A CASE OF ACUTE A CASE OF ACUTE PAINFUL PAINFUL

PARAPARESISPARAPARESISPROF S SHIVAKUMAR’S UNITPROF S SHIVAKUMAR’S UNIT

S MURUGANANTH MD PGS MURUGANANTH MD PG

ThomasThomas25 yrs / male25 yrs / maleAdm on 09/05/06 at 11.30pmAdm on 09/05/06 at 11.30pm

c/o c/o Acute onset of pain in both thighsAcute onset of pain in both thighsDifficulty in using both lower limbsDifficulty in using both lower limbsBurning and tingling both footBurning and tingling both foot

1 DAY

Patient was normal till yesterday nightPatient was normal till yesterday night

Patient had consumed 360 ml of whisky Patient had consumed 360 ml of whisky and 2 bottles of beer in the evening of and 2 bottles of beer in the evening of 08/05/0608/05/06

Went to sleep normally Went to sleep normally

Next day morning patient could not get up Next day morning patient could not get up from his bed and had severe pain in both from his bed and had severe pain in both the thighs.the thighs.

Had burning and tingling in the both feet Had burning and tingling in the both feet below the ankle below the ankle

H/o presenting illnessH/o presenting illness

Difficulty in walking since Difficulty in walking since morningmorning

H/o oliguria since morning and H/o oliguria since morning and the urine was dark in colourthe urine was dark in colour

No H/oNo H/o FeverFeverTraumaTrauma

Past H/oPast H/oNot a DM/ HTN/ PT/ BA/ RHDNot a DM/ HTN/ PT/ BA/ RHD

Personal H/oPersonal H/oOccasional alcoholic once or twice Occasional alcoholic once or twice

in a monthin a monthNot a smokerNot a smoker

Drug H/oDrug H/oNil Nil

GENERAL EXMINATIONGENERAL EXMINATION

ConsciousConscious OrientedOriented AfebrileAfebrile Not anemic/ jaundicedNot anemic/ jaundiced No cyanosis / clubbing/ pedal edemaNo cyanosis / clubbing/ pedal edema No lymph node enlargementNo lymph node enlargement PulsePulse - 90/ mt , felt in all peri - 90/ mt , felt in all peri

vesselsvessels BPBP - 130/80 mm Hg- 130/80 mm Hg

CVSCVS

RSRS NADNAD

ABDABD

CNSCNS HF & CNHF & CN – Normal – Normal

Weakness of both LL (proximal > distal)Weakness of both LL (proximal > distal)

DTR DTR -just present-just present

SensationSensation – pain / temp and vibration – pain / temp and vibration diminished below both anklesdiminished below both ankles

Plantar Plantar – not Elicitable– not Elicitable

Tenderness both thighs presentTenderness both thighs present

No spinal tendernessNo spinal tenderness

PROBLEMSPROBLEMSACUTE PROXIMAL WEAKNESS OF BOTH ACUTE PROXIMAL WEAKNESS OF BOTH

LLLLSEVERE PAIN BOTH THIGHSSEVERE PAIN BOTH THIGHSPERIPHERAL NEURITISPERIPHERAL NEURITISOLIGURIA ( ? RENAL FAILURE)OLIGURIA ( ? RENAL FAILURE)BLACK URINE BLACK URINE

PROVISIONAL DIAGNOSIS

1. RHABDOMYOLYSIS

2. PORPHYRIA

WITH THE BACKGROUND H/O ALCOHOL BINGE

Patient was not passing urine , so Patient was not passing urine , so catheterized to see the colour of catheterized to see the colour of urine.urine.

Around 50 ml of black coloured urine Around 50 ml of black coloured urine drained drained

Urgent RFTUrgent RFT UreaUrea -- 44 mg/dl44 mg/dl CreatCreat -- 1.4 mg/dl1.4 mg/dl SugarSugar -- 180 mg/dl180 mg/dl Na+Na+ -- 148 mq/ L148 mq/ L K+K+ -- 4.5 mq/ L4.5 mq/ L

PROGRESSPROGRESS

ECG & CXR were normalECG & CXR were normal

Fluid challengeFluid challenge1 litre of normal saline infused over 1 1 litre of normal saline infused over 1

hrhrThere was no increase in urine output There was no increase in urine output So inj Furesemide 40 mg givenSo inj Furesemide 40 mg givenAround 500 ml of urine drained till Around 500 ml of urine drained till

next day morningnext day morningUrine colour was still blackUrine colour was still black

