AN INTERESTING CASE OF CVA

DR.AMUDHAN M3 UNIT

A 35Y/FEMALE WAS BROUGHT TO THE HOSPITAL WITH

H/O DIMINISHED CONSCIOUSNESS-

1 DAY LOSS OF SPEECH

MORNING SHE WAS NOTICED TO HAVE DIMINISHED CONSIOUSNESS & LOSS OF SPEECH.

NOT ASSOC.WITH LOC,HEADACHE OR VOMITING

NOT ASSOC WITH BLURRING OF VISION

NOT ASSOC.WITH CHEST PAIN ASSOC.WITH SPEECH DIFFICULTY

H/O PRESENT ILLNESSH/O PRESENT ILLNESSH/O WEAKNESS IN USING RT UL AND LLH/O DEV.OF ANGLE OF MOUTH TO LEFT

SIDENO H/O BLURRING OF VISIONNO H/O VERTIGO/TINNITUSNO H/O LOSS OF SENSATION OVER THE

FACENO H/O NASAL REGURGITATIONNO H/O DEV OF TONGUENO H/O BLADDER AND BOWEL

INCONTINENCE

PAST HISTPAST HIST

K/C/O RHD/MS/POST CMC STATUS/AF/PHT 2 ½ YRS ON TREATMENT

K/C/O CONSTRICTIVE PERICARDITIS PERICARDECTOMY DONE 3 YEARS BACK. DETAILS NOT AVAILABLE.

ADMITED 2 MONTHS AGO WITH FEATURES OF FAILURE AND MASSIVE PLEURAL EFFUSION AND INVESTIGATED AND STARTED ON EMPIRICAL ATT.

NO H/O T2DM/SHT/IHDNO H/O SIMILAR ILLNESS IN FAMILY

PERSONAL H/OPERSONAL H/OMIXED DIETNO ANTI SOCIAL HABITSBOWEL & BLADDER HABITS

NORMAL

General ExaminationGeneral Examination

O/E PT. DROWSY, DISORIENTED AFEBRILE ANEMIC, BPPE + NO CL/ CY/J NO NEUROCUTANEOUS MARKER NO PERIPHERAL NERVE THICKENING ORAL ULCERS PRESENT

VITAL SIGNSVITAL SIGNSPULSE 78/MIN,IRREGULAR,NO

VESSEL WALL THICKENING,NO RADIOFEMORAL DELAY

RR-18/MINBP-140/90mm HgTEMP-NORMALPUPIL-3MM ERRLA

CNS EXAMINATIONCNS EXAMINATION

Pt DROWSY APHASIC HMF- COULD NOT BE ASSESED CRANIAL NERVES

RIGHT UMN VII N PALSY. OTHER CN-NORMAL

MOTOR FUNCTIONS RT LT

BULK UL N N LL N N

TONE UL EXT. HYPERTONIA N

LL FLEX. HYPERTONIA NPOWER UL 3 5 LL 4- 5 DTR BICEPS J 3+ 3+ TRICEPS J 3+ 3+ SUP. J 3+ 3+ KNEE 3+ 3+ ANKLE + +PLANTAR B/L EXTENSOR

SENSORY SYSTEM-COULD NOT BE TESTED

CEREBELLUM-COULDN’T BE TESTEDGAIT-HEMIPARETIC GAITCVS - S1S2 +,S1 VARIABLE,MDM +

APEX WITHOUT PRESYSTOLIC

ACCENTUATIONRS - NVBS+ BS DIMINISH IN LEFT

INFRA AXILLARY & INFRASCAPULARP/A DISTENDED. FF+ NO ORGANOMEGALY

PROVISIONAL DIAGNOSISPROVISIONAL DIAGNOSIS

RHD/ MS/ POST CMC/ AF/ CVA / RIGHT HEMIPARESIS/LEFT PLEURAL EFFUSION /ASCITES FOR EVALUATION

? EMBOLIC STROKE R/O CTD

INVESTIGATIONSINVESTIGATIONS CBC – HB 8 SR. ELECTROLYTES TC 6700 Na-124 DC N48L50E2 K-3.7 ESR 6/15 CL-98 PCV 25 HCO3-23 MCV 98 URINE MCH 28 ALB-++ MCHC 30 SUG-NIL RBC 3LAC DEP-1-3PUS CELS PLATLETS 1.5 24 hrs urine protein-608

mg/day RFT- SUGAR 96 URINE PCR-1.8 UREA 26 P. SMEAR-

normocytic , normochromic

CREATININE 0.7

CT BRAINCT BRAIN HYPODENSE LESION IN B/L PARIETAL LEFT TEMPORAL

LEFT CAUDATE LEFT CORONA

RADIATA RIGHT OCCIPITAL

FEATURES SUGGESTIVE OF ‘’MULTI INFARCT STATE’’

