The Diagnosis and Management of Epilepsy: Journal of Tropical Pediatrics January 2002
The Diagnosis and Management of Epilepsy: Journal of Tropical Pediatrics January 2002
The Diagnosis and Management of Epilepsy: Journal of Tropical Pediatrics January 2002
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Warren T. Blume
Abstract pected if syncope-like attacks occur in other circumstances,
particularly upon exercise.
THIS ARTICLE CONCISELY DESCRIBES the more common epilepsy con- Excessive daytime sleep, as episodes of microsleep, oc-
ditions and will enable physicians to efficiently evaluate and curs in children at school and in adults; it superficially re-
manage these disorders. Salient aspects of the history and exami- sembles temporal lobe seizures or absence attacks. The pa-
nation, together with electroencephalography, will usually deter- tient stares without specific warning or appears inattentive;
mine the epilepsy syndrome (category), forming the basis for any automatisms may occur. Unlike seizures, episodes of mi-
further investigation and possible antiepileptic therapy. Imaging crosleep can be reliably and instantly aborted with an affer-
may be required in some circumstances.
ent stimulus. Evidence of sleep deprivation includes less
CMAJ 2003;168(4):441-8 than 7 hours of sleep, hypnic jerks in drowsiness, frequent
dreaming, prominent snoring, morning arousal only with
E
pilepsy and stroke are the 2 most common neurologi- stimuli, morning irritability, excessive caffeine intake and
cal disorders: at any one time 7 in 1000 people in the prolonged sleeping on weekends.
general population have epilepsy. Epilepsy usually Psychogenic nonepileptic events may be defined as “a
begins in childhood, potentially impeding education, em- paroxysmal behavioural pattern mimicing epileptic seizures
ployment, social relationships and development of a sense of and initiated by psychological mechanisms”.1 Diagnosis de-
self-worth. Prompt, accurate diagnosis with appropriate so- pends principally on symptomatology (Table 2). A physician
cial and medical management will optimize the situation. A should suspect such events in any patient with an apparently
family physician, in conjunction with a neurologist, can as- intractable cryptogenic “seizure disorder,” except in infants
certain (a) if the episodes represent epileptic seizures and or elderly people. Psychogenic events may mimic any type of
(b) if so, which epileptic syndrome they represent. epileptic seizure and may occur as a pseudostatus epilepticus.
A harmonized partnership between family physician and Distinguishing between psychogenic and frontal lobe epilep-
neurologist will facilitate the recognition and care of tic seizures may be difficult although the latter are shorter
epileptic disorders. As the role of the family physician in and occur principally at night. Psychogenic events may su-
the care of patients with epilepsy increases, the principles pervene in some truly epileptic patients. Electroencephalo-
delineated in this article will be ever more utilized. gram (EEG) monitoring may be required. However, epilep-
tic seizures that arise from mesial or inferior cortical surfaces
Differential diagnoses may demonstrate no interictal or ictal EEG abnormality. At
the Epilepsy Programme in London, Ont., we have devel-
Before determining whether paroxysmal events repre- oped a system for identifying suspected psychogenic attacks
sent an epileptic disorder, one must consider 2 alternatives: that consists of taking a detailed description of the attack, 24-
(a) nonepileptic events mimicking epileptic seizures (Table hour telemetered EEG recordings over 2–3 days and a clini-
1) and (b) true epileptic seizures caused by a nonneurologi- cal psychological consultation including the Minnesota Mul-
cal condition (Box 1). Three conditions are common imita- tiphasic Personality Inventory-2 (MMPI-2). The MMPI-2
tors of epilepsy: syncope, excessive daytime sleep and pseu- contains profiles of significant sensitivity and specificity for
doseizures. anxiety, somatization and hysteria, components that predis-
Table 2 lists several distinguishing manifestations of pose a person to pseudoseizures. The evaluation concludes
syncope, which resembles a generalized tonic-clonic with an interview with the patient, one or more close rela-
(GTC) seizure in the middle of the attack but not at its on- tives, the clinical psychologist and the neurologist.
set or termination. Almost always while in an erect sitting An erroneous diagnosis of epilepsy carries serious conse-
or standing position, the patient feels faint, vision may blur, quences. Missing a cardiac arrhythmia could be fatal. The pa-
the face becomes pale, sweating may occur, and the patient tient could be unnecessarily exposed to side effects of
falls atonically with occasional bilaterally synchronous tonic antiepileptic medications; this occurs principally in emergent
or myoclonic phenomena followed by rapid recovery, albeit situations with pseudostatus epilepticus. Potentially treatable
with fatigue. The principal differential diagnosis is a treat- psychiatric conditions could be overlooked. The patient could
able cardiac arrhythmia, and this should be strongly sus- unnecessarily lose his or her driver’s licence and occupation.
