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Infantile Spasms

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Guidance for diagnosing and managing children with infantile spasms

Infantile spasms are a type of seizure seen in infants, but this term also represents a severe epilepsy syndrome of childhood. [Howell: 2021] The diagnosis of infantile spasms is given when an infant has some combination of spasms that are present in clusters and great numbers (many spasms in a cluster and sometimes many clusters a day), developmental delay, and a characteristic EEG pattern called hypsarrhythmia (high-amplitude chaotic abnormal pattern). Spasms typically begin at 3-6 months of age and are the main feature of a seizure syndrome known as West syndrome. Though the "West syndrome" and "infantile spasms" are used interchangeably, the latter is preferred. Infantile spasms are a final common syndrome for many etiologies, including brain abnormalities, genetic causes, and metabolic disorders.

A child with infantile spasms may have sudden stiffening of the legs and arms while bending forward at the waist; the movements may be asymmetrical. The spasms are brief, but tend to occur in clusters lasting several minutes or more. They do not usually occur during sleep but are often observed upon awakening. They can be subtle and are sometimes confused with the Moro reflex or colic.

Different types of infantile spasms are associated with varying outcomes (discussed later):

  • Symptomatic: A specific etiology can be identified through history, physical exam, or testing, including genetic testing. Of children with infantile spasms, 61-70% have the symptomatic type. Tuberous sclerosis and perinatal asphyxia are the most common etiologies. Other causes include cortical brain malformations, metabolic disorders (e.g., maple syrup urine disease, pyridoxine dependency, nonketotic hyperglycemia, phenylketonuria), chromosomal abnormalities, other neurocutaneous syndromes, infection, including prenatal infections (e.g., congenital CMV), and brain tumors. Of children with infantile spasms, 61-70% have the symptomatic type. [Wirrell: 1996]
  • Idiopathic: Children have normal development and neurologic exams at the onset of spasms and a higher likelihood of "growing out" of them, particularly if the spasms respond well to medication. This suggests that early diagnosis and treatment may affect the outcome in children with idiopathic infantile spasms.
  • Cryptogenic
    Some neurologists identify a third group, cryptogenic infantile spasms, in which children are either delayed developmentally or have an abnormal neurologic exam at the onset of the spasms, but no specific cause can be identified. The proportion of children in this category will likely decrease as genetic testing becomes more broadly useful.

Infants with infantile spasms may demonstrate a delay in or loss of previously achieved developmental milestones. Findings may include poor tone, poor head control, loss of eye contact, decreased responsiveness to sounds and eye contact, and decreased alertness. Additional seizure types are seen in 30-50% of infants with this syndrome. Spasms usually stop as the infant ages, but other seizure types often take their place.

Other Names

  • Hypsarrhythmia
  • Lightning spasms
  • West syndrome

Key Points

Delay in diagnosis/treatment
Infants with infantile spasms have better outcomes if spasms are treated early and stopped completely; however, the median time from onset to first visit with a pediatric neurologist is 24.5 days, and the parent’s concerns are often ignored initially. Developmental outcome may be better if the spasms are controlled quickly. [Nasuti: 2010] [Hussain: 2017] [O'Callaghan: 2011]

Clusters on awakening
Infantile spasm clusters on awakening, whether in the morning or after a nap. If the family describes unusual movements in their infant with this pattern, infantile spasms should be strongly considered

Infantile spasms and vaccines
Although it will sometimes seem to families that the onset of infantile spasms was caused by vaccine administration, no convincing evidence supports this causality. [Willmore: 2009]

Developmental assessments
Even a child who is developing typically when infantile spasms begin may show a slowing of milestone achievement or regression. The medical home clinician should consider frequent developmental evaluation while infantile spasms continue. Developmental assessments may be available through Early Intervention programs (See Services below).

Immunizations timing and high-dose steroids
Immunizations should be postponed until at least a month after discontinuing high-dose steroids used to treat infantile spasms.

Treatments if there are known etiologies
A few causes of infantile spasms have specific treatments, including pyridoxine-dependent epilepsy and glucose transporter deficiency.

