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Approach to myopathyApproach to myopathy
Hereditary Hereditary
acquiredacquired
historyhistory
Onset ageOnset age distributiondistribution CourseCourse MyalgiaMyalgia CrampCramp Contracture Contracture Dark urineDark urine Myotonia ,Stiffness /warming up phenomenaMyotonia ,Stiffness /warming up phenomena Aggravating: exercise /diet/temperature/drugAggravating: exercise /diet/temperature/drug
examexam
Limb girdleLimb girdle ScapuloperonealScapuloperoneal DistalDistal Ocular or pharyngealOcular or pharyngeal Neck extensorNeck extensor Atrophy or hypertrophyAtrophy or hypertrophy Myotonia or paramyotoniaMyotonia or paramyotonia
Ptosis usually without opthalmoplegiaPtosis usually without opthalmoplegiaMyotonic dystrophyMyotonic dystrophy
Congenital myopathyCongenital myopathy
Ptosis with opthalmoplegiaPtosis with opthalmoplegiaOculopharyngeal muscular dystrophyOculopharyngeal muscular dystrophy
Mitochondrial myopathyMitochondrial myopathy
WeaknessWeakness Constant fluctuationConstant fluctuation
Longlife acquiredLonglife acquired MG MG periodic Pperiodic P metabolic metabolic
Progressive Progressive staticstatic Dystrophy congenitalDystrophy congenital
Age Age DistributionDistribution InheritanceInheritance
Work upWork up Muscles enzymeMuscles enzyme R/o metabolic screenR/o metabolic screen EMG&NCS (myotonia ,fibrillation, R/o EMG&NCS (myotonia ,fibrillation, R/o
neuropathy)neuropathy) Muscle biopsy (dystrophy/ congenital Muscle biopsy (dystrophy/ congenital
myopathy, stain for enzyme level)myopathy, stain for enzyme level) Genetic studyGenetic study Other :Other : Forearm exercise testForearm exercise test specific Enzymes levelspecific Enzymes level
When do you suspectWhen do you suspect??
Hereditary myopathyHereditary myopathy
Structural Structural functionalfunctional
classificationclassification
DystrophyDystrophy Congenital myopathyCongenital myopathy Channelopathies & myotoniaChannelopathies & myotonia Metabolic (fatty acid/ Metabolic (fatty acid/
glycogensis/mitochondrial)glycogensis/mitochondrial)
muscular dystrophymuscular dystrophy
are inherited myopathy characterized by are inherited myopathy characterized by progressive muscles weakness progressive muscles weakness °eneration &subsequent replacement °eneration &subsequent replacement by fibrous & fatty connective tissueby fibrous & fatty connective tissue
Historically were categorized by their: Historically were categorized by their: Age onset /distribution of weakness& Age onset /distribution of weakness&
pattern of inheritancepattern of inheritance The genetic mutation &abnormal gene The genetic mutation &abnormal gene
product were defined for many of themproduct were defined for many of them
MDMDdiseasediseaseinheritanceinheritanceageageproteinprotein
duchenneduchenneX linkedX linked2y2ydystrophindystrophin
beckersbeckersX linkedX linked5-155-15....
