Overview of Pediatric Neurology

Dr. Judy Pipo-Deveza American Mission Hospital July 2012

History and Neuro PE Seizures, Epilepsy Headache Neurodiagnostics Increased ICP Neurodevelopmental disorders CNS Infections Neuromuscular disorders CNS tumors, SOL Immune mediated neurologic disorders Neurometabolic diseases Neurologic manifestations of systemic diseases

Epilepsy
Epilepsy refers to occurrence of two or more unprovoked seizures. Unprovoked seizures not precipitated by entities such as fever, electrolyte imbalance, CNS infection, trauma, etc

Why Classify?
Facilitate communication among professionals Facilitate communication between physician and patient Aid diagnosis Rational prescribing of AEDs based on accurate diagnosis of seizure type Prognosis

Simplified Classification of Epileptic Seizures

Partial seizures
Simple preservation of awareness Complex impairment of consciousness Secondary generalized

Generalized seizures
Absence Myoclonic Tonic-clonic Tonic Atonic

International Classification of Epileptic Seizures

I. Partial Seizures (Focal Seizures) A. Simple partial seizures 1. With motor signs 2. With somatosensory or special sensory symptoms 3. With autonomic symptoms 4. With psychic symptoms B. Complex partial seizures 1. Simple partial onset followed by impairment of consciousness 2. With impairment of consciousness at the onset

International Classification of Epileptic Seizures

I. C. Partial seizures evolving to secondarily generalized seizures 1. Simple partial seizures evolving to generalized seizures 2. Complex partial seizures evolving to generalized seizures 3. Simple partial seizures evolving to complex partial seizures evolving to generalized seizures

International Classification of Epileptic Seizures

II. Generalized Seizures (Convulsive or Nonconvulsive) A. 1. Typical absence seizures (petit mal) 2. Atypical B. Myoclonic seizures C. Clonic seizures D. Tonic seizures E. Tonic-clonic seizures (grand mal) F. Atonic seizures III. Unclassified Epileptic Seizures IV. Status Epilepticus

Epilepsia 1981; 22: 489-501

Videos

GTC video
Absence sz video

Tonic video
Simple partial Sz video Complex partial Sz video Infantile spasm Chorea video

Epilepsy syndromes
Severe Myoclonic Epilepsy of Infancy Infantile spasms Lennox-Gastaut Syndrome Childhood Absence Epilepsy Benign Rolandic Epilepsy Benign Occipital Epilepsy of Childhood Juvenile Absence Juvenile Myoclonic Epilepsy

Cellular basis of epileptogenesis Cellular basis of epileptogenesis

Astrocyte GABAergic interneuron
Glutamine synthase

Glutamate Transporter GABA-T

Ca++ channel

Na+ channel

Glutamate

NMDA receptor

GABA receptor

Non-NMDA receptor

Presynapse

Postsynapse

Treatment Issues
The goal is to prevent the recurrence of seizures while avoiding side effects of the drug Type of seizure Age of child Male or female Issues of compliances

HISTORY of Antiepileptics
1857 1912 Bromides Phenobarbital Ethosuximide 1937 50ies 1960 70ies Phenytoin
Carbamazepine

1989 Vigabatrin

Zonisamide

1990
Oxcarbazepine 1991

Lamotrigine

Felbamate

Benzodiazepine Valproate

1993
1995 1996 1999

Gabapentin
Topiramate Tiagabine Levetiracetam

Factors Influencing Decision to Treat

Benefits

Freedom from (or lower rate of) seizures

Reduced risk of potential injury or death

Psychological & social benefits of more security from seizures Educational, employment, driving benefits

Risks Potential drug interactions Cost Potential drug side effects (short- & long-term) Inconvenience

Mechanisms of Action and Indications of AEDs

Mechanism Of Action
Sodium channel drugs

Drug
Carbamazepine Phenytoin Oxcarbazepine Gabapentin Tiagabine Vigabatrin

Effective for
Localization related epilepsy

GABA enhancement drugs

Localization related epilepsy

Mechanisms of Action and Indications of AEDs

Mechanism Of Action Mixed mechanism (excitatory amino acid, Na channel, GABA)

Drug

Effective for

Valproate Lamotrigine

Localization related epilepsy & juvenile myoclonic epilepsy Lennox-Gastaut syndrome

Topiramate, Valproate, Clonazepam, Lamotrigine Felbamate

Mechanisms of Action and Indications of AEDs

Mechanism Of Action Mixed mechanism (excitatory amino acid, Na channel, GABA)

Drug

Effective for

Valproate Phenobarbital

Generalized epilepsies

Topiramate Lamotrigine Tiagabine

Clinical Practice GuidelineFebrile Seizures:

Guideline for the Neurodiagnostic Evaluation of the Child With a Simple Febrile Seizure
From the American Academy of Pediatrics
PEDIATRICS Volume 127, Number 2, February 2011
AAAMjjduyusayd FROM THE AMERICAN ACADEMY OF PEDIATRICSFROM THE AMERICAN ACADEMY OF PEDIATRICS

Clinical Practice GuidelineFebrile Seizures: Guideline for the Neurodiagnostic Evaluation of the Child With a Simple Febrile Seizure Febrile seizure is a seizure accompanied by fever (temperature 100.4F or 38C2 by any method), without central nervous system infection, that occurs in infants and children 6 through 60 months of age. Simple febrile seizures
primary generalized seizures lasting for less than 15 minutes

does not recur within 24 hours.

were defined as focal

Complex febrile seizures

prolonged ( 15 minutes)
and/or recurrent within 24 hours

Clinical Practice GuidelineFebrile Seizures: RECOMMENDATIONS:

1. A lumbar puncture should be performed in any child who presents with a seizure and a fever and has meningeal signs and symptoms (eg, neck stiffness, Kernig and/or Brudzinski signs) or in any child whose history or examination suggests the presence of meningitis or intracranial infection. 2. In any infant between 6 and 12 months of age who presents with a seizure and fever, a lumbar puncture is an option when the child is considered deficient in Haemophilus influenzae 3. A lumbar puncture is an option in the child who presents with a seizure and fever and is pretreated with antibiotics, because antibiotic treatment can mask the signs and symptoms of meningitis.

Clinical Practice GuidelineFebrile Seizures: RECOMMENDATIONS:

An electroencephalogram (EEG) should not be performed in the evaluation of a neurologically healthy child with a simple febrile seizure. The following tests should not be performed routinely for the sole purpose of identifying the cause of a simple febrile seizure: measurement of serum electrolytes, calcium, phosphorus, magnesium, or blood glucose or complete blood cell count. Neuroimaging should not be performed in the routine evaluation of the child with a simple febrile seizure.

Head Injuries

CT scan

Plain and contrast studies There are both benefits and risks associated with the use of CT

Risks :

Abnormal test results

---- or a benign or incidental finding, leading to unneeded, possibly invasive, follow-up tests that may present additional risks

increased possibility of cancer induction from x-ray radiation exposure.

Radiation Dose Comparison

Diagnostic Procedure
Chest x ray (PA film) Skull x ray Lumbar spine I.V. urogram Upper G.I. exam Barium enema CT head

Typical Effective Dose (mSv)

Number of Chest X rays (PA film) for Equivalent Effective Dose

1

Time Period for Equivalent Effective Dose from Natural Background Radiation

0.02

2.4 days

0.07 1.3 2.5 3.0 7.0 2.0

4 65 125 150 350 100

8.5 days 158 days 304 days 1.0 year 2.3 years 243 days

CT scan in head injuries

National Institute for Health and Clinical Excellence (NICE) Scottish Intercollegiate Guidelines Network (SIGN) Selection of children (under 16 years) for CT Scan

Urgent scan if any of the following:

Witnessed loss of consciousness >5 minutes Amnesia (antegrade or retrograde) >5 minutes Abnormal drowsiness 3 Discrete episodes of vomiting Clinical suspicion of nonaccidental injury Post-traumatic seizure (no PMH of epilepsy) GCS <14 in emergency room Suspected open or depressed skull fracture or tense fontanelle Signs of base of skull fracture* Focal neurological deficit

Aged <1 - bruise, swelling or laceration on head >5 cm

Dangerous mechanism of injury (high-speed RTA, fall from >3 m, high-speed projectile)

A CT scan is also recommended (within 8 hours of injury) if there is either:

More than 30 minutes of amnesia of events before impact Or any amnesia or loss of consciousness since injury if: Aged 65 years Coagulopathy or on warfarin Dangerous mechanism of injury Road traffic accident (RTA) as a pedestrian RTA - ejected from car Fall >1 m or >5 stairs

Subdural hemorrhage on Non contrast CT scan. Blood is hyperdense (white area)

When is cranial CT scan warranted ?

CT scan or MRI or ultrasound ?

Advantages of CT scan over MRI

Faster, shorter duration

Bone, calcium deposits, blood

Surgical clips, metallic fragments, pacemakers

Patients who are claustrophobic cheaper

Advantages of MRI over CT scan

No radiation Better detail for soft tissues Tumor dtection, epilepsy, MS Superior in the post fossa and Can change plane without moving the patient Contrast dye (gadolinium) is safer than iodine

parasellar regions

Ultrasound

Needs a window (AF, temporal bone)

Screening and follow ups

Limitation for posterior fossa structures

Headache

Worrisome Headache
SNOOP
S : Systemic symptoms (fever, weight loss) N : Neurologic symptoms (confusion, lateralizing signs, impaired consciousness) O : Onset - sudden, new onset, progressive O : Older - > 50 years old P : Previous headache history first headache, change in attack frequency

