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Pediatric Neurology Primer

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Pediatric Neurology Primer Powered By Docstoc
					        Pediatric Neurology
             A Primer




       Jules E.C. Constantinou, MD FRACP
        Comprehensive Epilepsy Program
Center For Autism and Developmental Disabilities
            Henry Ford Health System
The Patient Encounter




• Jai, a 9 year-old boy
• Hyperactivity and distractibility
• Headaches
• Habits
• Staring spells
Headaches


•   Beginning at the start of the school year
•   1 to 2 a week, at the end of the school day
•   Bifrontal, “stingy”, grade 8/10
•   Sometimes sick to his stomach, vomited on 2
    occasions
•   Sleeps for 3 hours, with relief
•   No aura
                       Pretest 1
                     True or False

• The headaches are of tension type, rather than
    migrainous, because of their onset with the start of the
    school year, their bilateral nature, the absence of aura
    and the absence of a family history
•   Triptans are not approved for the treatment of childhood
    migraine
•   MRI of the brain is indicated because of the early age of
    onset of headaches
The Patient Encounter



• Fourth grade: reading, math skills are “low
    average”
•   Tendency to talk out of turn, walk around has
    mellowed
•   Tied to school chair by teacher a few years ago!
•   Does best with one on one
•   Forgetful, loses assignments
•   Seems not to listen
The Patient Encounter
Personality Traits



• Perfectionistic, erases work over and over
• Makes bed a particular way
• Soft spoken and reserved, especially with
 strangers
The Patient Encounter



• Unusual noises in class and frequent eye
    blinking since the beginning of the year
•   Staring spells a few times a week, especially in
    tutoring
•   Mother very concerned because her brother had
    “petit mal” seizures in childhood
                     Pretest 2
                   True or False
• The child has Tourette’s syndrome because he
    has both motor and phonic tics and unusual
    personality traits
•   Tourette’s syndrome has a uniformly poor
    prognosis- the mother should be informed out of
    the hearing of the child
•   Treatment of the attentional disorder with a
    stimulant is contra-indicated because of the high
    risk of provocation of tics
                     Pretest 3
                   True or False
• The child has a 25% risk of typical absence
    epilepsy because of the family history?
•   The diagnosis of absence epilepsy is readily
    established in the office after a history is taken
    and the child is examined?
•   Treatment of the attentional disorder is contra-
    indicated because of the possibility of
    provocation of seizures?
Childhood Migraine

• Bille, 1962
• 1/3 of 7 year-old children, ½ of 15 year-old
    children at least one headache
•   Incidence of migraine
    -7 years          : 2.7%
    14 years (boys) : 6.4%
    14 years (girls) : 14.8%
• 20% experience first migraine before 5 years
• Carsickness, abdominal migraine in 45%
Childhood Migraine
Applicability of IHS Criteria?



• Unilateral: 25% to 66%
• Throbbing: 50% to 60%
• Duration: childhood migraine usually lasts 2-3
    hours
•   Aura: 10% to 30%
Childhood Migraine
The Sick Headache

•   Prensky’s criteria
•   Unilateral
•   Throbbing
•   Nausea, vomiting, autonomic phenomena
•   Photophobia, phonophobia
•   Relief by sleep, provocation by exercise
•   Aura
•   Family history (70% to 80%)
The Approach to the
Child With Headache



• Thorough medical history and physical
    examination
•   Measurement of vital signs, especially BP
•   Complete neurological examination including
    fundoscopy
The Child With Headache


• The role of imaging
• AAN review of six pediatric studies, 3% had
    surgically remediable lesions- all of whom had
    clinical signs
•   Focal findings, signs of raised ICP, alterations of
    consciousness, seizures
•   Recent onset of severe headaches, change in
    type of headache
Childhood Migraine
Rescue Treatment


