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p. 1272

Epilepsy is a disorder characterized by spontaneously recurring seizures.

Seizures can arise from a focal area of the brain (focal or partial

seizures) or arise diffusely from both brain hemispheres (primary

generalized seizures).

Case 60-1 (Question 1)

The optimal choice of antiepileptic drug (AED) treatment is based on

patient-specific considerations including seizure type (or epilepsy

syndrome, if defined), age, sex, concomitant medical conditions and

therapies, and AED adverse effects. Monotherapy is preferred;

polytherapy should be considered for patients with multiple seizure types

and/or when monotherapy (with 2 or 3 agents) fails at maximal tolerated

doses.

Case 60-1 (Questions 2, 3),

Case 60-6 (Question 1),

Case 60-7 (Questions 1, 3, 4)

Standard AEDs, such as carbamazepine, phenytoin, and valproate, are

used commonly for patients with newly diagnosed epilepsy. Newer

AEDs (e.g., lacosamide, lamotrigine, levetiracetam, oxcarbazepine,

pregabalin, topiramate, zonisamide, ezogabine, perampanel,

eslicarbazepine) are often initially approved for add-on therapy in

patients with partial-onset seizures who do not respond to other AEDs.

Lamotrigine, oxcarbazepine, topiramate, lacosamide, and felbamate are

indicated for monotherapy.

Case 60-1 (Question 3),

Case 60-2 (Question 1),

Case 60-6 (Question 1)

Enzyme-inducing AEDs (carbamazepine, phenobarbital, phenytoin)

increase the metabolism of many other drugs (e.g., warfarin,

contraceptive hormones). In addition, carbamazepine induces its own

metabolism and levels may decline during the first month of therapy

despite excellent adherence.

Case 60-1 (Question 5),

Case 60-11 (Question 1)

Serious idiosyncratic adverse effects have been associated with most

standard and new AEDs and include carbamazepine-associated

hematologic abnormalities; lamotrigine-associated skin rash; valproateinduced hepatotoxicity; and hypersensitivity syndrome seen with

carbamazepine, phenobarbital, and phenytoin. The role of routine

laboratory monitoring for detection of these adverse effects is

controversial. Patients should know the signs or symptoms that should

prompt them to seek medical attention.

Case 60-1 (Question 4),

Case 60-2 (Question 1),

Case 60-7 (Questions 8, 9),

Case 60-10 (Questions 1, 2)

Unlike other AEDs, phenytoin displays capacity-limited

pharmacokinetics at serum concentrations that are clinically useful for

Case 60-3 (Questions 2, 3)

epilepsy treatment. As a consequence, phenytoin blood levels often

change disproportionately to changes in dosage, and time to steady state

varies significantly in individual patients based on the phenytoin

concentration.

Serum concentration monitoring can be useful for selected AEDs when

there is a good correlation between concentration and therapeutic or

toxic responses. However, clinical criteria (seizure control, medication

tolerability) are the primary determinants of the need for dosage

adjustments.

Case 60-7 (Question 7)

The occurrence of seizure clusters (acute repetitive seizures) and status

epilepticus (prolonged or repeated seizures without recovery of

consciousness) warrants emergent AED therapy. Acute repetitive

seizures are often treated by parents or caregivers with rectal

diazepam. Status epilepticus is a life-threatening emergency and should

be treated with intravenous (IV) lorazepam as initial therapy.

Case 60-8 (Question 1),

Case 60-12 (Questions 1–4)

p. 1273

p. 1274

Incidence, Prevalence, and Epidemiology

Approximately 10% of the population will experience a seizure at some time in their

life. Up to 30% of all seizures are provoked by central nervous system (CNS)

disorders or insults (e.g., meningitis, trauma, tumors, and exposure to toxins); these

seizures may become recurrent and require chronic treatment with AEDs. Reversible

conditions such as alcohol withdrawal, fever, and metabolic disturbances may

provoke acute, isolated seizures. These seizures, along with drug-induced seizures,

are not considered to be epilepsy and usually do not require long-termAED therapy.

Approximately 1% of the general population has epilepsy.

1

Terminology, Classification, and Diagnosis of Epilepsies

CLASSIFICATION OF SEIZURES AND EPILEPSIES

A seizure is the “transient occurrence of signs and/or symptoms due to abnormal

excessive or synchronous neuronal activity in the brain” (p. 471).

2 These signs or

symptoms “may include alterations of consciousness, motor, sensory, autonomic, or

psychic events” (p. 593).

