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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