Neuro&Seizure&&&Epilepsy&

Epidemiology:! ! 5%!population!has!a!Seizure!by!20!yo!
! ! ! ! Epilepsy!Prevalence:! 0.5T1%!population!
Definition!(ILAE!2010):!
! Seizure:! ! Clinical!expression!of!abnormal,!excessive,!synchronous!discharges!of!neurons!
! ! ! ! ! residing!primarily!in!Cerebral!cortex!
! ! ! ! Intermittent!&!Usually!SelfTlimited,!lasting!seconds!to!a!few!minutes!
! ! ! ! Clinical!expression:! Motor,!Sensory,!Autonomic,!Higher!Cortical,!Psychiatric,!or!Complex!
! ! Unprovoked!Seizure:!
! ! ! ! Seizures!occurring!in!Absence!of!Precipitating!factors!
! Epilepsy:! ! ≥2!Unprovoked!(or!Reflex)!Seizures!occurring!>24h!apart!
! ! ! or! 1!Unprovoked!(or!Reflex)!Seizure!
! ! ! ! +!a!probability!of!further!Seizures!
! ! ! ! ! similar!to!General!recurrence!risk!(≥60%)!after!2!unprovoked!seizures,!
! ! ! ! ! occurring!over!next!10!years!
! ! ! ! (This!definition!is!in!the!new!ILAE!2010!guideline!but!Not!preferred!by!many!neurologists)!
! ! ! or! Dx!of!an!Epilepsy!syndrome!
! ! Considered!to!be!resolved!for!those!who:!
! ! ! ! Had!AgeTdependent!Epilepsy!syndrome!but!Now!past!the!Applicable!Age!
! ! ! or! Those!who!have!remained!SeizureTfree!for!last!10!years,!with!No!Seizure!drugs!for!last!5!years!
! ! Epilepsy!Syndrome:!
! ! ! ! Clinical!entities!–!ILAE!recognizes!>20!electroclinical!syndromes,!
! ! ! ! ! each!defined!by!a!distinctive!combination!of!Clinical!features,!S/S!&!EEG!patterns!
! Convulsion:! Lay!term!of!Motor!episodes!
Semiology!(S/S)!(ILAE!Commission!Report!2001):!
! ! ! ! Classified!by!predominant!Sx:! Motor,!Sensory!(Aura),!Autonomic,!Dialeptic!(Consciousness)!
1.!Motor:!
T!1.1!Elementary!Motor:!
! ! ! ! Single!type!of!contraction!that!is!usually!Stereotyped!&!Not!decomposable!into!phases!
! ! ! ! ! (Note!that!TonicTClonic!is!an!Elementary!Motor!sequence)!
! Tonic:! ! Sustained!↑!in!contraction!lasting!few!seconds!to!minutes!
! ! Epileptic!spasm!(formerly!Infantile!spasm):!
! ! ! ! Sudden!Flexion,!Extension,!or!Mixed!FlexionTExtension!
! ! ! ! ! of!predominantly!Proximal!&!Truncal!muscles!
! ! ! ! ! that!is!usually!more!sustained!than!Myoclonic!but!Not!as!sustained!as!Tonic!(ie.!~1s)!
! ! ! ! Limited!forms:! Grimacing,!Head!Nodding!
! ! ! ! Usually!occur!in!Clusters!
! ! Postural:! Adoption!of!a!posture!that!may!be!Bilaterally!Symmetrical!or!Asymmetrical!(“Fencing!posture”)!
! ! ! Versive:!
! ! ! ! Sustained,!Forced!Conjugate!Ocular,!Cephalic,!Truncal!rotation!or!lateral!deviation!from!midline!
! ! ! Dystonic:!

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! ! ! ! Sustained!contractions!of!both!Agonist!&!Antagonist!muscles!
! ! ! ! ! producing!Athetoid/Twisting!movements!
! ! ! ! When!prolonged,!may!produce!Abnormal!Postures!
! Myoclonic:! Sudden,!Brief!(<100ms)!Involuntary!single/multiple!contractions!of!variable!topography!
! ! –ve!Myoclonic:!
! ! ! ! Interruption!of!Tonic!muscular!activity!for!<500ms!without!evidence!of!preceding!Myoclonia!
! ! Clonic!(Rhythmic!Myoclonus):!
! ! ! ! Myoclonus!that!is!Regularly!Repetitive,!involves!Same!muscle!groups,!
! ! ! ! ! at!a!Frequency!of!~2T3!contractions/s,!and!is!Prolonged!
! ! ! Jacksonian!March:!
! ! ! ! Traditional!term;!Spread!of!Clonic!movements!through!Contiguous!body!parts!Unilaterally!
! TonicTClonic:! Sequence!consisting!of!a!Tonic!followed!by!a!Clonic!phase!
! ! ! ! Variants:! ClonicTTonicTClonic!
! ! Generalized!TonicTClonic!Seizure!(Formerly!Grand!Mal!Seizure):!
! ! ! ! Bilateral!Symmetrical!Tonic!contraction,!then!Bilateral!Clonic!contractions!
! ! ! ! Usually!asso.!with!Autonomic!phenomena!
! Atonic:! ! Sudden!Loss/Diminution!of!muscle!tone!without!apparent!preceding!Myoclonic/Tonic!event!
! ! ! ! ! lasting!≥1!to!2s,!involving!Head,!Trunk,!Jaw!or!Limb!musculature!
! Astatic!(Drop!attack):!
! ! ! ! Loss!of!Erect!Posture!that!results!from!Atonic,!Myoclonic!or!Tonic!mechanism!
T!1.2!Automatism:! Repetitive!Involuntary!SemiTpurposeful!(more!or!less!Coordinated)!motor!activity!
! ! ! ! Usually!occurring!when!Cognition!is!Impaired,!and!Pt!is!usually!Amnesic!for!it!afterward!
! ! ! ! Resembles!Voluntary!movement!
! ! ! ! May!consist!of!an!Inappropriate!Continuation!of!ongoing!preictal!motor!activity!
! Oroalimentary:! Lip!smacking,!Lip!pursing,!Chewing,!Licking,!Tooth!grinding,!Swallowing!
! Mimetic:! ! Facial!expression!suggesting!an!Emotional!state,!often!Fear!
! Manual/Pedal:! Indicates!principally!Distal!components,!Bilateral/Unilateral!
! ! ! ! Fumbling,!Tapping,!Manipulating!movements!
! Gestural:! ! Often!Unilateral!
! ! ! ! Fumbling/Exploratory!movements!with!Hand,!directed!toward!Self/Environment!
! ! ! ! Movements!resembling!those!intended!to!lend!further!Emotional!tone!to!speech!
! Hyperkinetic:! Involves!predominantly!Proximal!limb/Axial!muscles!
! ! ! ! ! producing!Irregular!Sequential!Ballistic!movements,!
! ! ! ! ! eg.!Pedaling,!Pelvic!thrusting,!Thrashing,!Rocking!movements!
! ! ! ! ↑!in!Rate!of!ongoing!movements!or!Inappropriately!Rapid!performance!of!a!movement!
! Hypokinetic:! ↓!in!Amplitude!and/or!Rate!or!Arrest!of!ongoing!motor!activity!
! Dysphasic!
! Dyspraxic!
! Gelastic:! ! Bursts!of!Laughter/Giggling,!usually!without!an!appropriate!affective!tone!
! Dacrystic!(Bursts!of!Crying)!
! Vocal!

