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Infantile Spasm (West Syndrome) Introduction
Clinical
Article Last Updated: Apr 10, 2006 Differentials
Section 1 of 11 Workup
AUTHOR AND EDITOR INFORMATION Treatment
Medication
Follow-up
Authors and Editors Introduction Clinical Differentials Workup Treatment Miscellaneous
Medication Follow-up Miscellaneous Multimedia References Multimedia
References
Author: Tracy A Glauser, MD, Professor, Departments of Pediatrics and Neurology, University of
Cincinnati College of Medicine, Children's Comprehensive Epilepsy Program, Children's Hospital Medical Related Articles
Center of Cincinnati
Epilepsy in Children
with Mental
Tracy A Glauser is a member of the following medical societies: American Academy of Neurology, Retardation
American Academy of Pediatrics, American Epilepsy Society, and Child Neurology Society
Epileptic and
Coauthor(s): Diego A Morita, MD, Assistant Professor of Pediatrics and Neurology, Department of Epileptiform
Pediatrics, Division of Neurology, Cincinnati Children's Hospital Medical Center, University of Cincinnati Encephalopathies

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

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Patient Education
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patient education.

Section 2 of 11
INTRODUCTION

Authors and Editors Introduction Clinical Differentials Workup Treatment

Medication Follow-up Miscellaneous Multimedia References

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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.

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.

Section 3 of 11
CLINICAL

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Medication Follow-up Miscellaneous Multimedia References

History

Ictal manifestations
Spasms begin with a sudden, rapid, tonic contraction of trunk and limb musculature that gradually relaxes over
0.5-2 seconds.

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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 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

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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 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.

Section 4 of 11
DIFFERENTIALS

Authors and Editors Introduction Clinical Differentials Workup Treatment

Medication Follow-up Miscellaneous Multimedia References

Epilepsy in Children with Mental Retardation

Epileptic and Epileptiform Encephalopathies

Other Problems to be Considered

Benign myoclonus of early infancy
Myoclonic-astatic epilepsy

Section 5 of 11
WORKUP

Authors and Editors Introduction Clinical Differentials Workup Treatment

Medication Follow-up Miscellaneous Multimedia References

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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 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.

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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.

Section 6 of 11
TREATMENT

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Medication Follow-up Miscellaneous Multimedia References

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

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ketogenic diet in the treatment of infants with West syndrome is not defined.

Section 7 of 11
MEDICATION

Authors and Editors Introduction Clinical Differentials Workup Treatment

Medication Follow-up Miscellaneous Multimedia References

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 better than vigabatrin
Description 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
Pediatric Dose mo suggested; some authors 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 insufficiency; hypercortisolism; active herpes infection;
Contraindications
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 diuretics can
Interactions
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
Pregnancy C - Safety for use during pregnancy has not been 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)

A 2004 American Academy of Neurology and Child Neurology
Society practice parameter concluded that "there is insufficient
evidence that oral corticosteroids are effective in the treatment of
infantile spasms (Level U)."
Few comparative studies between ACTH and 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
Description mg/kg/d) in suppressing clinical spasms and hypsarrhythmic EEG
in infants with infantile spasms.
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 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;
Contraindications hepatic dysfunction; connective tissue 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
Interactions
metabolism of prednisone; isoproterenol in patients with asthma
can increase risk of cardiac toxicity, clinical deterioration,
myocardial infarction, congestive heart failure, and death
Pregnancy B - Usually safe but benefits must outweigh the risks.
Prolonged therapy can affect metabolic, GI,
neurologic/behavioral, dermatologic, and endocrine systems;
metabolic adverse events can include (but are not limited to) fluid
Precautions
retention and electrolyte disturbances (eg, hypernatremia,
hypokalemia, hypokalemic metabolic alkalosis, hypocalcemia),
edema, hypertension, and hyperglycemia

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GI adverse events can include nausea, vomiting, abdominal pain,

anorexia, diarrhea, constipation, gastritis, esophageal ulceration,
weight loss, and delayed growth
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 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

A 2004 American Academy of Neurology and Child Neurology
Society practice parameter concluded that (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 studies)."
Not approved by FDA in US, but available in many countries
Description 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.
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 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;
Precautions idiosyncratic reactions include visual 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

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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 development of
Description 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 disease; acute
Contraindications
narrow-angle glaucoma
Decreases plasma levels of phenytoin, phenobarbital, and
carbamazepine; potentiates CNS depression induced by other
Interactions
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, dizziness, amnesic effect, and encephalopathy;
considered least-sedating benzodiazepine; long-term
Precautions (cumulative) adverse effects include tolerance and 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)

