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Authors & Editors
Introduction
Infantile Spasm (West Syndrome) Clinical
Differentials
Workup
Article Last Updated: Apr 10, 2006 Treatment
Medication
AUTHOR AND EDITOR INFORMATION Follow-up
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Section 1 of 11 References
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Authors and Editors
Introduction Related Articles
Clinical Epilepsy in Children
with Mental
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Epileptiform
Medication Encephalopathies
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Patient Education
Author: Tracy A Glauser, MD, Professor, Departments of Pediatrics and Click here for
Neurology, University of Cincinnati College of Medicine, Children's patient
Comprehensive Epilepsy Program, Children's Hospital Medical Center of education.
Cincinnati
Tracy A Glauser is a member of the following medical societies: American

Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, and Child
Neurology Society
Coauthor(s): Diego A Morita, MD, Assistant Professor of Pediatrics and Neurology, Department of
Pediatrics, Division of Neurology, Cincinnati Children's Hospital Medical Center, University of
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Cincinnati
Editors: Robert Baumann, MD, Program Director, Professor, Departments of Neurology and
Pediatrics, University of Kentucky; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor,
eMedicine; Kenneth J Mack, MD, PhD, Senior Associate Consultant, Department of Child and
Adolescent Neurology, Mayo Clinic; Selim R Benbadis, MD, Professor, Director of Comprehensive
Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of
Medicine, Tampa General Hospital; Nicholas Y Lorenzo, MD, Chief Editor, eMedicine Neurology;
Consulting Staff, Neurology Specialists and Consultants
Author and Editor Disclosure
Synonyms and related keywords: infantile spasms, hypsarrhythmia, developmental delay, West
syndrome, mental retardation, epilepsy syndrome
INTRODUCTION
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Background
West syndrome is composed of the triad of infantile spasms, an interictal EEG pattern termed
hypsarrhythmia, and mental retardation, although the diagnosis can be made even if one of the 3
elements is missing (according to the international classification). This severe epilepsy syndrome is an
age-dependent expression of a damaged brain. The term "infantile spasms" has been used to describe
the seizure type, the epilepsy syndrome, or both. In this article, the term "infantile spasms" is
synonymous with West syndrome.
The syndrome's namesake, Dr W J West, gave the first detailed description of infantile spasms, as they
occurred in his child. In a letter to the editor of The Lancet in 1841, West described the events as
"bobbings" that "cause a complete heaving of the head forward towards his knees, and then
immediately relaxing into the upright position … these bowings and relaxings would be repeated
alternately at intervals of a few seconds, and repeated from 10 to 20 or more times at each attack, which
attack would not continue more than 2 or 3 minutes; he sometimes has 2, 3 or more attacks in the day."
This detailed clinical description was followed approximately 100 years later by the report of the typical
interictal EEG pattern termed hypsarrhythmia. Most patients with infantile spasms have some degree of
developmental retardation.
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The eponym West syndrome was created in the early 1960s by Drs. Gastaut, Poirier, and Pampiglione.
Pathophysiology
Infantile spasms are believed to reflect abnormal interactions between the cortex and brainstem
structures. Focal lesions early in life may secondarily affect other sites in the brain, and hypsarrhythmia
may represent this abnormal activity arising from multiple brain sites. The frequent onset of infantile
spasms in infancy suggests that an immature central nervous system may be important in the
pathogenesis. The brain-adrenal axis also may be involved. One theory states that the effect of different
stressors in the immature brain produces an abnormal excessive secretion of corticotropin-releasing
hormone, causing spasms. The clinical response to adrenocorticotropic hormone (ACTH) and
glucocorticoids can be explained by suppression of corticotropin-releasing hormone (CRH) production.
Frequency
United States
Infantile spasm constitutes 2% of childhood epilepsies but 25% of epilepsy with onset in the first year
of life. The rate of infantile spasm is estimated between 2.5 to 6.0 per 10,000 live births. Its prevalence
rate is 1.5-2.0 per 10,000 children aged 10 years or younger.
International
Infantile spasm occurs in 0.05 (Estonia) to 0.41 (Oulu, Finland) of 1000 live births and in 1.4%
(Estonia), 4.2% (Odense, Denmark), and 7.6% (Tampere, Finland) of children with epilepsy.
Mortality/Morbidity
The premature death rate ranges from 5-31%. The upper limit comes from a study of 214 Finnish
children with a history of infantile spasms who were followed for a mean of 25 years (range, 20-30 y).
Most of the deaths (61%) occurred at or before age 10 years, while only 10% occurred after age 20
years.
Sex
Although males are affected slightly more often than females, no significant gender difference is noted.
Age
Ninety percent of infantile spasms begin in those younger than 12 months. Peak onset is at age 4-6
months.
CLINICAL
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History
Ictal manifestations
Spasms begin with a sudden, rapid, tonic contraction of trunk and limb musculature that
gradually relaxes over 0.5-2 seconds.
Contractions can last 5-10 seconds.
The intensity may vary from a subtle head nodding to a powerful contraction of the
body.
Infantile spasms usually occur in clusters, often several dozens, separated by 5-30
seconds.
Spasms frequently occur just before sleep or upon awakening. They can be observed
during sleep, although this is rare.
Spasms can be flexor, extensor, or a mixture of flexion and extension.
Flexor spasms consist of brief contractions of the flexor muscles of the neck, trunks,
and limbs, resulting in a brief jerk. They may resemble a self-hugging motion and
often are associated with a cry. The patient then relaxes, and the jerk repeats. These
attacks occur in clusters throughout the day and last anywhere from less than 1
minute to 10-15 minutes or longer in some patients.
Extensor spasms consist of contractions of the extensor musculature with sudden
extension of the neck and trunk with extension and abduction of the limbs. Extensor
spasms and asymmetric or unilateral spasms often are associated with symptomatic
cases.
Mixed spasms are the most common type, consisting of flexion of the neck and arms
with extension of the legs, or flexion of the legs with extension of the arms.
In different series the frequency of the 3 spasm types were 42-50% mixed, 34-42%
flexor, and 19-23% extensor.
Interictal manifestations: An arrest or regression in psychomotor development accompanies the
onset of spasms in 70-95% of patients.
Family history: A family history of infantile spasms is uncommon but as many as 17% of patients
may have a family history of any epilepsy.
Physical
General physical examination
Physical examination can be important in helping to identify specific etiologies that may
have both systemic and neurological symptoms (eg, tuberous sclerosis complex).
Often a patient with infantile spasms has normal findings on general physical examination.
No pathognomonic physical findings are present in patients with infantile spasms.
If abnormalities in the general physical examination are noted (eg, adenoma sebaceum, ash
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leaf macules), specific etiologies may be suggested.