1 Epilepsy is a “disorder of the brain characterized by an

enduring predisposition to generate epileptic seizures and by the neurobiologic,

cognitive, psychological and social consequence of this condition” (p. 471).

2 By

definition, epilepsy requires the occurrence of two or more seizures that are not

acutely provoked by other illnesses or conditions.

3 Guidelines have recently been

updated to include a patient who has had one unprovoked seizure and has a high risk

(>60%) of a second seizure to be included in the definition of a person with

epilepsy.

4 A commonly used classification scheme for epileptic seizures is shown in

Table 60-1.

5 Older terms such as “grand mal” and “petit mal” should not be used,

because their use may create confusion in the clinical setting. For example, it is

common for patients or caregivers to identify any seizure other than a generalized

tonic–clonic seizure as a “petit mal” seizure. This labeling may result in the selection

of an inappropriate medication.

Generalized tonic–clonic seizures are common. The patient loses consciousness

and falls at the onset. Simultaneously, tonic muscle spasms begin and may be

accompanied by a cry that results from air being forced through the larynx. Bilateral,

repetitive clonic movements follow. After the clonic phase, patients return to

consciousness but remain lethargic and may be confused for varying periods of time

(postictal state). Urinary incontinence and tongue biting is common. Primary

generalized tonic–clonic seizures affect both cerebral hemispheres from the outset.

Secondarily generalized tonic–clonic seizures begin as either simple or complex

partial seizures. The aura described by some patients before a generalized tonic–

clonic seizure represents an initial partial seizure that spreads to become a

secondarily generalized seizure. Identification of secondarily generalized tonic–

clonic seizures is important because some AEDs are more effective at controlling

primary generalized seizures than secondarily generalized seizures. In general,

partial seizures are often more difficult to control with AEDs as compared to primary

generalized seizures.

3

,

6

,

7

Absence seizures occur primarily in children and often remit during puberty;

affected patients may exhibit a second type of seizure. Absence seizures consist of a

brief loss of consciousness, usually lasting several seconds. Simple (typical) absence

seizures are not accompanied by motor symptoms; automatisms, muscle twitching,

myoclonic jerking, or autonomic manifestations may accompany atypical (complex)

absence seizures. Although consciousness is lost, muscle tone is maintained and

patients do not fall during absence seizures. Patients are unaware of their

surroundings and will have no recall of events during the seizure. Consciousness

returns immediately when the seizure ends, and postictal confusion does not occur.

Differentiation of atypical absence seizures from complex partial seizures may be

difficult if only a second-hand account of the episodes is available; identification of a

focal abnormality by an electroencephalogram (EEG) often is necessary to identify

complex partial seizures. This distinction is important for the proper selection of

AED.

Table 60-1

Classification of Epileptic Seizures

Partial Seizure (Focal)

Simple Partial Seizures (Without Impairment of Consciousness)

Motor symptoms

Specialsensory or somatosensory symptoms

Autonomic symptoms

Psychic symptoms

Complex Partial Seizures (With Impairment of Consciousness; “dyscognitive features”)

Progressing to impairment of consciousness

With no other features

With features as in simple partialseizures

With automatisms

With impaired consciousness at onset

With no other features

With features as in simple partialseizures

With automatisms

Partial Seizures That Evolve to Generalized Seizures

Simple partialseizures evolving to generalized seizures

Complex partialseizures evolving to generalized seizures

Simple partialseizures evolving to complex partialseizures to generalized seizures

Generalized Seizures (Convulsive or Nonconvulsive)

Absence Seizures

Typicalseizures (impaired consciousness only)

Atypical absence seizures

Myoclonic Seizures

Clonic Seizures

Tonic Seizures

Tonic–Clonic Seizures

Atonic (Astatic or Akinetic) Seizures

Unclassified Epileptic Seizures

Allseizures that cannot be classified because of inadequate or incomplete data and some that cannot be

classified in previously described categories

Simple partial (focal motor or sensory) seizures are localized in a single cerebral

hemisphere or portion of a hemisphere. Consciousness is not impaired during these

events. Various motor, sensory, or psychic manifestations may occur depending on

the area of the brain that is affected. A single part of the body may twitch, or the

patient may experience only an unusual sensory experience.

p. 1274

p. 1275

Complex partial seizures result from the spread of focal discharges to involve a

larger area. Consciousness is impaired and patients may exhibit complex but

inappropriate behavior (automatisms) such as lip smacking, picking at clothing, or

aimless wandering. A period of brief postictal lethargy or confusion is common.

