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Adverse effects of AEDs remain a major cause of morbidity and sometimes mortality in the course of treatment of epilepsy and hence considerably impact the QoL of people with epilepsy, pe

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Address for correspondence:

Dr Gagandeep Singh,

Department of Neurology,

Dayanand Medical College,

Ludhiana - 141 001, India

E-mail: gagandeep_si@yahoo.co.uk

Received : 25-01-2011

Review completed : 25-01-2011

Accepted : 25-01-2011

Do no harm – But first we need to know more: The case of adverse drug reactions with antiepileptic drugs

Gagandeep Singh

Department of Neurology, Dayanand Medical College, Ludhiana, India

Topic of the issue: Review Article

Access this article online

Quick Response Code: Website:

www.neurologyindia.com

PMID:

***

DOI:

10.4103/0028-3886.76859

Epilepsy is one of the most common neurological

disorders The mainstay of treatment of epilepsy is

antiepileptic drugs (AEDs), often for a long duration The

primary goals of treatment of epilepsy include complete

seizure remission, improvement in the the quality of

life (QoL), and do no harm, i.e., to avoid, minimize and

amend any adverse effects that might occur as a result of

treatment with AEDs Adverse effects of AEDs remain a

major cause of morbidity and sometimes mortality in the

course of treatment of epilepsy and hence considerably

impact the QoL of people with epilepsy, perhaps as

much as the seizure burden.[1] The exact incidence of

adverse effects of AEDs has not been determined as most people with epilepsy are managed as outpatients and are not hospitalized for either the epilepsy or for the adverse effects It is easy to estimate adverse effects in hospitalized patients and in patients who are hospitalized for the serious adverse effects However, the majority of adverse effects to AEDs are mild and

do not require admission In the outpatient setting, many of the mild adverse effects are either not reported

or not recorded; hence, it is difficult to determine the accurate incidence of adverse effects of AEDs One way

of evaluating the impact of adverse effects is to determine the proportion of patients who discontinue treatment (also referred to as treatment failure), for which there can be two reasons: (1) failure of the AED to control seizures adequately and (2) the occurrence of adverse effects during AED treatment It has been estimated that adverse effects of AED account for about 40% of treatment failures of AEDs in people with epilepsy.[2]

In a survey of selected patients with epilepsy, over 80%

Abstract

An adverse drug reaction (ADR) is defined by the World Health Organization as a noxious, unintended, and undesired drug effect, when used for therapeutic purposes in humans ADRs to anti-epileptic drugs (AEDs) significantly impact the quality of life of people with epilepsy and account for a little less than half of all recorded treatment failures with AEDs Hence prevention and early recognition of ADRs constitute an important aspect

of management of epilepsy Recent developments have improved our ability to predict and hence potentially prevent the occurrence of some of the serious ADRs to AEDs One example is the potential prediction of the risk of severe cutaneous hypersensitivity reactions including Stevens Johnson syndrome and toxic epidermal necrolysis by testing for expression

of HLA B*1502 allele in people of Asian origin who are prescribed carbamazepine The association between HLA B*1502 expression and carbamazepine skin reactions has been documented in India but the role of HLA testing in Indian populations needs to be clarified

in larger studies across different ethnic groups within the country

Key words: Adverse drug reaction, anti-epileptic drugs, Stevens Johnson syndrome, carbamazepine, HLA B*1502

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had reported at least one adverse event and the majority

had more than one adverse event.[3]

Definitions

The definition of an adverse drug reaction (ADR)

by the World Health Organization (WHO) is “any

noxious, unintended and undesired effect of a drug,

which occurs at doses used in humans for prophylaxis,

diagnosis or therapy” (World Health Organization,

1966) On the other hand, an “adverse event” refers

to any untoward experience that occurs during drug

treatment but which does not necessarily have a causal

relationship to the treatment.[4] The precise incidence of

ADRs in the community has never been estimated as

it is an onerous task due to inadequate documentation

and reporting It is easy to estimate the incidence of

ADRs in hospitalized patients In a meta-analysis of

ADRs to any medication, the overall incidence of ADRs

leading to hospitalization and in hospitalized patients

was 6.7% (95% CI 5.2–8.2%) and of fatal ADRs was

0.32% (95% CI 0.23–0.41%).[5]

