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Case 19
16-MONTH-OLD WITH A FIRST SEIZURE - IAN
Author: Sherman Alter, M.D., Wright State University School of Medicine; and
Michael Barone, M.D., Johns Hopkins University

Summary of clinical scenario: 16-month-old Ian is found unresponsive by


paramedics responding to a 911 call placed by Ians father after he demonstrated
a shaking episode. Students consider the differential diagnosis of an unresponsive
toddler. When Ian is brought to the emergency department, his behavior appears
postictal. The case explores how to differentiate true seizures from
pseudoseizures, as well as how to classify seizures. Various potential etiologies for
fever are considered, and indications for performing a lumbar puncture and the
work-up of fever without a source, are reviewed. The case includes a discussion of
simple febrile seizures, their recurrence risk, potential treatment, and subsequent
risk of epilepsy. The case concludes when Ian returns three days later for
follow-up with a blanching, non-pruritic, generalized maculopapular rash
consistent with roseola infantum (sixth disease).

Generalized seizure
History of irritability and possible
lethargy
Key Findings from
High fever on presentation
History
Normal developmental history
Positive family history of febrile
seizure

Febrile: 39.5 degrees C (rectal)


Dry mucous membranes
Key Findings from Neurologic exam: Alert, no ataxia
Physical Exam Deep tendon reflexes 2+ bilaterally,
good muscle tone bilaterally, toes
downgoing

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Febrile seizure
CNS infection (meningitis/encephalitis)
Differential
Idiopathic epilepsy
Diagnosis
Ingestion/poisoning
Head trauma

Complete blood count (CBC): Normal


Key Findings from
Cerebrospinal fluid (CSF): Normal
Testing
without organisms

Final Diagnosis Simple febrile seizure

Case highlights: Photo of a toddler with a maculopapular rash on his chest.

Key Teaching Points


Knowledge
Causes of unresponsiveness in a child:

Seizures

Causes include metabolic disturbances, head trauma, genetic syndromes,


developmental abnormalities, fever, idiopathic

Syncope due to breath-holding spells

Common between ages 1 and 3 years


May be cyanotic or pallid
Episodes quickly self-resolve; no postictal state

Infection (meningitis/encephalitis)

Up to 2040% of children with meningitis can present with seizure activity


Children with encephalitis will frequently present with fever and seizure
Enteroviral and herpes simplex virus infections are typical pathogens for
encephalitis

Toxic ingestions/poisoning

Most commonly seen between ages of 9 months and 3 years.


Various medications may cause unresponsiveness (e.g., opiates,
benzodiazepines, clonidine, oral diabetic agents)

Head trauma with loss of consciousness

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Intracranial processes (tumor/hemorrhage)

Intussusception

Lethargy with a near-unresponsive state may be seen between episodes of


colicky pain
Dehydration due to vomiting and third-spacing of fluids may lead to mental
status changes

Seizures: Result of excessive neuronal activity in the brain.

Classification

Generalized tonic-clonic seizure:


Most common type in children
Begins abruptly with tonic (rigid) stiffening of all extremities and
upward deviation of the eyes
Clonic jerks of all extremities follow the tonic phase
Finally, child becomes flaccid; urinary incontinence may occur
Absence epilepsy (petit mal seizures):
Generalized seizure, but consciousness is regained more quickly than
seen in a generalized tonic-clonic seizure
Seen in children starting around age 3
Characterized by loss of environmental awareness and automatisms
Not associated with loss of tone
May be precipitated by hyperventilation or photic stimulation
Simple partial seizures:
Motor signs in a single extremity or one side of the body
Focal onset seizure activity may spread to become generalized
Complex partial seizures:
Alteration of consciousness is hallmark feature
Signs and symptoms tend to localize around the eyes, the mouth, and
the abdomen
Commonly accompanied by automatisms, quasi-purposeful motor or
verbal behaviors that are repeated inappropriately
Lasts 30 seconds to 2 minutes and are associated with postictal phase
Secondary generalization can occur

Other conditions that mimic seizures

Motor tics
Myoclonus
Gastroesophageal reflux (Sandifers syndrome)
Pseudoseizure

Febrile seizures

Etiology:

Typically a benign and self-limited illness like a viral infection causes a fever

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that triggers a seizure in a susceptible host (young child, positive family


history, etc.).
With more serious central nervous system infection (such as meningitis or
encephalitis), the infection itself causes both fever and seizure.
Prolonged fever prior to the seizureespecially with irritability or
inconsolabilityis an indication of a more serious central nervous system
condition causing the seizure.