Emery-dreifussEmery-dreifussX linkedX linkedchildhchildhemerinemerin
LGDLGDAD/ARAD/ARsacroglycansacroglycan
Cong/CNSCong/CNSARARbirthbirth
Cong/noCNSCong/noCNSARAR....merosinmerosin
Distal MDDistal MDAD/ARAD/AR
bethlenbethlenADAD
FSHFSHADADChild&Child&adultadult
oculodystrophyoculodystrophyADAD55thth dec dec
Myotonic type1Myotonic type1ADAD2th,3th 2th,3th decadedecade
Myotonic type 2Myotonic type 2ADAD
myofibrillarmyofibrillarADADdesmmindesmmin
Duchenne MDDuchenne MD
Incidence: 1/3500 male birthIncidence: 1/3500 male birth 1/3 new mutation1/3 new mutation c/p:as early as 2-3y with delay c/p:as early as 2-3y with delay
milestonesmilestones Progressive limb girdle patternProgressive limb girdle pattern Fall 5-6y/difficult climb stair 8y, Fall 5-6y/difficult climb stair 8y,
confined to wheelchair 12yconfined to wheelchair 12y
Joint constructers 6-10yJoint constructers 6-10y Calf hypertrophy is earlyCalf hypertrophy is early Muscles atrophy lateMuscles atrophy late Progressive kyphscliosis due to Paraspinal Progressive kyphscliosis due to Paraspinal
muscles weaknessmuscles weakness Reflex: biceps/knee/lost by age 10yReflex: biceps/knee/lost by age 10y ankle preserved late in diseaseankle preserved late in disease Respiratory s/s after age 10 Respiratory s/s after age 10
Cardiac: generally asymptomaticCardiac: generally asymptomatic CHF, arrhythmia lateCHF, arrhythmia late 90% abnormal ECG :tall rt R 90% abnormal ECG :tall rt R
wave,deep left Q wavewave,deep left Q wave Echo: hypokinesia ,dilatation of Echo: hypokinesia ,dilatation of
ventricular wallventricular wall GI: intestinal pseudo obstructionGI: intestinal pseudo obstruction IQ: one SD below NIQ: one SD below N
lablab A dystrophin gene deletion can be detected by:A dystrophin gene deletion can be detected by: DNA analyses from leukocytes by PCR in 2/3 DNA analyses from leukocytes by PCR in 2/3
patient or DNA musclespatient or DNA muscles The other 1/3 DX by… muscles biopsy( dystrophin The other 1/3 DX by… muscles biopsy( dystrophin
def by stain &WB ,typical features of MD)def by stain &WB ,typical features of MD) CK:20-100 XN ,decline laterCK:20-100 XN ,decline later EMG:myopathic &fibrillationEMG:myopathic &fibrillation Note :if DNA study +ve no need for EMG Note :if DNA study +ve no need for EMG
&muscles biopsy&muscles biopsy
Beckers MDBeckers MD
Is milder form Is milder form 5/100,0005/100,000 Age :5-15yAge :5-15y Wheelchair at 30yWheelchair at 30y Cardiac similar to duchenneCardiac similar to duchenne Death by age 40Death by age 40 Dx: DNA, muscle biopsy decrease in Dx: DNA, muscle biopsy decrease in
dystrophindystrophin CK:moderatly elevatedCK:moderatly elevated
treatmenttreatment
No treatment prevent the No treatment prevent the progression progression corticosteroid :controlled trial with corticosteroid :controlled trial with predinsone 0,75mg/kg demonstrate predinsone 0,75mg/kg demonstrate moderate improvement in strength moderate improvement in strength &delay progression to wheel chair& &delay progression to wheel chair& respiratory compromiserespiratory compromise
Emery-dreifussEmery-dreifuss
X linkedX linked onset :childhoodonset :childhood Triad of: Triad of:
1-early contracture 1-early contracture elbow, ankle &posterior cervicalelbow, ankle &posterior cervical
2-progressive scapulohumroperoneal2-progressive scapulohumroperoneal
3-cardiomyopathy with atrial conduction 3-cardiomyopathy with atrial conduction defectdefect
CK :normal to or only moderate CK :normal to or only moderate elevatedelevated
The muscle biopsy :myopathic The muscle biopsy :myopathic &fewer dystrophic&fewer dystrophic
DNA:mutation gene in Xq28 code for DNA:mutation gene in Xq28 code for protien emerinprotien emerin
Limb girdle dystrophyLimb girdle dystrophy
AR majorityAR majority Onset: adolescence or lateOnset: adolescence or late
childhood: sever child recessive muscular childhood: sever child recessive muscular dystrophydystrophy
AR: defect in sacroglycan component of AR: defect in sacroglycan component of the DGC( sacroglycanopathythe DGC( sacroglycanopathy((
Alpha sacrglycan adhelin is account for Alpha sacrglycan adhelin is account for 20%20%
Onset:childhood& variableOnset:childhood& variable No intellectual impairment or cardiacNo intellectual impairment or cardiac Muscle biopsy :immune stain absent or Muscle biopsy :immune stain absent or
diminished for sacroglycandiminished for sacroglycan
AD: onset: second and third decadesAD: onset: second and third decades Protein defect:caveolin-3Protein defect:caveolin-3