PROCEEDED WITH OTHER PROCEEDED WITH OTHER INVESTIGATIONSINVESTIGATIONS

HEMOGRAMHEMOGRAMHbHb -- 10.8 g/dl10.8 g/dlTLCTLC -- 6100/ cu mm6100/ cu mmDLCDLC -- P 64 L 34 E 1 M 1P 64 L 34 E 1 M 1ESRESR -- 10/2210/22

URINEURINEAlb & sugarAlb & sugar -- nilnilMYOGLOBIN MYOGLOBIN -- +VE +VE

( BENZEDINE TEST( BENZEDINE TEST ) )

PHORPHOBILINOGEN-PHORPHOBILINOGEN- -VE-VE

Two cycles of FORCED ALKALINE Two cycles of FORCED ALKALINE DIURESIS was given in the DIURESIS was given in the morningmorning

Urine was clear after 2 pm.Urine was clear after 2 pm.

Adequate hydration continued Adequate hydration continued

10/ 0510/ 05 12/0512/05

TBTB 0.9mg/dl0.9mg/dl 1mg/dl1mg/dlSGOTSGOT 617 IU/L617 IU/L 824 IU/L824 IU/LSGPTSGPT 337 IU/L337 IU/L 451 IU/L451 IU/LSAPSAP 98 IU /L98 IU /L 75 IU/ L75 IU/ LT.proteinT.protein 7.2 g/dl7.2 g/dl 7.8 g/dl7.8 g/dlALBUMINALBUMIN 3.5g/dl3.5g/dl 4.2 g/dl4.2 g/dl

LFTLFT

CPKCPK -- 92,850 IU/L92,850 IU/L CALCIUMCALCIUM -- 9.9 mg/dl9.9 mg/dl PHOSPHORUSPHOSPHORUS -- 3.5 mg/dl3.5 mg/dl URIC ACIDURIC ACID -- 6.5 mg/dl6.5 mg/dl

PLASMA MYOGLOBINPLASMA MYOGLOBIN

POSITIVE BY POSITIVE BY SPECTROSCOPYSPECTROSCOPY

PROGRESSIVE RFTPROGRESSIVE RFT

RFTRFT 09/0509/05 10/0510/05 11/0511/05 12/0512/05

UREAUREA 4444 7272 8989 115115

CREATCREAT 1.41.4 2.42.4 3.33.3 2.72.7

Na +Na + 148148 136136 140140 142142

K+K+ 4.54.5 4.44.4 3.63.6 4.04.0

DIAGNOSISDIAGNOSIS

RHABDOMYOLYSIS-ALCOHOL INDUCEDACUTE RENAL FAILURE – PIGMENT NEPHROPATHY

Neurologist opinionNeurologist opinionRhabdomyolysis induced proximal Rhabdomyolysis induced proximal

muscle weaknessmuscle weaknessNephrologist opinionNephrologist opinion

ARF – myoglobin inducedARF – myoglobin induced

Renal function was improving Renal function was improving with adequate hydrationwith adequate hydration

Patient went against medical Patient went against medical adviceadvice

RHABDOMYOLYSIS- CAUSESRHABDOMYOLYSIS- CAUSES

ALCOHOL BINGEALCOHOL BINGE CRUSH INJURYCRUSH INJURY HYPOKALEMIAHYPOKALEMIA STATUS EPILEPTICUSSTATUS EPILEPTICUS HYPERTHERMIAHYPERTHERMIA PROLONGED IMMOBILISATIONPROLONGED IMMOBILISATION SHOCKSHOCK STRENUOUS EXERCISESTRENUOUS EXERCISE DRUGSDRUGS