In 2010, the International League Against Epilepsy recommended modifications to

the traditional seizure classification scheme and terminology. Whereas some of the

terminology remained unchanged, seizures limited to one hemisphere are now termed

“focal seizures” (instead of partial seizures) and the formal distinction between

complex partial and simple partial seizures is eliminated.

8 Because most of the

existing literature on epilepsy makes use of the traditional seizure terminology, we

have retained the use of “partial,” “complex partial,” and “simple partial” for this

chapter.

EPILEPSY SYNDROMES

Epilepsy can be classified based on seizure type as shown in Table 60-1. Epilepsy

syndromes can be defined on the basis of seizure type as well as cause (if known),

precipitating factors, age of onset, characteristic EEG patterns, severity, chronicity,

family history, and prognosis. Accurate diagnosis of epilepsy syndromes may better

guide clinicians regarding the need for drug therapy, the choice of appropriate

medication, and the likelihood of successful treatment.

1

,

6

,

7 Many epilepsy syndromes

have been defined; a complete listing is beyond the scope of this chapter. Several are

of interest with respect to pharmacotherapy and are described in Table 60-2.

7

Diagnosis

Optimal treatment of seizure disorders requires accurate classification (diagnosis) of

seizure type and appropriate choice and use of medications. Seizure classification

may be straightforward if an adequate history and description of the clinical seizure

are available. Physicians often do not observe patients’ seizures; thus, family

members, teachers, nurses, and others who have frequent direct contact with patients

should learn to observe accurately and objectively describe and record these events.

The onset, duration, and characteristics of a seizure should be described as

completely as possible. Several aspects of the events surrounding a seizure may be

especially significant: the patient’s behavior before the seizure (e.g., Did the patient

complain of feeling ill or describe an unusual sensation?), deviation of the eyes or

head to one side or localization of convulsive activity to one portion of the body,

impaired consciousness, loss of continence, and the patient’s behavior after the

seizure (e.g., Was there any postictal confusion?). In addition, it is helpful if the

observer can record the length of the event and how long it took for the patient to

return to baseline. A video of the event could be especially helpful. The patient and

caregivers should have a seizure calendar or diary to record events. Many options

exist to track seizures including online sites and smart phone applications. Those

who observe a seizure should not try to label the seizure but should be encouraged to

describe the event fully and objectively.

Accurate seizure diagnosis and identification of the type of epilepsy or epilepsy

syndrome also depend on neurologic examination, medical history, and diagnostic

techniques, such as EEG, computed tomography (CT), and magnetic resonance

imaging (MRI). The EEG often is critical for identifying specific seizure types. CT

scanning may help assess newly diagnosed patients, but MRI is preferred. MRI may

locate brain lesions or anatomic defects that are missed by conventional radiographs

or CT scans.

9

Table 60-2

Selected Epilepsy Syndromes

Syndrome

Seizure Patterns and

Characteristics

Preferred AED

Therapy Comments

Juvenile myoclonic

epilepsy

Myoclonic seizures often

precede generalized tonic–clonic

seizures.

sleep, fatigue, and

alcohol commonly precipitate

seizures

Valproate. Levetiracetam

FDA-approved as adjunct

for myoclonic seizures.

Lamotrigine, topiramate,

and zonisamide may be

effective

5%–10% of all epilepsies;

85%–90% response to

valproate. Lifelong

therapy usually needed.

High relapse rate with

attempts to discontinue

AED therapy

Lennox–Gastaut

syndrome

Generalized seizures: atypical

absence, atonic/akinetic,

myoclonic, and tonic most

common. Abnormal interictal

EEG with slow spike-wave

pattern. Cognitive dysfunction

and mental retardation. Status

epilepticus common

Valproate and

benzodiazepines may be

effective. Lamotrigine,

rufinamide, topiramate and

clobazam FDA-approved.

Felbamate also may be

effective, but potential

hematologic toxicity limits

use. Poorly responsive to

AED

Oversedation with

aggressive AED trials

may ↑ seizure frequency.

Tolerance to

benzodiazepines limits

their usefulness

Childhood absence

epilepsy

Typical absences often in

clusters of multiple seizures.

Tonic–clonic seizures in ˜40%.

Onset usually between ages 4

and 8 years. Significant genetic

component. EEG shows classic

3-Hz spike-and-wave pattern

Ethosuximide or valproate.

