Emergency Lecture Series Approach to Common Pediatric Neurology Consults A Case-Based Approach - PowerPoint PPT Presentation

Loading...

PPT – Emergency Lecture Series Approach to Common Pediatric Neurology Consults A Case-Based Approach PowerPoint presentation | free to download - id: 3d2c41-ZjVkM



Loading


The Adobe Flash plugin is needed to view this content

Get the plugin now

View by Category
About This Presentation
Title:

Emergency Lecture Series Approach to Common Pediatric Neurology Consults A Case-Based Approach

Description:

Emergency Lecture Series Approach to Common Pediatric Neurology Consults A Case-Based Approach Ruba Benini Pediatric Neurology (PGY2) McGill University – PowerPoint PPT presentation

Number of Views:253
Avg rating:3.0/5.0
Slides: 72
Provided by: neurology
Learn more at: http://neurology.mcgill.ca
Category:

less

Write a Comment
User Comments (0)
Transcript and Presenter's Notes

Title: Emergency Lecture Series Approach to Common Pediatric Neurology Consults A Case-Based Approach


1
Emergency Lecture SeriesApproach to Common
Pediatric Neurology ConsultsA Case-Based
Approach
  • Ruba Benini
  • Pediatric Neurology (PGY2)
  • McGill University
  • 4/August/2010

2
Outline
  • Febrile seizures
  • Afebrile Seizures (Childhood epilepsy syndromes)
  • Headaches
  • Ataxia
  • Hypotonia

3
  • Case 1
  • 14mo bb girl, history of irritability and
    lethargy for 3days. Brought to ER by parents
    because of sudden episode of body stiffening
    followed by limpness and fine shaking of all
    extremities for 1min. In ER, BP75/50 HR
    150/min RR40/min T38.6C rectal O2 sat98
    RA.
  • Case 2
  • 14mo bb girl, history of irritability and
    lethargy for 3days. Brought to ER by parents
    because of sudden episode of body stiffening
    followed by limpness and fine shaking of right
    arm and leg lasting 20min. In ER, BP75/50 HR
    150/min RR40/min T38.6C rectal O2 sat98
    RA.
  • Case 3
  • 14mo bb girl, ex-34weeker, known seizure
    disorder, on Frisium, history of irritability and
    lethargy for 3days. Brought to ER by parents
    because of sudden episode of body stiffening
    followed by limpness and fine shaking of all
    extremities for 1min. In ER, BP75/50 HR
    150/min RR40/min T38.6C rectal O2 sat98
    RA.

4
Approach to Febrile Seizures
  • Febrile seizures are defined as seizures that
    occur in association with fever, in the absence
    of CNS infection (meningitis, encephalitis) and
    in patients with no history of previous afebrile
    seizures
  • Occur in 3-4 of children between 3months 6
    years (peak age 18-24months)
  • High recurrence (30-40)
  • 10 of children experience ? 3 febrile seizures
  • Factors that increase risk of occurrence include
  • Young age at time of first febrile seizure
    (lt18months)
  • Family history of in first degree relative
  • Low degree of fever while in the emergency
    department
  • Brief duration between onset of fever and the
    first seizure
  • Etiology genetic predisposition
  • 40 concordance rate for monozygotic twins versus
    7 for dizygotic twins
  • 8 if sibling with febrile seizures, 22 if
    sibling parent
  • Mode of inheritance polygenic vs autosomal
    dominant with variable penetrance

5
Approach to Febrile Seizures
Classification
  • Simple (typical)
  • Generalized
  • lt15min in duration
  • No recurrence in a 24hr period
  • Normal neurological status before seizure
  • Complex (atypical)
  • Focal
  • gt15min in duration (status epilepticus)
  • Multiple episodes in a 24hr period
  • Abnormal preexisting neurological status before
    seizure
  • Risk of developing epilepsy in general
    population 1
  • Not increased with simple febrile seizures except
    might be mildly increased if multiple episodes,
    FHx of epilepsy, and age lt12months at first
    seizure
  • Increased to 2.4 with atypical febrile seizures
  • Risk of epilepsy increased with the presence of
    each atypical feature
  • One atypical feature 3
  • Two atypical features 6
  • Three atypical features 9
  • Four atypical features 12-15

6
Approach to Febrile Seizures
  • Treatment of febrile seizures and prevention of
    recurrences does not alter risk of later possible
    epilepsy - ??routine use of AEDs not recommended.
  • Parents can be advised to use anti-pyretics for
    comfort care, but there is no evidence that it
    prevents recurrence of febrile seizures
  • Intermittent benzodiazepine can be used when
    recurrence is expected excessive parental
    anxiety
  • Nitrazepam (Mogadon)
  • lt 2years 1.25mg TID
  • gt2years 2.5mg TID (Minimum 3days or until
    fever subsides)
  • What investigations are necessary
  • Simple febrile seizures nothing
  • Complex febrile seizures EEG neuroimaging
  • What do parents want to know
  • Is this harmful
  • Will it happen again
  • Can I prevent it?
  • Will my child develop epilepsy
  • Will it go away?