Children with infantile spasms and vaccines
Vaccines should be delayed in children with infantile spasms who are receiving treatment with hormonal therapies (prednisolone and ACTH) because they may be more likely to become infected from some types of vaccines, and more importantly, hormonal therapies greatly reduce the usefulness of vaccines. Children are generally on these medications short term: catch-up vaccination should occur several months after they are discontinued.
Certain causes of infantile spasms may lead to selecting one treatment over another
Children with infantile spasms due to tuberous sclerosis may be more likely to respond to vigabatrin than other treatments. Although children with Down syndrome respond to treatment as typical infants do, it is sometimes more difficult to recognize infantile spasms with existing hypotonia and developmental delays, and possibly more important to treat as early as possible since children with Down syndrome are likely to have baseline developmental delay even without the occurrence of infantile spasms.

Practice Guidelines

Standard of care guidelines were first published in 2010 and updated in 2018 to include new recommendations on the transition from pediatric to adult care, endocrine management, primary care, and emergency management. No practice guidelines have been published. Guidance based on expert opinion includes:

  • Go CY, Mackay MT, Weiss SK, Stephens D, Adams-Webber T, Ashwal S, Snead OC 3rd.
    Evidence-based guideline update: medical treatment of infantile spasms. Report of the Guideline Development Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society.
    Neurology. 2012;78(24):1974-80. PubMed abstract / Full Text

  • Wilmshurst JM, Gaillard WD, Vinayan KP, Tsuchida TN, Plouin P, Van Bogaert P, Carrizosa J, Elia M, Craiu D, Jovic NJ, Nordli D, Hirtz D, Wong V, Glauser T, Mizrahi EM, Cross JH.
    Summary of recommendations for the management of infantile seizures: Task Force Report for the ILAE Commission of Pediatrics.
    Epilepsia. 2015;56(8):1185-97. PubMed abstract

Diagnosis

Presentations

Characteristic symptoms include rapid muscular contractions of extremities in any of several patterns, including flexion, extension, and mixed patterns.

For example:

  • Extension/stiffening of the trunk, arms, and legs
  • Flexing at the waist, especially obvious when sitting
  • Thrusting arms to the side or across the chest
  • Repetitive head bobbing or nodding
  • Drawing up of legs to the chest when lying down

Individual spasms last only 1-2 seconds each and usually occur in clusters of just a few to a hundred. Clusters may occur many times a day, are common when the child is awakening in the morning or after a nap, and may be accompanied by irritability and crying. The child's developmental trajectory often slows or reverses around the time of spasm onset. Families may notice a decrease in developmental progress and interaction with the environment - or just that "something is wrong" with their child without being more specific. [Hussain: 2017] Video taken by parents of concerning behavior can be useful for the medical home provider and pediatric neurologist.

Diagnostic Criteria & Classifications

The term “infantile spasms” is often used somewhat interchangeably with "West syndrome." To meet criteria for West syndrome, the child must demonstrate the triad of spasms, intellectual disability, developmental delay, and hypsarrhythmia on EEG.

Diagnostic Testing & Screening

An EEG - wake and sleep - is particularly helpful when recorded during an episode thought to be infantile spasms. Hypsarrhythmia or modified hypsarrythmia, a very high-voltage chaotic pattern, is found in the majority of cases. Multifocal spike discharges (MSD) are found in the remainder.
Once the diagnosis of infantile spasms has been confirmed with EEG, testing for etiology is performed. Almost anything that causes brain damage can be associated with infantile spasms; an etiology can be found for about 3/4 of infants with infantile spasms, although this number continues to increase with the availability of genetic testing. The Infantile Spasms Diagnostic Algorithm (Primary Children's Hospital, Pediatric Neurology) (PDF Document 94 KB) (may guide the workup). The diagnostic evaluation should be tailored to the individual child, so not all studies are recommended for all children.

Labs

If no other cause is found for infantile spasms, metabolic causes should be considered, and newborn testing results confirmed. Testing may include serum amino acids, serum lactate, pyruvate, biotinidase, copper, ceruloplasmin, urine organic acids, urine purine and pyrimidine panel, urine sulfocysteine, and CSF neurotransmitter testing. Consultation with a metabolic geneticist may be valuable.

Imaging

Most infantile spasms with known etiologies are due to congenital, genetic, and acquired structural abnormalities, such as cortical dysplasia and stroke. Therefore, the first step recommended for testing for etiology is imaging, preferably MRI. In most cases, this would be done sedated and without contrast.

Other (sensory, genetic, other)

If the MRI is normal, genetic testing is recommended as being more likely to lead to a diagnosis. A microarray and/or epilepsy gene panel should be ordered first, with whole exome sequencing to follow if no etiology is found.