Use a Wood lamp to examine the skin.
Patients may exhibit moderate-to-severe growth delay; this is a nonspecific finding and
more a reflection of the underlying brain injury than of a specific epilepsy syndrome.
Neurologic examination
The neurologic examination in patients with infantile spasms demonstrates abnormalities

in mental status function, specifically deficits in cognitive function consistent with
developmental delay or regression.
Abnormalities in level of consciousness, cranial nerve function, and motor/sensory/reflex
examination are nonspecific findings and more a reflection of the underlying brain injury
or effect of anticonvulsant medications than of the syndrome.
No pathognomonic findings are present on neurologic examination in patients with
infantile spasms.
Causes
Infantile spasms (West syndrome) can be classified according to its suspected etiology as symptomatic,
cryptogenic, or idiopathic.
Symptomatic
Patients are diagnosed with symptomatic infantile spasms if an identifiable factor is

responsible for the syndrome. Virtually any disorder that can produce brain damage can be
associated with infantile spasms.
The list of etiologies can be subdivided into prenatal disorders, perinatal disorders, and
postnatal disorders.
Prenatal disorders include hydrocephalus, microcephaly, hydranencephaly,
schizencephaly, polymicrogyria, Sturge-Weber syndrome, incontinentia pigmenti,
tuberous sclerosis, trisomy 21, hypoxic-ischemic encephalopathies, congenital
infections, and trauma.
Perinatal disorders include hypoxic-ischemic encephalopathies, meningitis,
encephalitis, trauma, and intracranial hemorrhages.
Postnatal disorders include pyridoxine dependency, nonketotic hyperglycinemia,
maple syrup urine disease, phenylketonuria, mitochondrial encephalopathies,
meningitis, encephalitis, degenerative diseases, biotinidase deficiency, and trauma.
Evaluating children with infantile spasms for possible tuberous sclerosis is critical, as this
is the single most common disorder, comprising 10-30% of prenatal cases. Tuberosis
sclerosis is an autosomally dominant inherited disease with variable manifestations
including cardiac tumors, kidney tumors, cutaneous malformations such as ash-leaf
hypopigmented lesions, and seizures. In more than a few patients, the family diagnosis of
tuberous sclerosis is found only after a child presents with infantile spasms, and an
extensive workup of the child and subsequently the family reveals the genetic disease.
Of patients with infantile spasms, 70-75% have symptomatic epilepsy. This percentage
depends on the degree of sophistication of diagnostic studies. Development of more
exquisite neurodiagnostic techniques will alter the relative proportion of symptomatic,
cryptogenic, and idiopathic cases.
Cryptogenic
Patients have cryptogenic infantile spasms if no cause is identified but a cause is suspected
and the epilepsy is presumed to be symptomatic.
The proportion of cryptogenic cases varies from 8-42%. This wide range may be related to
variations in the definition of the term "cryptogenic" and the age of diagnosis, since
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assessment of developmental level in early infancy is difficult.