Lamotrigine less effective

80%–90% response rate

to AED therapy. Good

prognosis for remission.

Tonic–clonic seizures may

persist

Mesial temporal lobe

epilepsy

Complex partialseizures with

automatisms. Simple partial

seizures (auras) common;

secondary generalized seizures

occur in 50%

Carbamazepine, phenytoin,

valproate, gabapentin,

lamotrigine, topiramate,

tiagabine, levetiracetam,

oxcarbazepine,

zonisamide, pregabalin,

lacosamide, perampanel,

ezogabine, eslicarbazepine

Often incompletely

controlled with current

AEDs. Emotionalstress

may precipitate seizures;

surgical resection can be

effective when patient is

identified as a good

surgical candidate

AED, antiepileptic drug; EEG, electroencephalogram; FDA, US Food and Drug Administration.

p. 1275

p. 1276

Treatment

Early control of epileptic seizures is important because it allows normalization of

patients’ lives and prevents acute physical harm and long-term morbidity associated

with recurrent seizures. In addition, early control of tonic–clonic seizures is

associated with a reduced likelihood of seizure recurrence. Early control of epileptic

seizures also correlates with successful discontinuation of AED treatment after longterm seizure control.

10–12

NONPHARMACOLOGIC TREATMENT OF EPILEPSY

Alternatives or adjuncts to pharmacotherapy may be helpful in some patients. Surgery

is an extremely effective treatment in selected patients. Depending on the epilepsy

syndrome and procedure performed, up to 90% of patients treated surgically may

improve or become seizure-free. A study of 80 patients with medically refractory

temporal lobe epilepsy randomly assigned to either surgery or continued medical

treatment showed that after 1 year patients were more likely to be seizure-free after

surgery.

13 Surgery is advocated as early therapy for some patients with specific

epilepsy syndromes, such as mesial temporal sclerosis. Early surgical intervention

may prevent or lessen neurologic deterioration and developmental delay.

Dietary modification may be used for patients who cannot tolerate AEDs or to

treat seizures that are not completely responsive to AEDs. In most circumstances,

dietary modification consists of a ketogenic diet. This low-carbohydrate, high-fat diet

results in persistent ketosis, which is believed to play a major role in the therapeutic

effect. Ketogenic diets seem to be most beneficial in children; they are also used as

adjuncts to ongoing AED treatment.

14

,

15

The vagus nerve stimulator is an implantable device approved for treatment of

intractable partial seizures. This device uses electrodes attached around the left

branch of the vagus nerve. The electrodes are attached to a programmable stimulator

that delivers stimuli on a regular cycling basis; patients can also use “on demand”

stimulation at the onset of seizures by swiping the magnet over the subcutaneously

implanted stimulator. Approximately 30% to 40% of patients who are so treated have

a positive response (50% reduction in seizures).

16 The primary side effect of this

device is hoarseness during stimulation; infrequently, this is accompanied by left

vocal cord paralysis.

Responsive neurostimulation is a newer non-pharmacologic option for treating

intractable partial-onset seizures. Its role in therapy is yet to be determined.

Avoidance of Potential Seizure Precipitants

It is impossible to generalize about environmental and lifestyle precipitants of

seizure activity in persons with epilepsy. Individual patients or caregivers may

identify specific circumstances such as stress, sleep deprivation, acute illness, or

ingestion of excessive amounts of caffeine or alcohol, which may increase the

likelihood of a recurrent seizure event. Some women experience an increase in the

frequency and/or severity of seizures around the time of menstruation or ovulation.

Patients with epilepsy should avoid activities that seem to precipitate seizures; as

always, the goal is complete seizure control with as little alteration in quality of life

as possible.

ANTIEPILEPTIC DRUG THERAPY

Pharmacotherapy is the mainstay of treatment for epilepsy. Therefore, patient

education regarding medications and consultation among health care professionals

regarding the optimal use of AEDs are essential to quality patient care. Optimal AED

therapy completely controls seizures in approximately two-thirds of patients.

17

,

18

Optimization of drug therapy depends on several factors, with the choice of

appropriate AED, individualization of dosing, and adherence being the most

important.

Choice of Antiepileptic Drug

Many AEDs have a relatively narrow spectrum of efficacy against selected seizure

types; therefore, choice of appropriate drug therapy for a specific patient depends on

an accurate diagnosis of epilepsy. In addition, toxicity must be considered when

selecting an AED. Preferred drugs for specific types of seizures and common

epileptic syndromes are listed in Tables 60-2 and 60-3. Although certain drugs are

preferred, the identification of the most effective drug for a particular patient may be

a process of trial and error; several medication trials may be necessary before

success is achieved. It is important to keep in mind that certain AEDs can worsen

seizures.