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! Verbal!
2.!Sensory:! ! Aura:! ! Subjective!Ictal!phenomenon!that,!in!a!given!Pt,!may!Precede!an!observable!
seizure!
! ! ! ! ! ! If!alone,!constitutes!a!Sensory!Seizure!
! ! ! ! (cf!Prodrome:! Headache,!Vague!Nonascending!Longlasting!Abdominal!sensations,!Dysphoria!
! ! ! ! ! ! ! May!precede!(Focal)!seizures!by!many!minutes!(usually!>5!min))!
T!2.1!Elementary:! Single!Unformed!phenomenon!involving!1!primary!sensory!modality!
! Somatosensory:!Tingling,!Numbness,!ElectricTshock!sensation,!Pain,!Sense!of!movement,!Desire!to!move!
! Visual:! ! Flashing/Flickering!Lights,!Spots,!Simple!patterns,!Scotomata,!Amaurosis!
! Auditory:! ! Buzzing,!Drumming!sounds!or!Single!tones!
! Olfactory:! ! Odor,!usually!disagreeable!
! Gustatory:!! Acidic,!Bitter,!Salty,!Sweet,!Metallic!
! Epigastric:!! Abd!discomfort,!eg.!Nausea,!Emptiness,!Tightness,!Churning,!Butterflies,!Malaise,!Pain,!Hunger!
! ! ! ! Sensation!may!rise!to!Chest/Throat!
! ! ! ! Some!phenomenon!may!reflect!Ictal!Autonomic!dysfunction!
! ! ! ! (Closely!related!to!TLE)!
! Cephalic:! ! Sensation!in!Head,!eg.!LightTheadedness,!Tingling,!Headache!
! Autonomic:! Sensation!consistent!with!involvement!of!ANS!(Autonomic!Aura!=/=!Autonomic!events)!
T!2.2!Experiential:! Affective,!Mnemonic,!or!Composite!perceptual!phenomena!
! ! ! ! ! including!Illusory!or!composite!Hallucinatory!events!
! ! ! ! May!appear!alone/in!combination!
! ! ! ! Feelings!of!Depersonalization!included!
! ! ! ! Subjective!qualities!similar!to!those!experienced!in!life,!but!occurring!outside!of!actual!context!
! Affective:! ! Fear,!Depression,!Joy,!(rarely)!Anger!
! Mnemonic:! Reflect!Ictal!Dysmnesia,!eg.!Feelings!of!Familiarity!(dejaTvu),!Unfamiliarity!(jamaisTvu)!
! Hallucinatory!
! Illusory!
3.!Dyscognitive!
4.!Autonomic:!
! Autonomic!Aura:! Sensation!consistent!with!involvement!of!ANS!
! Autonomic!Seizure:! Objectively!documented!&!distinct!alteration!of!ANS!
! ! ! ! ! ! involving!CV,!Pupillary,!GI,!Sudomotor,!Vasomotor!&!Thermoregularity!functions!
Classification!of!Seizure:! Based!on!Clinical!&!EEG!findings!
! Old!Classification!by!ILAE!1981:!

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! ! ! ! !
! Classification!of!Epilepsy!syndromes!(ILAE!1989):! ! (Below!only!listed!few!of!them)!
! ! LocalizationTrelated!
! ! Generalized:!
! ! ! ! Childhood!Absence!Epilepsy!(pyknolepsy)!
! ! ! ! Juvenile!Myoclonic!Epilepsy!(JME)!
! ! ! ! LennoxTGastaut!syndrome!
! New!Classification!by!ILAE!2010:!
! ! ! ! (No!Longer!use!Simple/Complex!to!describe!Partial!Seizures:!Not!preferred!by!V!Wong,!etc)!

! ! !
! ! ! ! Focal:! ! Initial!activation!of!only!part!of!1!cerebral!hemisphere!
! ! ! ! Generalized:! Involvement!of!Both!cerebral!hemispheres!
! ! Electroclinical!syndromes:!
! ! ! Eg.! Neonatal!onset:!! ! Benign!Familial!Neonatal!Epilepsy!
! ! ! ! Infancy!onset:! ! ! West!syndrome,!Dravet!syndrome!
! ! ! ! Childhood!onset:! ! Febrile!seizure!plus,!Epilepsy!with!Myoclonic!Absences!
! ! ! ! AdolescenceTAdult!onset:! Juvenile!Absence!Epilepsy!
! ! ! ! LessTspecific!Age!group:! Reflex!Epilepsies!
Partial!Seizures:!
! Simple!Partial!Seizures:! No!impairment!of!Consciousness!
! ! ! ! ! ! EEG:!Local!Contralateral!discharge!over!corresponding!area!
! ! Motor:! Focal!Motor!with!March!(Jacksonian)!
! ! ! ! Versive!
! ! ! ! Postural!
! ! ! ! Phonatory!(Vocalization/Arrest!of!speech)!
! ! Sensory:! Somatosensory,!Visual,!Auditory,!Olfactory,!Gustatory,!Vertiginous!

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! ! Autonomic:!
! ! ! ! Eg.!Epigastric!sensation,!Pallor,!Sweating,!Flushing,!Piloerection,!Pupillary!dilation!
! ! Psychic:! Uncommon!(Usually!in!Complex!Partial!Seizures)!
! ! ! ! Dysphasic,!Dynamic!(eg.!Déjà!vu),!Cognitive!(eg.!Dreamy!states,!Distortion!of!Time!sense),!
! ! ! ! Affective!(eg.!Fear),!Illusions!(eg.!Macropsia),!Structured!Hallucinations!(eg.!Music,!Scenes)!
! Complex!Partial!Seizures:! With!impairment!in!Consciousness!(=/=!LOC!though)!
! ! ! ! ! ! EEG:!Unilateral/Bilateral!Asynchronous!discharge,!usually!in!Temporal/Frontal!
region!
! ! ! ! Impairment!of!Consciousness!can!be!present!at!Onset!or!after!a!Simple!Partial!onset!
! ! ! ! ! Not!necessarily!complete!LOC!
! ! ! ! ! Usually!Gradual,!Not!as!Abrupt!as!in!Absence!seizure!
! ! ! ! Preceded!by!Aura!
! ! ! ! Automatisms!
! ! ! ! PostTictal!drowsiness!
! ! ! ! (Often!originate!from!Temporal!lobe,!or!Frontal!lobe)!
! Partial!Seizures!evolving!to!Secondarily!Generalized!Seizures:!
! ! ! ! EEG:!Above!discharges!become!Secondarily!&!Rapidly!Generalized!
! ! ! ! Partial!Seizures!spread!within!1!cerebral!hemisphere!and!then,!via!Corpus!callosum,!
! ! ! ! ! spread!to!Opposite!hemisphere!
! ! ! ! 3!Ways:! Simple!Partial>!Generalized!
! ! ! ! ! ! Complex!Partial>!Generalized!
! ! ! ! ! ! Simple!Partial>!Complex!Partial>!Generalized!
! ! ! ! Types!of!Generalized!Seizures:! GTC,!Tonic,!Clonic!
! ! ! ! PostTictal!state!that!follows!is!often!prolonged!
! ! ! ! Pt!can’t!recall!any!of!this,!other!than!the!Aura!
Generalized!Seizures:!
! Absence!Seizures!(formerly!Petit!Mal!seizure):!
! ! ! ! Abrupt!onset!&!termination:! No!Aura,!No!PostTictal!drowsiness!
! ! ! ! Transient!LOC,!few!seconds!to!30s!
! ! ! ! Interruption!of!ongoing!activities:!
! ! ! ! ! Blank!stare/Brief!flickering!of!eyelids!
! ! ! ! ! Speech!slowed/interrupted,!Unresponsive!when!spoken!to!
! ! ! ! ! Stands!transfixed!if!walking!
! ! ! ! ! If!eating,!food!stops!on!the!way!to!mouth!
! ! ! ! ±!other!components:!Mild!Clonic/Atonic/Tonic,!Automatism,!Autonomic!
! ! ! ! Precipitated!by!Hyperventilation!
! ! ! ! EEG:!3Hz!spike!(Psychiatry!2006!MCQ!9)!
! ! Ddx!from!Daydreaming:!
! ! ! ! Can!occur!in!any!kind!of!setting,!Not!just!when!bored!
! ! ! ! Abrupt!onset!
! ! ! ! Can’t!be!interrupted!once!starts!off!