7
  • Case 4
  • 8 year old boy, developmentally normal, with
    previous history of febrile seizures (5 febrile
    seizures since age of 1), presenting with
    generalized tonic-clonic seizure lasting 1 minute
    in the context of a febrile illness.
  • Case 5
  • 3year old boy, with history of 5 febrile seizures
    in the past. First febrile seizure was atypical,
    at age of 7months. Subsequent seizures have
    different semiology (atonic,myoclonic). Was
    meeting developmental milestones until 1.5years
    ago when he began to deteriorate with regression
    in development. Myoclonic jerks at age 18months.

8
Approach to Febrile Seizures
  • Consider other entities when febrile seizures
  • Occur very frequently or
  • Past the conventional age of 6 years (sometimes 7
    or 8 years is used as upper limit) or
  • In the context of deteriorating development
  • Generalized Epilepsy with Febrile Seizures Plus
    (GEFS)
  • Febrile seizures begin in usual age range but
    persist beyond 6 years of age
  • Different seizure phenotypes may develop
    myoclonic, atonic, astatic, or partial complex
    seizure
  • Mutations in SCN1A, SCN2A, SCN1B, GABRG2
  • Severe Myoclonic Epilepsy of Infancy (Dravet
    Syndrome)
  • Intractable epilepsy
  • Begins in first year of life usually with
    prolonged hemiclonic febrile seizures
  • Over time seizures evolve into febrile/afebrile
    generalized seizure types (myoclonic, atypical
    absence and partial complex) which rapidly become
    refractory to AEDs
  • Myoclonic jerks between 12-36months
  • Prior to onset of seizures child is
    developmentally normal ?ataxia, psychomotor
    regression, mental retardation
  • EEG initially normal ?generalized spike wave
    abnormalities
  • 70 have mutation in SCN1A

Avoid AEDs that block Na channels lamotrigine,
CBZ, oxcarbazepine, phenytoin Rx clobazam, VPA
Stafstrom 2009
9
Approach to Febrile Seizures
10
Outline
  • Febrile seizures
  • Afebrile Seizures (Childhood epilepsy syndromes)
  • Headaches
  • Ataxia
  • Hypotonia

11
Approach to Afebrile Seizures
Seizure
Unprovoked
  • Provoked
  • Electrolyte abnormalities
  • Infection (meningitis)
  • Trauma
  • Toxic ingestion
  • Vasculitis
  • Inborn error of metabolism
  • CNS tumour
  • Does it fit any of the Childhood Epilepsy
    Syndromes?
  • Semiology of seizures
  • Age of onset
  • EEG features
  • Clinical features/progression
  • Response to Rx
  • Prognosis
  • History
  • Exam
  • Investigations lytes (Glc, Ca, P, Mg) CBC LP
    tox screen, etc)
  • Neuroimaging

12
Approach to Afebrile Seizures
13
Approach to Afebrile Seizures
Serologies/TORCH Preeclampsia/GDM/Infections Subst
ance abuse/meds Antenatal U/S
  • History
  • Antenatal History
  • Birth history
  • Developmental history
  • Family history
  • PMHx (?CNS infections)
  • Head trauma
  • Seizure description (aura, trigger, eyewitness
    description)

Fetal distress Apgars, Cord pH Need for postnatal
resuscitation
Normal vs delayed vs regressed
Consanguinity, hx of febrile seizures, epilepsy,
developmental delay, recurrent miscarriages, IEM
14
Approach to Afebrile Seizures
  • Physical Exam
  • Dysmorphism
  • Stigmata of Neurocutaneous disorders
  • Neurological exam including
  • HC
  • developmental
  • ?Liver, heart involvement (IEM)

15
Approach to Afebrile Seizures
  • Physical Exam
  • Dysmorphism
  • Stigmata of Neurocutaneous disorders
  • Neurological exam including
  • HC
  • developmental
  • ?Liver, heart involvement (IEM)

