An Interesting case of An Interesting case of QuadriparesisQuadriparesis

S.Karthikeyan.,S.Karthikeyan.,

M4M4

Prof.P.Vijayaraghavan`s unitProf.P.Vijayaraghavan`s unit

A 37 yr old female, admitted on 25/5/11 with A 37 yr old female, admitted on 25/5/11 with c/o difficulty in using both upper and c/o difficulty in using both upper and

lower limbs – past 5 hrslower limbs – past 5 hrs

On elaborating:On elaborating: Patient was apparently normal a few hrs Patient was apparently normal a few hrs

before admissionbefore admission

- she had 3—4 episodes of vomiting ,non - she had 3—4 episodes of vomiting ,non projectile, non bilious projectile, non bilious

- she went to bed normally and found - she went to bed normally and found herself after an hour to have difficulty in herself after an hour to have difficulty in

using both upper and lower limbs using both upper and lower limbs

CONTD…CONTD… Weakness --- flaccid type Weakness --- flaccid type

symmetricalsymmetrical

involved both UL&LL equallyinvolved both UL&LL equally

involved both proximal and distalinvolved both proximal and distal

muscles equallymuscles equally No h/o difficulty in lifting head from bedNo h/o difficulty in lifting head from bed No h/o difficulty in respirationNo h/o difficulty in respiration No history s/o sensory disturbancesNo history s/o sensory disturbances No h/o bladder disturbancesNo h/o bladder disturbances

No h/o altered sensoriumNo h/o altered sensorium No h/o headache ,blurring of vision diplopiaNo h/o headache ,blurring of vision diplopia No h/o involuntary movementsNo h/o involuntary movements No history s/o any cranial nerve involvementNo history s/o any cranial nerve involvement No h/o neck pain or radiating pain down the No h/o neck pain or radiating pain down the

limbs limbs h/o pain in distal & proximal parts of both ULs h/o pain in distal & proximal parts of both ULs

- crampy, intermittent, occurs - crampy, intermittent, occurs upon exercise, relieved by upon exercise, relieved by rest promptlyrest promptly

No h/o change in colour of hand/fingers upon No h/o change in colour of hand/fingers upon exercise or exposure to cold exercise or exposure to cold

but feels a sensation of cold upon exercisebut feels a sensation of cold upon exercise

No h/o joint pain, swelling,rashesNo h/o joint pain, swelling,rashes

No h/o chest pain,palpitation,syncopeNo h/o chest pain,palpitation,syncope

No h/o fever , weight loss or loss of appetite No h/o fever , weight loss or loss of appetite

No h/o traumaNo h/o trauma

No h/o recent vaccination,dog biteNo h/o recent vaccination,dog bite

Past history:Past history:

No h/o similar complaints in pastNo h/o similar complaints in past

No h/o HT/DM/CAD/BA/Epilepsy/TBNo h/o HT/DM/CAD/BA/Epilepsy/TB Personal history:Personal history: Not a smoker,alcoholic,tobacco chewerNot a smoker,alcoholic,tobacco chewer menstrual periods– normalmenstrual periods– normal Family history :Family history : no h/o similar illness in family no h/o similar illness in family

O/E:O/E:

Conscious,orientedConscious,oriented

pallor+,pallor+,

no icterus ,cyanosis,clubbing,pedal edemano icterus ,cyanosis,clubbing,pedal edema

no lymphadenopathyno lymphadenopathy

well hydratedwell hydrated

JVP not elevatedJVP not elevated

Pulse chart:Pulse chart:

Pulse: 64/mt, regular rhythm normal volume,no Pulse: 64/mt, regular rhythm normal volume,no specific characterspecific character