Idiopathic
Patients may be considered to have idiopathic infantile spasms if normal psychomotor

development occurs prior to the onset of symptoms, no underlying disorders or definite
presumptive causes are present, and no neurological or neuroradiological abnormalities
exist. Some investigators use the terms "idiopathic" and "cryptogenic" interchangeably.
The percentage of idiopathic cases reportedly is 9-14%.
Family history: A family history of infantile spasms is uncommon but as many as 17% of patients
may have a family history of any epilepsy.
DIFFERENTIALS
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Epilepsy in Children with Mental Retardation

Epileptic and Epileptiform Encephalopathies
Other Problems to be Considered
Benign myoclonus of early infancy

Myoclonic-astatic epilepsy
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Lab Studies
Prior to initiating therapy, consider obtaining some or all of the following laboratory studies:
Complete blood count with differential, liver panel, renal panel with electrolytes and
glucose, calcium, magnesium, phosphorus, and urinalysis with microscopic examination
Metabolic workup including glucose, liver panel, serum lactate and pyruvate, plasma
ammonia, serum and urine amino acids, urine organic acids, and serum biotinidase
Blood, urine, and cerebrospinal fluid cultures if an infection is suspected
Cerebrospinal fluid analysis for cell count, glucose, protein, bacterial and viral culture,
lactate, pyruvate, and amino acids
Imaging Studies
Overview
About 70-80% of patients have abnormal findings on neuroimaging studies.

Magnetic resonance imaging (MRI) of the brain provides a more detailed evaluation than
does a computed tomography (CT) scan of the brain.
Imaging studies should be obtained prior to starting ACTH or steroid therapy, as these
therapies are associated with the appearance of apparent brain atrophy as treatment
continues.
CT scan
Structural brain anomalies such as hydrocephalus, hydranencephaly, schizencephaly, and

agenesis of corpus callosum can be recognized easily by CT scans.
In addition, cerebral calcifications can be observed in patients with tuberous sclerosis or
congenital infections.
MRI: MRI scans are superior to CT scans in detecting areas of cortical dysgenesis, disorders of
neuronal migration, or disorders of myelination.
Other Tests
Electroencephalogram
Always perform an EEG in patients with suspected infantile spasms, since the diagnosis
depends on the presence of specific EEG findings.
If possible, obtain prolonged video-EEG telemetry to record both waking and sleep EEG to
assist in confirming a suspected diagnosis. A routine 20-minute EEG may not capture the
patient while both awake and asleep and thus may miss specific important EEG findings.
Interictal electroencephalogram
Hypsarrhythmia is the characteristic interictal EEG pattern and consists of chaotic, high- to
extremely high-voltage polymorphic delta and theta rhythms with superimposed multifocal
spikes and wave discharges (see Image 1). Multiple variations of this pattern are possible,
including focal or asymmetric hypsarrhythmia.
In one study of 77 patients with infantile spasms, unilateral hypsarrhythmia and
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asymmetric ictal EEG changes during spasms often occurred together and correlated with
focal or asymmetric cerebral lesions on imaging studies. Patients with symmetric
hypsarrhythmia and infantile spasms rarely had focal or asymmetric cerebral lesions on
imaging studies (most had structural diffuse brain lesions) and overall had better chances
for a normal outcome.
In a study of 26 patients with infantile spasms, 6 patients (23%) had asymmetric
hypsarrhythmia. All 6 had symptomatic infantile spasms and 5 had focal abnormalities on
examination or imaging study (4 ipsilateral to the lesion, 1 contralateral). These focal
abnormalities may identify a subset of patients with West syndrome who are candidates for
focal cortical resections.
Ictal electroencephalogram
Eleven different types of ictal patterns have been identified in patients with West
syndrome.
In one study, the most common pattern found in 38% of patients with seizures was a
high-voltage, frontal dominant, generalized slow-wave transient followed by voltage
attenuation, also termed an electrodecremental episode. These electrodecremental episodes
were a feature in 71% of the seizures.
No close correlation exists between the type of seizure and the EEG pattern.
Ophthalmic examination: Ophthalmic examination may reveal chorioretinitis from congenital
infections, chorioretinal lacunar defects in patients with Aicardi syndrome, or retinal tubers in
patients with tuberous sclerosis.
Wood lamp: Tuberous sclerosis is the single most common recognizable cause of West
syndrome. Therefore, a careful examination of the skin for the characteristic hypopigmented
lesions of tuberous sclerosis is mandatory. The unaided bedside identification of these lesions
may be more difficult in patients with light complexions.
Procedures
Lumbar puncture
In young infants with early onset of West syndrome, consider a lumbar puncture as part of
a full sepsis workup to look for signs of meningitis.
In older infants in whom no clear signs of infection are present, a lumbar puncture also is
useful in evaluating metabolic causes of West syndrome such as nonketotic
hyperglycinemia.
TREATMENT
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Medical Care
The goals of treatment for infants with West syndrome are the best quality of life with no
seizures, the fewest adverse effects from treatment, and the least number of medications.
Medications such as ACTH and conventional antiepileptic medications (AEDs) are the mainstay
of therapy for infants with West syndrome. Unfortunately, no one medical treatment gives
satisfactory relief for all infants with West syndrome.
The various medical treatment options for infants with West syndrome can be divided into 2
major groups:
Commonly used first-line treatments (ie, ACTH, prednisone, vigabatrin, pyridoxine