There are multiple subtypesThere are multiple subtypes AD type 1:1A,1B …AD type 1:1A,1B … AR type 2:AR type 2:
Congenital muscular dystrophyCongenital muscular dystrophy ARARPerinatal onset Perinatal onset c/p:hypotonia &proximal weakness,arthrogryposisc/p:hypotonia &proximal weakness,arthrogryposis Two types Two types CNS involvement: severCNS involvement: sever mental mental
retardation ,visual, seizure ..cerebrocular retardation ,visual, seizure ..cerebrocular dysplasia, progressive death by age 10-12dysplasia, progressive death by age 10-12
No CNS :classic typeNo CNS :classic type MRI (hypomyelination), MRI (hypomyelination), benign outcome, non progressivebenign outcome, non progressive
Muscle biopsy :dystrophy… Muscle biopsy :dystrophy…
FSHFSH InheritanceInheritance: AD: AD Variable expression within the familiesVariable expression within the families AgeAge: childhood or adult life: childhood or adult life C/PC/P: :
weakness early facial then descending to scapula stabilizer weakness early facial then descending to scapula stabilizer muscles &muscles of the upper limb& distal muscles &muscles of the upper limb& distal weakness ..peroneal ,the rate of progression to forearm weakness ..peroneal ,the rate of progression to forearm &pelvic girdle &pelvic girdle
Asymmetrical/Asymmetrical/ deltoid preserved / joint contracture are deltoid preserved / joint contracture are uncommonuncommon
Popeye handPopeye hand/ winging scapula/ / winging scapula/ no muscle hypertrophyno muscle hypertrophy Early onset worse prognosisEarly onset worse prognosis 20% require wheelchair20% require wheelchair
Work upWork up
CK:N or mild elevationCK:N or mild elevation Muscles biopsy: myopathic Muscles biopsy: myopathic
dystrophicdystrophic& occasionally prominent & occasionally prominent mononuclear infiltratemononuclear infiltrate
Gene: ch 4q35 gene deletion Gene: ch 4q35 gene deletion
Myotonic dystrophyMyotonic dystrophy AD, AD, CTG repeatCTG repeat Affect : Affect :
skeletal,cardiac, smooth muscles, eye,endocrine skeletal,cardiac, smooth muscles, eye,endocrine &brain&brain
Onset :at any age ,usually at late 2Onset :at any age ,usually at late 2ndnd decade decade Some individual can be symptoms free their Some individual can be symptoms free their
entire lifeentire life Sever form :congenital myotonic dystrophySever form :congenital myotonic dystrophy
C/P:weakness: C/P:weakness: (facial,temporalis wasting,ptosis,neck (facial,temporalis wasting,ptosis,neck flexor,distal weakness progress to flexor,distal weakness progress to involve limb girdle)involve limb girdle)
Weakness >myotoniaWeakness >myotonia May be areflexicMay be areflexic
systemicsystemic
Posterior sub scapular cataractPosterior sub scapular cataract Testicular atrophy& impotenceTesticular atrophy& impotence Intellectual impairmentIntellectual impairment Hypersomnia (central & obstructive)Hypersomnia (central & obstructive) Respiratory failureRespiratory failure Elevation of serum glu, rarely frank DMElevation of serum glu, rarely frank DM GI: dysphagea, pseudo obstructionGI: dysphagea, pseudo obstruction Cardiac conduction defect sudden deathCardiac conduction defect sudden death Fetal loss in femaleFetal loss in female
PROMMPROMM
ADAD Proximal weakness, no distal Proximal weakness, no distal
weaknessweakness Myotonia &myalgiaMyotonia &myalgia Less cardiac &other organ Less cardiac &other organ
involvement except cataractinvolvement except cataract
Work upWork up
CK:N or mild elevationCK:N or mild elevation EMG: myopathic & EMG: myopathic & myotoniamyotonia Muscle biopsy: atrophic, non specificMuscle biopsy: atrophic, non specific Gene :CTG repeat >50 in ch19q13.2Gene :CTG repeat >50 in ch19q13.2
tttttt
Myotonia rarely sever to require tt: Myotonia rarely sever to require tt: phenytoin is the only safe drugphenytoin is the only safe drug
Annual ECG ..pacemaker may Annual ECG ..pacemaker may requiredrequired
Positive pressure ventilation supportPositive pressure ventilation support High risk in surgery (cardiac High risk in surgery (cardiac
&respiratory)&respiratory) Sedation & opiod use with cautionSedation & opiod use with caution
Distal dystrophyDistal dystrophy
TypesTypes AD:4AD:4thth &6 &6thth decade decade AR:in early adult onset/late second or AR:in early adult onset/late second or
early 3early 3rdrd CK :elevated 200xN ARCK :elevated 200xN AR
oculopharengealoculopharengeal ADAD Onset:5Onset:5thth &6 &6thth decade decade Ptosis &dysphagea later all extra ocular Ptosis &dysphagea later all extra ocular