B.P:160/100 mm hg in both lower limbsB.P:160/100 mm hg in both lower limbs

ArteryArtery RightRight LeftLeft

CarotidCarotid + + ++

RadialRadial ---- ----

BrachialBrachial ---- ----

FemoralFemoral ++ ++

PoplitealPopliteal ++ ++

Dorsalis pedisDorsalis pedis ++ ++

CVS: S1S2 + , no murmersCVS: S1S2 + , no murmers RS: NVBSRS: NVBS ABDOMEN: soft, no organomegalyABDOMEN: soft, no organomegaly

CNS: conscious,oriented CNS: conscious,oriented

speech: normalspeech: normal

memory : intactmemory : intact

No e/o any cranial nerve involvementNo e/o any cranial nerve involvement

Motor system: Motor system:

R LR L

Bulk UL normal normal Bulk UL normal normal

LL normal normal LL normal normal

Tone UL decreased decreasedTone UL decreased decreased

LL decreased decreasedLL decreased decreased

Power UL 2/5 2/5Power UL 2/5 2/5

LL 2/5 2/5 LL 2/5 2/5

Superficial reflexesSuperficial reflexes

RR LL

cornealcorneal ++ ++

conjunctivalconjunctival ++ ++

gaggag ++ ++

palatalpalatal ++ ++

abdominalabdominal ++ ++

cremastriccremastric ++ ++

plantarplantar flexorflexor flexorflexor

DTRsDTRs

RR LL

bicepsbiceps ++ ++

tricepstriceps ++ ++

supinatorsupinator ++ ++

kneeknee ++ ++

ankleankle ++ ++

Gait and co ordination : not testedGait and co ordination : not tested

Sensory system:intactSensory system:intact

Cerebellar function test : not testedCerebellar function test : not tested

Spine & cranium:normalSpine & cranium:normal

No e/o meningeal signsNo e/o meningeal signs

ProblemsProblems:: Acute onset flaccid quadriparesis Acute onset flaccid quadriparesis Non palpable upper limb pulsesNon palpable upper limb pulses Hypertension Hypertension

Investigations Investigations CBC RFT:CBC RFT:Hb:11.0 Hb:11.0 RBS:95 mg RBS:95 mgTc:8600 B.Urea:22 mgTc:8600 B.Urea:22 mgDC:P68,L30,E2 Sr.creatinine:0.7 mgDC:P68,L30,E2 Sr.creatinine:0.7 mgRBC:3.7 Million Sr.electrolytes:RBC:3.7 Million Sr.electrolytes:MCV:90 Na--137MCV:90 Na--137MCH:30 MCH:30 K – 1.9K – 1.9ESR:5/12 Cl – 108ESR:5/12 Cl – 108 Mg- 2Mg- 2 Ca – 10Ca – 10Urine routine: normal studyUrine routine: normal studyCPK --- 150 U/LCPK --- 150 U/L

ECG: 60/mt,NSR, Normal Axis,ECG: 60/mt,NSR, Normal Axis,

prominent `U` waves,reduced `T` wave prominent `U` waves,reduced `T` wave amplitudeamplitude

CXR:Normal studyCXR:Normal study

INITIAL TREATMENTINITIAL TREATMENT

Oral fluids/feeds rich in potassiumOral fluids/feeds rich in potassium Syrup KCL 15—30 ml hourlySyrup KCL 15—30 ml hourly T.Amlodipine 2.5 mg odT.Amlodipine 2.5 mg od T.Rantac 150 mg bdT.Rantac 150 mg bd T.BCT 1 bdT.BCT 1 bd ECG monitoringECG monitoring Over the next few hours weakness improved Over the next few hours weakness improved

gradually and pt become ambulant next daygradually and pt become ambulant next day

SERIAL Potassium values:SERIAL Potassium values:

DAYDAY 25/525/5 26/526/5 28/528/5 30/5 30/5

Sr.KSr.K 1.91.9 3.23.2 3.23.2 3.43.4

Urine analysisUrine analysis

24 hr urine sample24 hr urine sample

30/530/5 4/64/6

Na ---- 180 meq/d Na----170 Na ---- 180 meq/d Na----170

K ---- 150 meq/d K ---- 148K ---- 150 meq/d K ---- 148

Ca ---- 144 mg/d Ca ----150Ca ---- 144 mg/d Ca ----150

Cl ---- 138 meq/d Cl ---- 142Cl ---- 138 meq/d Cl ---- 142

ABGABG

METABOLIC ALKALOSIS WITH METABOLIC ALKALOSIS WITH COMPENSATORY COMPENSATORY

RESPIRATORY ACIDOSIS WITH RESPIRATORY ACIDOSIS WITH NORMAL ANION GAPNORMAL ANION GAP

It was persistent when repeated thrice in a span It was persistent when repeated thrice in a span of 10 daysof 10 days

HypokalemiaHypokalemia

renal potassium wastingrenal potassium wasting

metabolic alkalosismetabolic alkalosis

hypertensionhypertension

The DDs are 1)Mineralocorticoid excessThe DDs are 1)Mineralocorticoid excess

2)Liddle syndrome2)Liddle syndrome

Next step…Next step…

“ “ GOT STUCK”GOT STUCK”

Sr.Renin and Sr.Renin and Aldosterone not doneAldosterone not done

IMAGINGIMAGING

USG abdomen/KUB: Normal studyUSG abdomen/KUB: Normal study CT Abd: normal studyCT Abd: normal study Renal artery doppler:Renal artery doppler: Resistive indexResistive index

Normal : Normal : less 0.8less 0.8 Impression: distal stenosis of renal artery ? Impression: distal stenosis of renal artery ?

Segmental arteries of right kidneySegmental arteries of right kidney

RR LL

Upper poleUpper pole 0.940.94 0.680.68

Mid poleMid pole 0.900.90 0.640.64

Lower poleLower pole 0.780.78 0.640.64

Ct angiogramCt angiogram

Report Report

Ct angiogram:B/L thinning of subclavian Ct angiogram:B/L thinning of subclavian artery with stenosis and tortuosity in its courseartery with stenosis and tortuosity in its course

Diffuse aortic wall thickeningDiffuse aortic wall thickening Normal appearing carotid , vertebral, Normal appearing carotid , vertebral,

abdominal aorta and proximal renal arteriesabdominal aorta and proximal renal arteries

““Disease affecting aorta, its branches and Disease affecting aorta, its branches and possibly distal renal arteries “possibly distal renal arteries “

POSSIBILITIES ARE………….POSSIBILITIES ARE………….

DISEASE TYPEDISEASE TYPE ENTITIESENTITIES

RHEUMATICRHEUMATIC GIANT CELL arteritis,Cogan GIANT CELL arteritis,Cogan syndrome,Relapsing syndrome,Relapsing polychondritis,Ankylosing polychondritis,Ankylosing spondylitis,Rheumatoid spondylitis,Rheumatoid arthritis,SLE,buerger`s diseasearthritis,SLE,buerger`s disease

INFECTIOUSINFECTIOUS Syphilis,TBSyphilis,TB

OTHERSOTHERS Atherosclerosis,ergotism,radiation Atherosclerosis,ergotism,radiation induced damage,sarcoidosis,marfan induced damage,sarcoidosis,marfan syndrome,Ehler Danlos syndrome,Ehler Danlos syndrome ,congenital co arctation of aorta syndrome ,congenital co arctation of aorta

Other investigations:Other investigations: Mantoux : negativeMantoux : negative VDRL:negativeVDRL:negative ANA: negativeANA: negative RA Factor:negativeRA Factor:negative CRP:5mg/LCRP:5mg/L Lipid profile: normal limitsLipid profile: normal limits Sr .T3,T4,TSH--wnlSr .T3,T4,TSH--wnl

CT chest: normal studyCT chest: normal study ECHO: normal studyECHO: normal study

Queries???????Queries???????

Why vessel wall biopsy not done in this case?Why vessel wall biopsy not done in this case?

CT –Angio doesn show e/o renal artery CT –Angio doesn show e/o renal artery stenosis.. How to explain? stenosis.. How to explain?