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! ! ! ! Duration!<30s!(Daydreaming!can!have!variable!duration)!
! Myoclonic!Seizures:!
! ! ! ! Brief!Irregular!ShockTlike!jerks,!over!Limbs,!Face,!Trunk!
! ! ! ! Symmetrical/Asymmetrical!
! ! ! ! Single/In!clusters!(If!Single,!No!acknowledged!LOC!or!Postictal!confusion)!
! ! ! ! Usually!occur!before!going!to!Bed/Just!awakening!from!Sleep!
! ! Syndrome:!
! ! ! ! Juvenile!Myoclonic!Epilepsy!(Janz!syndrome):!
! ! ! ! ! Family!Hx,!Onset!in!Teenage!years!
! ! ! ! Progressive!Myoclonic!Epilepsy:!
! ! ! ! ! Early!Teens,!Rapid!development!of!Ataxia!&!Dementia!
! ! ! ! Familial!Adult!Myoclonic!Epilepsy:!
! ! ! ! ! Adult!onset,!NonTdegenerative!
! Clonic!Seizure:! Repetitive!Rhythmic!Clonic!jerks!(1T2Hz)!
! ! ! ! Consciousness!Impaired!
! ! ! ! Short!PostTictal!phase!
! ! ! ! Cf!Myoclonic!Seizure:!! Sustained,!Rhythmical!jerks!
! Tonic!Seizure:! Generalized!↑!in!Tone;!Rigid,!Violent!Muscular!contractions!
! ! ! ! Limbs!fixed!in!a!strained!position!
! ! ! ! Deviation!of!eyes!&!head!toward!1!side!
! ! ! ! Respiration!arrested>!Cyanosis!
! ! ! ! Pupils!dilate,!Tongue!biting,!Urinary!Incontinence!
! TonicTClonic!Seizure!(formerly!Grand!Mal!seizure):!
! ! ! ! Sudden!onset,!LOC!
! ! ! ! If!with!Aura:! Indicates!a!Partial!onset!that!has!Secondarily!Generalized!
! ! Tonic!phase!(10T30s):!
! ! ! ! Sharp!Tonic!contractions!of!muscles!
! ! ! ! Fall!to!ground!+!Injury!
! ! ! ! Resp!arrest!with!Stridor/Cry,!Later!Cyanosis!
! ! ! ! Tongue!biting,!Urinary!incontinence!
! ! Clonic!phase!(30T60s):!
! ! ! ! Convulsive!movements!of!Limbs!
! ! ! ! Irregular!breathing!&!Remains!Cyanotic!
! ! ! ! Saliva!accumulates!at!mouth!
! ! Deep!Respiration,!Muscles!Relax!
! ! Postictal!phase!(2T30!min):!
! ! ! ! Postictal!drowsiness!(deep!sleep!for!several!hours)!
! ! Gradual!recovery:!
! ! ! ! Muscle!aches,!Headache!
! Atonic!Seizure:! Sudden!↓!in!Postural!tone:!
! ! ! ! ! Head!drop!

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! ! ! ! ! Slackening!of!Jaw!
! ! ! ! ! Dropping!of!a!Limb!
! ! ! ! ! Slumping!to!the!ground>!Injury!
! ! ! ! Lasts!for!<5s:! Very!brief!“Drop!attacks”!
! ! ! ! ! ! ! Brief!LOC!
! ! ! ! Often!follows!a!Myotonic!seizure!
Cause!of!Seizure:!
! NonTEpileptic:! Febrile!Seizure!
! ! ! ! CNS!Infection:! Meningitis,!Encephalitis!
! ! ! ! Metabolic:! ! Hypoglycemia!(need!to!rule!out),!HypoNa/HyperNa,!HypoCa,!HypoMg,!
! ! ! ! ! ! ! Vit!deficiency!(eg.!Vit!B6),!Inborn!Error!of!Metabolism,!Hypoxia,!Hypercapnia,!
! ! ! ! ! ! ! Organ!Failure!
! ! ! ! Head!Trauma!(Accidental/NonTaccidental)!
! ! ! ! Drugs,!Toxin:! Overdose:!! Alcohol,!Others!(eg.!Lignocaine,!AntiCholinergics,!Insulin)!
! ! ! ! ! ! ! Withdrawal:! Alcohol,!AED!
! ! ! ! ! ! ! Drug!abuse!
! ! ! ! ! ! ! Toxins:! ! Heavy!metals!
! Epilepsy:! ! Shift!of!Normal!balance!of!Excitation!&!Inhibition!in!CNS!
! ! ! ! Differences!of!Threshold!in!individuals:!
! ! ! ! ! Genetic!predisposition!
! ! ! ! ! In!Children:! Different!Maturational!stages!of!Brain!
! ! ! ! ! ! ! ! High!Fever!
! ! ! ! Dynamic!Interplay!between!Causes!(Epileptogenic!factors)!&!Triggers!
! ! Epileptogenic!factors:! Specific!changes!in!Neuronal!network!result!in!↓Seizure!threshold!
! ! ! ! *Idiopathic!
! ! ! ! Syndromal!
! ! ! ! Cerebral!damage:! HIE,!Stroke,!Malformation!
! ! ! ! Cerebral!tumor!
! ! ! ! Neurocutaneous!syndrome!(eg.!NF,!TS)!
! ! ! ! Neurodegenerative!disease!
! ! Triggers:!
! ! ! Endogenous!factors:!
! ! ! ! Physical!stress:!
! ! ! ! ! Recent!Febrile!illness,!Sleep!deprivation,!Hormones!(Menstrual!cycle!in!women),!
! ! ! ! ! Electrolyte!disturbance/Low!BG!
! ! ! ! Psychological!stress!
! ! ! Exogenous!factors:!
! ! ! ! Environment:! Specific!time!of!day/night,!Flash/Patterns!
! ! ! ! Substances:! Specific!foods/Caffeine/etc,!Drugs,!AED/Alcohol!withdrawal!
Causes!of!Epilepsy!by!Age:!
! Newborn:!! Perinatal!Hypoxia!&!Ischemia,!ICH,!Trauma,!Acute!CNS!Infection,!