Migraine in Children

Relatively

common in children

their

frequency increases through adolescence

The mean age of onset is 7.2 years for boys and 10.9 years for girls

International Headache Society

Criteria for Pediatric migraine without aura: A. Greater than or equal to five attacks fulfilling features B-D B. Headache attack lasting one to 72 hours C. Headache has at least two of the following four features:

At

Either bilateral or unilateral (frontal/temporal) location Pulsating quality Moderate to severe intensity Aggravated by routine physical activities Nausea and/or vomiting Photophobia and phonophobia (may be inferred from their behavior)

least one of the following accompanies headache:

2004 classification of headache disorders

Management of Headache
Diagnosis Acute

treatment Preventive treatment

Practice Parameter: Pharmacological Treatment of migraine headache in children and adolescents

Report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society
D. Lewis, MD; S. Ashwal, MD; A. Hershey, MD; D. Hirtz, MD; M. Yonker, MD; S. Silberstein, MD
Published in Neurology 2004;63:2215-2225

Recommendations
Practice Parameter: Pharmacological Treatment of migraine headache in children and adolescents

Ibuprofen is effective and should be considered for the acute treatment of migraine in children. (Class I, Level A) Acetaminophen is probably effective and should be considered for the acute treatment of migraine in children. (Class I, Level B)

Neurology 2004;63:2215-2225

Recommendations

Sumatriptan nasal spray is effective and should be considered for the acute treatment of migraine in adolescents. (Class I, Level A) There is no supporting data for the use of any oral triptan preparations in children or adolescents. (Class IV, Level U) There is inadequate data to make a judgement on the efficacy of subcutaneous sumatriptan. (Class IV, Level U)

Secondary Headaches by Category

C I T T E N D V M

Congenital anomalies (HCP, Arnold-Chiari) Infections (meningitis, abscess) Toxin (cocaine, amphetamine) Trauma (subdural, epidural) Endocrine (hypoglycemia) Neoplasm (brain tumor, leukemia) Degenerative disorder (Alexanders disease) Vascular (aneurysm, AVM, coagulation disorder) Metabolic (hypoxia, dehydration, fever)

Neurologic causes for headache in children

Variables that predict the presence of SOL : 1. headache of less than 1 month 2. Absence of family history of migraine 3. Abnormal neurologic functions on PE 4. Gait abnormalities 5. Presence of seizures
Practice parameter : Evaluation of children and adolescents with recurrent headache.
Neurology 2002; 59 : 490-498

Epilepsy
Can present as headache

Congenital malformations
Congenital Hydrocephalus Arnold Chiari malformation Congenital infection

Increased Intracranial Pressure

The brain occupies a rigid cranial container with a fixed volume with 3 components: Brain CSF 80 % 10 %

Blood

10 %

Increased Intracranial Pressure

Normal intracranial pressure : 0-10 mm Hg, not more than 20 mm Hg Beyond 20 mm Hg = cerebral perfusion pressure falls leading to ischemia Cerebral perfusion pressure- defined as mean arterial pressure minus ICP CPP = MAP - ICP = 50 -150 mm Hg

Management of Increased Intracranial Pressure (ICP)

Mannitol - 6 -carbon sugar similar to glucose
Dose: 0.25 to 1.0 g/kg body weight of a 20% solution Two Effects: 1) Direct Osmotic Effect

2) Decreases blood viscosity ,increases CBF and cerebral oxygenation

Onset of Mannitol: within 1- 5 minutes with a duration of action of 2-3 hours

Furosemide
Furosemide has a synergistic effect to mannitol in decreasing free water.

Hyperventilation
CO2 is a potent cerebral vasodilator Hyperventilation lowers cerebral vascular CO2 and causes rapid vasoconstriction leading to a decreased intracranial blood volume. caution : too much hyperventilation can cause cerebral ischemia

Hyperventilation
Maintain PaCO2 at 33+2 mm Hg (30-35) Aggressive hyperventilation with of PaCO2 <25mmHg can lead to abrupt vasoconstriction and a reduction in cerebral blood flow PaCo2 <30 mm Hg : can lead to vasoconstriction - induced ischemia in areas of uninjured brain thus poorer outcome

Positioning of the Neck

Head elevated at 30 degrees

Fluid & Electrolyte Management

CVP line if indicated
Indwelling catheter for urine output Avoid Hypotonic solutions - can precipitate a fluid shift into brain and exacerbate edema Use plain LR or normal saline for resuscitation and maintain with 5% dextrose with NSS, half saline or Ringers lactate Do not use D5 water

 Treat the cause of increase ICP!

Developmental Delays
Pervasive Development Disorders
ADHD

Autism
GDD Isolated speech delay

Shukran !!