muscles &extremities affected (limb muscles &extremities affected (limb girdle) but distal can be significant in some girdle) but distal can be significant in some variantvariant
Slow progressive ,death from aspiration Slow progressive ,death from aspiration pneumonia or starvationpneumonia or starvation
Ck:n or mild elevatedCk:n or mild elevated Muscle biopsy :Muscle biopsy :rim vacuolesrim vacuoles Genetic GCG repeat in ch14Genetic GCG repeat in ch14
Congenital myopathyCongenital myopathy
Are distinguished from dystrophy in Are distinguished from dystrophy in threethree respect:respect:
Characteristic morphologic alterationCharacteristic morphologic alteration At birthAt birth Non progressiveNon progressive However there are exception to all However there are exception to all
these generalizationthese generalization Inheritance: are variableInheritance: are variable
c/p: c/p: hypotonia with subsequent developmental delayhypotonia with subsequent developmental delay Reduce muscles bulk, slender body build &long narrow face Reduce muscles bulk, slender body build &long narrow face Skeletal abnormalities: high arched palate ,pectus Skeletal abnormalities: high arched palate ,pectus
exacavitum, kyphscliosis, dislocated hip, pes cavusexacavitum, kyphscliosis, dislocated hip, pes cavus))
Absent or reduced muscle stretch reflexAbsent or reduced muscle stretch reflex
Weakness: limb girdle mostly, butWeakness: limb girdle mostly, but distal distal weakness existweakness exist CK &EMG may be normalCK &EMG may be normal Muscle biopsy: the diagnostic methodMuscle biopsy: the diagnostic method
Central core myopathyCentral core myopathy
Characterized by discrete zones of Characterized by discrete zones of myofibrillar disruption in the center myofibrillar disruption in the center of muscles fiberof muscles fiber
AD but can be sporadicAD but can be sporadic Mutation ch 19,similar to malignant Mutation ch 19,similar to malignant
hyperthermia patienthyperthermia patient So anesthesia precaution are So anesthesia precaution are
necessarynecessary
Nemaline myopathyNemaline myopathy Pathology: thePathology: the presence of rods or presence of rods or
melamine bodies within muscles fibermelamine bodies within muscles fiber AD or ARAD or AR c/p:c/p: Sever neonatalSever neonatal form which is fatal in the form which is fatal in the
first year of lifefirst year of life Mild staticMild static Slowly progressiveSlowly progressive from birth or early from birth or early
childhoodchildhood Note :rods can present in HIV related Note :rods can present in HIV related
myopathy ,some inflammatorymyopathy ,some inflammatory
Centro nuclear (myotubular)Centro nuclear (myotubular)
Pathology: large central nuclei in the Pathology: large central nuclei in the muscle fibermuscle fiber
X linked/AD/ARX linked/AD/AR sever neonatal/static or slowly sever neonatal/static or slowly
progressiveprogressive c/p: ptosis & opthalmoparesisc/p: ptosis & opthalmoparesis Genetic defect: mutation in Genetic defect: mutation in
myotubularin gene Xp28myotubularin gene Xp28
Metabolic myopathyMetabolic myopathy
Metabolic myopathyMetabolic myopathy
Glucose/glycogen metabolismGlucose/glycogen metabolism Fattay acid metabolismFattay acid metabolism Purine nucleotidePurine nucleotide mitochondrialmitochondrial
Metabolic myopathyMetabolic myopathy
Clues to hereditary metabolic Clues to hereditary metabolic myopathymyopathy
Excersize induce weakness Excersize induce weakness &myoglobinuria…glycogen &lipid&myoglobinuria…glycogen &lipid
Part of diffuse neurological Part of diffuse neurological syndrome…mitochondrialsyndrome…mitochondrial
Glucose/glycogenGlucose/glycogen
Glucose &its storage is essential for the Glucose &its storage is essential for the short termshort term anaerobic energy (glycogensis)anaerobic energy (glycogensis)
Two clinical presentation:Two clinical presentation: 1-dynamic:type V/V11/V111/1X//XX11-dynamic:type V/V11/V111/1X//XX1 2-static:fix weakness2-static:fix weakness
1/111/1V1/111/1V Inheritance:AR except for Inheritance:AR except for
phosphoglycerate kinasephosphoglycerate kinase
Glycogensis with exerciseGlycogensis with exercise intolerance intolerance C/P: exercise intolerance in the childhood C/P: exercise intolerance in the childhood
followed by excertional induced muscle pain followed by excertional induced muscle pain &myoglobinurea in sec or 3&myoglobinurea in sec or 3rdrd decade.. decade..