Takayasu's arteritisTakayasu's arteritis Takayasu's arteritisTakayasu's arteritis is also known as "aortic arch  is also known as "aortic arch

syndrome", "nonspecific aortoarteritis" and the syndrome", "nonspecific aortoarteritis" and the "pulseless disease”."pulseless disease”.

It is a form of large vessel and medium It is a form of large vessel and medium granulomatous vasculitis with granulomatous vasculitis with massive intimal fibrosis and vascular narrowing.massive intimal fibrosis and vascular narrowing.

Incidence:0.2—2.5/million/yrIncidence:0.2—2.5/million/yr It affects young or middle-aged women M:F 8:1.It affects young or middle-aged women M:F 8:1. ASIANS commonly affectedASIANS commonly affected It mainly affects the aorta (the main blood vessel It mainly affects the aorta (the main blood vessel

leaving the heart) and its branches, as well as leaving the heart) and its branches, as well as the pulmonary arteries.the pulmonary arteries.

Pathogenesis Pathogenesis

TA is characterized by granulomatous inflammation of TA is characterized by granulomatous inflammation of the aorta and its major branches, leading to stenosis, the aorta and its major branches, leading to stenosis, thrombosis, and aneurysm formation. thrombosis, and aneurysm formation.

The lesions of TA are segmental with a patchy The lesions of TA are segmental with a patchy distribution.distribution.

Mononuclear infiltration of the adventitia occurs early Mononuclear infiltration of the adventitia occurs early in the course of the disease, with cuffing of the vasa in the course of the disease, with cuffing of the vasa vasorum. vasorum.

There is no inflammatory infiltrate of the intima; There is no inflammatory infiltrate of the intima; changes are thought to be reactive to inflammation in changes are thought to be reactive to inflammation in the media and adventitia. the media and adventitia.

Symptoms Symptoms Systemic symptoms in Takayasu arteritis (TA) include Systemic symptoms in Takayasu arteritis (TA) include

the following: the following: Fever, night sweatsFever, night sweats FatigueFatigue Weight lossWeight loss Myalgia and/or arthralgia and/or arthritisMyalgia and/or arthralgia and/or arthritis Skin rash (eg, erythema nodosum, pyoderma Skin rash (eg, erythema nodosum, pyoderma

gangrenosum)gangrenosum) Headaches and/or dizziness and/or syncopeHeadaches and/or dizziness and/or syncope Congestive heart failure, palpitations, anginaCongestive heart failure, palpitations, angina Hypertension (may be paroxysmal)Hypertension (may be paroxysmal)

1990 Criteria of American College of Rheumatology for 1990 Criteria of American College of Rheumatology for the Classification of Takayasu Arteritis the Classification of Takayasu Arteritis

Criteria Criteria Definition Definition

Age at disease onset Age at disease onset in year in year 

Development of symptoms or findings related to Development of symptoms or findings related to Takayasu arteritis at age <40 years. Takayasu arteritis at age <40 years. 

Claudication of Claudication of extremities extremities 

Development and worsening of fatigue and discomfort in Development and worsening of fatigue and discomfort in muscles of one or more extremity while in use, especially muscles of one or more extremity while in use, especially the upper extremities. the upper extremities. 

Decreased brachial Decreased brachial artery pulseartery pulse

Decreased pulsation of one or both the brachial arteriesDecreased pulsation of one or both the brachial arteries

BP difference BP difference >10mmHg>10mmHg

Difference of >10mmHg in systolic blood pressure Difference of >10mmHg in systolic blood pressure between arms between arms 

Bruit over subclavian Bruit over subclavian arteries or aortaarteries or aorta

Bruit audible on auscultation over one or both subclavian Bruit audible on auscultation over one or both subclavian arteries or abdominal aortaarteries or abdominal aorta