[vitamin B-6])
Second-line treatments (ie, benzodiazepines, valproic acid, lamotrigine, topiramate,
zonisamide)
Surgical Care
Focal cortical resection: In some patients, resection of a localized region can lead to freedom
from seizures.
Consultations
Pediatric neuropsychologists can assess intellectual function and educational needs and advise on
nonpharmacologic management of behavioral problems.
Pediatric psychiatrists can advise on pharmacologic management of behavioral problems.
Neurosurgeons can help assess whether the infant is a candidate for focal resection.
Dietitians can assist in the institution and maintenance of the ketogenic diet.
Diet
The ketogenic diet has been employed successfully to treat a variety of seizure types. However,
the role of the ketogenic diet in the treatment of infants with West syndrome is not defined.
MEDICATION
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The goals of treatment for infants with West syndrome are the best quality of life with no seizures, the
fewest adverse effects from treatment, and the least number of medications.
Drug Category: Hormonal agents
These agents cause profound and varied metabolic effects. Corticosteroids modify the body's immune
response to diverse stimuli.
Drug Name Corticotropin (Acthar, ACTH)

A 2004 American Academy of Neurology and Child
Neurology Society practice parameter concluded that
(i) "ACTH is probably effective for the short-term
treatment of infantile spasms and in resolution of
hypsarrhythmia (Level B)" and
(ii) "There is insufficient evidence to recommend the
optimum dosage and duration of treatment with
ACTH for the treatment of infantile spasms (Level
U)."
A 2004 multicenter, randomized, controlled trial in
the UK compared hormonal therapy (either oral
prednisolone or intramuscular tetracosactide depot, a
synthetic analogue of ACTH) to vigabatrin in 107
infants with infantile spasms. More infants assigned
hormonal treatments (73%) had no spasms on days
13 and 14 compared to infants assigned vigabatrin
(54%, p=0.043). A follow-up study demonstrated
that, although hormone treatment controlled spasms
Description better than vigabatrin initially, by age 12-14 months,
both groups had similar seizure-free rates. Older
studies suggest ACTH's efficacy (percentage of
infants with West syndrome reaching seizure
freedom) is between 50% and 67%. Associated with
serious, potentially life-threatening adverse effects.
Must be administered IM, which is painful to infant
and unpleasant for parent to perform.
Daily dosages expressed as U/d (most common),
U/m2/d, or U/kg/d.
Prospective single-blind study demonstrated no
difference in effectiveness of high-dose,
long-duration corticotropin (150 U/m2/d for 3 wk,
tapering over 9 wk) versus low-dose, short-duration
corticotropin (20-30 U/d for 2-6 wk, tapering over 1
wk). With respect to spasm cessation and
improvement in patient's EEG; hypertension was
more common with larger doses.
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Not established; 5-40 U/d IM for 1-6 wk to 40-160

U/d IM for 3-12 mo suggested; some authors
Pediatric Dose
recommend 150 U/m2/d IM for 6 wk or 5-8 U/kg/d
IM in divided doses for 2-3 wk
Documented hypersensitivity; porcine protein
hypersensitivity; scleroderma; recent surgery;
congestive heart failure; primary adrenal
Contraindications insufficiency; hypercortisolism; active herpes
infection; active tuberculosis; herpes simplex ocular
infection; thromboembolic disease; active serious
bacterial, viral, or fungal infection
Avoid vaccines and immunizations during therapy
Amphotericin B can decrease response;
acetazolamide or other carbonic anhydrase inhibitors
can cause hypernatremia, hypocalcemia,
hypokalemia, and edema; diuretics can reduce
natriuretic and diuretic effects; potassium-depleting
Interactions
diuretics can cause hypokalemia; phenytoin,
barbiturates, and rifampin can decrease effects;
estrogens can potentiate effects; salicylates or
NSAIDs can cause GI ulceration; can reduce growth
response to growth hormone (somatropin); warfarin
can decrease anticoagulation response
C - Safety for use during pregnancy has not been
Pregnancy
established.
Avoid vaccines and immunizations during therapy
Because of increased risk of infection, hypertension,
hypertrophic cardiomyopathy, and electrolyte
disturbances, careful and frequent clinical and
laboratory monitoring of patient is essential
Precautions
Caution in Cushing disease, hypertension,
hypokalemia, hypernatremia, diverticulitis,
ulcerative colitis or intestinal anastomosis, renal
disease, diabetes mellitus, hypothyroidism, hepatic
disease
Drug Name Prednisone (Deltasone, Orasone, Meticorten)