19

The consensus method was used to analyze expert opinion on treatment of three

epilepsy syndromes and status epilepticus.

20 The experts recommended monotherapy

first, followed by a second monotherapy agent if the first failed. If the second

monotherapy failed, the experts were not in agreement on whether to try a third

monotherapy agent or to combine two therapies. The experts recommended epilepsy

surgery evaluation after the third failed AED for patients with symptomatic

localization-related epilepsies.

To assess the evidence on efficacy, tolerability, and safety of many of the new

AEDs in treating children and adults with new-onset and refractory partial and

generalized epilepsies, a panel evaluated the available evidence.

21

,

22 They concluded

that AED choice depends on seizure and syndrome type; patient age; concomitant

medications; and AED tolerability, safety, and efficacy. The results of these two

evidence-based assessments provide guidelines for the use of newer AEDs in

patients with new-onset and refractory epilepsy.

Therapeutic End Points

The individual patient’s response to AED treatment (i.e., seizure frequency and

severity, and symptoms of toxicity) must be the major focus for therapy assessment.

In general, the goal of AED treatment is administration of sufficient medication to

completely prevent seizures without producing significant toxicity.

23 Realistically,

this goal may be compromised for many patients; it may not be possible to

completely prevent seizures without producing intolerable adverse effects. Thus, the

therapeutic end points achieved can vary among patients; optimization of AED

therapy for a specific person depends on tailoring therapy to the patient’s needs and

lifestyle. It is rarely optimal to administer “standard” or “usual” doses of an AED to

a patient or to adjust doses to achieve a “therapeutic blood level” without

considering the effect of the dose or serum concentration on the patient’s condition

and quality of life. As with many conditions requiring chronic drug therapy, patient

participation in developing and evaluating a therapeutic plan is extremely important.

Patients should be educated regarding the expected positive and negative effects of

their AED therapy, and they must be encouraged to communicate with their health

care provider regarding their responses to prescribed AEDs.

p. 1276

p. 1277

Table 60-3

Antiepileptic Drugs (AEDs) Useful for Various Seizure Types

Primary

Generalized

Tonic–Clonic

Secondarily

Generalized

Tonic–Clonic

Simple or

Complex Partial

(Focal) Absence

Myoclonic,

Atonic/Akinetic

Most Effective With Least Toxicity

a

Valproate Carbamazepine Carbamazepine Ethosuximide Valproate

Levetiracetam Oxcarbazepine Oxcarbazepine Valproate Clonazepam

Lamotrigine Levetiracetam Levetiracetam Lamotrigine Rufinamide

(Lennox–Gastaut

Syndrome)

Levetiracetam Lamotrigine Lamotrigine

(Topiramate)

b Levetiracetam

(Juvenile Myoclonic

Epilepsy)

Levetiracetam Valproate Valproate Lamotrigine

b

Levetiracetam Gabapentin Gabapentin Clobazam (Lennox–

Gastaut Syndrome)

(Oxcarbazepine)

b Topiramate

(Topiramate)

b

Perampanel Tiagabine

Zonisamide

Levetiracetam

Pregabalin

Lacosamide

Ezogabine

Perampanel

Topiramate

b

Tiagabine

Pregabalin

Zonisamide

Lacosamide

Ezogabine

Perampanel

Eslicarbazepine Eslicarbazepine

Effective, but Often Poorly Tolerated or Cause Unacceptable Toxicity

Phenobarbital Phenobarbital Clorazepate Clonazepam (Felbamate)

c

Primidone Primidone Phenobarbital

(Felbamate)

c

(Felbamate)

c Primidone

Phenytoin Phenytoin

(Vigabatrin)

d

(Felbamate)

c

Phenytoin

(Vigabatrin)

d

aDrugs are listed in general order of preference within each category. Recommendations by various authorities

may differ. The use of phenobarbital and primidone is discouraged.

bThe place of some AEDs for select seizure types is yet to be determined. More clinical experience is needed

before their roles as possible primary AEDs are clarified.

cFelbamate is placed on this table only to indicate the types of seizures for which it appears to be effective.