(Second wind phenomena)(Second wind phenomena) work up: CK/EMG work up: CK/EMG
normal between the attack in early stage but normal between the attack in early stage but after attack( myopathic &fibrillation)after attack( myopathic &fibrillation)
Forearm exercise testForearm exercise test Enzyme assayEnzyme assay Muscle biopsyMuscle biopsy Genetic for mutation Genetic for mutation
What is 0ther causes of What is 0ther causes of myoglobinureamyoglobinurea??
Glycogensis with fixed weaknessGlycogensis with fixed weakness
Acid maltase deficiency:Acid maltase deficiency: Enzyme convert glycogen to glucoseEnzyme convert glycogen to glucose Three clinical variant:Three clinical variant: Infantile: pompes: progressive Infantile: pompes: progressive
weakness ,enlargement of heart, tongue &liverweakness ,enlargement of heart, tongue &liver death by age 2death by age 2
Juvenile type: proximal weakness, may calf Juvenile type: proximal weakness, may calf hypertrophy death by age 20 from respiratory hypertrophy death by age 20 from respiratory failurefailure
Adult type:2&7Adult type:2&7thth progressive limb girdle or progressive limb girdle or scapuloperoneal .no liver ,no heart scapuloperoneal .no liver ,no heart involvementinvolvement
Work upWork up
CK :moderately increasedCK :moderately increased EMG: myopathic changes &EMG: myopathic changes &myotonic myotonic
discharge in paraspinusdischarge in paraspinus Enzyme assay:Enzyme assay: Muscle biopsy: a vacuolar myopathy Muscle biopsy: a vacuolar myopathy
with high glycogen contentwith high glycogen content Genetic: mutation in ch 17Genetic: mutation in ch 17
Fatty acid metabolismFatty acid metabolism
Lipids are essential for aerobic Lipids are essential for aerobic metabolismmetabolism
Dynamic & staticDynamic & static CPT:carnitine palmitoyl transeferase CPT:carnitine palmitoyl transeferase
deficiencydeficiency Carnitine deficiencyCarnitine deficiency
CPTCPT Type 1:infancy &child hood with hepatic Type 1:infancy &child hood with hepatic
dysfunctiondysfunction Type 2:exertional myalgia &myoglobinurea, Type 2:exertional myalgia &myoglobinurea,
it is the most frequently definable it is the most frequently definable metabolic defect presenting with myoglobinureametabolic defect presenting with myoglobinurea
AR ,gene 1p32AR ,gene 1p32 The attacks occur after prolonged exercise, The attacks occur after prolonged exercise,
fasting, febrile illnessfasting, febrile illness Unlike mecardle disease the patient can tolerate Unlike mecardle disease the patient can tolerate
brief exercise ,no second wind phenomenabrief exercise ,no second wind phenomena Muscle strength are normal at restMuscle strength are normal at rest
lablab
CK:n at restCK:n at rest Forearm exercise test :NForearm exercise test :N EMG: n at rest ,&myopathic during the EMG: n at rest ,&myopathic during the
attackattack Muscle biopsy: usually N ,except of Muscle biopsy: usually N ,except of
myopathic changes after rhabdomylsismyopathic changes after rhabdomylsis Enzyme assayEnzyme assay ttt &meal frequency: increase CHO intakettt &meal frequency: increase CHO intake
&education about fasting &exercise&education about fasting &exercise
MitochondrialMitochondrial Kearns-sayer: Kearns-sayer: opthalmoplegia, retinitis pigmentosa, heart opthalmoplegia, retinitis pigmentosa, heart
block, hearing loss, short stature, ataxia, delayed 2block, hearing loss, short stature, ataxia, delayed 2ndnd sexual sexual characteristic, PN, respiratory characteristic, PN, respiratory
MERFMERF: myoclonic epilepsy, generalized seizure, ataxia, : myoclonic epilepsy, generalized seizure, ataxia, dementia, hearing loss, optic atrophy ,PN, cardiomyopathy dementia, hearing loss, optic atrophy ,PN, cardiomyopathy &cutenous lipoma&cutenous lipoma
MNGLEMNGLE: mitochondrial neurogastrointestinal : mitochondrial neurogastrointestinal encephalomyopathyencephalomyopathy
POLIP :POLIP :polyneuropathy,opthalmoplasia,leukoencephalopathpolyneuropathy,opthalmoplasia,leukoencephalopathy& intestinal pseudo obstructiony& intestinal pseudo obstruction