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! ! ! ! Metabolic!causes!(Hypoglycemia,!HypoCa/HypoMg,!Pyridoxine!deficiency),!
! ! ! ! Inborn!errors!of!metabolism,!Maternal!drug!use!
! Infants/Children:!
! ! ! ! Trauma,!CNS!Infection,!Idiopathic,!Syndrome!(Down,!Angelman),!
! ! ! ! Neurocutaneous!syndrome!(NF,!TS),!
! ! ! ! Other!Genetic!disorders!(Primary!Epilepsy!syndrome,!Metabolic/Neurodegenerative!disease),!
! ! ! ! Developmental!disorder,!Progressive!Brain!disease!(Rare),!Illicit!drug!use!(Adolescents)!
Approach!to!Clinical!Dx:!
! Step!1:! ! Describe!the!Paroxysmal!event!
! Step!2:! ! Is!this!a!Seizure?!
! Step!3:! ! NonTepileptic/Epileptic?!
! Step!4:! ! Underlying!Cause?!
Hx:! ! ! ! Describe!the!episode!
! ! ! ! Time!course!(onset,!duration,!frequency,!progression)!
! ! ! ! Previous!episodes!(details,!Tx!given,!any!FU)!
! ! ! ! Asso.!Sx!
! ! ! ! Birth!Hx!(prenatal,!perinatal,!postnatal)!
! ! ! ! G&D,!Vaccination!
! ! ! ! PMH,!Drugs,!Allergy!
! ! ! ! FHx,!Social!Hx!
! Details!of!Seizure!Episodes:!
! ! ! ! PreTictal,!Ictal,!PostTictal!
! ! PreTictal:! Aura!
! ! ! ! Prodrome!
! ! ! ! Trigger:! ! Trauma,!Fever,!Stress,!Sleep!deprivation,!Flashing!Light,!Noise,!Drug,!etc!
! ! ! ! ! ! ! ! (May!guide!Tx!before!giving!AED,!eg.!Restore!Sleep,!Avoid!Flashing!light)!
! ! Ictal:!! Motor:! ! Posture,!Muscle!contraction,!Tongue!bite,!Head/Eye!movement,!Lip!smacking,!
! ! ! ! ! ! ! Focal!Neurological!Sx,!etc!
! ! ! ! Sensory:! ! Visual,!Auditory,!etc!
! ! ! ! Autonomic:! Urinary!Incontinence,!etc!
! ! ! ! Color:! ! Pallor,!Cyanosis!
! ! ! ! LOC!
! ! PostTictal:! Confusion,!Drowsiness!
! ! ! ! Headache!
! ! ! ! Todd’s!paralysis:! Transient!(<48h)!focal!weakness!of!the!part!affected!after!Partial!Seizure!
! ! ! ! Recovery!time!
! ! ! ! Injury!(esp!Head!injury)!
! ! ! ! Amnesia!of!the!episodes!
! Asso.!Sx:!
! ! Febrile!Seizure:!
! ! ! ! Fever,!Infection!foci!

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! ! CNS!Infection:!
! ! ! ! Can!be!NonTspecific!in!Infants:! Poor!feeding,!Lethargy,!Irritability,!Inconsolable!crying!
! ! ! ! Confusion,!Headache,!Photophobia,!Vomiting!
! ! Metabolic:! Hypoglycemic!Sx,!Vomiting/Diarrhea!(Electrolyte!disturbance),!etc!
! ! Brain!SOL:!Headache,!Morning!Vomiting,!Unsteady!gait,!Motor!weakness!
PE:! ! ! ! Aims:! Look!for!underlying!causes!
! ! ! ! ! ! Identify!PostTictal!sequelae:! Eg.!Todd’s!paralysis,!head/tongue/other!injuries!
! ! ! ! Full!Exam:!General,!Neurological!
! ! ! ! ! ! Resp,!Abd,!CVS!
! General:! ! Vital!signs,!GC!
! ! ! ! Dysmorphism,!Neurocutaneous!markers!
! ! ! ! Growth!
! ! ! ! Signs!of!Traumatic!injury!
! Neurological:! Observe!Seizure!episodes!(if!possible)!
! ! ! ! Signs!of!Meningeal!Irritation!
! ! ! ! Signs!of!↑ICP!(eg.!Bulging!Fontanelle,!Papilledema)!
! ! ! ! Focal!Neurological!deficits!
! ! ! ! Neurodevelopment!
Ix:! Simple!Blood!tests:!
! ! ! ! CBC:!! ↑WBC!(Infection),!↓Platelet!
! ! ! ! LRFT:!! Electrolyte!
! ! ! ! BG:! ! Hypoglycemia,!Also!for!comparing!with!LP!glucose!value!
! ! ! ! Ca:! ! HypoCa!
! ! ! ! PO4,!ABG,!etc!
! Septic!workup!if!Fever:!
! ! ! ! Blood!Culture,!LP,!Urinalysis!
! ! ! ! CXR,!NPA,!Throat!swab!
! Metabolic!&!Toxicology!Screening!
! Neuroimaging:! To!detect!&!localize!epileptogenic!lesions!in!brain!
! ! ! ! May!Not!be!indicated!in!Febrile!Seizures,!or!when!Extracranial!causes!are!evident,!
! ! ! ! ! or!when!1st!episode!of!unsuspected!Afebrile!Seizures!
! ! ! ! Can!be!Normal!in!many!causes!of!Seizures/Epilepsy!
! ! Modality:! Structural!scan:!MRI:!! More!Sensitive!than!CT!(eg.!Small!Tumors,!Cortical!dysplasia,!etc)!
! ! ! ! ! ! ! CT:! ! Faster,!Show!Hemorrhage!better!
! ! ! ! Functional!scan!(eg.!PET/SPECT)!
! ! ! MRI:!

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! ! ! ! !
! ! ! ! ! Mesial!Temporal!Sclerosis:!Scarring/Atrophy!within!Hippocampus>!Complex!Partial!
seizure!
! EEG:!! ! Support!Dx,!Guide!Tx!
! ! ! ! Limitations:! Prone!to!Interference!
! ! ! ! ! ! ! High!variability!with!respect!to!Timing!
! ! ! ! ! ! ! 1st!EEG!falsely!–ve!in!50%!Epileptic!Pt!(can!Not!rule!out!Epilepsy)!
! ! ! ! Methods:! Resting!EEG:! With!Stress,!eg.!Hyperventilation,!Photic!stimulation!
! ! ! ! ! ! Videotelemetry!
! Genetic!test!
LT!Mx:!
! Principle:! ! Dx!made!by!a!specialist!
! ! ! ! Tx!guided!by!Seizure!type,!Epileptic!syndrome,!Comedication!&!Comorbidities!
! ! ! ! Begin!with!explanation!&!advice!to!the!family!member!in!decision!making!
! ! ! ! Not!all!Pt!require!Tx,!but!mostly!require!Lifestyle!changes!&!Education!
! ! NB:! ! 25%!Pt!with!a!Single!seizure!will!have!a!Recurrence!seizure!without!following!RF!
! ! ! ! Risk!↑!to!40%!with!≥1!RF!of:! Epileptiform!EEG!pattern!
! ! ! ! ! ! ! ! ! ! Known!Cause!
! Modalities:! NonTpharmacological:! Diet!&!Lifestyle!changes!
! ! ! ! Pharmacological:! ! AED!
! ! ! ! Surgical!&!other!Radical!Tx!
1.!AED:!
! Principle:! ! Indicated!only!when!>1!unprovoked!seizure!
! ! ! ! Monotherapy!recommended!(Fewer!S/E!&!Drug!interaction,!Lower!Cost)!
! ! ! ! Lowest!dosage!possible!
! ! ! ! Only!↓Risk!but!Not!eliminate!
! Risk!of!Recurrence:!
! ! ! ! In!2!years!after!1!unprovoked!seizure:! ! 15T70%!
! ! ! ! RF:! Abnormal!MRI:! Focal!lesion!in!Cortex!&!Limbic!centres!
! ! ! ! ! Abnormal!EEG:! Epileptiform!abnormalities,!Focal!slowing!waves!
! ! ! ! ! PartialTonset!seizure!
! Types!of!Seizure:!
! ! Absence:! Ethosuximide!(Fewer!S/E!than!Valproate)!

