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    Epilepsy 22th Oct

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    هلا دلبح
    This document discusses differentiating between epileptic and non-epileptic behaviors in children. It outlines key questions to ask about episodes to determine their nature, like duration and triggers. Common childhood seizure types and non-epileptic conditions are described, including their typical presentations and diagnostic testing approaches. The document emphasizes that not all stereotyped behaviors are epileptic, and that a thorough history is most important for diagnosis over reliance on tests like EEG alone. It cautions against therapeutic trials of anticonvulsants for diagnostic purposes.

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    Epilepsy 22th Oct

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    هلا دلبح
    38 views26 pages
    This document discusses differentiating between epileptic and non-epileptic behaviors in children. It outlines key questions to ask about episodes to determine their nature, like duration and triggers. Common childhood seizure types and non-epileptic conditions are described, including their typical presentations and diagnostic testing approaches. The document emphasizes that not all stereotyped behaviors are epileptic, and that a thorough history is most important for diagnosis over reliance on tests like EEG alone. It cautions against therapeutic trials of anticonvulsants for diagnostic purposes.

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    This document discusses differentiating between epileptic and non-epileptic behaviors in children. It outlines key questions to ask about episodes to determine their nature, like duration and triggers. Common childhood seizure types and non-epileptic conditions are described, including their typical presentations and diagnostic testing approaches. The document emphasizes that not all stereotyped behaviors are epileptic, and that a thorough history is most important for diagnosis over reliance on tests like EEG alone. It cautions against therapeutic trials of anticonvulsants for diagnostic purposes.

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    38 views26 pages

    Epilepsy 22th Oct

    Uploaded by

    هلا دلبح
    This document discusses differentiating between epileptic and non-epileptic behaviors in children. It outlines key questions to ask about episodes to determine their nature, like duration and triggers. Common childhood seizure types and non-epileptic conditions are described, including their typical presentations and diagnostic testing approaches. The document emphasizes that not all stereotyped behaviors are epileptic, and that a thorough history is most important for diagnosis over reliance on tests like EEG alone. It cautions against therapeutic trials of anticonvulsants for diagnostic purposes.

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    Epileptic and non-epileptic

    behaviours in children's

    Motee Ashhab,MD,DIU
    Pediatric Neurologist/ Neurophysiologist
    Makassed hospital- Jerusalem
    Caritas Baby hospital –Bethlehem
    A child presents with
    stereotyped ‘neurological’ behaviours

     How many episodes?


     Different types of episodes?
     What time of the day? Relationship to sleep?
     Triggers?
     How does it begin? Paroxysmal? How long does it last?
     Color change?
     Motor or non-motor phenomena?
     What did the child itself realize?
     Post-ictal symptoms? Tiredness, focal weakness?

    2
    Clinical symptoms of seizures
    Focal seizures:
    Motor
    Sensory
    Autonomic
    Psychotic

    33
    Causes of Epilepsy

    1-Secondary(Lesional,organic ]

    2-Primary Functional [genetic] syndromic


    Familial and Idiopathic Epilepsy (32 Genes)
    ADRA2B, ALDH7A1, CACNA1A, CACNB4, CHRNA2, CHRNA4, CHRNB2,CNTN2, CPA6, DEPDC5, GABRA1, GABRB3, GABRG2, GAL,
    GRIN2A,KCNA1, KCNMA1, KCNQ2, KCNQ3, KCNT1, LGI1, PNKD, PRRT2, RELN,SCN1A, SCN1B, SCN2A, SCN8A, SLC1A3, SLC2A1,
    STX1B, TBC1D24
    Epileptic Encephalopathies (75 Genes)
    AARS, ALDH7A1, ALG13, AMT, ARHGEF9, ARX, BRAT1, CACNA1A, CASK,CDKL5, CHD2, CNNM2, DNM1, DOCK7, EEF1A2, FOXG1,
    GABRA1,GABRB3, GAMT, GLDC, GNAO1, GNB1, GPHN, GRIN1, GRIN2A, GRIN2B,HACE1, HCN1, IQSEC2, ITPA, KCNA2, KCNB1,
    KCNQ2, KCNT1, MBD5,MECP2, MEF2C, MOCS1, MOCS2, NECAP1, PCDH19, PIGA, PLCB1,PNKP, PNPO, POLG, PURA, QARS,
    ROGDI, SCN1A, SCN2A, SCN8A, SIK1,SLC12A5, SLC13A5, SLC25A22, SLC2A1, SLC35A2, SLC6A1, SLC6A8,
    SLC9A6, SPTAN1, ST3GAL3, ST3GAL5, STXBP1, SYN1, SYNGAP1, SZT2,TBC1D24, TSC1, TSC2, UBE3A, WDR45, WWOX, ZEB2
    Progressive Myoclonic Epilepsy (29 Genes)
    AFG3L2, ASAH1, ATP13A2, CARS2, CERS1, CLN3, CLN5, CLN6, CLN8,CSTB, CTSD, CTSF, DNAJC5, EPM2A, GOSR2, GRN, KCNC1,
    KCTD7,LMNB2, MFSD8, NEU1, NHLRC1, PPT1, PRDM8, PRICKLE1, PRICKLE2,SCARB2, SERPINI1, TPP1