Epidemiology:

One of the most common reasons for seizures in children


Febrile seizures occur in children ages 660 months at a frequency of 24%
and tend to occur early in the febrile illness (often on the first day).
Febrile seizures are hereditary, but mode of inheritance is unclear.

Classification:

Simple febrile seizure: More common, last < 15 minutes, occur once in a
24-hour period, generalized
Complex febrile seizure: Less common, last > 15 minutes, occur more than
once in a 24-hour period, focal

Recurrence risk:

If a child has a first febrile seizure before age 12 months, the recurrence
risk for a second febrile seizure is about 50%. If first seizure is after 12
months of age, the recurrence risk is about 30%. Parents should be
reassured that recurrent, simple febrile seizures have no long-term effects
on child development.

Risk of epilepsy

Risk of developing epilepsy in children with simple febrile seizures is slightly


increased above the 0.51% baseline population risk.
Epilepsy more common among children with early, recurrent febrile
seizures, especially if there is a family history of epilepsy.
Children with complex febrile seizures and those with pre-existing
developmental abnormalities are at increased risk.

Fever without a source

Viral infection

Many common viral infections (e.g., enterovirus, adenovirus) may cause


significant fever in a young child without any additional clinical signs or
symptoms (e.g., congestion, cough, diarrhea, or rash).

Occult bacteremia

Occult bacteremia may also cause fever without additional clinical signs or
symptoms.

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Febrile children ages 336 months without a discernible focus of infection


may have an "occult" bacteremia, usually caused by Streptococcus
pneumoniae or Hemophilus influenza, type B.
Since the introduction of the protein-polysaccharide conjugate
pneumococcal vaccine (PCV-7), the rates of invasive pneumococcal
infections have declined. And Hemophilus influenzae, type Bonce a feared
pathogenis now uncommon in the U.S. due to universal vaccination.
Undiagnosed, the child with occult bacteremia is at risk for the development
of a more serious bacterial infection, such as meningitis or osteomyelitis,
through bacterial seeding of these distant sites.

Urinary tract infection (UTI)

Common cause of fever in children


Because small children cannot complain of dysuria, frequency, or costo-
vertebral angle (CVA) tenderness, UTI must be ruled out by laboratory
testing.
Clinical practice guidelines suggest when it is appropriate to obtain a urine
specimen in the workup of a fever without a source.

Bacterial meningitis: One of the most potentially serious infections in infants


and children.

Epidemiology:

Increasingly uncommon due to immunization (and herd immunity), but


potentially devastating.

Etiology:

Bacterial meningitis in immunized children 2 months to 12 years of age is


usually due to Streptococcus pneumoniae or Neisseria meningitides.
In younger infants, gram negative organisms, such as Escherichia coli,and
organisms like Group B Strep (Streptococcus agalactiae) need to be
considered.

Presentation:

May present with increasing lethargy and irritability, as well as signs of


meningeal irritation (nuchal rigidity, or meningismus).
Alternatively, non-specific findings, including fever (in 9095% of cases),
anorexia, poor feeding, symptoms of an upper respiratory infection,
myalgias, and tachycardia may predominate.

Treatment:

In cases in which meningitis is highly suspected, antibiotics are generally


given empirically as soon as the cerebrospinal fluid culture is obtained (and
in severe cases, even beforehand).
Start with high-dose intravenous antibiotics directed at the most likely

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organisms (usually a third-generation cephalosporin and vancomycin). Then


tailor antibiotics based on sensitivities.
Treatment duration: 714 days.

Complications:

Stroke, subdural effusions, and syndrome of inappropriate anti-diuretic


hormone (SIADH) secretion
Developmental delay, seizures, and hearing loss
Unusual for treated meningitis to be fatal

Bacterial and viral meningitis findings in CSF:

Bacterial
Viral meningitis
meningitis
CSF laboratory results (as compared to
(as compared to
normal)
normal)

CSF glucose Decreased Normal

CSF glucose: blood


Decreased Normal
glucose ratio

CSF protein Increased Normal

CSF WBC count Increased Increased

Percentage of
polymorphonuclear
Increased Variable
cells
in CSF WBCs
Skills
History:

Obtaining a seizure history:

Remember that parents or other observers may be upset and have difficulty
recalling details.
Ask open-ended questions:
Why did you suspect something was wrong?
What was the first thing you noticed?
Did you notice any movements in his arms or legs? How would you
describe them?