The sources for determining ADRs in humans can be

many Prior to approval, pharmaceutical agents are

subjected to controlled clinical trials These clinical

trials, although rigorously regulated, might not bring

to light all possible ADRs as these are conducted for

short periods of time and in highly selected patient

populations While in the post-marketing phase, when

the drugs are used in much larger and unrestricted

patient population and often for unapproved

indications, several of the ADRs not reported in the

clinical trials come to light This underscores the

importance of meticulous and careful reporting of

adverse effects observed in the clinical practice as in

four of the case reports in this issue of the journal.[6-9]

The United States Food and Drug Administration (FDA)

has in place a well-established system, the FDA Safety

Information and Adverse Events Reporting Program

(also known as MedWatch), to which ADRs can be

reported in writing, telephonically or electronically

by prescribing physicians, other health-care workers,

patients and pharmaceutical companies (http://www

fda.gov/Safety/MedWatch) Each report is made in

a standard format and entered into a computerized

database and then analyzed by experts in order to

establish the causality A review of adverse event

reporting to the FDA from 1969 through 2002 revealed

about 2.3 million reports.[10] Likewise, in India, the

National Pharmacovigilance Advisory Committee

(NPAC) set up a system in 2004 to monitor safety of

medicinal products in India The committee has so

far appointed two zonal, five regional and about 25

peripheral pharmacovigilance centers ADRs can be

reported to the NPAC either directly or through one of the appointed centers (www.pharmacovigilance.co.in)

Antiepileptic Drugs – Incidence of Adverse Drug Reactions

The precise incidence of adverse effects to AEDs ideally needs to be determined in the community setting, but this apparently is an arduous task It might be possible to obtain community-based data from databases of general practices (e.g., the General Practice Research Database

in the UK) linked to pharmacy databases, but the information obtained thereof is likely to be incomplete

as many adverse effects are not reported and/or recorded It has been shown that systematic screening for adverse effects to AEDs using standardized and validated adverse effects profile-questionnaire provides

a greater yield of adverse effects experienced by the patients in comparison to spontaneous reporting by patients as is the usual practice in clinics.[11] Not only this, it results in better rationalization of AED treatment

as well as better QoL experienced by people with epilepsy The adverse effect profile-questionnaire is

a 19- or 21-item validated questionnaire, in which all items are scored on a 4-point scale (1 – never or rarely experienced adverse effect; 4 – common adverse effect)

by the patient.[11]

Systematic screening in pre-regulatory clinical trials results in a greater yield of adverse effects On the other hand, post-marketing studies might underestimate the magnitude of adverse effects to AEDs, but it is possible that new and hitherto unknown adverse effects come to light during this phase A large multicenter survey undertaken in Europe with over 5000 patients determined that 88% of the patients experienced at least one adverse effect and about one-third reported change of the AEDs in the previous one year on account

of adverse effects.[12]

Classification of Adverse Drug Reactions and Determination of Causality

In order to understand better the impact of ADRs, so frequently reported in many scientific journals, it is pertinent to review various classifications of ADRs The original Rawlins and Thompson’s classification (1977) of ADRs into Type A (augmented) and Type

B (bizarre) has been expanded to Types A through F [Table 1].[4,13] Other classifications are based on the frequency [Table 2] and severity of the ADRs An important concern is the assessment of causality of the reported ADRs Many standardized qualitative

or semi-quantitative approaches toward causality determination and classification are available

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Table 1: Classification of ADRs [4]

Type A Predictable, dose-related ADRs With most AEDs (with some interindividual variations between AEDs and patients): sedation,

somnolence, fatigue, tiredness, dizziness, unsteadiness, depression, agitation, nervousness, blurred vision, diplopia, ataxia, headache