channelopathychannelopathy
Non dystrophic myotoniaNon dystrophic myotonia Periodic paralysisPeriodic paralysis It due to mutation in different It due to mutation in different
channels gene leading to :channels gene leading to : Hyper excitability :myotoniaHyper excitability :myotonia In excitability: paralysisIn excitability: paralysis
Chloride channelopathyChloride channelopathy
Mutation in CL Mutation in CL channel..hyperexcitability after channel..hyperexcitability after depolarizationdepolarization
Myotonia congenita:Myotonia congenita: AD..thomsen /AR:beckerAD..thomsen /AR:becker C/P:C/P:
muscle hypertrophy, muscle hypertrophy, myotonia/becker type has fluctuating myotonia/becker type has fluctuating limb girdle weaknesslimb girdle weakness
Sodium channleopathySodium channleopathy ADAD Onset: first decadeOnset: first decade Phenotypic types:Phenotypic types: Paramyotonia congenitaParamyotonia congenita
Hyperkalemic periodic paralysisHyperkalemic periodic paralysis
myotonia :Potassium sensitive disorder : myotonia :Potassium sensitive disorder : myotonia fluctuant myotonia fluctuant
myotonia permanentmyotonia permanent acetazolamide responsive myotoniaacetazolamide responsive myotonia
Calcium channelopathyCalcium channelopathy
HypokalemicHypokalemic
malignant hyperthermiamalignant hyperthermia
C/P of channelopathyC/P of channelopathy
Paramytonia congenitaParamytonia congenita
ADAD Onset :1Onset :1stst decade decade Paradoxical myotonia (Aggravated by Paradoxical myotonia (Aggravated by
warm as well cold)warm as well cold) Face ,neck,forearmFace ,neck,forearm After several attempt of eye closure the After several attempt of eye closure the
patient can not open the eyepatient can not open the eye ttt: Na channels blocker mexiletinettt: Na channels blocker mexiletine
Hyperkalamic periodic paralysisHyperkalamic periodic paralysis K sensitive periodic paralysisK sensitive periodic paralysis Onset :1Onset :1stst decade decade Attack last:1-2 hAttack last:1-2 h During attack: areflexic with no ocular or During attack: areflexic with no ocular or
respiratory muscles weaknessrespiratory muscles weakness Strength is n between the attack, but some Strength is n between the attack, but some
patient has interictal limb girdle weaknesspatient has interictal limb girdle weakness Some families have myotonia ¶myotoniaSome families have myotonia ¶myotonia Aggravated: fasting/cold, shortly after exercise, K Aggravated: fasting/cold, shortly after exercise, K
load, early AMload, early AM
Episodes are rarely serous enough to Episodes are rarely serous enough to require acute tttrequire acute ttt
ttt: ttt: oral CHO oral CHO Prevention: thiazide,B agonist, low Prevention: thiazide,B agonist, low
K,high CHOK,high CHO Avoid fasting, strenuous exercise/Avoid fasting, strenuous exercise/
myotoniamyotonia
No weaknessNo weakness Aggravated by K diet/ excretionAggravated by K diet/ excretion Can response to acetazolamideCan response to acetazolamide
hypokalemiahypokalemia
AD:AD: It is the most frequent form of periodic It is the most frequent form of periodic
paralysisparalysis Common in maleCommon in male Age: adolescenceAge: adolescence The attacks 3-24h/vague prodorme of The attacks 3-24h/vague prodorme of
stiffness &heaviness& rarely ocular, stiffness &heaviness& rarely ocular, bulbar, respiratory involvedbulbar, respiratory involved
Early Myotonia of eyelid & late interictal Early Myotonia of eyelid & late interictal proximal weakness proximal weakness
AggravatedAggravated: CHO meal, cold,hrs post : CHO meal, cold,hrs post exercise, sleepexercise, sleep
Work up:K level q 30min /TFT/ Work up:K level q 30min /TFT/ R/O 2R/O 2ndnd causes of hypokalemia causes of hypokalemia Tttt:Tttt: Acute: Acute: oral K Q30min ,if symptoms sever oral K Q30min ,if symptoms sever
iv Kiv K Prevention:Prevention: Low CHO, low sodium Low CHO, low sodium
diet ,spirnolactone, trimetrinediet ,spirnolactone, trimetrine