Felbamate has been associated with aplastic anemia and hepatic failure; until a possible causative role is clarified,

felbamate cannot be recommended for treatment of epilepsy unless all other, potentially less toxic, treatment

options have been exhausted.

dVigabatrin causes progressive and permanent visual field constriction that is related to total dose and duration of

exposure. Vigabatrin cannot be recommended for treatment of epilepsy unless other potentially less toxic

treatment options have been exhausted.

Serum Drug Concentrations

Relation to Clinical Response

For selected AEDs, proper use and interpretation of serum concentrations are

important for optimizing treatment regimens in epilepsy.

24

,

25 An individual patient’s

clinical response to AED treatment must be the major focus for therapy assessment.

Individual patients often differ dramatically in their response to a particular serum

drug concentration; therefore, therapeutic serum concentrations should be considered

only as guidelines for treatment. Many patients’ condition may be controlled with

serum drug concentrations above or below the usual therapeutic range.

26

In these

patients, dosage adjustment to get the patient “in range” is not warranted. It is better

to “treat the patient, not the level.”

Interestingly, a recent Cochrane Review found no evidence that measuring AED

concentrations routinely to inform dose adjustments is superior to dose adjustments

based on clinical information.

27 However, the authors do state that their review does

not exclude the possibility that AED serum concentration might be useful in special

situations or in selected patients.

Indications for Use

Measurement of serum drug concentrations may provide clinically useful information

in the following situations:

Uncontrolled seizures despite administration of greater-than-average doses: Serum

concentrations of AED may help distinguish drug resistance from subtherapeutic

drug concentrations caused by malabsorption, nonadherence, or rapid

metabolism.

Seizure recurrence in a patient whose seizures were previously controlled: This is

often owing to nonadherence with the prescribed medication regimen.

Documentation of intoxication: In patients who exhibit signs or symptoms of doserelated AED toxicity, documentation

p. 1277

p. 1278

of the dose and serum concentration of the responsible drug is helpful.

Assessment of patient adherence: Although monitoring AED serum concentrations

can be used to assess patient adherence with therapy, conclusions must be based

on comparisons with previous steady state serum concentrations that reflected

reliable intake of a given dose of AED.

Documentation of desired results from a dose change or other therapeutic maneuver

(e.g., administration of a loading dose): When patients are receiving multiple

AEDs, it is often appropriate to measure serum concentrations of all drugs after a

change in the dose of one agent because changes to one drug frequently affect the

pharmacokinetic disposition of other drugs.

When precise dosage changes are required: On occasion, small changes in the dose

of a drug (e.g., phenytoin) can result in large changes in both the serum

concentration and clinical response. In addition, cautious titration of dosage and

serum concentration may be necessary to avoid intoxication. Knowledge of the

serum drug concentration before the dosage change may allow the clinician to

select a more appropriate new maintenance dose.

During pregnancy, AED serum concentrations often decline and dosage adjustments

may be warranted to maintain adequate seizure protection. Free (unbound)

concentrations should be monitored for highly protein-bound AEDs. AED serum

concentrations should be monitored after delivery, particularly when dosage

escalations have been made during pregnancy.

Frequent, “routine” determinations of serum AED concentrations are costly and not

warranted for patients whose clinical status is stable. Clinicians may tend to focus

attention on normal variability in serum concentrations rather than on the patient’s

clinical status; as a result, unnecessary dosage adjustments may be made to make

serum concentrations fit the “normal range.” A plan of action for what the clinician is

going to do with the information once it is obtained should be in place before

obtaining the sample. Therefore, the results of individual serum concentration

determinations must be evaluated carefully to decide whether a significant, clinically

meaningful change has occurred.

28

Interpretation of Serum Concentrations

Several factors can alter the relationship between AED serum concentration and the

patient’s response to the drug. Whenever a change in serum concentration is apparent,

pharmacokinetic factors (Table 60-4) should be considered (along with the patient’s

clinical status) before a decision is made to adjust the AED dosage. Laboratory

variability can cause minor fluctuations in reported AED serum concentrations.

Under the best conditions, reported values for serum concentrations may be within

plus or minus 10% of “true” values.

29

,

30 Therefore, the magnitude of any apparent

change must be considered. Published therapeutic ranges may have been determined

in small numbers of patients or may more accurately represent average serum

concentrations at usual doses. Inappropriate sample timing can result in inconsistent

and clinically meaningless changes in AED serum concentrations.

24 Generally, serum

concentrations of AED should not be measured until a minimum of four to five halflives have elapsed since initiation of therapy or a dosage change. Blood samples

should be obtained in the morning, before any doses of the AED have been taken; this

practice provides reproducible, postabsorptive (i.e., “trough”) serum concentrations.