With specific AEDs: leukopenia (carbamazepine), thrombocytopenia and deranged coagulation profile (valproate), hyponatremia (carbamazepine, oxcarbazepine), tremors (valproate), hypohiderosis (topiramate, zonimsamide), anxiety (levetericetam), paresthesiae (topiramate) Type B Unpredictable, idiosynchratic ADRs Skin rash both-, benign and anticonvulsant hypersensitivity syndrome (phenytoin,

carbamazepine, phenobarbital, lamotrigine, zonisamide) Aplastic anemia (phenytoin, carbamazepine, felbamate), angle closure glaucoma (topiramate), liver failure (valproate, felbamate, carbamazepine, phenytoin)

Type C Chronic, cumulative ADRs Osteomalacia and osteoporosis (phenytoin, carbamazepine, phenobarbital, valproate), gingivial

hyperplasia (phenytoin), hirsutism (phenytoin), weight gain (valproate, pregabalin, gabapentin), visual field loss (vigabatrin)

Type D Delayed ADRs Carcinogenesis (unproven in most cases, e.g., phenytoin-induced myeloma and lymphoma),

teratogenesis Type E ADRs evident only after withdrawal of drug NA

Type F Therapeutic failure of drug NA

Table 3: Assessment of causality of reported ADRs (adapted from Reference 2)

Category Explanation

Certain An ADR (clinical or laboratory) with definite time

relationship to drug administration, with appropriate response to drug withdrawal and documented by re-challenge with the drug The occurrence of the event cannot be explained by the underlying disease or other drugs

Probable An ADR (clinical or laboratory) with definite time

relationship to drug administration, with appropriate response to drug withdrawal but not documented by re-challenge with the drug The occurrence of the event cannot be explained by the underlying disease or other drugs

Possible An adverse event with plausible time relationship to

drug administration but might also be explained by the underlying disease or another drug Information on response to drug withdrawal is lacking

Unlikely An adverse event without plausible time relationship to

drug administration and which might be explicable by underlying disease or other drugs

Unclassified/

unclassified

A reported adverse event for which more data are required for causality assessment or not available

Table 2: Classification of ADRs to AEDs based on the frequency

of occurrence

Category Frequency (%) Examples

Very

common

>10 Dizziness, fatigue, tiredness, difficulty

in concentration

depression Uncommon 0.1–1 Ataxia, somnolence, skin rash

nephrolithiasis (topiramate) Very rare <0.01 Aplastic anemia (phenytoin), glaucoma

(topiramate)

[Table 3].[4,14] The assignment of causality is mostly

based on the temporal relationship of the adverse event

to drug administration, plausibility of its occurrence,

absence of a better explanation and consistency with

other reports of similar ADRs with the drug Going

by the above listed criteria, the ADRs reported in four

case reports in this issue of the journal are very rare

ADRs with a frequency of less than 0.01%.[6-9] With

the exception of the report of refractory hiccups due

to phenytoin therapy, in which case re-challenge with

phenytoin led to reappearance of hiccups, the ADRs

in the case reports might be classified as probable

inasmuch as re-challenge was not undertaken in the

patients.[9]

Patterns of Adverse Drug Reactions to

Antiepileptic Drugs

It remains unclear whether certain AEDs are more often

associated with ADRs Some adverse effects such as

dizziness, unsteadiness, sleepiness and tiredness occur

with nearly equal frequency during the use of any of the

conventional AEDs.[12] Other adverse effects such as hair

loss and weight gain are more specifically associated with

only certain AEDs such as valproate-sodium Attempts

to classify ADRs according to whether certain effects segregate along with certain others have generally not yielded any concrete pattern In one study of patients with refractory epilepsy, certain adverse effects tended to

go along with each other, e.g., restlessness with disturbed sleep, depression with nervousness or agitation, memory problems with difficulty in concentrating and sleepiness with tiredness.[3] The authors of this study classified ADRs to AEDs into five groups using factor analysis: disturbances of cognition and co-ordination, disorders of mood and emotion, sleep disturbances, weight changes and adverse effects related to the skin and mucosa The most common Type A (dose-related) adverse effects

