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  • 8/11/2019 Pedia - CNS Infection, Seizures, NMD [Agrava]

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    Highlight = Memorize per Dr. Agrava

    Table of ContentsInfections of the CNS ................................... 2

    DefinitionsBacterial Meningitis

    EtiologyNewborn period2 months 12 years

    Neisseria meningitidesHaemophilus influenza type bRisk FactorsPathophysiology of Bacterial Meningitis .................... 3Clinical ManifestationsLumbar PunctureContraindications for LP

    5 Absolute, 1 relative CINormal CSFTypical CSF Findings in Meningi tis ............................ .......... 4T602-1(partial) CSF Findings in Meningitis

    Techniques in DiagnosisNeurodiagnostic Procedures

    Treatment ................................................................. 5Recommendations for Empiric antibiotic Therapy in Bacterial

    MeningitisPathogen-specific therapy for Meningitis

    Complications of Bacterial MeningitisPrognosis

    Poor PrognosisPrevention ................................................................ 6

    N. meningitidesH. Influenzae Type BS. Pneumoniae

    Tuberculous MeningitisPathophysiologyClinical Features of TB MeningitisRadio diagnostic criter ia ............. .............. ............... .. 7Diagnosis

    ClinicalCT ScanLumbar puncture

    InvestigationOther Forms of CNS TBManagement ............................................................. 8Treatment

    Viral Meningoencephalitis ................................... 9VE Etiology

    Enteroviruses

    Mumps virusArbovirusesHerpes Family

    Pathology and pathogenesisTissue sectionsPredilection

    Clinical manifestationDiagnosis

    Tb Diagnostic proceduresLaboratory findings ............................................................ 10

    TreatmentPrognosis

    Possible ComplicationsEosinophilic meningitis

    EtiologyOthersNoninfectious causes include

    Clinical ManifestationsDiagnosisTreatmentPrognosis

    Brain Abscess .................................................. 11CausesPathology

    Location and sourceClinical manifestationDiagnosis

    CT with contrast and MRITreatment

    Surgery is indicatedPrognosis

    Seizures in Chi ldhood ................................ 12EpidemiologyEvaluation of the First Seizures

    HistorySeizure typeExamination

    Febrile seizures ................................................ 13FS Recurrent Seizures

    Factors assoc with !recurrence risk!Risk of developing epilepsy

    FS Treatment

    Unprovoked SeizuresUS First seizure

    Recurrent SeizuresRS Etiology

    Diagnostic Evaluation of First SeizuresFebrileUnprovokedRecurrent

    Intl Classification of Epileptic Seizures .................. 14Epileptic syndromesPartial seizures

    SIMPLE partial seizuresCOMPLEX partial seizures

    AutomatismComplex partial seizures with 2generalizationDiagnosis

    Benign Partial Epilepsy w/Centrotemporal Spikes(BPEC)

    Clinical FeaturesDiagnosisTreatment

    Rasmussen EncephalitisGeneralized Seizures ......................................... 15

    Absence seizuresAbsence TypicalAbsence Atypical

    Generalized Tonic-clonic SeizuresGeneralized Seizures Precipitating factors

    Myoclonic Epilepsies of ChildhoodBenign myoclonus in infancyTypical myoclonic epilepsy of early childhoodComplex Myoclonic Epilepsies of ChildhoodJuvenile Myoclonic Epilepsy (Janz syndrome)Progressive Myoclonic Epilepsies

    Infantile spasms ...................................................... 16CryptogenicSymptomaticTheories

    Landau-Kleffner Syndrome (LKS)LKS DxLKS Tx

    Mechanism of SeizuresTwo hypotheses

    Use of EEG in diagnosing EpilepsyProlonged EEG monitoring with simultaneous closed-circuit video recording

    Treatment of Epil epsy ........................ ................ 17Pharmacologic

    Indications for anticonvulsant drug monitoringProminent risk factorsNon pharmacologic

    Ketogenic dietSurgery for epilepsyVagal nerve stimulation

    Neonatal Seizures ............................................. 18Focal seizuresMultifocal clonicTonic seizuresMyoclonic seizuresSubtle seizures

    NS EEG ClassificationClinical sz w/ consistent EEG eventClinical sz w/ inconsistent EEG eventElectrical sz w/ absent clinicalseizures