"there is insufficient evidence that oral
corticosteroids are effective in the treatment of
infantile spasms (Level U)."
Few comparative studies between ACTH and
Description prednisone have been performed; one double-blind,
placebo-controlled, crossover study demonstrated no
difference between low-dose ACTH (20-30 U/d) and
prednisone (2 mg/kg/d), while second prospective,
randomized, single-blinded study demonstrated
high-dose ACTH at 150 U/m2/d was superior to
prednisone (2 mg/kg/d) in suppressing clinical
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spasms and hypsarrhythmic EEG in infants with

infantile spasms.
better than vigabatrin initially, by age 12-14 months,
both groups had similar seizure-free rates.
Pediatric Dose 2 mg/kg/d PO for 2-4 wk
Documented hypersensitivity; viral infection; peptic
ulcer disease; hepatic dysfunction; connective tissue
Contraindications
infections; fungal or tubercular skin infections; GI
disease
Barbiturates, phenytoin, rifabutin, and rifampin can
increase metabolism of prednisone; hyperthyroidism
can increase metabolism of prednisone;
hypothyroidism can decrease metabolism of
Interactions
prednisone; isoproterenol in patients with asthma
can increase risk of cardiac toxicity, clinical
deterioration, myocardial infarction, congestive heart
failure, and death
B - Usually safe but benefits must outweigh the
Pregnancy
risks.
Prolonged therapy can affect metabolic, GI,
neurologic/behavioral, dermatologic, and endocrine
systems; metabolic adverse events can include (but
are not limited to) fluid retention and electrolyte
disturbances (eg, hypernatremia, hypokalemia,
hypokalemic metabolic alkalosis, hypocalcemia),
edema, hypertension, and hyperglycemia
GI adverse events can include nausea, vomiting,
abdominal pain, anorexia, diarrhea, constipation,
gastritis, esophageal ulceration, weight loss, and
delayed growth
Precautions Neurological and behavioral adverse events reported
during prolonged administration can include
headache, insomnia, restlessness, mood lability,
anxiety, personality changes, and psychosis
Visual adverse events may include exophthalmos,
retinopathy, posterior subcapsular cataracts, and
ocular hypertension
Dermatological adverse events reported during
therapy can include skin atrophy, diaphoresis,
impaired wound healing, facial erythema, hirsutism,
ecchymosis, and easy bruising
Endocrinological adverse events from prolonged use
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include hypercorticism and physiologic dependence

Idiosyncratic reactions include pancreatitis and
dermatological hypersensitivity reactions (allergic
dermatitis, angioedema, urticaria); avoid vaccination
with live-virus vaccines; avoid abrupt
discontinuation if patient has been on long-term
therapy
Caution in congestive heart failure, hypertension,
glaucoma, GI disease, diverticulitis, intestinal
anastomosis, hepatic disease, hypoalbuminemia,
peptic ulcer disease, renal disease, osteoporosis,
diabetes mellitus, hypothyroidism, coagulopathy or
thromboembolic disease, or potential impending GI
perforation
Drug Category: Anticonvulsants
These agents are used to manage severe muscle spasms.
Drug Name Vigabatrin

(i) "Vigabatrin is possibly effective for short-term
treatment of infantile spasms (Level C, Class III and
IV evidence)." (ii) "Vigabatrin is also possibly
effective for short-term treatment of infantile spasms
in majority of children with tuberous sclerosis
(Level C, Class III and IV evidence)." (iii) "Serious
concerns about retinal toxicity in adults suggest that
serial ophthalmologic screening is required in
patients on vigabatrin. However, data are
insufficient to make recommendations regarding the
frequency or type of screening that would be of
value in reducing the prevalence of this
complication in children (Level U, Class IV
Description studies)."
Not approved by FDA in US, but available in many
countries worldwide. Multiple studies (both open
label and double blind) have reported some
effectiveness in stopping seizures in infants with
West syndrome, especially when caused by tuberous
sclerosis.
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better than vigabatrin initially, by age 12-14 months,

both groups had similar seizure-free rates.
Initial dose: 40 mg/kg/d in 2 divided doses
Pediatric Dose
Maintenance doses: 40-150 mg/kg/d
Contraindications Documented hypersensitivity
Interactions None reported
Pregnancy
Dose-dependent adverse effects include
hyperactivity, agitation, sedation, depression,
psychosis, drowsiness, insomnia, facial edema,
ataxia, nausea and/or vomiting, stupor, and
somnolence; idiosyncratic reactions include visual
Precautions
field constriction; may exacerbate myoclonic and
absence seizures in some patients; long-term
reactions (ie, cumulative adverse effects) include
weight gain; lower doses in patients with renal
dysfunction
Drug Category: Benzodiazepines
A 2004 American Academy of Neurology and Child Neurology Society practice parameter concluded
that "there is insufficient evidence to recommend benzodiazepines for the treatment of infantile spasms
(Level U, Class III and IV evidence)."
By binding to specific receptor sites, these agents appear to potentiate the effects of GABA and
facilitate inhibitory GABA neurotransmission and other inhibitory transmitters.
Drug Name Clonazepam (Klonopin)

