A Practical Approach To Common Child Neurology Problems

A
PRACTICAL APPROACH TO

COMMON CHILD NEUROLOGY
PROBLEMS

Myrna S. Fojas, MD

Child Neurologist
Diplomate, American Academy of Neurology and Psychiatry with Special Qualification in Child Neurology
Diplomate, American Academy of Pediatrics
Diplomate, Philippine Board of Neurology and Pediatrics

SEIZURES
HEADACHES
APPROACH TO A PATIENT WITH
SEIZURE
IS IT A SEIZURE?
NO
SEIZURE
IS IT A SEIZURE?
NO
SEIZURE
(not a seizure)

-‐Apnea -‐Breathholding -‐Tics

-‐Jitteriness -‐Benign Myoclonus -‐Syncope
-‐Benign Neonatal -‐Shuddering -‐Pseudoseizure
Sleep Myoclonus -‐Sandifer -‐Parasomnia
-‐Hyperekplexia -‐Dystonia -‐Paroxysmal
Dyskinesia
-‐Paroxysmal
Vertigo
SEIZURE
IS IT A SEIZURE?
NO
SEIZURE
SEIZURE
SEIZURE
-‐Hypoglycemia
-‐Hyponatremia
-‐Hypocalcemia
-‐Hypomagnasemia
-‐Hypoxia (Hypoxic seizure secondary to syncope/breathholding
-‐Benign Febrile Convulsion
Approach to a patient with
seizure

§  -‐Subtle seizures may present as apnea,
swimming motion
§  *May need a video EEG to prove or
dispute
SEIZURE
AED’s
required?
YES
Need for
YES maintenance
AED’s NO
NO
SEIZURE
Common causes for unprovoked
seizures
Brain
injury
Genetically Unprovoked Congenital

acquired Seizures Malformations
Structural
SEIZURE
EPILEPSY
Conceptual definition of seizure and epilepsy-‐(ILAE,
2014)
§  An epileptic seizure is a transient occurrence of signs
and/or symptoms due to abnormal excessive or
synchronous neuronal activity in the brain
§  Epilepsy is a disorder of the brain characterized by
an enduring predisposition to generate epileptic
seizures, and by the neurobiologic, cognitive,
psychological and social consequences of the
condition.
§  The definition of epilepsy requires the occurrence of
at least one epileptic seizure.
EPILEPSY
Operational (Practical) Clinical Definition of
Epilepsy (ILAE, 2014)

1.  At least two unprovoked (or reflex) seizures
occurring more than 24 hours apart;
2.  One unprovoked (or reflex) seizure and a
probability of further seizures similar to the
general recurrence risk (at least 60%) after two
unprovoked seizures, occurring over the next 10
years
CLASSIFICATION OF EPILEPSY
CLASSIFICATION OF SEIZURES
UPDATES ON NOVEL
TERMINOLOGIES
EPILEPSY SYNDROMES
(Electroclinical Syndromes)
§  Complex of clinical features, signs and

symptoms that together define a distinctive,
recognizable clinical disorder
§  Defined on the basis of age of onset, specific
EEG findings, seizure types and other
features which combine to form a specific
diagnosis
INFANTILE SPASMS

ú  also known as West Syndrome
ú  consist of:
  Infantile spasm
  developmental regression
  specific pattern on electroencephalography (EEG)
testing called hypsarrhythmia (chaotic brain waves).
Infantile spasms
§  Age of onset:
ú  < 1 year old, typically between 4-‐8 months
§  Seizure description:
ú  primarily consist of a sudden bending forward of
the body with stiffening of the arms and legs;
some children arch their backs as they extend
their arms and legs
ú  Spasms tend to occur upon awakening or after
feeding, and often occur in clusters of up to 100
spasms at a time. Infants may have dozens of
clusters and several hundred spasms per day
Infantile spasms
§  EEG findings:

ú  Hypsarrhythmia
  chaotic
  high-‐ to extremely high-‐voltage
  multifocal spikes and wave discharges
Infantile spasms
§  Prognosis:
ú  usually stop by age 1-‐2 only to be replaced by
other types of sz
ú  mental retardation
ú  close relationship between IS and Lennox-‐Gastaut
Syndrome
ABSENCE SEIZURES
§  Brief, sudden lapses of consciousness which

usually present with a vacant stare, which
may be mistaken for a lapse in attention that
lasts 10 to 15 seconds, without any
subsequent confusion, headache or
drowsiness, followed immediately by full
recovery
Absence Seizures
§  Age of Onset:

ú  More common in children between the ages 4 and
10
§  Seizure description:
ú  Blank stare with any of the following:
  Sudden stop in motion without falling, lipsmacking,
eye flutters, chewing motions, finger rubbing or
small movements of both hands
Absence Seizures

ú  Normal background activity
ú  Bursts of frontally predominant, generalized 3-‐Hz
spine-‐and-‐wave complexes
ú  Hyperventilation: often provokes these seizures
and should be a routine part of all EEGs in children
Absence Seizures
§  Prognosis:
ú  Remission rate is good: 80% respond to
medications
ú  Early onset of absence seizures, quick response to
therapy and normal EEG background are good
prognostic signs

BENIGN ROLANDIC EPILEPSY
§  Also known as Benign Epilepsy with

Centrotemporal Spikes (BECTS)
§  Represents about 15% of all epilepsies in
children
Benign Rolandic Epilepsy
§  Age of Onset:

ú  Typically begins at around ages 6-‐8 (as early as 4yo)
§  Seizure Type:

ú  Characterized by twitching, numbness or tingling of
the face or tongue, which may interfere with speech
and cause drooling
ú  Seizures may spread and become generalized
seizures
ú  Usually occur during sleep, and lasts no more than 2
minutes
ú  If sz remain focal, child may remain aware and
conscious
ú  Diphasic consisting of high-‐voltage, spikes or

sharp waves followed by slow waves (‘centro
temporal’ or ‘rolandic spikes’ which tend to occur
in clusters but an also be isolated
ú  30% of them appear only during sleep
§  Prognosis:
ú  Age of remission is 10-‐14 years

Callenbach, P.M.C., et al. (2010). Long term outcome of benign childhood epilepsy
with centrotemporal spikes: Dutch study of Epilepsy in Childood. Seizure. Vol 18,
Issue 8, pp 501-‐506
JUVENILE MYOCLONIC EPILEPSY
§  Idiopathic generalized epileptic syndrome

characterized by myoclonic jerks, generalized
tonic-‐clonic seizures and sometimes absence
seizures
§  Myoclonic jerks or seizures without
impairment of consciousness
Juvenile Myoclonic Epilepsy
Age of onset:

§  Typically begins in adolescence, peak age
of 12-‐18 years
ú  If absence seizure is a feature: usually begin
between 5-‐16 years
ú  Mypclonic jerks follow 1-‐9 years later, usually
around the age of 15 years
ú  GTCs typically appear a few years later than
myoclonic jerks

ú  Generalized 4-‐ to 6-‐Hz spike or polyspike and
slow-‐wave discharges lasting 1-‐20 seconds
ú  Usually, 1-‐3 spikes precede each slow wave
ú  When absence seizures are also present, 3-‐Hz
spike-‐and-‐wave activity may be see in addition to
the polyspike-‐and-‐wave pattern
§  Prognosis:
ú  Good when treated with appropriate drugs, but
with a well-‐known tendency to relapse after
withdrawal
ú  Majority of patients have continuing seizures after
a follow-‐up of two decades
ú  17% are able to discontinue medication and
remain seizure-‐free thereafter

Baykan, B., et al. (2013). Lifetime prognosis of Juvenile Myoclonic
Epilepsy. Epilepsy and Behavior. Volume 28, Supplement 1, pp S18-‐S24
DIAGNOSTICS
§  EEG
§  (?) Video EEG
§  Neuroimaging
§  Bloodworks (CBC, electrolytes, liver function
test, assays)
PRINCIPLES OF TREATMENT
Drug Interactions

CURE (ILAE, 2014)
§  Epilepsy is considered to be resolved for

individuals who:
ú  had age-‐dependent epilepsy syndrome but are
now past the applicable age
ú  those who have remained seizure-‐free for the last
10 years, with no seizure medicines for the last 5
years
APPROACH TO A PERSON WITH
HEADACHE
HEADACHE
ACUTE CHRONIC
HEADACHE
HEADACHE
ACUTE CHRONIC
ACUTE HEADACHE
Patient febrile Patient afebrile
No nuchal Abnormal neurologic Normal neurologic