16
Approach to Afebrile Seizures
  • Physical Exam
  • Dysmorphism
  • Stigmata of Neurocutaneous disorders
  • Neurological exam including
  • HC
  • developmental
  • ?Liver, heart involvement (IEM)

Shagreen patch (Tuber Sclerosis)
Hypopigmented macule (Tuberous sclerosis)
Café -au -lait macule (Neurofibromatosis)
Port Wine Stain (SturgeWeber)
17
Developmental Approach to Common Childhood
Epilepsy Syndromes
ILAE 2009
18
Common Childhood Epilepsy Syndromes
http//www.ilae-epilepsy.org/Visitors/Centre/ctf/C
TFsyndromes.cfm
19
Neonate (lt28days)
  • Case 6
  • Called from NICU to rule out seizures in a 5day
    old baby boy, ex-34 weeker. Unremarkable
    antenatal history except baby was born at 34weeks
    because of PROM. GBS status unknown. No maternal
    fever. Mom received antibiotics 5hrs prior to
    delivery which was by SVD. BB born with Apgars
    7,9. Cord pH 7.24. On DOL 1 baby started having
    apneic episodes lasting 10secs, associated with
    bradycardia and desaturation. Might have had one
    episode of generalized tonic-clonic movements of
    extremities lasting 3secs.

20
Common Childhood Epilepsy Syndromes
  • Neonatal Period (lt28 days)
  • Seizures in newborns are often difficult to
    distinguish from normal activity
  • Most commonly occur within the first week of life
  • 2/3 of neonatal seizures are due to
    Hypoxic-ischemic encephalopathy (HIE)
  • Other causes infection, electrolyte
    abnormalities, inborn errors of metabolism,
    structural
  • The clinical and electroencephalographic features
    of neonatal seizures differ considerably from
    those in older children and adults.
  • Some seizures can be quite subtle making
    diagnosis difficult
  • Important to note that in neonates 50-80 of
    prolonged epileptiform discharges on EEG are not
    associated with visible clinical changes
  • This electroclinical dissociation is due to the
    Incomplete myelination of white matter tracts and
    immaturity of regional brain interconnectivity
  • Leads to only modest behavioural manifestations
    of seizures and explains why unlikely to get
    tonic-clonic seizures in newborns

Feinichel
21
Common Childhood Epilepsy Syndromes
  • Sudden jerking movements during sleep only
  • Can be stopped with gentle restraint
  • Normal EEG
  • No Rx
  • Excessive response to stimulation
  • Low frequency, high amplitude shaking of limbs
    and jaw in response to touch, noise or motion
  • Low threshold for Moro reflex

Feinichel
22
Common Childhood Epilepsy Syndromes
  • Almost never a seizure manifestation unless
    associated with eye deviation, tonic stiffening
  • Prolonged apnea without bradycardia with
    tachycardia is a seizure until proven otherwise
  • Often associated with HIE

ILAE 2009
23
Common Childhood Epilepsy Syndromes
  • Approach to Neonatal Seizures

Hypoxic-Ischemic Encephalopathy Infection Electrol
yte abnormalities Structural Inborn errors of
metabolism Stroke
Epilepsy syndromes
Benign
Epileptic encephalopathies
Feinichel
24
Common Childhood Epilepsy Syndromes
  • Neonatal Period (lt28 days)
  • Benign Familial Neonatal Seizures
  • FHx of seizures in first weeks of life with no
    epilepsy or neurologic abnormalities
  • Autosomal dominant, mutations in voltage-gated K
    channels
  • Brief multifocal clonic seizures during the first
    week apnea
  • Seizures stop spontaneously within 6weeks
  • Healthy newborn, normal interictal EEG, clinical
    events are associated with flattening of the EEG
  • Rx Phenobarbital, then taper off when seizure
    free for 4weeks
  • Benign Familial Neonatal-Infantile Seizures
  • Onset between 6days of life to 3months
  • Seizures are partial in onset then become
    generalized
  • Seizures stop spontaneously by age 12months

Feinichel
25
Common Childhood Epilepsy Syndromes
  • Neonatal Period (lt28 days)

Epileptic encephalopathies seizures lead to
severe cognitive and behavioral impairment
  • Ohtahara Syndrome
  • (Early Infantile Epileptic Encephalopathy)
  • Frequent extensor tonic spasms
  • EEG shows burst suppression pattern
  • Usually abnormal MRI findings (lissencephaly,
    focal cortical dysplasia,etc)
  • Medically intractable seizures
  • Early Myoclonic Encephalopathy
  • Erratic/fragmentary myoclonus that typically is
    not associated with an EEG correlate
  • EEG shows burst suppression pattern
  • Usually normal MRI findings
  • Sometimes associated with certain inborn errors
    of metabolism ex. Nonketotic hyperglcemia, Menkes
    disease, proprionic acidemia, etc