CT CHESTCT CHESTLEFT PLEURAL EFFUSION WITH

MULTIPLE LOCULATION WITH UNDRELYING LUNG COLLAPSE.

LFT TOTAL BILIRUBIN-1mg/dl IDB-0.6 DB-0.4 SERUM ALBUMIN-3.6mg/dl SERUM ALP-WNL SERUM ALT.AST-WNL

ECHOCARDIOGRAMECHOCARDIOGRAMRHD (Post CMC)MVA 1.7Cm2MS- Mod.MR- MildTR- MildPHT-MildAR – TrivialNo LA ClotNormal LV SYS. FunctionNO PERIC.EFFUSION

Ascitic fluid Analysis Ascitic fluid Analysis C/S-No GrowthGM Stain-No Org.TC- 100 Cells/m3

Lymp.-40%N-30%Reactive mesothel.-30%Sugar-76Protein-3

AFB-Negative

Pleural Fluid AnalysisPleural Fluid AnalysisTC-30 CELLLYMP.-90%REACTIVE MESOTHEL.-10%CYTOLOGY

SHEETS OF LYMPHOCYTES & REACTIVE MESOTHEL.CELLS IN PROTEINACEOUS BACKGROUND

S/O REACTIVE EFFUSION.

OTHER INVESTIGATIONS OTHER INVESTIGATIONS

RA - NEGATIVE CRP -12U VDRL -NEGATIVE ANA - POSITIVE 1 : 100+VE RIM PATTERN

ANTI DS DNA - POSITIVE.

ORAL ULCERPOLY SEROSITISPROTEINURIA in a women of child bearing

age with STROKE IMMUNOLOGICAL

EVIDENCE

FINAL DIAGNOSISFINAL DIAGNOSIS

RHD/ MS/ POST CMC STATUS/ AF/ CVD / RIGHT HEMIPARESIS/ SYSTEMIC LUPUS ERYTHREMATOSUS

DEFINITIONDEFINITIONSLE is an autoimmune disease in

which organs and cells undergo damage mediated by tissue binding autoantibodies and immune complexes.

99% are women of child bearing years.

EPIDEMIOLOGY

Prevalence influenced by age, gender, race, and genetics◦Prevalence: 1:2000◦Peak incidence 14-45 years◦Black > White (1:250 vs. 1:1000)◦Female predominance 10:1◦HLA DR3 association, Family History

Severity is equal in male and female

Etiology

Genetic (HLA DR3 association)◦Abnormal immune response

Environmental◦UV◦Viruses◦Hormones (Estrogen)

PATHOGENESISPATHOGENESIS Gene-environment interaction

Abnormal immune response

Induces pathogenic autoantibodies and immune complexes.

Activates complement causing inflammation

Irreversible organ damage.

GENE ENVIRONMENT GENE ENVIRONMENT INTERACTIONINTERACTION GENES … C1q,c2,c4 HLA-D2,3,8 MBL FcR 2A,3A,2B MCP-1 . ENVIRONMENT FACTORS UV LIGHT,gender ?infection ?EBV

ABNORMAL IMMUNE ABNORMAL IMMUNE RESPONSERESPONSE1) Activation of innate immunity

by DNA/RNA2)Lowered threshold of adaptive

immunity cells.3) ineffective regulatory and

inhibitory CD4+ and CD8+TCELLS.

4)reduced clearence of apoptotic cells.

INFLAMMATIONINFLAMMATIONImmune activation of cells

Increased proinflammatory factors like TNFalpha,IFN,IL10

Sustained production of pathogenic autoantibodies and immune complexes.

Activation of compliment and phagocytic cells leading to irreversible tissue damage.