    NS Etiologic DiagnosisDiagnostic ExamsF593-3 An approach to the child with a suspected convulsive

    dsoCauses by AgeInborn errors of metabolism ................................................19Unintentional injection of local anestheticBenign familial neonatal seizuresFifth-day fitsPyridoxine dependency

    Drug withdrawal seizuresStatus epilepticusSE Major subtypes

    Febrile seizureIdiopathic status epilepticusSymptomatic status epilepticus

    SE PathophysiologyGeneral Principles in the Management of StatusEpilepticusSE Treatment

    First line drugSecond line Drugs

    Conditions that MimicSeizures ............................ 20Benign paroxysmal vertigoNight terrorsBreath holding spells

    Cyanotic spellsPallid spells

    Paroxysmal kinesigenic choeoathetosisShuddering attacks

    Benign paroxysmal torticollis of infancyHereditary chin tremblingNarcolepsy & CataplexyRage attacks or episodic dyscontrol syndromeMasturbationPseudoseizuresSyncope .................................................................. 2

    Simple syncopeCough syncopeProlonged QT syndrome

    Neuromuscular Diseases.............................. 22Evaluation and Investigation

    Components of the motor unitsInnervation

    Nervous systemClinical Manifestations

    Neuropathic or MyopathicLaboratory Findings

    Serum Creatinine kinase (CK) levelsOtherDistinguishing Features of Dsos of Motor System ............... 23

    Patterns of Weakness and Localization in Floppy InfantsDevelopmental Dso of Muscle ............................. 24

    DiseasesDisease

    Transmission LocusMuscular Dystrophies

    Duchenne & Beckers Muscular Dystrophy .............. 25Duchenne muscular dystrophyBecker muscular dystrophyD&B Clinical M anifestationLab Findings ...................................................................... 26

    DiagnosisGenetic Etiology and PathogenesisTreatmentNutritional State

    Emery-Dreifuss Muscular DystrophyClinical ManifestationsDiagnosisTreatment

    Myotonic Muscular Dystrophy (Steinert dse)MMD TypesClinical Manifestations

    Periodic Paralyses (Potassium-Related) .................... 27PP Treatment

    Congenital Myotonic DystrophyLab FindingsDiagnosis ........................................................................... 2

    Treatment

    Myotonic chondrodystrophy (Schwartz-Jampel dse)Myotonia congenital (Thomsen disease)Paramyotonia

    Guillain-Barr SyndromeClinical Manifestations

    Bulbar Involvement 50%GBS vs. Polio

    GBS VariantsMiller-Fisher SyndromeChronic Relapsing/Unremitting Polyradiculoneuropathy .... 29

    Congenital Guillain-Barr syndromeLab Findings and Diagnosis

    CSF studiesOther

    TreatmentPrognosis

    Endocrine and Toxic MyopathiesThyroid Myopathies

    HyperthyroidismHypothyroidismTreatment

    HyperparathyroidismSteroid-Induced MyopathyHyperaldosteronism (Conn syndrome)

    Chronic Growth Hormone ExcessToxic Myopathies .................................... ......... 3

    Malignant HyperthermiaDSO of NM Transmission & of Motor Neurons

    NMJ dso: Myasthenia GravisJuvenile MGTransient MGCongenital MGDiagnosis

    Anterior Horn Cell: Spinal Muscular Atrophy (SMA)Perinatal SMA (Type 0)Infantile SMA (Type1, Werdnig-Hoffman Dse)Juvenile SMA (Type 2)Adult SMA (Type 3, Kugelberg-Welander Dse)DiagnosisTreatment

    Course: Pediatrics

    Week: 7

    Lecturer: Dr. Agrava

    Topic: CNS Infections

    Seizures and Neuromuscular

    Diseases

    Source: Lecture

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    Infections of the CNS

    DEFINITIONS

    BACTERIAL MENINGITIS

    Etiology

    o

    Newborn period

    2 months 12 years

    !

    o

    o

    ! o o

    o o o

    Neisseria meningitides

    o

    o

    o o

    Haemophilus influenza type b

    o

    !