Genetics

Many causes of infantile spasms are genetic, including tuberous sclerosis, metabolic disorders, and chromosomal abnormalities. ARX and CDKL5, as well as other genes, are associated with infantile spasms in some infants with cryptogenic infantile spasms. [Paciorkowski: 2011]

Incidence & Prevalence

The prevalence rate is 0.15 to 0.2 per 1000 children 10 years or younger. [Mackay: 2004]

Differential Diagnosis

Infantile spasms are diagnosed by the appearance of the seizures (seizure "semiology"), the characteristic age at which they appear, and distinct abnormalities on EEG. The diagnosis should be reconsidered if an EEG obtained in a child with suspected infantile spasms is normal.
Other differential diagnoses to consider:
Moro reflex is a normal finding in infants up to about 3 or 4 months. When the infant is quickly moved, he/she will look startled, fling his/her arms out (palms up), and then draw the arms back to the body and relax. See Illustration of Moro Reflex (MedlinePlus).
Bobble-headed doll syndrome is a movement disorder seen in children with episodic up-and-down or side-to-side movement at 2-3 Hz. This disorder is often associated with third ventricle tumors, aqueductal stenosis, communicating hydrocephalus, and other structural brain disorders that can be diagnosed with brain MRI.
Spasmus nutans is a disorder seen in infants and young children. It consists of rapid, uncontrolled eye movements, head bobbing, and sometimes abnormal head positioning. Although an MRI of the brain is often performed to rule out brain abnormalities, a cause for this movement disorder is rarely found, and it usually resolves without treatment after several months. [Dugdale: 2009]
Other seizure types - Although infantile spasms may appear similar to myoclonic and tonic seizures, they are distinct and will require different treatments. It may be helpful for the family to record episodes on video and look for the initial contraction followed by a longer tonic phase typical for infantile spasms. Look for the combination of developmental delay and the characteristic EEG pattern.
Sandifer syndrome is body stiffening due to gastroesophageal reflux, but it may look similar to the tonic spasms seen in infantile spasms. Differentiating them may require additional workup, including a pH probe and an upper GI series. Sandifer syndrome may be seen with severe reflux, and it lacks the EEG findings of infantile spasms.

Comorbid Conditions

Additional seizure types are seen in 30 to 50% of infants with this syndrome. Spasms usually stop as the infant ages, but other seizure types often take their place.

Prognosis

In general, the prognosis depends on the etiology of the spasms and whether there is developmental delay or an abnormal neurologic exam at the time of presentation. A good outcome is associated with a lack of identifiable etiology, normal neurologic exam and development at the time of presentation, older age at onset, and a relatively quick and complete response to treatment of the spasms.

Most infants with infantile spasms have poor outcomes. In long-term follow-up studies, normal intellectual development is observed in only about 1/4 of children, complete seizure freedom is only observed in about 1/3 of children, and autism is very common. In those who develop other seizure types, a substantial portion may develop Lennox-Gastaut syndrome, an epileptic encephalopathy of childhood. [Riikonen: 2020] Development may be improved with early, effective treatment, although the optimal regimen is not known.

Treatment & Management

Etiology often determines the prognosis for seizure cessation and eventual developmental outcome. Specific therapies exist for a few of the underlying disorders, including pyridoxine-dependent seizures and focal cortical dysplasias (treated with vitamin B6 and surgical resection, respectively).
Because recent research suggests that hypsarrhythmia is difficult to recognize on EEG and experts may disagree, treatment should be initiated in infants with seizures consistent with infantile spasms and an abnormal EEG even if it is not read as hypsarrhythmia. [Demarest: 2017]

