DIFFERENTIAL: (1) CNS infection especially if the child has had fever for 1 to 2 d and then develops seizures. (2) Underlying seizure disorder where fever triggered an event. (3) Febrile seizure: an age-limited, genetic epilepsy in which seizures occur only with fever.

DEFINITIONS: Simple febrile seizure: brief (<15 min), generalized (non-focal), once in 24 h. Complex febrile seizure: duration >15 min (hard to define for parents; good rule of thumb is whether still seizing after the parents have left the house), focal or secondarily generalized, or > once in 24 h. EPIDEMIOLOGY: Most common between ages 6 mo to 6 yo. Risk factors: close relative with a hx of a febrile seizure. Roseola and AOM are common precipitants. EXAM: Simple febrile: normal exam. Meningitis: headaches ± stiff neck. Encephalitis: altered mental status (AMS) before the seizure (somnolence, not following commands, not acting appropriate, and irritable). Children with meningitis/encephalitis do not tend to wake up after a seizure. Other: If focality on exam consider stroke, infection, or Todd paralysis (see “Weakness/Gait Difficulties” section below).

DIAGNOSTIC STUDIES: (1) Fever workup: strongly consider LP: if <12 mo (meningismus frequently absent); 12 to 18 mo where meningismus is subtle; >18 mo if recently on antibiotics (partially treated meningitis) or meningeal
signs. (2) EEG: not recommended for first simple febrile seizure. EEG indicated for complex febrile seizure. Also consider if child not neurologically normal at baseline, has developmental delay, or family history of epilepsy. (3) Neuroimaging: not recommended for first simple febrile seizure. For complex febrile seizure, brain MRI is indicated to evaluate for a mass, hemorrhage, or CNS dysgenesis.

TREATMENT: (1) Around-the-clock antipyretics not shown to decrease risk of recurrent seizures, can be discussed with the family as they may feel more comfortable knowing about this option; remember that antipyretics can mask a serious infection. (2) Simple febrile seizure: no need for long-term treatment with anticonvulsive medication. Consider giving family rectal diazepam prescription for seizure lasting >5 min (0.5 mg/kg if <5 y; 0.3 mg/ kg if 6-11 y; 0.2 mg/kg if >11 y). (3) Complex febrile seizure: consider prophylactic AEDs if family history of epilepsy, child does not recover rapidly, or frequent complex febrile seizures. Consider Depakote (avoid in children <2 yo due to risk of liver failure), levetiracetam, topiramate; needs follow up with neurologist, brain MRI, and EEG. If a child presents with frequent complex febrile seizures, consider Dravet syndrome, which is associated with developmental delay in the first 1 to 2 years.

PROGNOSIS: Good. Risk of epilepsy: 1% after simple febrile seizure (same as general population), ˜2% if recurrent febrile seizures, abnormal neurologic exam or developmental delay is an important predictor of epilepsy risk. RECURRENCE RISK: Febrile seizures tend to recur (30% if first seizure occurs in >12 mo; 50% if <12 mo). Simple febrile seizures tend to decrease in frequency as the child gets older. Risk increases with family history of febrile seizure or history of low temperature with seizure onset.

DEFINITIONS AND CONSIDERATIONS: Non-febrile seizure, non-provoked (not due to trauma, illness, or other medical condition) in a child not known to have seizures. Characterize seizure type: preserved or AMS, focal vs. generalized, increase tone (tonic), jerking (clonic, myoclonic), or loss of tone (atonic).

MAJOR RISKS: Congenital malformations (cortical dysplasia, TORCH infection), neonatal seizures, or family history of epilepsy (genetic). If a child presents with partial seizures, strongly consider focal brain lesion. Intractable seizures in children <2 yo are associated with mental retardation. Greatest probability of mental retardation in descending order is myoclonic > tonicclonic > complex partial > simple partial. HISTORY: Decide if seizure vs. non-seizure paroxysmal event. Until the event is proven to be a seizure, better to define the event as “spell.” Of importance: What happened before the event (more important than what happened after)? What do the parents believe is the cause? What time did it happen? Was there an aura? Has this happened before? SEMIOLOGY: Changes in behavior, cry (seizure cry is usually at the beginning and then stops), slurred speech, head/eye deviation (eyes should look away from the seizure focus), posturing, jerking, automatism, generalized or focal movements, apnea/cyanosis, and autonomic signs (pupil size, drooling, heart rate, incontinence, pallor, vomiting, LOC). Postictal period: amnesia of the event, confusion, lethargy, sleepiness, headache, and transient focal weakness (Todd paralysis).