Chapman and Rho
26
Infant (lt2yrs)
  • Case 7
  • 8mo bb girl, developmentally normal, presents to
    ER with 3 weeks history intermittent sudden jerky
    movements of the head and upper extremities,
    lasting 1-2secs, worsening over the course of the
    past 3 weeks. Multiple episodes a day, sometimes
    in clusters. Resumes activity right after jerk
    with no apparent alteration. No other unusual
    movements. Unremarkable perinatal history. No FHx
    of epilepsy or other seizure disorders. Exam
    normal except for myoclonic jerks noted.
    Video-EEG normal, with no associated changes
    during myoclonic episodes.
  • Case 8
  • 8mo bb girl, developmentally delayed, presents to
    ER with episodes of stiffening of upper
    extremities and abduction of arms. Multiple
    episodes a day, sometimes in clusters lasting
    5-10min since 6months of age. Exam abnormal
    unable to sit without support, truncal and lower
    extremity weakness. Flexor spasms of upper
    extremities noted. One hypopigmented macule on
    back. EEG shows hypsarrhythmia with
    electrodecrimental changes associated with
    spasms. MRI shows subcortical tubers.

27
Common Childhood Epilepsy Syndromes
  • Infancy (lt2yrs)

Benign myoclonus of infancy
Benign myoclonic epilepsy
West Syndrome (infantile spasms)
Severe Myoclonic Epilepsy of Infancy (Dravet
syndrome)
ILAE 2009
28
Common Childhood Epilepsy Syndromes
  • Infancy (lt2yrs)

ILAE 2009
29
Common Childhood Epilepsy Syndromes
  • Infancy (lt2yrs)
  • West Syndrome (infantile spasms)
  • Triad of infantile spasms, hypsarrythmia on EEG,
    and developmental arrest/regression
  • Peak age of onset 3-7months
  • Most common epileptic encephalopathy
  • Etiologies
  • Cerebral dysgenesis
  • Tuberous sclerosis
  • Preexisting injury 2o ischemia, infection or
    trauma
  • Down syndrome
  • IEM

http//www.youtube.com/watch?vfEgAjCv7VQoNR1
ILAE 2009
30
Child (2yrs - adolescence)
Common Childhood Epilepsy Syndromes
  • Case 9
  • Healthy 7 year old boy, brought to ER because
    parents noticed unusual event after patient went
    to bed. Heard gurgling noises from his room,
    found him sitting in bed with right lower face
    jerking, excessive drooling and unable to speak.
    Lasted 2min and was completely back to baseline.
    Developmentally normal. Exam normal. EEG shows
  • Case 10
  • Healthy 6year old girl, brought to ER because
    parents noticed unusual event. Patient hurt
    herself whilst playing outside. Came indoors to
    mom, crying then suddenly she stopped crying,
    eyes staring ahead, some blinking movements.
    Lasted 5secs then she snapped out of it and
    continued crying. Developmentally normal except
    parents report she is often dans la lune. Also,
    teachers complain that her grades have dropped
    during this academic year and that she
    continuously stares off into space. Exam
    completely normal.

31
Common Childhood Epilepsy Syndromes
  • Childhood

BECTS
Childhood Absence Epilepsy
Lennox-Gastaut
Landau-Kleffner
ILAE 2009
32
Common Childhood Epilepsy Syndromes
  • Childhood (2yrs adolescence)
  • Benign Epilepsy with Centrotemporal spikes
    (BECTS/Rolandic epilepsy)
  • Most common form of idiopathic epilepsy in
    childhood
  • Peak age of onset 5-10years (range 3yrs-13yrs)
  • Developmentally intellectually normal
  • Strong genetic predisposition
  • Seizures
  • brief (1-2min)
  • infrequent
  • 10 of children will only have one seizure ?
    Majority (70) will seize 2-6x
  • Majority have nocturnal seizures only
  • Hemifacial clonic movements, speech arrest,
    dysarthria and excessive drooling
  • Preceding paresthesias in mouth, gum, cheeks or
    lips may occur
  • May have involvement of ipsilateral limbs or even
    generalization