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! ! ! ! V!Wong:! Valproate!is!preferred;!Can!treat!both!Absence!&!GTC!too!
! ! Absence!+!other!types!(eg.!TonicTClonic,!Myoclonic):!
! ! ! ! Valproate,!Lamotrigine,!Topiramate!
! ! ! ! Contraindicated:! Carbamazepine,!Gabapentin,!Tiagabine!
! ! Tonic,!Atonic!(eg.!Lennox!Gastaut!syndrome):!
! ! ! ! Broad!spectrum:! Valproate,!Lamotrigine,!Topiramate!
! ! ! ! Others!
! ! Myoclonic,!JME:!
! ! ! ! Broad!spectrum:! Valproate,!Lamotrigine,!Topiramate!
! ! ! ! Adjunctive:! ! Levetiracetam!
! ! ! ! Phenytoin!&!Carbamazepine!contraindicated!
! ! Generalized!unclassified:!
! ! ! ! Valproate,!Lamotrigine,!Topiramate!
! ! Focal!onset:!
! ! ! ! 1st!line:! Carbamazepine!
! ! ! ! 2nd!line:! Lamotrigine,!Topiramate,!Oxcarbazepine,!Tiagabine,!Gabapentin,!Levetiracetam,!
! ! ! ! ! ! Pregabalin,!Zonisamide!
! Drugs:!
! ! Ethosuximide:!
! ! ! ! Action:! Succinimide!
! ! ! ! ! ! Depress!nerve!transmission!in!Motor!cortex,!↑Convulsive!stimuli!threshold!in!CNS!
! ! ! ! Indication:!Only!in!Absence!
! ! ! ! S/E:! ! Common:! N/V,!GI!upset,!Dizziness!
! ! ! ! ! ! Rare:!! Suicidal!thought,!SJS,!SLE!
! ! Valproate:!Action:! ↑GABA!level!in!brain!
! ! ! ! ! ! May!enhance/mimic!action!of!GABA!at!postsynaptic!receptor!sites!
! ! ! ! ! ! May!also!inhibit!Na!&!Ca!channels!
! ! ! ! Indication:!GTC,!Myoclonus,!Generalized!unclassified!
! ! ! ! S/E:! ! Weight!gain,!Hair!Loss,!Sedation,!Thrombocytopenia!
! ! ! ! ! ! Hepatotoxicity,!Teratogenicity,!Pancreatitis!
! ! ! ! ! ! Caution:! Check!Platelet!count!&!Clotting!profile!before!Surgery!
! ! Topiramate:!
! ! ! ! Action:! Inhibit!Neuronal!VoltageTdependent!Na!channels!
! ! ! ! ! ! Enhance!NT!inhibitory!activity!of!GABA!
! ! ! ! S/E:! ! ↓Serum!Bicarbonate,!HypoK,!Liver!Failure,!SJS!
! ! Lamotrigine:!
! ! ! ! Action:! Inhibit!release!of!excitatory!amino!acid!Glutamate!
! ! ! ! ! ! Inhibit!VoltageTsensitive!Na!channel,!which!stabilizes!neuronal!membranes!
! ! ! ! S/E:! ! Be!cautious!of!SJS!
! ! Carbamazepine:!
! ! ! ! Action:! Stabilize!inactivated!state!of!Na!channels,!thereby!making!neurons!less!excitable!

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! ! ! ! Indication:!Focal!seizure,!GTC!
! ! ! ! S/E:! ! Common!>10%:!! N/V!
! ! ! ! ! ! Moderate:!! ! Dry!mouth!
! ! ! ! ! ! Rare:!! ! ! Rash,!SJS,!Hepatic!Failure,!SIADH!
! ! ! ! Check!HLATB*1502!status!to!avoid!TEN!&!SJS!!
! Medically!Refractory!Epilepsy:!
! ! ! ! Definition:!! Failure!to!respond!to!3!AntiConvulsants!
! ! ! ! Newly!proposed!definition!by!ILAE:!
! ! ! ! ! Failure!of!Seizure!freedom!from!trial!of!2!drugs,!either!Monotherapy!or!in!Combination!
! ! ! ! ! Failure!due!to!Poor!Efficacy,!Not!Adverse!effects!
! ! ! ! New!drug!options:! Immunotherapy:!IV!Steroids,!IVIG,!Rituximab!
! Discontinuation:!
! ! ! ! Usually!considered!when!seizure!free!for!2T5!years!
! ! ! ! Relapse!rate!in!Children:! 25%!
! ! ! ! Risks!&!Benefits!of!continuing/withdrawing!AED!therapy!should!be!discussed!
! ! ! ! Should!be!carried!out!slowly!(at!least!2T3!months),!1!drug!at!a!time!
! Monitoring:! Check!Adherence!&!S/E:! Drug!choice,!Dosage!
! ! ! ! No.!of!Recurrent!attacks!
! ! ! ! Blood!tests:! CBC,!Electrolytes,!LFT,!Vit!D!
2.!Surgery:!! ! For!Intractable!Seizures!&!Epilepsy!
! ! ! ! For!wellTlocalized!with!good!EEG,!MRI!&!other!Functional!imaging!findings,!
! ! ! ! ! which!a!surgical!intervention!is!proved!to!be!curative!
! ! ! ! (Not!commonly!done!in!HK)!
! Modalities:! Resection:!! Lesionectomy,!Lobectomy,!Hemispherectomy,!Corticectomy!
! ! ! ! Disconnection:! Callostomy,!Multiple!Subpial!Transection!
! ! ! ! Augmentation:! Vagal!nerve!stimulation!
! ! Resection:!To!resect!an!accurately!localized!part!of!the!brain!
! ! ! ! Lesionectomy:! ! ! Low!grade!Astrocytomas,!Area!of!Focal!Atrophy,!Cortical!dysplasias!
! ! ! ! Temporal!Lobectomy:! Anteromedial!Temporal!Resection!(AMTR)!
! ! ! ! ! ! ! ! ! ! (to!remove!Mesial!structures)!
! ! ! ! Extra!Temporal!Resection!
! ! ! ! Hemispherectomy:! Used!to!do!in!Pt!with!Congenital!Hemiplegia,!SturgeTWeber!syndrome!
! ! ! ! ! Hemispherotomy:!
! ! ! ! ! ! ! ! Leave!Frontal!&!Occipital!lobe!in!places!with!total!nerve!transection!
! ! ! ! ! ! ! ! ! to!prevent!serious!Cx!
! ! Disconnection:!
! ! ! ! Corpus!Callostomy:! Disruption!of!1!or!more!major!CNS!pathway!for!seizure!generation!
! ! ! ! Multiple!Subpial!Transection!(MST):!
! ! ! ! ! ! ! ! Disruption!of!Interneuronal!Synchrony!of!Epileptic!focus!
! ! ! ! ! ! ! ! ! (For!areas!which!dare!not!to!cut!directly;!Make!multiple!slices)!
! ! Nerve!stimulation:!