(1) Without prominent alteration of awareness: benign neonatal sleep myoclonus (infant already asleep), GERD, shuddering attacks, hyperekplexia (marked susceptibility to startle), benign paroxysmal torticollis, benign
paroxysmal vertigo, self-gratification phenomena (“infantile masturbation”), tics, behavioral stereotypies, hyperventilation and anxiety attacks, paroxysmal dyskinesias (kinesigenic, non-kinesigenic), and migraine. (2) With alteration of awareness: syncope, breath-holding spells, daydreaming, non-epileptic attacks (pseudoseizures), and narcolepsy/catatonia. EXAM: Special focus on vital signs—elevated BP (posterior reversible encephalopathy syndrome/PRES), head circumference in children <3 yo, skin exam, including hypochromatic or café au lait spots (neurocutaneous syndromes), evidence of trauma.

DIAGNOSTIC STUDIES: Depends on history: if back to baseline, >6 mo, and history not suggestive of seizure, generally not necessary. (1) LP: if possibility of meningitis/encephalitis. (2) EEG: used to define an epilepsy syndrome, sometimes when there is difficulty determining if spell was seizure by history. Normal EEG associated with favorable outcomes, can be done as outpatient; sleep deprivation increases the sensitivity. If high suspicion of seizures and EEG negative, consider repeating. (3) Neuroimaging: emergent NCHCT if prolonged focal deficit. MRI (outpatient OK) with seizure protocol if: focal seizure, history of developmental delay, cognitive problems, abnormal neurologic exam, abnormal EEG, or <1 yo. TREATMENT: AEDs not indicated after first nonfebrile seizure, normal EEG, and imaging. If abnormal EEG and/or brain MRI, consider starting AEDs. Will need neurologic follow-up. COMPLICATIONS: Most children with 1st unprovoked seizure have no or few recurrences (30% to 50% recurrence within 2 y after 1st seizure). Epilepsy risk increased if abnormal EEG findings or history/findings of remote brain injury.

DEFINITION: Seizure lasting > 30 min or > two sequential seizures with no return to baseline, medical emergency. Current understanding: recurrent seizures become harder to treat, most seizures last 2 to 3 min; therefore, current recommendation is to treat seizures lasting >5 min. Classification: Generalized SE and focal SE (epilepsia partialis continua, aura continua, limbic SE, and hemiconvulsive status with hemiparesis). Epidemiology: Most common age group is <3 yo. 1/3 is initial presentation of epilepsy, 1/3 have preexisting epilepsy, and 1/3 are acute symptomatic. If seizure lasts >60 min, it is defined as refractory SE. ETIOLOGY: Febrile seizures account for >1/3 of all SE in childhood. Other causes: Epilepsy, withdrawal or change in AEDs, CNS infection, cerebral hypoxia/metabolic disturbance, and acute insults (stroke, trauma). EXAM ON PRESENTATION: Vital signs: BP/HR— bradycardia (increased ICP?), hypertension may be the cause of seizures (hypertensive encephalopathy, PRES). Body temperature: cold/clammy (sepsis, hypoglycemia), warm (meningitis/encephalitis). Head: otorrhea, rhinorrhea (skull fracture, trauma). Skin: rash (sepsis). Brief neuro exam: pupils/posture – decorticate/decerebrate posturing may be confused with tonic seizure, focality (mass, herniation). DIAGNOSTIC STUDIES: (1) Labs (before giving any treatment): glucose, Chem 20, CBC/diff, U/A, blood/urine cultures (if febrile), toxicology (full serum and urine), AED levels on patients (asses compliance; for phenytoin/phenobarbital level corresponds with efficacy). (2) LP: if concern for CNS infection. (3) Neuroimaging: NCHCT, when unclear reason for SE, concern for trauma. EEG, when patient stable, or for SE lasting >30 min, may need LTM. INITIAL TREATMENT: ABCs: O₂ by mask, CV monitor, correct metabolic derangement. MEDICATION PREPARATION: Always be one step ahead: when med is given, order the next med, hold in case needed. Do not wait to find out whether the 1st med is effective or not before ordering the next. Time is of the essence! Maintain
euglycemia (start normal saline with dextrose), treat pyrexia; when safe, place nasogastric tube to empty stomach contents.

Alternative medications: Can be used instead of fosphenytoin or phenobarbital if patient is already known to be taking them. (1) Valproic acid: 30 mg/kg load, caution if liver or mitochondrial disease. (2) Levetiracetam: 20 to 40 mg/kg load. (preferred given little interaction with other AEDs, good side effect profile, no need for monitoring drug levels).

If refractory SE: Intubate (if not already), EEG to monitor for burst suppression. (1) Pentobarbital: load 20 mg/kg IV push, maintenance dose 1 to 2 mg/kg/h. Titrate to burst suppression. (2) Midazolam: load 0.15 to 0.3 mg/kg IV push, maintenance dose 0.1 mg/kg/h, but may need to increase due to tachyphylaxis, titrate to burst suppression. Disposition: Admit to ICU. Complications: Cardiac arrhythmia, cerebral edema, hypotension, pneum onia, rhabdomyolysis, and dehydration. Outcome is etiology-dependent.