ILAE 2009
33
Common Childhood Epilepsy Syndromes
  • Childhood (2yrs adolescence)
  • Benign Epilepsy with Centrotemporal spikes
    (BECTS/Rolandic epilepsy)
  • EEG
  • Normal background in awake and sleep
  • Epileptiform discharges focal, diphasic
    spike-and-slow-wave discharges over
    rolandic/centrotemporal regions unilaterally or
    independentally bilaterally
  • Horizontal dipole with maximum spike negativity
    over central/temporal regions and maximum
    positivity over frontal region.
  • Rx
  • Often not necessary unless frequent and
    disruptive to childs life
  • Carbamazepine, Clobazam
  • Spontaneous resolution by adulthood (18yrs)
  • Neuroimaging if EEG findings are focal

ILAE 2009
34
Common Childhood Epilepsy Syndromes
  • Childhood (2yrs adolescence)
  • Childhood Absence Epilepsy
  • Idiopathic generalized epilepsy syndrome
  • Age of onset 4yr to 10 yrs (peak 5-7yrs)
  • Onset before age 3yrs associated with an
    increased likelihood of neurodevelopmental
    abnormalities probably represents another
    epilepsy syndrome
  • More frequent in girls
  • Developmentally and intellectually normal
    children
  • Seizures are brief (4-20secs) abrupt onset of
    impaired consciousness and unresponsiveness
  • Typically sudden onset and interruption of
    activity with blank stare
  • Abrupt end and child continues ongoing activity
    unaware that a seizure occurred
  • If other seizure types present (myoclonic,
    atonic, tonic-clonic) then not CAE!
  • Frequent (up to 100s/day)
  • Provoked by hyperventilation in 90 of children

ILAE 2009
35
Common Childhood Epilepsy Syndromes
  • Childhood (2yrs adolescence)
  • Childhood Absence Epilepsy
  • EEG ictal events show generalized symmetric 3HZ
    spike-wave discharges
  • Prognosis excellent
  • Complete remission 2-6yrs after onset
  • Rx Ethosuximide, VPA
  • However,
  • Up to 30 can continue into adulthood, and these
    have a greater chance (40) to get generalized
    tonic-clonic seizures
  • CAE can precede juvenile myoclonic epilepsy in
    11-18 of cases
  • Must be differentiated from Juvenile Absence
    Epilepsy (JAE)
  • Older age of onset 10yrs to 16yrs
  • Infrequent absences with longer duration
    (gt20secs)
  • More likely to experience generalized
    tonic-clonic seizures
  • EEG 3.5Hz to 4Hz generalized spike-wave
    discharges
  • Good response to Rx, but usually lifelong

http//www.youtube.com/watch?vH3iLQi6wt94feature
related
ILAE 2009
36
Common Childhood Epilepsy Syndromes
  • Childhood (2yrs adolescence)
  • Lennox-Gastaut syndrome
  • Intractable pediatric epilepsy
  • Onset 2 to 8yrs (peak 3 to 5years)
  • Male predominance
  • 2/3 of cases are symptomatic i.e. have
    pre-existing brain abnormalities
  • 1/3 of cases have history of infantile spasms
  • 1/3 are cryptogenic affecting children who are
    initially developmentally neurologically normal
  • Classic triad
  • Multiple generalized seizure types (tonic,
    atonic, myoclonic, atypical absence)
  • Interictal EEG diffuse slow spike-wave
    discharges
  • Cognitive dysfunction (which may not be present
    at onset)
  • Poor response to AEDs (VPA, lamotrigine,
    topiramate)
  • Ketogenic diet significant reduction in seizures
    in 50 of patients

ILAE 2009
37
Common Childhood Epilepsy Syndromes
  • Childhood (2yrs adolescence)
  • Electrical Status Epilepticus in slow sleep
    (ESES)
  • Comprises of 2 clinically related syndromes
    Continous spike-wave in sleep (CSWS) and Landau
    Kleffner syndrome
  • Age of onset 3 to 8 years
  • Usually history of normal development with
    regression in preschool years
  • CSWS global regression, decreased intellectual
    level, poor memory, hyperkinesis, motor
    impairment, psychosis
  • LKS acquired auditory agnosia
  • EEG
  • CSWS frontal and multifocal sharp waves that
    become continuous during sleep
  • LKS centrotemporal sharp waves that increase
    during sleep (85 or more of sleep)
  • These entities are believed to represent the
    severe spectrum of BECTs
  • If clinical suspicion, need to admit for 24hr
    telemetry
  • Rx oral steroids and high dose diazepam

ILAE 2009
38
Adolescence
  • Case 11
  • 15year old girl, previously healthy and
    developmentally normal, experienced a 2minute
    generalized tonic-clonic seizure in the morning
    while on vacation with parents. Admitted to
    staying up later than usual. Denied
    alcohol/substance abuse. Mom reports that for the
    past couple of years, has had episodes when she
    would drop her dishes. Also reported early
    morning limb jerks. Otherwise, developmentally
    normal. Exam normal.