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! ! ! ! Cerebellar!&!Vagal!nerve!
! Cx:! AMTR:! Hemiparesis,!VF!deficit,!CN!palsy,!Fever,!Verbal!deficits,!Memory!problems!
! ! Corpus!Callosotomy:!
! ! ! ! Hydrocephalus,!Aseptic!Meningitis,!Bleeding!from!the!Superior!Sagittal!sinus,!
! ! ! ! Frontal!lobe!Edema,!Venous!Infarction,!Air!Embolism!
! ! Functional!Hemispherectomy:!
! ! ! ! Hemogenic!Meningitis,!Ventriculitis,!CSF!Leakage,!Hydrocephalus!
! ! ! ! Less!commonly!Stroke,!Infection,!Coma,!PostTop!Hemorrhage!
3.!Diet!&!Lifestyle!modifications:!
! Diet:!Ketogenic!diet:!
! ! ! ! For!those!Refractory!to!AED,!typically!used!in!Children!
! ! ! ! Content:! High!Fat!(nuts,!cream,!butter),!Adequate!Protein,!Low!Carbohydrate!
! ! ! ! Concept:! Low!Insulin>!Burn!Fat>!Ketosis!
! ! ! ! Compliance:! Difficult!to!maintain;!<10%!Pt!continue!the!diet!after!1!year!
! ! ! ! ! ! ! Any!Small!Carbohydrate!intake!resets!Ketone!metabolism!for!2!weeks!
! ! ! ! S/E:! ! Constipation,!Low!grade!Acidosis,!Hypoglycemia,!HyperLipidemia!
! ! ! ! Risks:! Slowed!Growth,!Bone!Fractures,!Kidney!Stones!
! ! ! ! Efficacy:! >50%!children!have!at!least!50%!reduction!in!No.!of!their!seizures!
! ! ! ! ! ! Some!children,!usually!10T15%,!even!become!SeizureTfree!
! ! ! ! ! ! Syndromes,!eg.!Infantile!spasms,!Rett!syndrome,!respond!particularly!well!
! ! ! ! ! ! If!Seizure!well!controlled!for!some!time!(eg.!2!years),!might!suggest!going!off!the!
diet!
! ! Atkins!diet:!
! ! ! ! Low!Carbohydrate!diet!
! ! ! ! No!limit!on!calories/protein!
! ! ! ! Similar!Efficacy!as!Ketogenic!diet!
! Lifestyle!Modifications:!
! ! Assistance:!
! ! ! ! Promote!Independence!&!Confidence!
! ! ! ! Empower!Parents!
! ! ! ! Psychological!help!
! ! ! ! Inform!School!&!Teachers!
! ! Avoidance:!
! ! ! ! Avoid!deep!baths!
! ! ! ! Avoid!swimming!alone!in!deep!water!
! ! ! ! Sit!at!a!distance!from!TV!(for!those!with!Photosensitivity)!
! ! ! ! Power!tools!&!cooking!
! ! ! For!Adolescents!&!Adults:!
! ! ! ! Driving!(only!after!1Tyear!free!of!seizures)!
! ! ! ! Contraception!&!Pregnancy!
! ! ! ! Avoid!Alcohol/Poor!Sleep!

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! ! Lifestyle!Modifications:!
! ! ! ! Noise:! ! ! Use!Earplugs/Ear!phones,!Listen!to!relaxing!music/sounds!
! ! ! ! Bright!Flashing!lights:!Use!Polarized!glasses,!Avoid!Discos!
! ! ! ! Sleep:! ! ! Regulate!Sleeping!habits!
! ! ! ! Exercise:! ! ! Avoid!Hyperventilation,!Stay!well!hydrated!
NB:! ! ! ! Epilepsy!is!protected!under!Disability!Discrimination!Ordinance!
Misc:!
V!Wong:! Juvenile!Myoclonic!Epilepsy:!
! ! ! ! Often!MixDx!as!Partial!Seizure!&!given!Carbamazepine!
! ! ! ! Although!still!Conscious,!can!progress!to!Generalized!Epilepsy!in!the!future!
! ! ! ! Often!precipitated!by!Alcohol,!Sleep!deprivation!
!

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Febrile!Seizure:!
! ! ! ! Occur!in!~3%!children!
! ! ! ! Genetic!predisposition:! 10%!risk!if!1°!relative!with!Febrile!Seizure!
! ! ! ! Usually!occurs!Early!in!Viral!Infection!when!Temp!is!↑ing!rapidly!
Type:!
! Simple:! ! Age!6!months!–!5!years!
! ! ! ! Brief!duration!<15!min!
! ! ! ! Only!once!in!a!24h!period!
! ! ! ! Generalized!(*TonicTClonic)!
! ! ! ! Accompanied!by!Fever!but!without!Intracranial!Infection!(uncommon!but!important!ddx)!
! ! ! ! NeuroDevelopmentally!Normal!
! Complex:! ! Eg.!Focal,!Prolonged,!Repeated!in!the!same!illness!
! ! ! ! ↑Risk!of!4T12%!of!subsequent!Epilepsy!
Ddx:! ! ! ! CNS!Infection:! ! Meningitis,!Encephalitis!
! ! ! ! Encephalopathy:! PostTinfectious!Encephalomyelitis!(ADEM)!
! ! ! ! ! ! ! ! (Precipitated!by!URI,!but!Afebrile!by!the!time;!Diffuse!Slow!waves!in!EEG)!
Mx:! Reassurance!&!Education!(First!Aid!Mx!of!Convulsion)!
! ! Counseling:!
! ! ! ! Risk!of!Recurrence!of!Simple!Febrile!Seizure:!
! ! ! ! ! 1st!Seizure!at!<1!yo:! 50%!
! ! ! ! ! 1st!Seizure!at!>1!yo:! 30%!after!1st!attack,!50%!after!2nd!attack!
! ! ! ! Risk!of!Epilepsy:!
! ! ! ! ! 2%!(vs!1%!in!general!population)!
! Consider!EEG!if!Recurrent!Febrile!Seizures!with!Complex!features!
! Drug:!
! ! AntiPyretic:!
! ! ! ! Only!to!improve!comfort!of!child!
! ! ! ! Ineffective!at!preventing!Recurrent!Febrile!Seizures!
! ! AntiConvulsant:!(Usually!the!current!convulsion!was!already!over;!Mainly!for!future!event)!
! ! ! Tx:! May!prescribe!Rectal!Diazepam!(Rectal!Valium!5!mg!prn,!for!convulsions!>5!min)!
! ! ! ! For!future!abortion!of!an!attack!for!Pt!with!Recurrent!Febrile!Seizures;!Educate!how!to!use!
! ! ! Prophylaxis:!
! ! ! ! Continuous!(Phenobarbital,!Valproate)!or!Intermittent!(Oral!Diazepam!at!onset!of!Fever)!
! ! ! ! Can!prevent!Recurrent!Febrile!Seizure!
! ! ! ! But!Not!generally!recommended,!as!potential!S/E!more!than!benefits!
! ! ! ! ! Phenobarbital:! Behavioral!problems,!Hypersensitivity!
! ! ! ! ! Valproate:!! Fatal!Hepatotoxicity!esp!in!Young!Children!<3!yo,!
! ! ! ! ! ! ! ! Thrombocytopenia,!Weight!Gain,!GI!disturbances,!Pancreatitis!
! ! ! ! ! Diazepam:!! Lethargy,!Drowsiness,!Ataxia!
! ! ! ! ! ! ! ! Sedation!may!mask!evolving!signs!of!CNS!Infection!