    4 4
    Functional (Genetic)Epielpsy

    5
    Genetic (syndromic) Epilepsy

    6
    Benign myoclonic epilepsy in
    infancy
    Developmentally normal infants
    Age 4 months to 3 years
    Short rapid myoclonic seizures – shock like , jerks
    Interictal EEG could be normal
    Ictal EEG shows generalised polyspikes
    Good response to AED
    Usually good prognosis

    77
    Infantile spasms (West-syndrome)

    4th to 9th month of life


    Characteristic attacks
     Nodding attacks: Convulsions of neck flexor muscles
     Salaam attacks: a flexor spasm with rapid bending of the head
    Occurring in clusters
    Loss of alertness, irritability
    EEG: interictal hypsarrhythmia 50 % , SB .
    tx. Steriods , Vigabatrin , KD

    88
    Absence epilepsy
     age: 3-8 years
     Developmentally normal children
     Symptoms:
     Brief arrest of speech, activity and awareness (usually < 5 sec)
     hundreds of episodes a day
     Often mistaken for „day-dreaming“

     EEG: typical 3/sec spike-wave-patterns, can be provoked by


    hyperventilation
     Good response to anticonvulsive treatment ( ESM,VPA)
     Good prognosis 80 %

    99
    10
    Myoclonic-Astatic Epilepsy
    (Doose syndrome)

    Age 3 – 5 years
    Prior development normal
    Short bilateral myocloni, followed by loss of tonus (drop
    attack)
    Often progressive with generalized tonic clonic seizures
    Prognosis variable
    tx; VPA,CLB, LTG,LEV,
    Role of KD

    11
    11
    12
    Rolandic epilepsy
    = Benign epilepsy of childhood
    with centrotemporal spikes

     The most common type of epilepsy in childhood


     Onset between the ages of 5 and 12 years
     Mostly nocturnal seizures
     Perioral/facial onset, numbness or paraesthesia of the tongue,
    inability to speak,drooling; secondary generalization possible.
     Prognosis excellent regarding seizures (remission after
    puberty)

    13
    13
    Landau-Kleffner syndrome
     Age of onset: 3 – 8 years
     Main symptoms: regression in NL speech development ,
    behavioural problems,
    mental regression
     Nocturnal epileptic seizures
     EEG awake : normal
     EEG sleep : bioelectrical status epilepticus during sleep
     Therapy: difficult
     Prognosis:
     Good regarding epilepsy
     Poor regarding speech and cognitive development

    14
    14
    Landau-Kleffner syndrome: sleep
    EEG

    15
    Juvenile myoclonic epilepsy
    (Janz-syndrome)
     Onset: Age 12-18 years
     Symptoms:
     Mainly myoclonic seizures
     Generalized tonic-clonic seizures (80-95%)
     Absences (35%)
     EEG : polyspikes slow wave
     Triggering factors: sleep deprivation, alcohol, flickering light
     Therapy:
     Similar as absence epilepsy
     In the majority lifelong therapy required

    16
    16
    Benign neonatal sleep myoclonus

    Neonatal onset
    In sleep only – terminated by waking
    Coarse myoclonus of one or more limbs
    Face spared
    Well neurologically normal child
    Spontaneous remission
    Gratification phenomena

    Normal infants and toddlers


    Stereotyped behaviours – often rhythmic
    Usually if inactive/bored
    May appear flushed, grunting
    Interruptible
    Resolve by 3-4 years old

     Long-term outcome of infantile gratification phenomena.


    Can J Neurol Sci 2013 May;40(3)
    ‘Breath-holding attacks’Blue breath-holding
    attacks, Reflex anoxic seizures

    Well toddlers
    Triggered
    Duration - crying followed by apnea and LOC for
    seconds to a few minutes some times followed by
    motor convulsions
    Self-limiting( role of Iron Tx
    Tics

     Motor plus/minus vocal


     Mainly head and neck
     Predominantly boys
     Onset in primary school years
     Within spectrum of semivoluntary movement – but
    repeated and stereotyped
     Suppressible temporarily
     May have co-morbidities
    Cardiac arrythmias
    Exercise-related - sometimes
    Palpitations - sometimes
    Drop attacks (with brief tonic-clonic)
    ECG ( QT interval
    Vasovagal syncope
    Teenagers
    Situation-specific
    Prodrome
    Fine tonic-clonic movements possible at end
    Recovery within minutes
    ECG – but not EEG!
    Psychogenic attack

    Usually teenagers
     Variable seizures (lack of stereotypy)
    Atypical features – e.g. long seizure without post-ictal
    phase; unusual patterns of movement
     Social and emotional history
     Value of video
     Value of ambulatory EEG
    Diagnostic testing -EEG
    Role misunderstood
    Detection of abnormality is no proof of epilepsy
    Absence of interictal abnomality does not exclude
    epielspy
    Ictal EEG could also be normal

    24
    Take-home messages ?

    Not all stereotyped behaviours are epileptic


    It’s the history that matters!
    Video can be helpful
    EEG may mislead
    No place for therapeutic trials of anticonvulsants
    In a child with resistant epilepsy – is it epilepsy?
    26

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