Considered second-line AED therapy against spasms
associated with West syndrome. Adverse effects and
Description development of tolerance limit usefulness over time.
Nitrazepam and clobazam not approved by FDA in
US but are available in many countries worldwide.
Pediatric Dose Maintenance dose: 0.01-0.2 mg/kg/d PO
Documented hypersensitivity; significant liver
Contraindications
disease; acute narrow-angle glaucoma
Decreases plasma levels of phenytoin,
phenobarbital, and carbamazepine; potentiates CNS
Interactions depression induced by other anticonvulsants and
alcohol; may reduce renal clearance of digoxin;
cimetidine and erythromycin decrease clearance
Pregnancy D - Unsafe in pregnancy
Dose-dependent adverse effects include
hyperactivity, sedation, drooling, incoordination,
drowsiness, ataxia, fatigue, confusion, vertigo,
Precautions
dizziness, amnesic effect, and encephalopathy;
considered least-sedating benzodiazepine; long-term
(cumulative) adverse effects include tolerance and
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dependence; considered to have longest time to

development of tolerance; adjust dose or discontinue
therapy in presence of renal or liver function
impairment, since metabolism occurs in liver and
metabolites are excreted in urine
Drug Category: Anticonvulsants
These agents prevent seizure recurrence and terminate clinical and electrical seizure activity.
Drug Name Valproic acid (Depakote, Depakene, Depacon)

"there is insufficient evidence to recommend
Description valproic acid for treatment of infantile spasms
Considered effective second-line AED therapy
against spasms associated with West syndrome.
Initial dose: 10-15 mg/kg/d PO divided bid/tid
Titration: 5-10 mg/kg/d increments at weekly
Pediatric Dose intervals until therapeutic effect achieved or toxic
effects occur
Maintenance dose: 15-60 mg/kg/d PO
Documented hypersensitivity; history of
hepatotoxicity or pancreatitis (patients at high risk
for hepatotoxicity include <2 y, multiple
Contraindications
concomitant AEDs including phenobarbital,
underlying metabolic disease such as defect in fatty
acid oxidation, and developmental delay)
Cimetidine, salicylates, felbamate, and erythromycin
may increase toxicity; rifampin may significantly
reduce levels; in children, salicylates decrease
protein binding and metabolism of valproate; may
result in variable changes of carbamazepine
concentrations, with possible loss of seizure control;
Interactions may increase diazepam and ethosuximide toxicity
(monitor closely); may increase phenobarbital and
phenytoin levels while either one may decrease
valproate levels; may displace warfarin from protein
binding sites (monitor coagulation tests); may
increase zidovudine levels in HIV-seropositive
patients
Pregnancy D - Unsafe in pregnancy
Dose-dependent adverse effects include asthenia,
nausea, vomiting, somnolence, tremor, and
dizziness; less common adverse effects include
Precautions thrombocytopenia and parotid swelling;
idiosyncratic reactions include hepatotoxicity and
pancreatitis; long-term (cumulative) adverse effects
include hair loss and weight gain
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Drug Name Lamotrigine (Lamictal)

lamotrigine for the treatment of infantile spasms
Lamotrigine inhibits release of glutamate and
Description inhibits voltage-sensitive sodium channels, leading
to stabilization of neuronal membrane. Effectiveness
in West syndrome has been investigated in
open-label studies with promising results.
Initial dose, maintenance dose, titration intervals,
and titration increments depend on concomitant
medications.
Combination with AEDs that induce hepatic
CYP-450 enzyme system WITHOUT valproate
Initial starting dose: 0.6 mg/kg/d PO for 2 wk; 1.2
mg/kg/d for wk 3-4; 5-15 mg/kg/d thereafter; after
week 4, dosage increment not to exceed 1.2 mg/kg/d
q1-2wk until maintenance dose achieved; maximum
daily dose is 400 mg/d
Pediatric Dose
Combination WITH valproate with or without other
AEDs that induce hepatic CYP-450 enzyme system
Initial starting dose: 0.15 mg/kg/d PO for 2 wk; 0.3
mg/kg/d for weeks 3-4; 1-5 mg/kg/d thereafter; after
week 4, dosage increment not to exceed 0.3 mg/kg/d
q1-2wk until maintenance dose achieved; usual
maximum daily dose is 200 mg/d
Documented hypersensitivity; history of erythema
multiforme, Stevens-Johnson syndrome, or toxic
Contraindications epidermal necrolysis; erythema multiforme;
Stevens-Johnson syndrome; toxic epidermal
necrolysis
Affected by concomitant AEDs; when used in
conjunction with medications that induce hepatic
CYP-450 microsomal enzymes (eg, phenobarbital,
carbamazepine, phenytoin), clearance enhanced;
conversely, when used in conjunction with
Interactions
medications that inhibit hepatic CYP-450
microsomal enzymes (eg, valproate), clearance
diminished; lower starting doses, slow titration rate
(ie, 2-wk or greater intervals between dosage
increases), and smaller increments are needed
Pregnancy
established.
Dose-dependent adverse effects include ataxia,
diplopia, dizziness, headache, nausea, and
Precautions
somnolence; idiosyncratic reactions include
Stevens-Johnson syndrome and toxic epidermal
16 of 23 6/16/2008 5:35 PM
necrolysis; no long-term (cumulative) adverse