The diagnosis is based on EEG, the common cause of coma in the pediatric ICU. Consider NCSE when a known epileptic patient presents with persistent AMS, or after the patient presents with GTC, movement stops after AEDs are administered but patient not back to baseline after reasonable time (related to dose/type of medication given). CLASSIFICATION AND TREATMENT: (1) Subtle SE: following cessation of motoric convulsions but mental status not back to baseline. Most common, needs to be treated as SE with same medications, typically needs burst suppression on EEG for at least 24 to 48 h. Prognosis depends on time between onset and diagnosis as well as underlying etiology. (2) Absence SE: presents with confusion, disorientation, and decreased speech. Can present with minor motor disturbances including jerks, head drops, automatism, and autonomic features. Typically does not have long-term sequelae even if it lasts for days. Treat as SE. Valproic acid used over fosphenytoin, as later can exacerbate SE. Levetiracetam currently being studied. (3) Complex partial SE: fluctuating, with or without automatism or eye deviation, no significant sequelae after resolution. Treat as SE. Good response to benzodiazepines and fosphenytoin. (4) Epileptic encephalopathy: early myoclonic encephalopathy, infantile spasms, Otahara, Dravet, or Lennox-Gastaut syndromes, myoclonic status in nonprogressive encephalopathy, Landau-Kleffner syndrome, and epilepsy with continuous spike and wave during slow wave sleep (ECSWS). EEG needed for diagnosis and to direct therapy. Consider in children with developmental regression including autistic regression (see Chapter 5 for extended discussion).

QUESTIONS TO ASK IF CONCERN FOR MIGRAINE: General: Car sickness when younger; HA associated with nausea/emesis; aura preceding the attack (visual, paresthesias, weakness); photo- or phonophobia; aggravated with stress, fatigue, lack of sleep, certain foods, menstrual cycle; alleviated with sleep/dark room. Family history: Do not ask, “Does anyone in your family have migraines?” Ask instead, “Does anyone suffer from HAs?” Some parents consider “sinus HAs” different from migraines or don’t realize family members’ HAs are classified as migraines (because infrequent, mild, uncomplicated, etc.).

MIGRAINE: Presents in children with either uni- or bi-hemispheric HA, pulsating/throbbing pain, and free interval time during attacks. Common migraine has no aura; classic migraine has an aura (prodrome). Can present without HA, e.g., nausea and brainstem signs (“Alice in Wonderland” syndrome, opthalmoplegia). BASILAR MIGRAINE: HA associated with nausea/emesis and either vertigo, nystagmus, visual disturbances, ataxia, diplopia, decreased level of consciousness, decreased hearing, tinnitus, dysarthria, or other bulbar symptoms. FAMILIAL HEMIPLEGIC MIGRAINE (FHM): Usually presents with HA followed by slowly (over few minutes) migrating pattern of weakness or sensory deficits (e.g., weakness starts in the face and migrates to the arm/leg). Differentiate from stroke where weakness is complete at onset (however, there are exceptions including stuttering lacunar stroke). Be cautious of diagnosing hemiplegic migraine on first presentation without ruling out stroke if history
unclear. PRECURSORS OF MIGRAINE: Cyclic vomiting, benign paroxysmal vertigo of childhood, and abdominal migraine/nausea (car sickness).

TENSION HA: Usually presents as symmetric head pain, band-like, typically with almost no free interval period and constant ache. CLUSTER HA: Intense HA occurring for a period of weeks/months, with long pain-free intervals (years), often unilateral, associated with rhinorrhea, tearing, congestion, eyelid edema, myosis, and ptosis.

HISTORY: Time pattern, frequency, duration, warning signs, location, quality, associated symptoms, alleviating/aggravating factors, and degree of debilitation. EXAM: BP, temp, sinuses, teeth, fundoscopic examination (presence of venous pulsations implies normal ICP; however, absent in ˜10% of the population), visual fields, cranial nerve exam, signs of meningismus (neck pain, Kernig and Brudzinski signs), strength, gait. DIAGNOSTIC STUDIES: None needed if history classic for migraines, family history of migraines, and nonfocal neurologic exam. NEUROIMAGING: If any neurologic deficit with acute presenting HA, worrisome signs, or changes in HA characteristics. Consider NCHCT for head trauma, acute weakness, or signs suggestive of stroke; SDH; SAH; epidural hemorrhage; and acute changes in mental status. Consider MRI for chronic progressive HA to rule out brain mass or other subtle etiology. LP: if history of fevers or changes in mental status and neuroimaging is negative. If needed, consider performing LP after MRI to avoid leptomeningeal enhancement (however, use clinical judgment).