39
Teaching Point 2 Common Childhood Epilepsy
Syndromes
  • Adolescent

Juvenile Myoclonic Epilepsy (JME)
  • Progressive myoclonic epilepsies (PME)
  • Encompass various metabolic and neurodegenerative
    conditions that present with progressive
    myoclonus, gen. tonic-clonic and other seizures,
    with mental deterioration and cerebellar
    dysfunction.
  • Onset infancy to adulthood
  • Etiologies MERRF, Lafora body disease, Neuronal
    ceroid lipofuscinosis

ILAE 2009
40
Common Childhood Epilepsy Syndromes
  • Childhood (2yrs adolescence)
  • Juvenile Myoclonic Epilepsy (JME)
  • Most common form of idiopathic generalized
    epilepsy
  • Age of onset 12 to 18years (peak 15yrs)
  • Neurological and developmentally normal
  • Typically first seizures noted are early morning
    generalized tonic-clonic seizures precipitated by
    slep deprivation
  • Often there is a preceding history of Myoclonic
    jerks and absence seizures
  • EEG 4Hz to 6Hz generalized atypical spike and
    polyspike-and-wave discharges with normal
    background
  • Photosensitivity in 30-90 of cases
  • Triggers
  • Sleep deprivation
  • Alcohol consumption
  • Menstruation

ILAE 2009
41
Common Childhood Epilepsy Syndromes
  • Childhood (2yrs adolescence)

Show videos
42
  • Not all that shakes or stiffens is seizures .

Case 11 Youre asked to see a 6month old baby
girl who was admitted for ALTE (Altered
life-threatening event) to rule out seizure.
Parents describe an episode lasting 2min when
the baby became blue and limp, lost
consciousness and had trembling of her arms and
legs. Episode was preceded by intense crying. No
FHx of epilepsy. Developmentally normal. Exam
Normal. EEG was done Normal.
Case 12 5month old bb boy, developmentally
normal, presenting with episodes of arching,
lasting a few seconds. Occur several times during
the day. Mostly related to feeds. ROS Negative,
except baby is known for frequent regurgitation
of feeds.
43
Nonepileptic Paroxysmal events
  • Nonepilepic paroxysmal events in childhood are
    common and pose a diagnostic challenge
  • Key to diagnosis is a detailed history and
    careful observation

DiMario 2006
44
Nonepileptic Paroxysmal events
DiMario 2006
45
Nonepileptic Paroxysmal events
46
Nonepileptic Paroxysmal events
  • Breathholding spells
  • Represent a prolonged expiratory apnea
  • Inciting event provokes emotional upset/crying in
    child
  • Followed by noiseless pause
  • Change in colour (pale or cyanotic)
  • Severe spells can have loss of consciousness ?
    loss of postural tone ? opisthotonic posturing
    with myoclonic jerks
  • In 15 of children, can have a generalized
    convulsion ? anoxic epileptic seizure
  • BHS can be divided into
  • Pallid BHS pallor with rapid progression to loss
    of consciousness
  • Cyanotic BHS protracted crying prior to LOC
  • Age of onset 6-12months with worsening in second
    year of life
  • Resolution between 3yrs to 8 yrs
  • BHS can be exacerbated by anemia

http//www.youtube.com/watch?v2bKVHSe6hVQ
http//www.youtube.com/watch?v-ne9i2a0lqkfeature
related
47
Nonepileptic Paroxysmal events
  • Gastroesophageal reflux disease (Sandifer
    syndrome)
  • Can present as ALTE
  • Neck and head extension with opisthotonic
    posturing associated with GER (Sandifer syndrome)
  • Typically associated with feeding and vomitting
  • Treatment of reflux with anti-reflux medications
    reduces frequency of episodes

48
Outline
  • Febrile seizures
  • Afebrile Seizures (Childhood epilepsy syndromes)
  • Headaches
  • Ataxia
  • Hypotonia