Autonomic features
Micturition Frequent Rare Occasional
Eyes Open Closed Open
Pupils Dilated or hippus during Normal Dilated
attacks
Colour Cyanotic or grey Rubor or normal Pale
Pulse Rapid, strong Normal Slow if vasovagal, weak if
vasodepressor; that of
arrhythmias if cardiogenic
Cognitive and behavioural aspects
Awareness Lost Preserved Lost or impaired
Talking None Occasional None
Restraint necessary To prevent injury; 1 person To control violence; many people Never
suffices required
Timing
Usual duration 1–5 min 5–60 min 1–2 min
Onset Sudden Gradual Gradual; possibly sudden if
cardiogenic
Sequence of symptoms Stereotyped Variable Stereotyped
Termination Spontaneous Spontaneous or induced by supra- Rapid
orbital pressure, suggestion
Sequelae
Injury Frequent, mild; scalp, face, Rare, but multiple bruises If sudden onset
common possible; scalp, face, rare
Postictal Tired, confused, sleeps Alert, emotional outburst Regains consciousness in
2–3 min; alert but tired
protein binding and compliance. It is prudent to measure that of maternal serum.24 Ultrasonography at 16–18 weeks’
antiepileptic serum levels before conception, at the begin- gestation may be necessary as well.
ning of each trimester and during the last month in patients Hemorrhagic disease of the newborn may occur in an in-
with moderately severe seizure disorders. fant whose mother has lower than normal levels of vitamin
K–dependent clotting factors. This can be prevented with
Teratogenic effects 10–20 mg per day of vitamin K orally in the last month of
pregnancy23,25 (Dr. Renato Natale, Associate Chief, St.
The risk of major malformations in babies of mothers Joseph’s Health Centre and London Health Sciences Cen-
taking antiepileptic drugs is about 4%–8% as compared tre — University Campus, London, Ont.: personal commu-
with a baseline of 1%–3%.25 Most of this increased risk can nication, 2002). Oral vitamin K can be obtained in Canada
be attributed to unfavourable lifestyle, inadequate nutri- through the Special Access Programme.27,28
tion, high antiepileptic drug levels and polypharmacy.24,25
Therefore, if possible, change gradually to monotherapy, Postpartum considerations
which is usually a safe procedure.
As no single antiepileptic drug has been shown to be more Although antiepileptic drugs are detectable in breast
teratogenic than another, a pregnant woman should keep tak- milk, their concentrations are usually lower than those in
ing her current drug, which is pre- maternal serum. Breast-feeding
sumably the best antiepileptic should not be discouraged in
drug for her epilepsy. Barbitu- women with epilepsy, because
rates, phenytoin and ethosuximide Antiepileptic drug therapy: key points its advantages appear to out-
have been associated with con- weigh the rare (5%–10%) ad-
genital heart, cleft lip and palate • Monotherapy suffices for most seizure dis- verse effects to the baby of seda-
abnormalities. 24 Valproate and orders. tion, hypotonia and feeding
• Twice-daily dosing is most practical except
carbamazepine may produce difficulty. Drug withdrawal
in pregnancy, when dosing 4 times daily
neural tube defects and hydro- symptoms have been reported
prevents a serum level surge and therefore
cephalus, with an incidence of sporadically. 23 Antiepileptic
has less effect on the fetus.
neural tube defects of 1%–2% for • The severity of the seizure disorder, not the drug levels may gradually in-
valproate and 0.5%–1% for laboratory numbers, determines the “thera- crease over the first few weeks
carbamazepine.24,25 Effects of ox- peutic range.” Whatever serum drug level after birth as enzymatic induc-
carbazepine, topiramate and lam- renders the patient seizure free is adequate tion will have decreased.
otrigine are unknown. Minor mal- for that patient, even if it is below the labo- Mothers with incompletely
formations such as hypertelorism, ratory range. controlled seizures should avoid
low-set ears and nail-bed hypopla- • Dual therapy with most antiepileptic drugs at bathing an infant in the bathtub
sia may occur, but these usually do serum levels in the middle of the laboratory without another person present
not cause serious medical or cos- range impairs cognition. and should change the infant on
metic effects.23 • Effectiveness and side effects both depend the floor.