    Risk Factors

    o o

    Risk Factors for Common Orgs

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    Pathophysiology of Bacterial Meningitis

    o

    o

    o

    ! !

    o !!

    o

    "

    "

    " "

    o

    o

    "

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    Clinical Manifestations

    Neonates Infants Older Children and Adults

    Very non-specific

    Fever or hypothermia

    Seizures

    Lethargy or irritability

    Vomiting, poor feeding or feeding intolerance

    Respiratory distress, apnea

    Diarrhea

    Fever - 50%

    Seizures - 40%

    Poor feeding

    Abdominal distention

    Respiratory distress

    Bulging anterior fontanelle - 30%

    Altered sensorium

    Classic Triad:

    Fever

    Headache

    Nuchal rigidity

    Lumbar Puncture

    Contraindications for LP

    5 Absolute, 1 relative CI !

    Normal CSFPRETERM TERM INFANTS/CHILD

    WBC (cell/mm3)Mean

    -2SD%PMN

    9.0

    0-25.457.2%

    8.2

    0-22.461.3%

    0

    0-70

    Glucose (mg/dl)

    MeanRange

    5024.63

    5232-119

    >4040-90

    CSF glucose/Blood glucose

    MeanRange

    7455-105

    8144-248

    5040-60

    Protein (mg.dl)

    MeanRange

    11565-150

    9020-170

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    Typical CSF Findings in Meningitis

    o

    Bacterial Viral Fungal Tuberculous

    Opening pressure Normal or High Normal Normal or High Usu High

    WBC count (cells/mm3) 1,000 10,000 80

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    Subdural effusion !focal seizures; note thickened sulci = pus

    Meningitis; thickened; whitish material is exudate

    TreatmentRecommendations for Empiric antibiotic Therapy in Bacterial Meningitis

    Pathogen-specific therapy for Meningitis

    Complications of Bacterial Meningitis

    Prognosis !"

    Poor Prognosis #

    !

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    Sensorineural hearing loss

    o o

    o

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    PreventionN. meningitides H. Influenzae Type B S. Pneumoniae

    o

    o

    TUBERCULOUS MENINGITIS

    Pathophysiology #

    ! $

    Rupture of these encapsulated foci results in spread of infection intothe subarachnoid space

    Clinical Features of TB Meningitis

    Inferior brain surface showing exudate in basal cisterns

    Stage

    INon-specificsymptoms -fever, lethargy

    Stage

    2Confusion,cranial nerveparesis,`meningism!arteritic

    Complications:hemiparesis,ataxia,dysarthria

    Stage

    3Coma localising,flexing orextending topain,opisthotonos

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    Radio diagnostic criteria

    !

    Normal vs Tuberculous Meningitis

    Enhancement in basal exudates of Tb meningitis as shown in CT Scan

    DiagnosisClinicalCT Scan

    Lumbar puncture

    o

    " o

    Investigation

    o "

    Other Forms of CNS TB

    o

    4yo/F, prolonged fever, ataxia, vomiting, headache; thought it was atumor in the cerebellum but it was actually caseation necrosis

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    VIRAL MENINGOENCEPHALITIS

    o

    o

    VE Etiology

    Enteroviruses

    Mumps virus

    Arboviruses

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    Pathology and pathogenesis

    Tissue sections

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    Predilection $ $

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    Clinical manifestation

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    Diagnosis

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    Laboratory findings>6R

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    Treatment

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    Prognosis

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    Possible Complications

    o ! o

    EOSINOPHILIC MENINGITIS

    Etiology

    Others

    Noninfectious causes include:

    Clinical Manifestations !

    o

    o o o o

    o o

    o o

    o o o o

    Diagnosis

    o

    o

    Treatment

    o

    ! ! "

    Prognosis

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    BRAIN ABSCESS

    Causes

    Pathology

    o

    Location and source

    Clinical manifestation

    o

    o o

    o

    Diagnosis

    o $

    o o

    o

    CT with contrast and MRI

    o

    o

    Large multiloculated frontal lobeabscess showing ring enhancement andmidline shift as seen on CT scan

    Hypoechoic 6.7 x 5.2 cm.Oval ParietoTemporal Lesion

    Treatment

    !

    !

    ! !

    Surgery is indicated

    o !

    Prognosis

    o o o

    o o o o o

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    BacterialMeningitis

    Tuberculous Meningitis Viral Encepahlitis

    S/sx

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    FEBRILE SEIZURES

    o o

    FS Recurrent Seizures

    Factors assoc with !recurrence risk

    !Risk of developing epilepsy

    FS Treatment

    o

    o o o

    o

    UNPROVOKED SEIZURES

    o

    US First seizure

    RECURRENT SEIZURES

    RS Etiology !

    !

    o

    !o o o o

    o

    DIAGNOSTIC EVALUATION OF FIRST SEIZURES

    Febrile Unprovoked Recurrent

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