A 2004 American Academy of Neurology and Child Neurology
Society practice parameter concluded that "there is insufficient
evidence to recommend valproic acid for treatment of infantile
Description
spasms (Level U, Class III and IV evidence)."
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 intervals until
Pediatric Dose
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,
Contraindications multiple 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; may
Interactions
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 thrombocytopenia and parotid swelling;
Precautions
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)

A 2004 American Academy of Neurology and Child Neurology
Society practice parameter concluded that "there is insufficient
evidence to recommend lamotrigine for the treatment of infantile
spasms (Level U, Class III and IV evidence)."
Lamotrigine inhibits release of glutamate and inhibits
Description
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 epidermal necrolysis;
Contraindications
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 C - Safety for use during pregnancy has not been established.
Dose-dependent adverse effects include ataxia, diplopia,
dizziness, headache, nausea, and somnolence; idiosyncratic
reactions include Stevens-Johnson syndrome and toxic
epidermal necrolysis; no long-term (cumulative) adverse effects
noted to date
Risk factors for associated severe dermatologic reactions include
Precautions 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)

A 2004 American Academy of Neurology and Child Neurology
Society practice parameter concluded that "there is insufficient
evidence to recommend topiramate for the treatment of infantile
spasms (Level U, Class III and IV evidence)."
Topiramate is a sulfamate-substituted monosaccharide with
Description broad spectrum of 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 2-3 mg/kg
Adult Dose q3-4d
Maintenance dose: 15-20 mg/kg/d PO
Initial starting dose: 2-3 mg/kg/d PO; increment of 2-3 mg/kg
Pediatric Dose q3-4d
Maintenance dose: 15-20 mg/kg/d PO

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Contraindications Documented hypersensitivity

May increase phenytoin plasma levels; may decrease valproate
Interactions
plasma levels; phenytoin and carbamazepine decrease levels
Pregnancy C - Safety for use during pregnancy has not been established.
Dose-dependent adverse effects include irritability, ataxia,
dizziness, fatigue, nausea, somnolence, psychomotor slowing,
concentration, constipation, and speech problems; if CNS
Precautions
adverse effects occur, reduce concomitant AEDs, slow titration,
or reduce dose; no idiosyncratic reactions noted; oligohidrosis
and nephrolithiasis reported

Drug Name Zonisamide (Zonegran)

A 2004 American Academy of Neurology and Child Neurology
Society practice parameter concluded that "there is insufficient
evidence to recommend zonisamide for the treatment of infantile
Description
spasms (Level U, Class III and IV evidence)."
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 q2wk
Pediatric Dose
Maintenance dose: 8-12 mg/kg/d PO
Contraindications Documented hypersensitivity
Phenytoin, phenobarbital, carbamazepine, and valproate
Interactions decrease half-life; no effect on steady-state plasma
concentrations of other AEDs
Pregnancy C - Safety for use during pregnancy has not been established.
Dose-dependent adverse effects include headache, anorexia,
nausea, dizziness, ataxia, paresthesia, difficulty concentrating,
irritability, and somnolence; idiosyncratic reactions include severe
Precautions
rash (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)

A 2004 American Academy of Neurology and Child Neurology
Society practice parameter concluded that "there is insufficient
evidence to recommend pyridoxine for the treatment of infantile
spasms (Level U, Class III and IV evidence)."
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 abnormalities are related to
Description
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 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
Maintenance dose: 15-50 mg/kg/d PO (approximately 100-400
mg/d)
Documented hypersensitivity; do not administer IV to infants with
Contraindications
cardiac disease
Interactions Can decrease phenobarbital and phenytoin serum concentrations
Pregnancy C - Safety for use during pregnancy has not been established.
Usually well tolerated; adverse events include decreased
appetite, nausea, vomiting, paresthesias, diarrhea, somnolence,
and headache; abnormal liver function tests and low serum folic
Precautions
acid levels have been noted in some patients; long-term
(cumulative) adverse effects can include severe sensory
peripheral neuropathy, movement disorders, and ataxia

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Section 8 of 11
FOLLOW-UP

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 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.

Section 9 of 11
MISCELLANEOUS

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 select an appropriate AED
with proven efficacy; this could increase the risk for uncontrolled seizures that in turn increase the risk for injury and

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death

Section 10 of 11
MULTIMEDIA

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.

View Full Size Image

Media type: Rhythm Strip

Section 11 of 11
REFERENCES

Authors and Editors Introduction Clinical Differentials Workup Treatment

Medication Follow-up Miscellaneous Multimedia References

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Infantile Spasm (West Syndrome) excerpt

Article Last Updated: Apr 10, 2006

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