effects noted to date
Risk factors for associated severe dermatologic
reactions include younger age (children more than
adults), co-medication with valproic acid, rapid rate
of titration, and high starting dose; give careful
attention to initial starting dose, titration rate, and
co-medications; prompt evaluation of any rash is
prudent and imperative; approximately 10-12% of
patients develop non–life-threatening rash that
usually resolves rapidly upon withdrawal and
occasionally without changing dosage
Drug Name Topiramate (Topamax)

topiramate for the treatment of infantile spasms
Topiramate is a sulfamate-substituted
monosaccharide with broad spectrum of
Description
antiepileptic activity that may have state-dependent
sodium channel blocking action, potentiates
inhibitory activity of neurotransmitter GABA. May
block glutamate activity.
Effectiveness in West syndrome has been
investigated in one open-label study with promising
results.
Initial starting dose: 2-3 mg/kg/d PO; increment of
Adult Dose 2-3 mg/kg q3-4d
Initial starting dose: 2-3 mg/kg/d PO; increment of
Pediatric Dose 2-3 mg/kg q3-4d
May increase phenytoin plasma levels; may decrease
Interactions valproate plasma levels; phenytoin and
carbamazepine decrease levels
Pregnancy
established.
Dose-dependent adverse effects include irritability,
ataxia, dizziness, fatigue, nausea, somnolence,
psychomotor slowing, concentration, constipation,
Precautions and speech problems; if CNS adverse effects occur,
reduce concomitant AEDs, slow titration, or reduce
dose; no idiosyncratic reactions noted; oligohidrosis
and nephrolithiasis reported
Drug Name Zonisamide (Zonegran)
17 of 23 6/16/2008 5:35 PM

zonisamide for the treatment of infantile spasms
Description
Effectiveness in West syndrome has been
investigated in 5 open-label studies with promising
results.
Initial dose: 1-2 mg/kg/d PO; increase 1-2 mg/kg/d
Pediatric Dose q2wk
Phenytoin, phenobarbital, carbamazepine, and
Interactions valproate decrease half-life; no effect on steady-state
plasma concentrations of other AEDs
Pregnancy
established.
Dose-dependent adverse effects include headache,
anorexia, nausea, dizziness, ataxia, paresthesia,
difficulty concentrating, irritability, and somnolence;
idiosyncratic reactions include severe rash
Precautions
(Stevens-Johnson syndrome, toxic epidermal
necrolysis) with reporting rate of 46 per million
patient-years of exposure; oligohidrosis and
nephrolithiasis reported
Drug Category: Vitamins
These agents are essential for normal metabolic processes.
Drug Name Pyridoxine (vitamin B-6)

pyridoxine for the treatment of infantile spasms
Two distinct treatment situations exist in which
pyridoxine is used in patients with West syndrome:
(1) IV administration during diagnostic EEG to
assess whether patient's seizures and EEG
Description abnormalities are related to pyridoxine deficiency. In
this approach, administer 50-100 mg IV during
diagnostic EEG; if dramatic improvement noted in
EEG, patient believed to have pyridoxine-dependent
seizures
(2) Long-term oral administration: Effectiveness of
long-term oral high-dose pyridoxine in West
syndrome has been investigated in multiple
open-label studies with promising results; most
patients who respond to long-term oral high-dose
18 of 23 6/16/2008 5:35 PM
pyridoxine do so within 1-2 wk of initiation.

Initial dose: 10-20 mg/kg/d PO
Titration: Increase by 10 mg/kg q3d
Pediatric Dose
(approximately 100-400 mg/d)
Documented hypersensitivity; do not administer IV
Contraindications
to infants with cardiac disease
Can decrease phenobarbital and phenytoin serum
Interactions
concentrations
Pregnancy
established.
Usually well tolerated; adverse events include
decreased appetite, nausea, vomiting, paresthesias,
diarrhea, somnolence, and headache; abnormal liver
Precautions function tests and low serum folic acid levels have
been noted in some patients; long-term (cumulative)
adverse effects can include severe sensory peripheral
neuropathy, movement disorders, and ataxia
FOLLOW-UP
Section 8 of 11
ClickClickClick
Authors and Editors
Introduction
Clinical
Differentials
Workup
Treatment
Medication
Follow-up
Miscellaneous
Multimedia
References
Complications
Complications include dose-related, idiosyncratic, or long-term adverse effects from medications,

including death.
Prognosis
The long-term overall prognosis is poor and is related directly to the etiology.
Infants with idiopathic West syndrome have a better prognosis than do infants with
symptomatic West syndrome. Only 14% of infants with symptomatic West syndrome have
19 of 23 6/16/2008 5:35 PM
normal or borderline normal cognitive development, compared to 28-50% of infants with

