new-onset neurologic signs and symptoms
Hence early diagnosis is the key !!!
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In a study conducted in france, 2008 on 4 cases of SLE I, ALL cases, had renal involvement and hypertension . Neurological symptoms were typical. Magnetic resonance imaging showed posterior cerebral edema and in one case hemorrhagic complication. With symptomatic treatment and immunosuppressor withdrawal when they were previously used, symptoms fully resolved within 15 days in all cases
In a study aimed Los angels at clinical and imaging characteristics; associated risk factors and neurological outcome in sle patients ,identified 22 episodes of PRES in 21 patients; 20 (95.2%) . Acute hypertension was observed in 18 episodes (81.8%), and renal failure in 16 (72.7%); only 3 patients were on cyclophosphamide at the time of the onset of PRES. Persistent neurological deficit was observed in 2 cases; one patient died during the acute episode.
study in USA on PRES as CNSmanifestation of SLE. yielded 26 SLE cases reported with PRES. The study proposed that PRES should be considered in the differential diagnosis in SLE patients with new-onset neurologic signs and symptoms.
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ReviewPosterior reversible encephalopathy syndrome during systemic lupus erythematosus: four new cases and review of the literature. Lupus. 2008; 17(2):139-47
Reversible posterior leukoencephalopathy syndrome: a retrospective study in King Chulalongkorn Memorial Hospital.J Med Assoc Thai. 2008 Mar; 91(3):427-32.
W.S.Bartynski, AJNREvaluation of nine children with reversible posterior encephalopathy syndrome.29,JUN –JULY 2008
Posterior reversible encephalopathy syndrome during systemic lupus erythematosus: four new cases and review of the literature.Leroux G,2006,33;2178-83
Posterior reversible encephalopathy syndrome as a complication of acute lupus activity.Baizabal-Carvallo JF,2006.OCT ,38;338-41
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