49
Approach to Headaches in Pediatric population
50
Approach to Headaches in Pediatric population
51
Approach to Headaches in Pediatric population
Brain Tumour
Migraine
Infection (meningitis)
SAH
Secondary Headache
Primary Headache
Vasculitis
Sinus disease
Cluster headaches
Head injury
Tension headaches
?ICP (pseudotumour cerebri)
52
Approach to Headaches in Pediatric population
  • Red Flags
  • Worse headache ever/onset with exertion/thundercla
    p headache
  • Fever, nuchal rigidity, behavioural changes
  • Headade worse in morning/straining/change in head
    position
  • Associated posterior fossa signs (diplopia,
    facial weakness, hearing loss, dysarthria,
    dysphagia, ataxia, dysmmetria
  • Papilledema
  • Worsening or changing quality
  • Protracted vomiting
  • B symptoms

Brain Tumour
Infection (meningitis)
SAH
Secondary Headache
Vasculitis
Sinus disease
Head injury
?ICP (pseudotumour cerebri)
Investigations tailored towards suspected etiology
53
Approach to Headaches in Pediatric population
  • Migraine with aura
  • Migraine without aura
  • Migraine equivalents
  • Acute confusional migraine
  • Basilar migraine
  • Cyclic vomitting
  • Hemiplegic migraine
  • Opthalmoplegic migraine
  • Benign paroxysmal vertigo

Migraine
Primary Headache
Cluster headaches
Tension headaches
54

Approach to Headaches in Pediatric population
Lewis D et al 2004
55

Approach to Headaches in Pediatric population
Abortive
Prophylactic/Preventative Agents
Behavioural interventions
  • NSAIDs (Ibuprofen)
  • Acetominophen
  • Triptans (nasal sumatriptan)
  • Flunarizine (Ca channel blocker)
  • Topiramate
  • Amitryptyline
  • Valproic acid
  • Beta blockers (pronalolol)
  • Cyproheptadine

Avoid triggers
VITAMIN B2
Lewis D et al 2004
56
Outline
  • Febrile seizures
  • Afebrile Seizures (Childhood epilepsy syndromes)
  • Headaches
  • Ataxia
  • Hypotonia

57
Approach to Ataxia
  • Case 13
  • 3year old girl, previously healthy, brought to ER
    by mother because for the past 24 hrs she has
    been unsteady on her feet and walking like a
    drunk. PMHx is unremarkable except for low
    grade fever and cold symptoms 2 weeks prior to
    presentation. O/E VSS and afebrile. Patient
    alert and sitting up in bed. Neck supple.
    Language appropriate. CN exam normal except for
    nystagmus on lateral gaze. Truncal ataxia.
    Dysmmetria (upper and lower extremities).
    Wide-based ataxic gait.

58
Approach to Childhood ataxia
Acute
Chronic/Progressive
  • Toxic ingestion
  • CNS Infections (rhomboencephalitis)
  • Post-infectious/immune
  • Acute cerebellar ataxia
  • Miller-Fisher
  • ADEM
  • Myoclonus-opsoclonus
  • Migraine (Basilar, BPV)
  • Trauma
  • Vascular
  • cerebellar hemorrhage, PCA stroke)
  • Genetic
  • Episodic Ataxia 1/2
  • Investigations
  • Urine toxicology screen, drugs levels, CBC, LFTs,
    lytes
  • LP CSF cell counts, protein, glucose, bacterial
    culture PCR testing for varicella, mycoplasma
    pneumonia or other viruses
  • Neuroimaging (CT head MRI with and without
    gadolinum to rule out structural, demyelinating
    and infectious etiologies
  • Metabolic workup for IEM

59
Approach to Childhood ataxia
Acute
Chronic/Progressive
  • Neoplastic
  • Cerebellar astrocytomas
  • Cerebellar hemangioblastomas
  • Medulloblastoma
  • Ependymoma
  • Congenital malformation
  • Cerebellar aplasia
  • Dandy-Walker malformation
  • Chiari malformation
  • Hereditary ataxia
  • Autosomal dominant
  • Machado-Joseph
  • Olivopontocerebellar
  • Autosomal recessive
  • Abetalipoproteinemia
  • Ataxia-telengiectasia
  • Fredreich ataxia

60
Outline
  • Febrile seizures
  • Afebrile Seizures (Childhood epilepsy syndromes)
  • Headaches
  • Ataxia
  • Hypotonia

61
Approach to Hypotonia
  • Case 6
  • 6month old baby boy who was admitted on the ward
    for FTT and feeding difficulties. Neurology
    consulted by pediatrics team who was concerned
    about the gross motor development of this baby.
    At 6months, he still cannot support his head, is
    not sitting, even with support. On exam, high
    prominent forehead, narrow bifrontal diameter,
    downslated fissures, almond-shaped eyes,
    downturned corners of the mouth, micrognathia,
    dysplasic ears and diminished spontaneous
    activity of limbs profound axial and appendigeal
    hypotonia. Reflexes bilaterally symmetrical 2,
    upgoing plantars. No fasciculations. No muscle
    atrophy.