do not vary much in their frequency of occurrence at

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least among the conventional AEDs (carbamazepine,

valproate, phenytoin and phenobarbital) Certain ADRs

(especially, certain Type B adverse effects) are AED

specific (e.g., weight gain with certain AEDs including

valproate-sodium, pregabalin and gabapentin) It is

generally believed that the second- and third-generation

AEDs produce lesser adverse effects than the conventional

AEDs In the Standard and New Antiepileptic Drugs

(SANAD) trial, while carbamazepine was found to be

significantly better than lamotrigine in terms of efficacy,

the latter was found better than the former in terms of

tolerability On the other hand, in people with either

generalized or unclassified seizures, valproate-sodium,

though significantly better than lamotrigine (a

second-generation AED) in terms of efficacy, was also superior

to another second-generation AED, topiramate, in terms

of tolerability In addition, certain newer AEDs have

been shown to have very serious and severe adverse

effects For instance, felbamate was approved by the

FDA in 1993 for use in patients with refractory partial

epilepsy with or without secondary generalization and

in children with Lennox Gastaut syndrome Within 1

year, following the reports of about 10 cases of aplastic

anemia and another 10 of hepatic failure (of which four

cases were fatal), the approval was withdrawn.[15,16] At

that time, an estimated 10,000–15,000 patients were on

treatment with the drug in the United States Hence,

letters were sent to several physicians warning them

about these potentially fatal adverse effects At present,

felbamate is approved for use only in those conditions

in which the benefit of administration far outweighs

the risks Another second-generation AED, vigabatrin,

was available in several countries for use in children

with infantile spasms and adults with complex partial

seizures not responding to other AEDs However,

post-marketing use in these countries revealed irreversible

peripheral visual field defects inasmuch as 30% of the

patients using the AED.[17] Hence, approval for use in

the United States by the FDA was withheld till as late as

2009 Hence, it might not be true that the newer AEDs

have lesser adverse effects than the conventional AEDs

The incidence of adverse effects is expected to vary

according to the patient population Hence, a recent

study in a cohort of patients with epilepsy, 50% of

whom were on more than one AED, revealed that 83%

experienced two or more adverse effects, and in this

subgroup, the mean number of adverse effects was seven.[3]

However, in a different study, with a cross-sectional

design, the frequency and pattern of ADRs did not differ

among patients on monotherapy and polytherapy.[18]

Hence, although it might be theoretically plausible that

polytherapy leads to more ADRs than monotherapy in

patients with refractory epilepsy and this contention

might be supported by data from pre-approval clinical

trials, in the pragmatic situation involving routine clinical

practice, polytherapy might not be disadvantageous in comparison to monotherapy in terms of adverse effect profiles at least in patients with refractory epilepsy

The Special Case of Carbamazepine Hypersensitivity Among People of Asian Origin

An unrelated but important issue concerns the occurrence

of severe hypersensitivity reactions including Stevens Johnson Syndrome and Toxic Epidermal Necrolysis (TEN) among people of Asian origin The association between the HLA B*1502 allele and the occurrence

of carbamazepine-associated severe hypersensitivity reactions was first reported in the Hans Chinese in a controlled study from Taiwan.[19] In this report, the HLA B*1502 allele was expressed in all of the 44 cases with carbamazepine-induced severe hypersensitivity reactions, and in comparison, in only 3% of 101 carbamazepine-tolerant patients and 9% of 93 healthy, unexposed controls The odds ratio for the association was very high (2504; 95% CI 126–49,522) Similar associations, albeit less strong, were found among people

of Chinese origin from mainland China, Hong Kong, and among Thai and Malaysian patients.[20-22] One small study

of eight patients with carbamazepine-associated Stevens Johnson Syndrome from Gujarat in India also revealed HLA B*1502 expression in six.[23] However, no significant association was demonstrated in studies in Japanese and Caucasian (from Europe) people.[24,25] The FDA recently issued recommendations for screening of all people of Asian origin for the HLA B*1502 allele before initiating treatment with carbamazepine.[26] It recommended that those Asians who tested positive for the allele should avoid exposure to carbamazepine