PSYCHOTROPICS- NEUROLEPTIC MALIG PSYCHOTROPICS- NEUROLEPTIC MALIG SYNDROMESYNDROME

STATINS STATINS - REDUCED CO- ENZYME Q - REDUCED CO- ENZYME Q

NORMAL CELL METABOLISM

Na K ATPASE

GLUCOSE

GLYCOLYSIS

PYRUVATE LACTATE

GLUCONEOGENESIS

FFA

BETA OXIDATION

ACETYL CO A

GLUCOSE

TCA CYCLE

ATP

ATP

ATP

Na+ K+

70 % TOTAL CELLULAR ATP IS UTILIZED FOR THIS REACTION

NADH

ALCOHOL ON METABOLISM

Na K ATPASE

GLYCOLYSIS

PYRUVATE LACTATE

GLUCONEOGENESIS OF FFABETA OXIDATION

TCA CYCLE

Na+ K+

ALCOHOL ACETALDEHYDE ACETATE

NAD

NADH

NADH:NAD

OXALOACETATE

INCREASEDACETYL COA

REDUCED ATP

KETOGENESIS

LIPOGENESISHYPOGLYCEMIA

ALCOHOLDIRECT SARCOLEMMAL INJURY

INCREASED SODIUM PERMEABILITY

REDUCED ATP SYNTHESIS

INCREASED ATPaseACTIVITY

INCREASED ICF Na+

REDUCED ATP SUPPLY

Na+K+ATPase EXHAUSTED

ICF Na +

ICF Ca+

Calmodulin proteasesactivated

CELL SWELLING

CELL INJURY

MYOLYSIS

K+ P

ENZYMESCPK LDHSGOTSGPT

MYOGLOBIN CREATININE

HYPERURICEMIA

HYPERKALEMIA

HYPERPHOSPHOTEMIAHYPOCALCEMIA

ARF RAISEDDISPROPOPRTIONATETO RENAL FAILURE

PURINES

FILTRATION OFMYOGLOBIN

ACIDIC URINE

INTRALUMINAL CAST FORMATION

FREE IRON RELEASE FROM HEME

FREE RADICALS

ATN

ARFARF

FENTON RXN

PRECIPITATION

DEHYDRATIONRENAL VASOCONSTRICTIONHYPER URECEMIA

COMPLICATIONSCOMPLICATIONSARFARFARRYTHMIAS DUE TO ARRYTHMIAS DUE TO

HYPERKALEMIAHYPERKALEMIAMYOCARDIAL INVOLVEMENTMYOCARDIAL INVOLVEMENTCOMPARTMENTAL SYNDROMECOMPARTMENTAL SYNDROMERESPIRATORY MUSCLE RESPIRATORY MUSCLE

WEAKNESSWEAKNESSHYPOCALCEMIAHYPOCALCEMIA

CLINICAL FEATURESCLINICAL FEATURESTRIADTRIAD

MUSCLE PAINMUSCLE PAINWEAKNESSWEAKNESSDARK URINEDARK URINE

MUSCLE SWELLING AND TENDERNESS MUSCLE SWELLING AND TENDERNESS MAY BE PRESENTMAY BE PRESENT

THIGH AND BACK MUSCLES INVOLVED THIGH AND BACK MUSCLES INVOLVED PREDOMINANTLYPREDOMINANTLY

DIAGNOSISDIAGNOSIS PLASMA/ URINE MYOGLOBINPLASMA/ URINE MYOGLOBIN

PositivePositive Disappears within 6 hrsDisappears within 6 hrs

CPKCPK Increases after 12 hrsIncreases after 12 hrs Normalized after 3 – 5 daysNormalized after 3 – 5 days

CARBONIC ANHYDRASE/ MYOSIN HEAVY CARBONIC ANHYDRASE/ MYOSIN HEAVY CHAINCHAIN

More specificMore specific Increased in 4 – 7 daysIncreased in 4 – 7 days Detected up to 12 daysDetected up to 12 days

OTHER ENZYMES/ RENAL PARAMETERSOTHER ENZYMES/ RENAL PARAMETERS SGOT, SGPT and LDHSGOT, SGPT and LDH

MANAGEMENTMANAGEMENT GENERAL SUPPORTIVE MEASURESGENERAL SUPPORTIVE MEASURES

HydrationHydration Treatment of precipitating causeTreatment of precipitating cause

PREVENTION OF ARFPREVENTION OF ARF Maintain urine out put > 300 ml/ hrMaintain urine out put > 300 ml/ hr Forced alkaline diuresisForced alkaline diuresis MannitolMannitol

TREATMENT OF ARFTREATMENT OF ARF ConservativeConservative Dialysis Dialysis

CONCLUSIONCONCLUSIONDIAGNOSIS OF RHABDOMYOLYSIS DIAGNOSIS OF RHABDOMYOLYSIS

SHOULD BE SUSPECTED WITH A H/oSHOULD BE SUSPECTED WITH A H/o

ALCOHOLIC BINGE, SEVERE MUSCLE ALCOHOLIC BINGE, SEVERE MUSCLE PAIN AND DARK COLOURED URINEPAIN AND DARK COLOURED URINE

EARLY RECOGNITION AND EARLY RECOGNITION AND

ADEQUATE HYDRATION/ FAD WILL ADEQUATE HYDRATION/ FAD WILL PREVENT RENAL FAILURE.PREVENT RENAL FAILURE.