Interindividual variability in response to a given serum concentration of medication

is common. Excellent therapeutic response or even symptoms of intoxication may be

associated with AED serum concentrations that are classified as “subtherapeutic.”

31

Binding to serum proteins is significant for some AEDs (e.g., phenytoin, valproate,

tiagabine). Changes in protein binding can result from drug interaction, renal failure,

pregnancy, or changes in nutritional status. These changes can alter the usual

relationship between the measured total drug concentration (bound and unbound to

plasma proteins) and the unbound (pharmacologically active) drug concentration.

This change may not be apparent when only total serum concentrations are measured.

Determination of serum concentrations of free (i.e., unbound) AEDs is available from

many commercial laboratories; these determinations are expensive and results may

not be available for several days. If significant changes in protein binding are

suspected, measurement of free concentrations of AED may provide additional

information useful for adjustment of doses or interpretation of the patient’s

symptoms.

29

,

30

,

32

Monotherapy Versus Polytherapy

Decades ago, epilepsy was often treated initially with multiple AEDs (polytherapy).

A second, third, or even fourth drug was added when seizures were incompletely

controlled with a single AED. Evaluation of the effectiveness of polytherapy in

subsequent years has shown little advantage for most patients. Use of a single drug at

optimal tolerated serum concentrations produces excellent therapeutic results and

minimal side effects in most patients. Addition of a second AED significantly

improves seizure control in only 10% to 20% of patients.

33

,

34 Reduction or

elimination of existing polytherapy in patients with longstanding seizure disorders

often lessens or eliminates cognitive impairment and other side effects; seizure

control actually may improve.

33

,

35–38

Most experts advocate the use of a single AED (monotherapy) whenever possible.

Successful monotherapy may require higher-than-usual AED doses or serum

concentrations greater than the upper limit of the usual therapeutic range.

39

,

40

Addition of a second drug may be necessary in some patients; however, polytherapy

should be reserved for patients with multiple seizure types or for patients in whom

first-line AEDs have failed to control seizures when titrated to maximal tolerated

doses.

20

,

36

,

39 When a new AED is added to a patient’s regimen with the goal of

improving seizure control, the existing AED regimen should be scrutinized for

continued value. In some cases, a patient’s AED regimen can accumulate drugs that

may be unnecessary because they were started and never re-evaluated. Continued

vigilance and critical assessment of every drug in a patient’s regimen is important.

Use of polytherapy creates several disadvantages that must be weighed against

possible benefits. Seizure control may not significantly improve. Health care costs,

for medications and for increased laboratory monitoring, may increase significantly

with polytherapy. In addition, drug interactions among AEDs can complicate

assessment of the patient’s response and serum concentrations. Patient adherence

often is worsened when multiple medications are prescribed, and adverse effects

often increase.

Although AED monotherapy is preferred whenever feasible, the recent

introduction of several new AEDs has increased the use of polytherapy.

40 Owing to

limitations on the patient populations used for clinical trials of new drugs (i.e.,

patients with seizure disorders not completely controlled by previous medications),

most new AEDs are labeled only for use as add-on therapy. Although reports exist on

the efficacy of the new AEDs as monotherapy,

41–44 only felbamate, lacosamide,

lamotrigine, oxcarbazepine, and topiramate are US Food and Drug Administration

(FDA) approved for monotherapy. More AEDs will undoubtedly follow with

monotherapy indications.

Duration of Therapy and Discontinuation of Antiepileptic Drugs

A diagnosis of epilepsy may not necessitate lifelong drug therapy. Several long-term

studies have shown that AED therapy may be successfully withdrawn from some

patients after a seizure-free period of 2 to 5 years.

10–12 Seizures recurred in only 12%

to 36% of patients who were followed for up to 23 years after AED withdrawal.