Adequate nutrition and folic on dosage. Small changes in dosage can
acid supplementation by about produce dramatic effects. Epilepsy in elderly patients
4–5 mg/d in any sexually active • Traditional antiepileptic drugs may be as
woman of childbearing age low- effective as new ones. Unfortunately, the incidence
ers the risk of major fetal malfor- • Fatigue is the most common side effect of and prevalence of epilepsy in-
most antiepileptic drugs.
mations, especially neural tube creases in elderly people because
• Phenytoin is the only antiepileptic drug
defects in babies of young women hemorrhagic and ischemic
that can be started at full dose.
taking antiepileptic drugs.23,24,26 As stroke, primary or secondary tu-
neural tube and cardiac malfor- mours, trauma, dementia and
mations occur during the first 5 metabolic disorders occur com-
weeks of pregnancy, adequate folic acid levels should be es- monly in this population. Fortunately, such epilepsy is sel-
tablished before conception. dom intractable. The consequences of uncontrolled
Because of high fetal demand, folic acid levels decline seizures may be greater in elderly patients: a fall may frac-
in pregnancy, reaching a nadir at term.23 Women who ture a hip or create a subdural hematoma, whereas a GTC
smoke have lower folic acid levels than those who do not seizure may crush a vertebra, giving back pain. A postictal
smoke. High folic acid levels do not appear to exacerbate state may manifest as memory loss, cognitive impairment,
a seizure disorder. or an increase in a hemiparesis or dysphasia.
An expert obstetric opinion is needed to monitor for The principal differential diagnoses are syncope, sudden
congenital defects. This may involve α-fetoprotein screen- falls of elderly people, transient ischemic attacks or even
ing: that of amniotic fluid is apparently more reliable than sleep disturbances. Nonconvulsive status epilepticus ap-
pears more often in elderly people, manifesting as mild ders in elderly patients are focal with possible secondary
confusion and forgetfulness or total unresponsiveness for generalization, carbamazepine and phenytoin would be ap-
hours or days. propriate drugs.14,29
Management Driving
Diagnostic tests include EEG, CT scanning and meta- The loss or suspension of a driving licence significantly
bolic studies. The need for antiepileptic drugs and ongo- disrupts life, but the medical, emotional and legal impacts of
ing medication should be reviewed to diminish polyphar- a medically related driving injury to others or self potentially
macy and its complications. The choice of any needed produce greater anguish. These opposing considerations
antiepileptic drug is guided by efficacy, ease of introduc- have led to the development by the Canadian Medical Asso-
tion and administration, potential drug interactions and ciation of guidelines for physicians (Table 4).30 Although
likelihood of significant side effects. As most seizure disor- studies have shown the risk of motor vehicle crashes to be
only equal or one-third greater among drivers with epilepsy 13. Loiseau P. Idiopathic and benign partial epilepsies. In: Wyllie E, editor. The
treatment of epilepsy. Principles and practice. 3rd ed. Philadelphia: Lippincott
as compared with the general population,31 this near equality Williams & Wilkins; 2001. p. 475-84.
may have been achieved by the implementation of the 14. Karceski S, Morrell M, Carpenter D. The expert consensus guideline series.
Treatment of epilepsy. Epilepsy Behav 2001;(Suppl 2):A1-50.
CMA’s guidelines. Risk assessment should include seizure 15. Friis ML, Lund M. Stress convulsions. Arch Neurol 1974;31:155-9.
frequency and loss of awareness or other faculty during the 16. Bardy AH. Decisions after first seizure. Acta Neurol Scand 1991;83:294-6.
17. Berg AT, Shinnar S. The risk of seizure recurrence following a first unpro-
events. Legal responsibility for failing to report possibly in- voked seizure: a quantitative review. Neurology 1991;41:965-72.
capable drivers is being placed ever more upon physicians. 18. Shinnar S, Berg AT, Moshe SL, Petix M, Maytal J, Kang H, et al. Risk of
seizure recurrence following a first unprovoked seizure in childhood: a
prospective study. Pediatrics 1990;85:1076-85.
This article has been peer reviewed. 19. Camfield PR, Camfield CS, Dooley JM, Tibbles JA, Fung T, Garner B. Epilepsy
after a first unprovoked seizure in childhood. Neurology 1985;35:1657-60.
Dr. Blume is Professor, Department of Clinical Neurological Sciences, Epilepsy 20. Camfield C, Camfield P, Gordon K, Dooley J. Does the number of seizures
and Clinical Neurophysiology, London Health Sciences Centre — University before treatment influence ease of control or remission of childhood epilepsy?