Neurology

Treatment is generally provided by a pediatric neurologist in communication with the medical home clinician, who will perform much of the ongoing monitoring. Treatment is pursued early and aggressively since the prognosis is extremely poor without treatment, and early treatment may affect the outcome. The goal of treatment is cessation of infantile spasms and normalization of the EEG. Treatment options include ACTH, vigabatrin (Sabril), prednisolone/prednisone, anticonvulsants (e.g., topiramate), other medications, and the Ketogenic diet. [Prezioso: 2018] Surgery may also be an option for a minority of patients. Dual therapy with a steroid (ACTH or prednisone/prednisolone) and vigabatrin is increasingly used nationwide. [Riikonen: 2020] One example of a dual therapy protocol is given here (from the University of Utah Health Pediatric Neurology Division).
Patient characteristics and local expertise will determine which treatment option to choose. Some considerations when choosing medication may be:
  • Dual or combo therapy: Many centers are now using both a steroid (prednisolone or prednisone) AND vigabatrin to aggressively treat infantile spasms.
  • In infants without tuberous sclerosis, hormonal treatments appear to be superior to vigabatrin for achieving seizure cessation, with short-term success in about 3/4 of infants, compared to about 1/2 with vigabatrin. Excluding effects on vision, adverse effects were similar in the hormonal groups and the vigabatrin groups.
  • Cessation of spasms may occur more quickly in infants treated with ACTH or prednisolone, which may have a beneficial effect on development. Although prednisolone and ACTH appear to be of similar efficacy and have about the same incidence of adverse effects, prednisolone is easier to administer and much less expensive. However, treatment with ACTH has been the gold standard and further studies are needed.
  • Vigabatrin is thought to be more effective in infants with infantile spasms due to tuberous sclerosis, and in these cases, it is often the first-line treatment even though there are no evidence-based recommendations for appropriate dosing. [Pellock: 2010][
Whatever regimen is chosen first, if spasms do not stop, an increase in dose or a new medicine is tried to stop the spasms.
ACTH
Dosing: 50-91% of children stop having infantile spasms with a 40 IU/m2 dose; a higher dose may be tried in infants who respond incompletely. ACTH is typically continued for 4 weeks after the spasms stop. If relapses occur, a second course of ACTH may stop the spasms again, or a different treatment might be tried.
  • Side effects: ACTH must be given by IM injections. It has many potentially serious side effects. Possible side effects include weight gain, hypertension, metabolic abnormalities, ulcers leading to gastric hemorrhage, irritability, sepsis, osteoporosis, and heart failure caused by dilated cardiomyopathy (some series show up to a 5% mortality rate).
  • Children on prednisolone or ACTH for infantile spasms may have an altered immune system, and care should be taken if the child becomes ill.
  • Screen for fecal occult blood and urine for glucose periodically in infants on high-dose steroids.
  • Steroids should be deferred for 4 weeks after administration of live vaccines and for 21 days if the infant has been exposed to varicella.
Infants are usually started on GI prophylaxis (e.g., ranitidine) to prevent gastrointestinal bleeding and a low-salt diet to prevent hypertension. The medical home clinician monitors children, sometimes with the help of home health, to check weight, irritability, blood pressure, fecal occult blood, and urine glucose. See Acthar GEL Manufacturer Information (Mallinckrodt Pharmaceuticals) for important safety information.
Vigabatrin
If the infant is on vigabatrin, perform ophthalmological assessments approximately every 3 months after the baseline evaluation to follow for retinal changes associated with vision loss.
In 2009, vigabatrin was licensed by the FDA to treat infantile spasms in children 1 month to 2 years of age. Due to the high incidence of vision loss, generic and brand name (Sabril) vigabatrin is restricted to patients registered with the manufacturer. Medications require dispensing from a specialty pharmacy.
  • Dosing: Use vigabatrin at the lowest dose possible for the shortest duration possible, and stop it if there is no observed benefit after 2-4 weeks. Vigabatrin is given to families in packets of powder, which they reconstitute. Detailed instructions are provided, but the medical home clinician may need to ensure that families are giving the medication correctly. When stopping vigabatrin, the dosage should be tapered gradually. Vigabatrin Manufacturer Information (Lundbeck Pharmaceuticals) for prescribing information.
  • Side effects/other: MRI changes have been observed in some infants receiving vigabatrin for infantile spasms. These changes, which are of uncertain significance, involve increased T2 signal and a restricted, symmetric, diffusion pattern in the thalamus, basal ganglia, brain stem, and cerebellum. There is no suggested screening for these changes. Other side effects have been observed in adults, including somnolence, fatigue, weight gain, edema, anemia, and peripheral neuropathy.
Oral prednisolone
Although more studies may be needed, many studies comparing oral prednisolone to ACTH have found the two likely equivalent. [Li: 2020] .
  • Dosing: Dosage ranges from 6 to 8 mg/kg/day, maximum daily dose of 60 mg, although there are variations in the schedule. The duration of therapy is short, and response is usually achieved within 2 weeks, although the dose needs to be tapered down over several weeks.
  • Side effects: Irritability, increased appetite, weight gain, hypertension (high blood pressure), and hyperglycemia (high blood sugar), immune system dysfunction
  • Considerations: Because of the prohibitive cost of ACTH, oral prednisolone has recently been investigated and may be equivalent to ACTH treatment, with fewer side effects and lower cost.
Ketogenic diet
One study found the ketogenic diet effective in fewer patients than ACTH (62% of infants became spasm-free with the ketogenic diet vs. 90% of infants given ACTH), although side effects also occurred less often (31% with the ketogenic diet vs. 80% for ACTH). [Kossoff: 2008] Since the nutritional content of infant formula is easier to control than that of a varied solid and liquid diet, the ketogenic diet is easier to manage in infants than in children. Further studies with variations of the ketogenic diet are necessary to understand its role in treatment. [Prezioso: 2018] See Ketogenic Diet for further details.
High-dose topiramate
Some clinicians use topiramate if prednisolone/ACTH are not appropriate or by physician choice. [Song: 2017]
  • Dosing: At least 6 mg/kg/day may also be effective, with about 1/2 of infants becoming seizure-free.
  • Side effects occurred in approximately 39%. [Zou: 2008] Also see [Peltzer: 2009]
Other medications
Newer antiepileptic medications and high-dose intravenous immunoglobulin are also being studied as possible treatments, and trials of everolimus for infants with tuberous sclerosis and infantile spasms are ongoing. [Samueli: 2018]