Although not based on reliable comparative data, the frequency of carbamazepine-induced severe hypersensitivity reactions appears to be higher among people of Asian origin in comparison to Caucasians Furthermore, the strength of association between carbamazepine hypersensitivity and HLA B*1502 expression apparently depends on the background frequency of expression of this allele in the general population The highest frequencies of expression on the allele outside India have been reported among Filipinos (from Philippines), Chinese from Taiwan, mainland China and Hong Kong and among the Thai and Malaysians, in whom background rates are in the order of 10–20% Apparently, HLA B*1502 expression

is of very low order among Europeans In concordance with the background rates of allele expression, the association between HLA B*1502 and carbamazepine hypersensitivity is strongest among the Hans Chinese (from Taiwan), other Chinese, Thai and Malaysians

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Within India, in the ethnic population from Kandhesh

Pawra in Maharashtra in Western India, the frequency

of expression of this allele is as high as 6% In North

Indian populations from Delhi and Punjab, the frequency

of expression is about 1%.[23,26,27] Since India comprises

a very large and multiethnic population, the frequency

of expression of the allele is likely to be highly variable

Accordingly, the strength of association between HLA

B*1502 expression and carbamazepine hypersensitivity

is expected to vary by ethnic regions within India

Can the Impact of Adverse Drug Reactions be

Reduced?

Most Type A adverse effects are dose related Hence,

the incidence of Type A ADRs can be reduced by

keeping the dose of the required AED as low as possible,

whilst not compromising its efficacy At the same

time, although unproven, tolerance often develops

to many of the dose-related adverse effects of many

of the AEDs, such as dizziness, tiredness, fatigue and

sedation It makes sense, therefore, to slowly titrate

the doses of AEDs while initiating treatment, i.e., go

slow, keep low Even for certain Type B adverse effects,

for instance, lamotrigine-induced skin rash and

topiramate-induced glaucoma, slow upward titration of

doses helps In addition, recognizing patient subgroups

that are more predisposed to a particular adverse

effect is an important aid in reducing the incidence

of adverse effects For instance, valproate-induced

hepatotoxicity appears to be more common among

children <2 years of age, on AED polytherapy and

with underlying mental retardation and developmental

delay indicative of organic brain disease or inborn errors

of metabolism Hence, valproate should be started with

caution in such individuals Likewise, levetericetam

administration leads to psychiatric adverse effects

in some patients Careful analysis of these cases has

led to the understanding that psychiatric adverse

events appear to be more common among individuals

with prior psychiatric conditions or a family history

of psychiatric disease Hence again, levetericetam

should be started with caution in individuals with

prior or family history of psychiatric disorders A more

definitive example where screening helps in avoiding

untoward effects has been hitherto described – the

case of carbamazepine-induced hypersensitivity in

association with HLA B*1502 expression Finally, it

is most crucial to counsel patients and their families

regarding the possibility of occurrence of adverse

effects, their early recognition, the time frame in which

these are expected to occur and the importance of

reporting these adverse effects to the physician early,

while at the same time reassuring her/him that most

side effects are uncommon or rare

Conclusions

What do we learn and conclude from the contained case reports in this issue of the journal[6-9] and the limited data available on the association between HLA B*1502 allele expression and carbamazepine hypersensitivity from India? In the first place, we learn that as responsible physicians involved in the care of people with epilepsy,

we are justifiably obliged to report as completely as possible any suspected adverse event occurring during treatment with AEDs to the appropriate authority This will go a long way to ensure safety of the people with epilepsy that we care for Secondly, it is desirable to have multicenter studies from across the country on the association between HLA B*1502 expression and carbamazepine hypersensitivity The association studies might be extended to include a large number of single nucleotide polymorphisms that might be related to ADRs

to AEDs These are the take-home messages

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Source of Support: Nil, Conflict of Interest: None declared.

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