•   Ibuprofen : 7.5 to 10 mg/kg
•   Acetaminophen : 15 mg/kg
•   Triptans safe and effective in adolescence
•   Almotriptan approved by FDA
•   Studies often muddied by placebo effect
•   Rizatriptan melt: 5mg < 40kg, 10mg > 40kg
•   Zolmitriptan melt
Childhood Migraine
Control Treatment



•   Indications
•   Lifestyle issues
•   Comorbid issues
•   Choices of medication – amitriptyline, SSRI,
    topiramate, et al
Tic Disorders




• Prevalence
  – Transient tic disorder: 3 to 15%
  – Tourette’s syndrome: 0.72%
Tic Disorder
Tourette’s Syndrome


•   Multiple motor and phonic tics
•   Many times a day, in bouts, for at least a year
•   Begin at 8 years, peak in pre-adolescence
•   Onset under the age of 18 years
•   Comorbid ADHD, OCD in 50%
•   Complete resolution by age 18 in 50%
Tourette’s Syndrome
Simple Motor Tics
• Clonic – eye blinking, head jerking, nose
    twitching
•   Dystonic – blepharospasm, bruxism, shoulder
    rotation, sustained jaw opening
•   Tonic – abdominal contraction, limb extension,
    limb flexion

Complex Motor Tics
• Hitting, jumping, kicking, head shaking,
  touching, smelling objects, burping, retching,
  vomiting, copropraxia
Tourette’s Syndrome



Simple Phonic Tics – sniffing, throat clearing,
  grunting, coughing, squeaking

Complex Phonic Tics – coprolalia, echolalia,
  paliphrasia
Tourette’s Syndrome
A Disorder of the Basal Ganglia



• Decreased volume of caudate nucleus
• Activation of striatal neurones that share
    functional homogeneity (matrisomes)
•   Dopaminergic and serotonergic neurones
Tourette’s Syndrome
            To Treat or Not To Treat

• Medical treatment not necessary in majority

• Annoying, embarrassing, uncomfortable
Tic Disorders
Treatment Options


• Target the comorbidity
• Noradrenergic drugs: clonidine, guanfacine
• Dopamine receptor blocking drugs: pimozide,
    haloperidol, olanzapine, risperdal
•   ADHD – stimulants (TSSG), atomoxetine
•   OCD, anxiety, SSRI
•   AED’s: topiramate, levetiracetam
Typical Absence Epilepsy
• Idiopathic, generalized epilepsy
• Age related: neurologically and developmentally normal
    children between 5 to 10 years
•   Seizure types
    -absence seizures (pyknolepsy)
    -generalized tonic-clonic seizures
•   EEG: generalized 3Hz spike and slow waves
•   Remission in 90%
•   Treatment choices: ethosuximide, lamotrigine, valproate
                    Post Test 1
                   True or False
• The headaches are of tension type, rather than
    migrainous, because of their onset with the start
    of the school year, their bilateral nature, the
    absence of aura and the absence of a family
    history
•   Triptans are not approved for the treatment of
    childhood migraine
•   MRI of the brain is indicated because of the
    early age of onset of headaches
                    Post Test 2
                   True or False
• The child has Tourette’s syndrome because he
    has both motor and phonic tics and unusual
    personality traits
•   Tourette’s syndrome has a uniformly poor
    pronosis – the mother should be informed out of
    the hearing of the child
•   Treatment of the attentional disorder with a
    stimulant in contra-indicated because of the high
    risk of provocation of tics
                    Post Test 3
                   True or False
• The child has a 25% risk of typical absence
    epilepsy because of the family history
•   The diagnosis of absence epilepsy is readily
    established in the office after a history is taken
    and the child is examined
•   Treatment of the attentional disorder is contra-
    indicated because of the possibility of
    provocation of seizures
Back to the Patient



•   Migraine
•   ADHD
•   Minor obsessive compulsive traits
•   Transient tic disorder
•   No evidence active epilepsy

				
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posted:4/25/2011
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