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Infantile!Spasm:!
! ! ! ! Can!be!used!to!describe!the!specific!Seizure!manifestation!or!as!a!synonym!for!syndrome!itself!
! ! ! ! West!syndrome:! 2!out!of!the!Triad!
! ! ! ! ! Triad!of:! Infantile!Spasms!
! ! ! ! ! ! ! Pathognomonic!EEG!pattern!(Hypsarrhythmia)!
! ! ! ! ! ! ! ! Chaotic!pattern!of!HighTvoltage!Slow!waves!with!Sharp!Multifocal!waves!
! ! ! ! ! ! ! Mental!Retardation!
! ! ! ! Usually!begins!at!4T8!months!
Cause:!
! Symptomatic:! Related!directly!to!several!prenatal,!perinatal!&!postnatal!factors! !
! ! ! ! Virtually!any!disorder!that!can!produce!brain!damage!can!be!asso.!with!Infantile!spasms!
! ! Examples:!Neurocutaneous!syndromes!esp!Tuberous!sclerosis!
! ! ! ! HypoxicTischemic!Encephalopathy!
! ! ! ! Congenital!Infection/CNS!Infection!
! ! ! ! Head!Trauma!
! ! ! ! Inborn!errors!of!metabolism!
! ! ! ! Cytoarchitectural!abnormalities!including!Lissencephaly!&!Schizencephaly!
! ! ! ! Down!syndrome!(Clinically!more!likely!Milder!&!Respond!better!to!Drugs)!
! ! ! ! SturgeTWeber!syndrome!
! Cryptogenic!
! (Idiopathic):! Some!may!classify!Cryptogenic!as!with!Occult!cause,!while!Idiopathic!is!truly!Idiopathic!
! ! ! ! Some!may!just!classify!both!as!Cryptogenic!
Clinical:! ! ! Triad!of!Attack!types!(Wiki):! Lightning!attacks,!Nodding!attacks,!Salaam!attacks!
! ! ! ! Often!Violent!Flexor!spasms!of!Head,!Trunk!&!Limbs!
! ! ! ! ! followed!by!Extension!of!Arms!(‘Salaam!spasms’)!

! ! ! ! ! !
! ! ! ! Flexor!spasms!1T2s,!often!Multiple!bursts!of!20T30!spasms!many!times!a!day,!often!on!Waking!
Tx:! ! ! ! Vigabatrin!
! ! ! ! Steroid,!ACTH;!Suppress!CRH!production!(Excessive!CRH!secretion!is!1!theory!of!causing!Spasm)!
! ! ! ! Others!(eg.!Ketogenic!diet!in!refractory!cases)!
Poor!Prognosis:!! Mental!Retardation!
! ! ! ! Some!Pt!may!suffer!from!Epilepsy!later!in!life!
! ! ! ! ! May!turn!into!Focal/Generalized!Epilepsy!
! ! ! ! ! May!develop!LennoxTGastaut!syndrome!(classically!have!Drop!attacks)!

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Benign!Rolandic!Epilepsy:!
! ! ! ! (2015!SAQ!6)!
! ! ! ! Most!common!Epilepsy!syndrome!in!Childhood!(15%!of!all!childhood!epilepsies)!
! ! ! ! Benign!as!most!children!will!outgrow!it!
! ! ! ! ! Starts!at!Age!3T13,!Peak!at!8T9,!Stops!at!Age!14T18!
Clinical:! ! ! Infrequent,!often!Single!Focal!Seizures!
! ! ! ! ! Unilateral!Facial!SensoriMotor!Sx!(30%)!
! ! ! ! ! OropharyngoLaryngeal!manifestations!(53%)!
! ! ! ! ! Speech!Arrest!(40%)!
! ! ! ! ! Hypersalivation!(30%)!
! ! ! ! Lasts!<2!min;!Child!is!Fully!Conscious!
Ddx:! ! ! ! Temporal!lobe!Epilepsy,!LandauTKleffner!syndrome!(Aphasia!with!Convulsive!disorder)!
EEG:!! ! ! CentroTemporal!spikes!(Typically!High!voltage!spikes!followed!by!Slow!waves)!
Tx:! ! ! ! May!Not!be!needed!
! ! ! ! AntiEpileptics!may!be!considered!(eg.!Carbamazepine)!
Prognosis:!! ! Most!children!stop!having!seizures!2T4!years!after!they!begin!
! ! ! ! Virtually!all!children!will!outgrow!it!by!adolescence!(Age!of!15)!

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 Rheumat&JIA&
Terminology!of!Juvenile!Arthritis:!
! ! ! ! Juvenile!Chronic!Arthritis!(JCA)!
! ! ! ! ! European!League!of!Associations!for!Rheumatology!(EULAR)!
! ! ! ! Juvenile!Rheumatoid!Arthritis!(JRA)!
! ! ! ! ! American!College!of!Rheumatology!(ACR)!
! ! ! ! Juvenile!Idiopathic!Arthritis!(JIA)!(Newest!term)!
! ! ! ! ! International!League!of!Associations!for!Rheumatology!(ILAR)!
Classification:! ! Dx!of!JIA!can!only!be!made!with!Age!limit!(Onset!<16!yo)!&!Duration!limit!(≥6!weeks)!
! ACR!1977:!! PauciTarticular!(≤4!joints)!
! ! ! ! ! Type!1:! Girls!>!Boys!
! ! ! ! ! ! ! Associated!with!ANA!Iritis!
! ! ! ! ! Type!2:! Boys!>!Girls!
! ! ! ! ! ! ! Associated!with!HLA!B27!
! ! ! ! Polyarticular!(≥5!joints)!
! ! ! ! ! RF!+ve!
! ! ! ! ! RF!–ve!
! ! ! ! Systemic!onset!
! ILAR!1997:!! Oligoarticular:! F>M!
! ! ! ! ! *Persistent:! ≤4!joints!for!course!of!disease!
! ! ! ! ! Extended:!! >4!joints!after!6!months!
! ! ! ! Polyarticular!(>4!joints):! F>M!
! ! ! ! ! RF!–ve!(usually!<10!yo)!
! ! ! ! ! RF!+ve!(usually!≥10!yo)!
! ! ! ! Systemic!onset:!F~M!
! ! ! ! Psoriatic!
! ! ! ! EnthesitisTrelated:! M>F!
! ! ! ! Undifferentiated!
Oligoarticular!JIA:! ≤4!affected!joints!during!first!6!months!of!illness!
! Epidemiology:! F>M!
! ! ! ! Peak!Incidence:!2T3!yo!
! Clinical:!
! ! Joint:! Affect!Larger!joints!(and!esp!Weight!bearing!joints):! Knees,!Ankles;!Also!Wrists,!Elbows!
! ! ! ! ! <4!joints,!often!just!1!joint!
! ! ! ! ! Virtually!never!begins!in!Hips!
! ! ! ! Appear!well!despite!ambulating!with!a!Limp!
! ! ! ! ! Typical!complaint!is!Morning!Stiffness/Gelling!(Stiffness!after!long!periods!of!Inactivity)!
! ! ! ! ! Joint!Pain!may!Not!be!a!predominant!complaint!in!Small!Children!
! ! ! –ve:! Small!joints!involvement!in!Hands!suggests!eventual!Polyarticular!JIA!or!Psoriatic!Arthritis!
! ! ! ! Dactylitis/Diffuse!Tenosynovitis!of!digits!(Sausage!digit)!suggests!Psoriatic/EnthesitisTrelated!
! ! ! ! Systemic!manifestations!(other!than!Uveitis)!are!characteristically!Absent!