Campus, London, Ont. Not if the number is 10 or less. Neurology 1996;46:41-4.
21. First Seizure Trial Group. Randomized clinical trial on the efficacy of anti-
Competing interests: None declared.
epileptic drugs in reducing the risk of relapse after a first unprovoked tonic-
clonic seizure. Neurology 1993;43:478-83.
22. Klein P, Herzog AG. Hormones and epilepsy. In: Schmidt D, Schachter SC,
References editors. Epilepsy: problem solving in clinical practice. London: Martin Dunitz;
2000. p. 413-33.
1. Kuyk J, Leijten F, Meinardi H, Spinhoven, Van Dyck R. The diagnosis of 23. Foldvary N. Treatment of epilepsy during pregnancy. In: Wyllie E, editor.
psychogenic non-epileptic seizures: a review. Seizure 1997;6:243-53. The treatment of epilepsy principles and practice. 3rd ed. Philadelphia: Lippincott
2. Canadian Pharmacists Association. Tegretol. Compendium of pharmaceuticals Williams & Wilkins; 2001. p. 775-86.
and specialties. Ottawa: The Association; 2002. p. 1666-8. 24. Lindhout D. Pregnancy and epilepsy. In: Schmidt D, Schachter SC, editors.
3. Canadian Pharmacists Association. Lamictal. Compendium of pharmaceuticals Epilepsy: problem solving in clinical practice. London: Martin Dunitz; 2000.
and specialties. Ottawa: The Association; 2002. p. 866-70. p. 241-52.
4. Canadian Pharmacists Association. Dilantin. Compendium of pharmaceuticals 25. Sadler RM. Women’s issues and epilepsy. Mississauga (ON): The Medicine
and specialties. Ottawa: The Association; 2002. p. 509-12. Group; 2002. Merritt-Putnam Neurology Residents’ Course.
5. Canadian Pharmacists Association. Topamax. Compendium of pharmaceuticals 26. Canadian Pharmacists Association. Folic acid. Compendium of pharmaceuticals
and specialties. Ottawa: The Association; 2002. p. 1709-13. and specialties. Ottawa: The Association; 2002. p. 658-9.
6. Canadian Pharmacists Association. Epival. Compendium of Pharmaceuticals and 27. Canadian Pharmacists Association. Vitamin K. Compendium of pharmaceuticals
Specialties. Ottawa: The Association; 2002. p. 582-6. and specialties. Ottawa: The Association; 2002. p. 1842-3.
7. Canadian Pharmacists Association. Zarontin. Compendium of Pharmaceuticals 28. Canadian Pharmacists Association. Special Access Programme. Compendium
and Specialties. Ottawa: The Association; 2002. p. 1899. of pharmaceuticals and specialties. Ottawa: The Association; 2002. p. A7.
8. Levy RH, Dreifuss FE, Mattson RH, Meldrum BS, Penry JK. Antiepileptic 29. Parker BM, Vestal RE. Pharmacokinetics of anticonvulsant drugs in the el-
drugs. 3rd ed. New York: Raven Press; 1989. derly. In: Wyllie E, editor. The treatment of epilepsy, principles and practice.
9. Levy RH, Mattson RH, Meldrum BS. Antiepileptic drugs. 4th ed. New York: Philadelphia: Lea & Febiger; 1993. p. 769-74.
Raven Press; 1995. 30. Canadian Medical Association. Determining medical fitness to drive. 6th ed. Ot-
10. Wyllie E. The treatment of epilepsy, principles & practice. 3rd ed. Philadelphia: tawa: The Association; 2000.
Lippincott Williams & Wilkins; 2001. 31. McLachlan R. Epilepsy and driving. Mississauga (ON): The Medicine Group;
11. Blume WT. Epilepsy with generalised tonic-clonic seizures on awakening and 2002. p. 44-7. Merritt-Putnam Neurology Residents’ Course.
other idiopathic generalised epilepsies. In: Meinardi H, editor. The epilepsies,
Part II. Vol 73(29) of Handbook of clinical neurology series. Amsterdam: Elsevier
Science; 2000. p. 175-82.
12. Van Huffelen AC, van der Meij W. Idiopathic partial epilepsies. In: Meinardi
Correspondence to: Dr. Warren T. Blume, London Health Sciences
H, editor. The epilepsies, Part II. Vol 73(29) of Handbook of clinical neurology se- Centre — University Campus, 339 Windermere Rd., London ON
ries. Amsterdam: Elsevier Science; 2000. p. 5-35. N6A 5A5; fax 519 663-3753; warren.blume@lhsc.on.ca