Nuchal
rigidity findings (nuchal rigidity) findings
rigidity
CT scan Ask about drug use;
General examination Lumbar Puncture check BP and CBC
Sinus xray views if No mass Mass
indicated If normal, follow for possible
Lumbar Puncture chronic headache or
Treat underlying Normal CSF
intracranial mass lesion
Meningitis
illness Urinalysis
RBC in CSF
Follow until well Chest roentgenograms WBC in CSF
Look for head and neck infections Subarachnoid hemorrhage
HEADACHE
HEADACHE
ACUTE
CHRONIC
History: CHRONIC HEADACHE
Description of headache,
change over time; Family history;
Examination
Normal neurologic
findings
Episodic nonprogressive Increasingly severe Abnormal

headache headache CT scan neurologic findings
Criteria for migraine Mass Normal
Lumbar Puncture
Present Absent
Normal Increased pressure

Migraine Episodic headcahe Persistent headache Cells
Normal cells
Consider: Occipital neuralgia; TM
Migraine protein, glucose
Consider: Tension headache
Rule out hypertensive joint dysfunction; Muscle Chronic
Tension
headcahe contraction; Sinus disease; Ocular Pseudotumor meningitis
Muscle contraction
disorder Psychogenic disorder
PEDIATRIC MIGRAINE WITHOUT AURA
DIAGNOSTIC CRITERIA (American
Headache Society)
§  A. At least 5 attacks fulfilling B-‐D
§  B. Headache attack lasting 30 minutes to 48
hours
§  C. Headache has at least 2 of the following:
ú  1. Bilateral (frontal/temporal) or unilateral location
ú  2. Pulsating quality
ú  3. Moderate to severe intensity
ú  4. Aggravation by routine physical activity
§  D. During headache, at least 1 of the folowing:
ú  1. Nausea and/or vomiting
ú  2. Photohobia and phonophobia
Pediatric vs. Adult Migraine
PEDIATRIC ADULT
•  1-‐72 hours duration •  4-‐72 hours duration

•  Unilateral or bilateral (frontal/ •  Typically unilateral
temporal) •  Pain intensity (moderate/severe) and
•  Younger children often cannot quality (pulsatile) included in the
describe pain; quality/intensity; diagnostic criteria
otherwise similar to adults
•  Comorbidity: motion sickness (49%
of patients)
MANAGEMENT OF PEDIATRIC
MIGRAINE
§  Treatment Goals:
ú  Educate parents and patients regarding migraine
triggers
ú  Formulate a plan of treatment for acute attacks
ú  Consider prophylaxis for patients with frequent
migraines
Management of Acute Attacks
§  Sleep: most potent antimigraine treatment

§  Simple analgesics: Paracetamol/Ibuprofen
§  Promethazine: diminishes nausea, causes
drowsiness and decreases pain

Migraine prophylactics

§  Propranolol
§  Anticonvulsants (Topiramate)

A Practical Approach To Common Child Neurology Problems

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A

PRACTICAL APPROACH TO

Myrna S. Fojas, MD

-­‐Apnea -­‐Breathholding -­‐Tics

Genetically Unprovoked Congenital

§ Complex of clinical features, signs and

§ EEG ﬁndings:

§ Brief, sudden lapses of consciousness which

§ Age of Onset:

§ EEG ﬁndings:

§ Also known as Benign Epilepsy with

§ Age of Onset:

§ Seizure Type:

§ EEG ﬁndings:

ú Diphasic consisting of high-­‐voltage, spikes or

§ Idiopathic generalized epileptic syndrome

Age of onset:

§ EEG ﬁndings:

§ Epilepsy is considered to be resolved for

Patient febrile Patient afebrile

No nuchal Abnormal neurologic Normal neurologic

Episodic nonprogressive Increasingly severe Abnormal

Criteria for migraine Mass Normal

Normal Increased pressure

• 1-­‐72 hours duration • 4-­‐72 hours duration

§ Sleep: most potent antimigraine treatment

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-‐Apnea -‐Breathholding -‐Tics

§  Complex of clinical features, signs and

§  EEG ﬁndings:

§  Brief, sudden lapses of consciousness which

§  Age of Onset:

§  EEG ﬁndings:

§  Also known as Benign Epilepsy with

§  Age of Onset:

§  Seizure Type:

§  EEG ﬁndings:

ú  Diphasic consisting of high-‐voltage, spikes or

§  Idiopathic generalized epileptic syndrome

§  EEG ﬁndings:

§  Epilepsy is considered to be resolved for

•  1-‐72 hours duration •  4-‐72 hours duration

§  Sleep: most potent antimigraine treatment