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Establish a clear timeline of events leading up to and through the episode,


including how child recovered.
Supportive evidence for a true seizure (as opposed to a pseudoseizure)
includes:
History of alteration or loss of consciousness
Incontinence
Deviation of eyes
Rhythmic motor movements that cannot be stopped by touching or
holding the child
Postictal state
Also ask about precipitating events (possibility of toxic ingestion, head
injury [recent or distant], illnessespecially with fever), patients mental
status prior to the event, and whether the symptoms are generalized or
focal.
Additional history should include relevant medical information such as a past
history of seizures, developmental delay, prenatal and perinatal history, and
family history of seizure disorders or neurologic problems.

Differential diagnosis
1. Febrile seizure: Febrile seizures usually occur on the first day of the
illness. Fever > 38 degrees C is typically seen. Children with febrile seizures
are developmentally normal and often have a positive family history for
other first-degree relatives with febrile seizures as children.

2. Infection (meningitis/encephalitis): Approximately 30% of infants with


meningitis present with a seizure. However, they typically have other signs
of illness (vomiting, persistent lethargy, behavior change).Because clinical
signs and symptoms of meningitiscan be subtle in young children, a febrile
child with irritability and a seizure (especially if he has not recovered to
normal activity and behavior after a period of observation) should have
meningitis included in the differential diagnosis, even when the physical
exam does not demonstrate a stiff neck.

3. Epilepsy: A fever often triggers the first seizure in a child with epilepsy.
Although some children with epilepsy also have developmental delay due
genetic, congenital or acquired disorders (symptomatic epilepsy), many
children with epilepsy are developmentally normal (idiopathic epilepsy).

4. Ingestion/poisoning: A poisoning (accidental ingestion) may lead to a


generalized seizure. Examples include hypoglycemia due to a diabetic
medication, lead poisoning, and acute alcohol poisoning.

5. Post-traumatic seizure: These generally occur one to two hours after a


head injury. Important to keep non-accidental injury in mind.

Studies

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Diagnostic studies for fever without a source with central nervous system
symptoms:

White blood cell count and differential: A low or high white blood cell count,
as well as a left shift, are sensitive indicators of a possible bacterial infection.

Complete blood count (CBC) and blood culture: In many cases occult
bacteremia resolves it on its own without sequelae; however, there is the
possibility that untreated occult bacteremia may go on to seed other sites, such as
the meninges or a bone or joint.Risk of disease is low but burden of disease may
be high (e.g., missed bacteremia progressing to bacterial meningitis). Especially
important to obtain a CBC and blood culture when immunization status is unclear.

Urinalysis: A urinalysis, looking for evidence of pyuria and bacteria, should be


checked routinely in males < 6 months old (< 12 months old if uncircumcised)
and females < 12 months old.

Urine culture: A positive culture is the gold standard for diagnosing infection. A
urine culture should be obtained from males < 6 months old (< 12 months old if
uncircumcised) and females < 12 months old. Cultures are only as good as the
sample, so most pediatricians will catheterize infants and toddlers to obtain the
specimen.

Lumbar puncture:T he younger the child, the more subtle the signs of
meningitis may beVery young infants (<3-under 6 months) with bacterial
meningitis may not show signs of nuchal rigidity and should undergo lumbar
puncture for spinal fluid. Strongly consider lumbar puncture after the first seizure
in a child < 12 months. Potentially consider lumbar puncture in children 12 to 18
months. A lumbar puncture should also be a strong consideration in children
older than 6-12 months with atypical seizures, children whose immunization
status is incomplete or unknown, or if there is persistent irritability in children
slow return to baseline behavior.

Management
Fever:

If child is uncomfortable, may give nonsteroidal anti-inflammatory


medicines (i.e., acetaminophen, ibuprofen).
However, studies show that this does not prevent recurrence of febrile
seizure.

Seizure:

A child experiencing seizure should be placed on his side to avoid choking


on stomach contents or saliva.
Keep child in safe setting to minimize risk of injury while seizing.
Do not put anything in his mouth.
Do not restrain movements.

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Call 911 if seizure lasts > 5 minutes.

Anti-seizure prophylaxis

Should not be used for children with simple febrile seizures due to serious
side effects

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