62
Approach to Hypotonia
Central Nervous System
Spinal Cord
Anterior Horn cell
Peripheral Nerve
Neuromuscular junction
Muscle
63
Approach to Hypotonia
  • Central Nervous System
  • Benign congenital hypotonia
  • Genetic syndromes (Prader-Willi, trisomies)
  • Hypoxic-ischemic encephalopathy
  • Cerebral malformations
  • Cortical migration abnormalities (lissencephaly,
    schizencephaly)
  • Inborn errors of metabolism (leukodystrophies)
  • History (traumatic birth ?HIE)
  • Seizures
  • Cognitive delay
  • Dysmorphism
  • Hemiparesis
  • Hyperreflexia
  • Limb spasticity

64
Approach to Hypotonia
  • Spinal cord
  • Trauma
  • Spinal cord ischemia

65
Approach to Hypotonia
  • Anterior Horn Cell
  • Spinal muscle atrophy
  • Neonatal poliomyelitis
  • Profound weakness
  • Areflexia
  • Feeding difficulties
  • Respiratory difficulties
  • Tongue fasciculations
  • No sensory deficits
  • Alert, interactive

66
Approach to Hypotonia
  • Peripheral Nerve
  • Hereditary motor and sensory neuropathies
  • Acute inflammatory demyelinating polyneuropathy
  • Familial dysautonomia syndromes
  • Giant axonal neuropathy
  • Inborn errors of metabolism
  • Distal gt proximal weakness
  • Hyporeflexia
  • Facial weakness unusual
  • Sensory deficits

67
Approach to Hypotonia
  • Neuromuscular Junction
  • Infantile botulism
  • Transient neonatal myasthenia gravis
  • Congenital myasthenia gravis
  • Generalized weakness
  • Fatiguability
  • Hyporeflexia
  • Feeding problems
  • Respiratory compromise
  • Ptosis

68
Approach to Hypotonia
  • Muscle
  • Congenital myopathy
  • Congenital muscular dystrophy
  • Syndromic and non-syndromic
  • Congenital myotonic dystrophy
  • Metabolic myopathy
  • History of poor fetal movement/polyhydramnios
  • Proximal muscle weakness
  • Hyporeflexia
  • Feeding problems
  • Respiratory compromise
  • Facial diplegia
  • Arthrogryposis/bilateral club feet
  • Other organ involvement (ex. Heart in Pompe
    disease)

69
Approach to Hypotonia
  • Head/spine imaging
  • Genetic testing
  • Karyotype
  • Prader-Willi (methylation 15q11-13)
  • SMA testing
  • CSF evaluation
  • EMG/NCS
  • Muscle/nerve biopsy
  • Tensilon testing
  • Metabolic w/u
  • Lactate/pyruvate
  • Plasma a.acids
  • Urine organic acids
  • Acyl carnitine profile
  • VLCFA

Central Nervous System
Spinal Cord
Anterior Horn cell
Peripheral Nerve
Neuromuscular junction
Muscle
70
References
  • Febrile seizures clinical practice guideline for
    the long-term management of the child with simple
    febrile seizures. Pediatrics. 2008
    Jun121(6)1281-6.
  • Stafstrom CE. (2009) Severe epilepsy syndromes
    of early childhood the link between genetics and
    pathophysiology with a focus on SCN1A mutations.
    J Child Neurol. 2009 Aug24(8 Suppl)15S-23S.
  • DiMario FJ Jr.(2006) Paroxysmal nonepileptic
    events of childhood. Semin Pediatr Neurol. 2006
    Dec13(4)208-21.
  • Fenichel GM (2005). Clinical Pediatric Neurology
    A signs and symptoms approach.
  • Guberman A and Bruni J (1999) Essentials of
    Clinical Epilepsy. Chapter 3.
  • Lewis D, Ashwal S, Hershey A, Hirtz D, Yonker M,
    Silberstein S American Academy of Neurology
    Quality Standards Subcommittee Practice
    Committee of the Child Neurology Society.
    Practice parameter pharmacological treatment of
    migraine headache in children and adolescents
    report of the American Academy of Neurology
    Quality Standards Subcommittee and the Practice
    Committee of the Child Neurology Society.
    Neurology. 2004 Dec 2863(12)2215-24. Review

71
References
  • To download protocols from MUHC portal
About PowerShow.com