Overactive B-cellsOveractive B-cellsEstrogen is a stimulator of B-cell

activity◦ Lupus is much more prevalent in females

of ages 15-45 Height of Estrogen production

IL-10, also a B-cell stimulator is in high concentration in lupus patient serum. ◦ High concentration linked to cell damage

caused by inflammation

AUTOANTIBODIES IN SLEAUTOANTIBODIES IN SLE

ANTIBODY CLINICAL IMPORTANCE

1)ANTINUCLEAR(ANA)

2)ANTI-DsDNA

3)ANTI_SM

BEST SCREENING TEST(98%PREVALENCE)

SLE SPECIFIC, CORRELATES WITH DISEASE ACTIVITY, NEPHRITIS,VASCULITIS

SPECIFIC FOR SLE

ANTIBODIES CLINICAL IMPORTANCE

4)ANTI-RNP

5)ANTI-RO(SS-A)

NOT SPECIFIC

ASSOC.WITH SICCA SYNDR,SUBACUTE CUTANEOUS LUPUS,NEONATAL LUPUS WITH CONG.HEART BLOCK,DECREASED RISK OF NEPHRITIS

ANTIBODIES CLINICAL IMPORTANCE

6)ANTI-La(SS-B)

7)ANTI HISTONE

8)ANTIPHOSPHOLIPID

ASSOC.WITH ANTI-RO,DECREASED RISK OF NEPHRITIS

IN DRUG INDUCED LUPUS

PREDISPOSE TO THROMBOCYTOPENIA, FETAL LOSS

ANTIBODIES CLINICAL IMPORTANCE

9)ANTI ERYTHROCYTE

10)ANTIPLATELET

11)ANTI NEURONAL

12)ANTIRIBOSOMAL P

MEASURED AS DIRECT COOMBS TEST

ASSOC.WITH THROMBOCYTOPENIA

ACTIVE CNS LUPUS

DEPRESSION OR PSYCHOSIS

DIAGNOSTIC CRITERIADIAGNOSTIC CRITERIA

MALAR RASH-ERYTHEMA OVER MALAR EMINENCE DISCOID RASH-ERYTHEMATOUS RAISED PATCH

WITH FOLLICULAR PLUGGING PHOTOSENSITIVITY ORAL ULCERS ARTHRITIS-NONEROSIVE ARTHRITIS SEROSITIS-PLEURITIS , PERICARDITIS RENAL DISORDER-PROTEINURIA>0.5G/DAY OR

CAST HEMATOLOGICAL DISORDER-HEMOLYTIC ANEMIA

OR LEUCOPENIA OR THROMBOCYTOPENIA IMMUNOLOGICAL DISORDER-ANTIBODIES ANA NEUROLOGICAL- PSYCOSIS, SEIZURES

CLASSIFICATION CRITERIA

Must have 4 of 11 for Classification◦Sensitivity 75%Sensitivity 75%◦Specificity 95%Specificity 95%

Like RA, diagnosis is ultimately clinical

Not all “Lupus” is SLE◦Discoid Lupus◦Overlap syndrome◦Drug induced lupus◦Subacute Cutaneous Lupus

Behavior/Personality changes, depressionCognitive dysfunctionPsychosisSeizuresStrokeChoreaPseudotumor cerebriTransverse myelitisPeripheral neuropathyTotal of 19 manifestations describedTotal of 19 manifestations described

May be difficult to distinguish from steroid psychosis or primary psychiatric disease

CLINICAL FEATURES: Neurologic

CLINICAL FEATURES: Gastrointestinal & Hepatic

◦Uncommon SLE manifestations◦ mesenteric vasculitis, resembling

medium vessel vasculitis (PAN)◦Diverticulitis may be masked by steroids◦Hepatic abnormalities more often

IATROGENIC than to SLE itself

Treatment.

Mild cases : NSAID, local treatment, hydroxy-chloroquin

Cases of intermediate severity : corticosteroid (12-64 mg methylprednisolon), azathioprin, methotrexat

SLE – treatment

Severe, life-threatening organ involvements : High dose IV corticosteroid +

iv.cyclophosphamide .Plasmapheresis or iv. Immunoglobulin.

Some cases of nephritis (especially membranous), myositis, thrombocytopenia: cyclosporine

WOMEN OF CHILDBEARING AGEMULTISYSTEM INVOLVEMENTNO RELATED CAUSEEVEN WITH SUBTLE

MANIFESTATION EVALUATE C T D RULE OUT S L E

THANK U