eMedicine Specialties > Neurology > Pediatric Neurology

Infantile Spasm (West Syndrome): Treatment & Medication

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 Center of Cincinnati
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 Cincinnati
Contributor Information and Disclosures

Updated: Apr 10, 2006

Treatment

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

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.

Hormonal agents

These agents cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.


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 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.

Adult

Pediatric

Not established; 5-40 U/d IM for 1-6 wk to 40-160 U/d IM for 3-12 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

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 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

Documented hypersensitivity; porcine protein hypersensitivity; scleroderma; recent surgery; congestive heart failure; primary adrenal insufficiency; hypercortisolism; active herpes infection; active tuberculosis; herpes simplex ocular infection; thromboembolic disease; active serious bacterial, viral, or fungal infection

Pregnancy

C - Safety for use during pregnancy has not been established.

Precautions

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
Caution in Cushing disease, hypertension, hypokalemia, hypernatremia, diverticulitis, ulcerative colitis or intestinal anastomosis, renal disease, diabetes mellitus, hypothyroidism, hepatic disease


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 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.

Adult

Pediatric

2 mg/kg/d PO for 2-4 wk

Barbiturates, phenytoin, rifabutin, and rifampin can increase metabolism of prednisone; hyperthyroidism can increase metabolism of prednisone; hypothyroidism can decrease metabolism of prednisone; isoproterenol in patients with asthma can increase risk of cardiac toxicity, clinical deterioration, myocardial infarction, congestive heart failure, and death

Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; GI disease

Pregnancy

B - Usually safe but benefits must outweigh the risks.

Precautions

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
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

Anticonvulsants

These agents are used to manage severe muscle spasms.


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 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.

Adult

Pediatric

Initial dose: 40 mg/kg/d in 2 divided doses
Maintenance doses: 40-150 mg/kg/d

Pregnancy
Precautions

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 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

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.


Clonazepam (Klonopin)

Considered second-line AED therapy against spasms associated with West syndrome. Adverse effects and development of tolerance limit usefulness over time. Nitrazepam and clobazam not approved by FDA in US but are available in many countries worldwide.

Adult

Pediatric

Maintenance dose: 0.01-0.2 mg/kg/d PO

Decreases plasma levels of phenytoin, phenobarbital, and carbamazepine; potentiates CNS depression induced by other anticonvulsants and alcohol; may reduce renal clearance of digoxin; cimetidine and erythromycin decrease clearance

Documented hypersensitivity; significant liver disease; acute narrow-angle glaucoma

Pregnancy

D - Unsafe in pregnancy

Precautions

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 (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

Anticonvulsants

These agents prevent seizure recurrence and terminate clinical and electrical seizure activity.


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 spasms (Level U, Class III and IV evidence)."
Considered effective second-line AED therapy against spasms associated with West syndrome.

Adult

Pediatric

Initial dose: 10-15 mg/kg/d PO divided bid/tid
Titration: 5-10 mg/kg/d increments at weekly intervals until therapeutic effect achieved or toxic effects occur
Maintenance dose: 15-60 mg/kg/d PO

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 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

Documented hypersensitivity; history of hepatotoxicity or pancreatitis (patients at high risk for hepatotoxicity include <2 y, multiple concomitant AEDs including phenobarbital, underlying metabolic disease such as defect in fatty acid oxidation, and developmental delay)

Pregnancy

D - Unsafe in pregnancy

Precautions

Dose-dependent adverse effects include asthenia, nausea, vomiting, somnolence, tremor, and dizziness; less common adverse effects include thrombocytopenia and parotid swelling; idiosyncratic reactions include hepatotoxicity and pancreatitis; long-term (cumulative) adverse effects include hair loss and weight gain


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 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.

Adult

Pediatric

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

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

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 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

Documented hypersensitivity; history of erythema multiforme, Stevens-Johnson syndrome, or toxic epidermal necrolysis; erythema multiforme; Stevens-Johnson syndrome; toxic epidermal necrolysis

Pregnancy

C - Safety for use during pregnancy has not been established.

Precautions

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 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


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 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.

Adult

Initial starting dose: 2-3 mg/kg/d PO; increment of 2-3 mg/kg q3-4d
Maintenance dose: 15-20 mg/kg/d PO

Pediatric

Initial starting dose: 2-3 mg/kg/d PO; increment of 2-3 mg/kg q3-4d
Maintenance dose: 15-20 mg/kg/d PO

May increase phenytoin plasma levels; may decrease valproate plasma levels; phenytoin and carbamazepine decrease levels

Pregnancy

C - Safety for use during pregnancy has not been established.

Precautions

Dose-dependent adverse effects include irritability, ataxia, dizziness, fatigue, nausea, somnolence, psychomotor slowing, concentration, constipation, and speech problems; if CNS adverse effects occur, reduce concomitant AEDs, slow titration, or reduce dose; no idiosyncratic reactions noted; oligohidrosis and nephrolithiasis reported


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 spasms (Level U, Class III and IV evidence)."
Effectiveness in West syndrome has been investigated in 5 open-label studies with promising results.

Adult

Pediatric

Initial dose: 1-2 mg/kg/d PO; increase 1-2 mg/kg/d q2wk
Maintenance dose: 8-12 mg/kg/d PO

Phenytoin, phenobarbital, carbamazepine, and valproate decrease half-life; no effect on steady-state plasma concentrations of other AEDs

Pregnancy

C - Safety for use during pregnancy has not been established.

Precautions

Dose-dependent adverse effects include headache, anorexia, nausea, dizziness, ataxia, paresthesia, difficulty concentrating, irritability, and somnolence; idiosyncratic reactions include severe rash (Stevens-Johnson syndrome, toxic epidermal necrolysis) with reporting rate of 46 per million patient-years of exposure; oligohidrosis and nephrolithiasis reported

Vitamins

These agents are essential for normal metabolic processes.


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 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.

Adult

Pediatric

Initial dose: 10-20 mg/kg/d PO
Titration: Increase by 10 mg/kg q3d
Maintenance dose: 15-50 mg/kg/d PO (approximately 100-400 mg/d)

Can decrease phenobarbital and phenytoin serum concentrations

Documented hypersensitivity; do not administer IV to infants with cardiac disease

Pregnancy

C - Safety for use during pregnancy has not been established.

Precautions

Usually well tolerated; adverse events include decreased appetite, nausea, vomiting, paresthesias, diarrhea, somnolence, and headache; abnormal liver 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

More on Infantile Spasm (West Syndrome)

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Follow-up: Infantile Spasm (West Syndrome)
Multimedia: Infantile Spasm (West Syndrome)
References
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Further Reading

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Keywords

infantile spasms, hypsarrhythmia, developmental delay, West syndrome, mental retardation, epilepsy syndrome

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Contributor Information and Disclosures

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 Center of Cincinnati
Tracy A Glauser, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, and Child Neurology Society
Disclosure: Nothing to disclose.

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 Cincinnati
Diego A Morita, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Robert Baumann, MD, Program Director, Professor, Departments of Neurology and Pediatrics, University of Kentucky
Robert Baumann, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American College of Epidemiology, American Epilepsy Society, and Child Neurology Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Kenneth J Mack, MD, PhD, Senior Associate Consultant, Department of Child and Adolescent Neurology, Mayo Clinic
Kenneth J Mack, MD, PhD is a member of the following medical societies: American Academy of Neurology, Child Neurology Society, Phi Beta Kappa, and Society for Neuroscience
Disclosure: Nothing to disclose.

CME Editor

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
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Nicholas Y Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants
Nicholas Y Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology
Disclosure: Nothing to disclose.

 
 
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