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Juvenile! idiopathic! Arthritis!
! ! ! Cx:! Leg!Length!Discrepancy:!
! ! ! ! ! Asymmetrical!Arthritis>!Chronic!InflammationTrelated!Hyperemia>!Overgrowth!of!limb!
! ! ! ! Muscle!Atrophy!(esp!Extensor!muscles)!
! ! ! ! Flexion!Contractures!
! ! Eye:! ! Anterior!Uveitis:! Present!in!<20%!Pt,!usually!for!those!with!ANA!+ve!
! ! ! ! ! ! ! ! Typically!Asymptomatic!at!onset!(Need!screening)!
! ! ! ! ! ! ! ! (Acute!Symptomatic!Anterior!Uveitis!may!suggest!EnthesitisTrelated)!
Polyarticular!JIA:! >4!affected!joints!during!first!6!months!of!illness!
! ! ! ! Subclassified!by!Presence/Absence!of!RF!
! Epidemiology:! F>M!
! ! ! ! Bimodal!distribution:!1st!Peak:! 2T5!yo!
! ! ! ! ! ! ! ! 2nd!Peak:! 10T14!yo!
! Clinical:!
! ! Joint:! Weight!bearing!joints!(Knees,!Ankles,!Wrists!most!typical)!
! ! ! ! Symmetrical!involvement!of!Small!joints!in!Hands!common!
! ! ! ! Cervical!spine:! Often!Asymptomatic;!May!lead!to!Cervical!spine!Fusion!(↓Extension)!
! ! ! ! TMJ:!! ! Often!Asymptomatic;!May!lead!to!Micrognathia!
! ! Eye:! ! Uveitis!can!occur!but!Less!common!than!in!Oligoarticular!JIA;!↑Risk!if!ANA!+ve!
! ! Rheumatoid!Nodules:!
! ! ! ! May!be!present!in!RF!+ve!Polyarticular!JIA!
Systemic!onset!JIA:! Aka!Still’s!disease!
! Epidemiology:! F~M!
! ! ! ! No!particular!peak!age!of!onset!
! Clinical:! ! Most!difficult!to!diagnose!
! ! ! ! ! Arthritis,!although!necessary!for!a!definitive!Dx,!may!Not!be!evident!in!Early!disease!
! ! ! ! ! Constitutional!Sx!++!
! ! ! ! ! ! IllTlooking,!Spiking!Fever,!Rashes,!High!WBC,!Anemia!
! ! ! ! ! ! Major!Ddx:! Infection,!Malignancy!(Leukemia)!
! ! Joint:! Any!No.!of!joints!may!be!involved!(similar!to!Polyarticular)!
! ! ! ! May!be!present!at!onset!of!Fever!&!Rash!or!may!lag!by!months!
! ! ! ! ! (Even!present!at!Onset,!usually!just!Arthralgia!at!the!beginning)!
! ! Fever:! High!Spiking!(Swinging)!Fever!(SoJIA!commonly!presents!as!FUO)!
! ! ! ! ! Predictable!pattern:! Typically!once/twice!each!day,!at!about!the!same!time!of!day!
! ! ! ! ! ! ! ! ! Temp!will!return!to!Normal/Below!Normal!after!the!spike!
! ! ! ! Children!are!characteristically!illTappearing!when!Febrile,!
! ! ! ! ! but!seem!to!improve!dramatically!when!their!Temp!is!Normal!
! ! Rash:! Salmon!Pink!Macular!Rash:!
! ! ! ! ! Often!Linear;!Mainly!over!Trunk!&!Extremities,!but!may!present!anywhere!
! ! ! ! ! Asso.!with!Fever!spikes!
! ! ! ! ! ! Brought!out!by!Heat;!Evanescent,!lasting!only!a!few!hours!
! ! ! ! ! Usually!Not!Itchy!

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! ! Others:! HepatoSplenomegaly,!Lymphadenopathy!(esp!Axillary!LN)!
! ! ! ! Generalized!Myalgia!(May!have!Muscle!Tenderness!on!Palpation)!
! ! ! ! Serositis:! Pericardial!Effusion,!Pleural!Effusion!
! ! ! ! (Chronic!Headaches!&!other!Neurological!Sx)!
! ! ! ! (Very!Low!risk!of!Uveitis)!
! Cx:! Pericarditis!(often!present!with!Orthopnea!&!respond!to!IV!Steroid)!
! ! Hemolytic!Anemia!
! ! Macrophage!Activation!syndrome!(Rare!but!Severe!Cx):!
! ! ! ! High!Fever,!HepatoSplenomegaly,!Lymphadenopathy!
! ! ! ! Pancytopenia!(cf!SoJIA!originally!should!just!be!Anemia!with!↑Neutrophils!&!Platelets)!
! ! ! ! ↑Liver!enzymes,!↑Ferritin,!↑Triglyceride,!↓Fibrinogen,!DICTlike!picture!
! ! ! ! ESR!Low!despite!Marked!Systemic!Inflammation!due!to!Low!Fibrinogen!level!
! ! ! ! Release!of!massive!amount!of!Cytokines!can!lead!to!Hypotension!&!CNS!disease!
! ! Endarteritis!
Tx:! Medical:!
! ! NSAID:! 1st!line!
! ! ! ! Eg.!Naproxen,!Ibuprofen!
! ! Steroid:! IntraTarticular!Steroid!Injection:!
! ! ! ! ! Useful!for!Monoarticular/Oligoarticular!Arthritis!
! ! ! ! ! Sometimes!indicated!in!Polyarticular!JIA!with!particular!symptomatic!joints!too!
! ! ! ! Systemic!Steroid:!
! ! ! ! ! Generally!Avoided!
! ! ! ! ! (May!consider!in!Systemic!JIA!with!severe!Sx!Not!controlled!by!NSAID,!
! ! ! ! ! ! eg.!MAS,!Symptomatic!Serositis,!Myocarditis)!
! ! DMARD:!
! ! ! Conventional:!
! ! ! ! Methotrexate:! 1st!choice!of!DMARD!
! ! ! ! ! ! ! Folic!acid!may!be!given!to!alleviate!S/E!of!GI!upset!&!Mucosal!Stomatitis!
! ! ! ! Leflunomide,!Sulphasalazine!(Avoid!in!SoJIA>!MAS),!Hydroxychloroquine,!Cyclosporine!
! ! ! Biologics:!
! ! ! ! AntiTTNFα!(Etanercept,!Infliximab),!AntiTIL1!(Anakinra),!AntiTIL6!(Tocilizumab)!
! ! Autologous!HSCT:!
! ! ! ! Experimental;!Reserved!for!Pt!with!most!severe!disease!unresponsive!to!standard!Tx!
! ! ! ! Procedure!carries!Mortality!risk,!usually!from!MAS!
! Physical!&!Occupational!therapy:!
! ! ! ! Maintenance!of!Joint!function:!
! ! ! ! ! Splints,!ROM!Exercise!(Active/Passive),!Posture/ProneTlying!Exercise!
! ! ! ! Adequate!Rest/Exercise:! Balanced!PA,!Selected!Peer!Sports!(Tricycle/Swimming)!
Course!&!Prognosis:! Arthritis!resolves!in!40T50%!Children!
! ! ! ! If!well!after!6!months,!likely!to!remain!well!
! ! ! ! 1/3!Children!with!Systemic!JIA!remain!ill!for!a!prolonged!period!of!time!–!Guarded!Prognosis!

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