A Case of CVA with Polyserositis
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Transcript of A Case of CVA with Polyserositis
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