idiopathic West syndrome. Mental retardation is severe in 70% of patients, often with
psychiatric problems such as autistic features or hyperactivity. Infrequently spasms may
persist in adulthood. Fifty to seventy percent of patients develop other seizure types.
Eighteen to fifty percent of patients will develop Lennox Gastaut syndrome.
Subsets of patients among the symptomatic West syndrome group seem to have a better
prognosis. A retrospective study of 17 children with trisomy 21 and infantile spasms found
that 13 of 16 survivors were seizure free for more than 1 year and 10 no longer were taking
anticonvulsants.
A study of 15 children with neurofibromatosis type 1 and infantile spasms also reported a
relatively benign seizure and cognitive outcome.
Prognosis appears to be worse in infants with other seizure types, persistent EEG
abnormalities, poor response to ACTH, and delayed initiation of treatment. One study
showed that later onset, normal-to-mild psychomotor delay at the time of diagnosis, and
good seizure control were factors related to better prognosis.
MISCELLANEOUS
Section 9 of 11
ClickClickClick
Authors and Editors
Introduction
Clinical
Differentials
Workup
Treatment
Medication
Follow-up
Miscellaneous
Multimedia
References
Medical/Legal Pitfalls
Failure to inform the patient's family of the risk for severe adverse effects, including death, from
the use of either ACTH or oral steroids
Failure to inform the patient's family of the risk for severe idiosyncratic reactions from two
commonly used antiepileptic medications for West syndrome
Valproate - Hepatotoxicity, pancreatitis

Lamotrigine - Steven-Johnson syndrome, toxic epidermal necrolysis
Failure to inform the patient's family of signs and symptoms to watch for that indicate severe
adverse effects or idiosyncratic reactions
Failure to instruct the family on what to do if they notice signs and symptoms indicating severe
adverse effects or idiosyncratic reactions
Failure to fully investigate and identify possible causes of the patient's West syndrome, including
identification of tuberous sclerosis (a common cause of West syndrome), which can have
implications for the entire family
Failure to recognize signs and symptoms of West syndrome, which could result in failure to
20 of 23 6/16/2008 5:35 PM
select an appropriate AED with proven efficacy; this could increase the risk for uncontrolled
seizures that in turn increase the risk for injury and death
MULTIMEDIA
Section 10 of 11
ClickClickClick
Authors and Editors
Introduction
Clinical
Differentials
Workup
Treatment
Medication
Follow-up
Miscellaneous
Multimedia
References
Media file 1: Infantile spasm (West syndrome). Mountainous chaotic disorganized

rhythms with superimposed multifocal spikes demonstrating hypsarrhythmia in an
8-month-old boy with infantile spasms and developmental delay. Courtesy of E Wyllie.
Click to see larger picture Click to see detailView Full Size Image
Media type: Rhythm Strip
REFERENCES
Section 11 of 11
ClickClick
Authors and Editors
Introduction
Clinical
Differentials
Workup
Treatment
Medication
Follow-up
Miscellaneous
Multimedia
References
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Tracy A Glauser is a member of the following medical societies: American

Author and Editor Disclosure

General physical examination

leaf macules), specific etiologies may be suggested.

The neurologic examination in patients with infantile spasms demonstrates abnormalities

Patients are diagnosed with symptomatic infantile spasms if an identifiable factor is

assessment of developmental level in early infancy is difficult.

Patients may be considered to have idiopathic infantile spasms if normal psychomotor

Epilepsy in Children with Mental Retardation

Other Problems to be Considered

Benign myoclonus of early infancy

About 70-80% of patients have abnormal findings on neuroimaging studies.

Structural brain anomalies such as hydrocephalus, hydranencephaly, schizencephaly, and

Commonly used first-line treatments (ie, ACTH, prednisone, vigabatrin, pyridoxine

Drug Category: Hormonal agents

Drug Name Corticotropin (Acthar, ACTH)

Not established; 5-40 U/d IM for 1-6 wk to 40-160

Drug Name Prednisone (Deltasone, Orasone, Meticorten)

spasms and hypsarrhythmic EEG in infants with

include hypercorticism and physiologic dependence

Drug Category: Anticonvulsants

These agents are used to manage severe muscle spasms.

Drug Name Vigabatrin

better than vigabatrin initially, by age 12-14 months,

Drug Category: Benzodiazepines

Drug Name Clonazepam (Klonopin)

dependence; considered to have longest time to

Drug Category: Anticonvulsants

Drug Name Valproic acid (Depakote, Depakene, Depacon)

Drug Name Lamotrigine (Lamictal)

necrolysis; no long-term (cumulative) adverse

Drug Name Topiramate (Topamax)

Drug Name Zonisamide (Zonegran)

A 2004 American Academy of Neurology and Child

Drug Category: Vitamins

These agents are essential for normal metabolic processes.

Drug Name Pyridoxine (vitamin B-6)

pyridoxine do so within 1-2 wk of initiation.

Complications include dose-related, idiosyncratic, or long-term adverse effects from medications,

normal or borderline normal cognitive development, compared to 28-50% of infants with

Valproate - Hepatotoxicity, pancreatitis

Media file 1: Infantile spasm (West syndrome). Mountainous chaotic disorganized

Media type: Rhythm Strip

Groups. Epilepsia. Jul 1996;37(7):638-42. [Medline].

hormone. Epilepsia. Mar 2004;45(3):255-62. [Medline].

Infantile Spasm (West Syndrome) excerpt

Article Last Updated: Apr 10, 2006

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