Therefore, many patients whose epilepsy is completely controlled with medication

can stop therapy after a seizure-free period of at least 2 years.

p. 1278

p. 1279

Table 60-4

Pharmacokinetic Properties of Antiepileptic Drugs (AEDs)

Drug

Oral

Absorption

(%)

Half-Life

(hours)

Time to

Steady

State

a

Dosage

Schedule

Usual

Therapeutic

Serum

Concentration

Plasma

Protein

Binding

(%)

Volume of

Distribution

(L/kg)

Carbamazepine 90–100 Chronic: 5–25 2–4

days

BID to

TID

5–12 mcg/mL 75 (50–

90)

0.8–1.6

Eslicarbazepine >90 Normal renal

function: 13–

20

4–5

days

QD Not determined <40 0.8

Ethosuximide 90–100 Pediatric:

30Adult: 60

5–10

days

Daily

(BID)

40–100

mcg/mL

0 0.7

Ezogabine 60 Normal renal

and hepatic

function: 7–11

2–4

days

TID Not determined 80 2–3

Felbamate 90 12–20 3–4

days

BID to

TID

50–110

mcg/mL

24 0.7–0.8

Gabapentin 40–60;

with ↑ dose

Normal renal

function: 5–9;

↑ with

renal

function

Normal

renal

function:

1–1.5

days

TID to

QID

(every 6–

8 hours)

2 mcg/mL

(proposed)

0 ≈0.8

Lacosamide 100 13 ↑ slightly

with renal

impairment

2–3

days

BID Not determined <15 0.6

Lamotrigine 90–100 Monotherapy:

24–29Enzyme

inducers:

15Enzyme

inhibitor

(VPA): 59

4–9

days

BID 4–18 mcg/mL

(proposed)

55 0.9–1.2

Levetiracetam 100 Normal renal

function: 6–8;

↑ with

renal

function

Normal

renal

function:

1–1.5

days

BID Not determined <10 ≈0.7

Oxcarbazepine 100 8–13 2–3

days

BID to

TID

Not determined 40

Perampanel 100 Normal

hepatic

function: 105

2–3

weeks

QD Not determined 95 NA

Phenobarbital 90–100 2–4 days 8–16

days

Daily 15–40 mcg/mL 50 0.5–0.6

Phenytoin 90–100 Varies with

dose

5–30

days

Daily to

BID

10–20 mcg/mL 95 0.5–0.7

Pregabalin ≥90 Normal renal

function: 6; ↑

with

renal

function

24 hours BID to

TID

Not determined 0 0.5

Rufinamide 85 9 1–2

days

BID Not determined <35 Dose

dependent

Tiagabine 90 Monotherapy:

7–9Enzyme

inducers: 4–7

1–2

days

BID to

QID

Not determined 96 1.1

Topiramate ≥80 12–24 3–4

days

BID Not determined 10–15 0.7

Valproate 100 (≈80%

with

divalproex

ER)

10–16 2–3

days

BID to

QID

(daily

with

divalproex

× ER)

50–150

mcg/mL

90+ 0.09–0.17

Vigabatrin 80–90 8–12 (not

clinically

important.

Irreversible

enzyme

inhibitor)

NA Daily to

BID

NA NA NA

Zonisamide ≈80 Monotherapy:

≈60Enzyme

inducers: 27–

36

2 weeks Daily to

BID

Not determined 50–60 1.3

aBased on four half-lives. This lag time should allow determination of steady state serum concentrations within

limits of most assay sensitivities.

BID, twice daily; NA, not available; TID, three times daily; QID, four times daily; VPA, valproic acid.

p. 1279

p. 1280

Table 60-5

Risk Factors Possibly Predicting Seizure Recurrence After Antiepileptic Drug

(AED) Withdrawal

<2 years seizure-free before withdrawal

Onset of seizures after age 12

History of atypical febrile seizures

Family history of seizures

2–6 years before seizures controlled

Large number of seizures (>30) before control or total of >100 seizures

Partialseizures (simple or complex)

Abnormal EEG persisting throughout treatment

Slowing on EEG before medication withdrawal

Organic neurologic disorder

Moderate to severe mental retardation

EEG, electroencephalogram.

Discontinuation of medications is advantageous for economic, medical, and

psychosocial reasons. Costs associated with health care visits, serum concentration

determinations, and the medications themselves are eliminated or reduced. The risk

of adverse effects from long-term medication use is eliminated, and patients can

expect fewer lifestyle restrictions. Attempts to withdraw AED therapy are associated

with risks, however. Primary among them is the reappearance of seizure activity,

which can result in status epilepticus, loss of driving privileges, employment

difficulties, and/or physical injury.

Risk factors for seizure recurrence after discontinuation of AED have been

identified in observational studies; complete agreement, however, is not found among

studies regarding the nature and importance of specific risk factors. Opinions and

data also differ regarding the optimal duration of the seizure-free period before

discontinuation of AED is attempted. Nevertheless, at least some consensus has been

reached regarding certain factors that may predict a higher risk of seizure recurrence

(Table 60-5).

10–12,45,46

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