Arteriogram Arteriogram abnormality abnormality 

Arteriographic narrowing or occlusion of the entire aorta, Arteriographic narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the proximal its primary branches, or large arteries in the proximal uppper or lower extremities, not due arteriosclerosis, uppper or lower extremities, not due arteriosclerosis, fibro-muscular dysplasia, or similar causes: changes fibro-muscular dysplasia, or similar causes: changes usually focal or segmental usually focal or segmental 

For purposes of classification, a patient shall be said to have Takayasu's arteritis if at least three of For purposes of classification, a patient shall be said to have Takayasu's arteritis if at least three of these six criteria are present. The presence of any three or more criteria yields a sensitivity of these six criteria are present. The presence of any three or more criteria yields a sensitivity of 90.5% and a specificity of 97.8%. BP = blood pressure (systolic) difference between arms90.5% and a specificity of 97.8%. BP = blood pressure (systolic) difference between arms

TYPESTYPES

TYPESTYPES

11 Aortic arch and branchesAortic arch and branches

22 Abdominal aortaAbdominal aorta

33 Both arch and abdominal aortaBoth arch and abdominal aorta

44 Pulmonary arteryPulmonary artery

Treatment Treatment Daily high-dose corticosteroid administration is the mainstay of Daily high-dose corticosteroid administration is the mainstay of

initial therapy for active disease. initial therapy for active disease. Prednisone at 1-2 mg/kg/d for 4-6 weeks.Prednisone at 1-2 mg/kg/d for 4-6 weeks. Patients not responding to corticosteroids or who relapse during Patients not responding to corticosteroids or who relapse during

corticosteroid taper require an additional agent. corticosteroid taper require an additional agent. Regimens including weekly methotrexate or daily or monthly Regimens including weekly methotrexate or daily or monthly

intravenous cyclophosphamide .intravenous cyclophosphamide . Cyclosporine may be an alternative therapy offering lower ovarian Cyclosporine may be an alternative therapy offering lower ovarian

toxicity than cyclophosphamide. toxicity than cyclophosphamide. Mycophenolate mofetil may be useful to treat individuals with Mycophenolate mofetil may be useful to treat individuals with

glucocorticoid-resistant disease.glucocorticoid-resistant disease. Infliximab has been used in children with Takayasu arteritis.Infliximab has been used in children with Takayasu arteritis. Anecdotal reports of matrix metalloproteinase inhibition using Anecdotal reports of matrix metalloproteinase inhibition using

minocycline propose that this may be a useful adjunctive therapy.minocycline propose that this may be a useful adjunctive therapy.

Surgical therapy:Surgical therapy: Bypass surgeries for stenotic lesionsBypass surgeries for stenotic lesions

PTCA for stenotic renal arteriesPTCA for stenotic renal arteries

Aortic valve replacement for ARAortic valve replacement for AR

PROGNOSISPROGNOSIS

20% have self limited disease20% have self limited disease

Rest have relapsing and remitting diseaseRest have relapsing and remitting disease

5yr survival rate is 92.5%5yr survival rate is 92.5%

Death is due to cardiac and renal failureDeath is due to cardiac and renal failure

Carry home points……..Carry home points……..

TA is a vasculitis affecting TA is a vasculitis affecting large and medium large and medium sized arteriessized arteries

Young females Young females more commonly affected more commonly affected AngiographyAngiography is an important tool in diagnosis is an important tool in diagnosis Vessel biopsy is not Vessel biopsy is not mandatorymandatory CorticosteroidsCorticosteroids are the mainstay in treatment are the mainstay in treatment InfliximabInfliximab – a newer modality in treatment – a newer modality in treatment Cardiac and renal failure Cardiac and renal failure are common causes of are common causes of

deathdeath

References References

Harrison`s Principles of Internal MedicineHarrison`s Principles of Internal Medicine

17 edition17 edition

Kelly`s Text book of RheumatologyKelly`s Text book of Rheumatology

eMedicineeMedicine

Rheumatology case reportsRheumatology case reports

Thank youThank you