Development

Children who present with infantile spasms may have slow development or may have met early milestones and then show slower progress or regression after infantile spasms start. All children with infantile spasms should have periodic developmental assessment and be referred to Early Intervention. Physical, occupational, and speech therapy may be helpful for some patients.

Services & Referrals

Neuromuscular Clinics (see NW providers [1])
A multidisciplinary approach for care of boys with DMD is preferred. These clinics also may be involved in research protocols for treatment of children with DMD. List of MDA Care Centers (MDA) has clinic locations and local details.

Pediatric Orthopedics (see NW providers [4])
Consider referral for baseline evaluation, routine spine X-rays, and management of contractures, gait problems, scoliosis, and the need for equipment for ambulation, such as walkers. Initially, these visits may be every year, but as the disease progresses, the child may need to be seen at 6-month intervals.

Pediatric Endocrinology (see NW providers [1])
Consider referral if vertebral fractures are found on spine X-rays, even non-symptomatic ones, for consideration of IV bisphosphonate therapy. Endocrinology referral may also be important if puberty is delayed or if there is concern for adrenal insufficiency or growth hormone deficiency.

Pediatric Physical Medicine & Rehabilitation (see NW providers [3])
A referral may help in the evaluation of contractures, gait problems, and obtaining aids for ambulation. Physical medicine and rehabilitation may be available at MDA Clinics.

Physical Therapy (see NW providers [0])
Periodic visits can help to evaluate and maintain abilities. Frequency of visits should be based on many factors (need, financial resources, availability, and access) and balanced with treatment goals (ranging from post-surgical PT to a home-therapy program taught to the parents).

Pediatric Cardiology (see NW providers [0])
Boys with DMD and BMD should receive cardiac evaluation with echocardiogram (or MRI) and EKG at diagnosis and then yearly unless clinical circumstances mandate more frequent visits.

Pediatric Pulmonology (see NW providers [0])
Boys with DMD should initially see a pulmonary specialist for a baseline evaluation and then visit regularly after loss of ambulation. Periodic screening may include pulmonary function testing and/or overnight oximetry. If overnight oximetry is abnormal, an overnight sleep study determines if NIPPV (non-invasive positive pressure ventilation) is needed. If needed, a specialist will fit the child with NIPPV equipment and determine settings. Cough strength should also be evaluated. Cough assist devices should be prescribed soon after the child becomes non-ambulatory.

Dieticians and Nutritionists (see NW providers [1])
Early referral should be made for patients who become overweight (which makes it more difficult for already weak muscles to move the body) or underweight (no reserve, risk of pressure ulcers). Ideally, dieticians should be available at Neuromuscular Clinics.

Pediatric Ophthalmology (see NW providers [1])
Children taking steroids should have periodic eye exams for cataracts.

ICD-10 Coding

G40.821, Epileptic spasms not intractable, with status epilepticus

G40.822, Epileptic spasms not intractable, without status epilepticus

G40.823, Epileptic spasms intractable, with status epilepticus

G40.824, Epileptic spasms intractable, without status epilepticus

Further coding details can be found by using the search feature at ICD10Data.com.

Resources

Information & Support

Related Portal Content
The Medical Home Portal provides related general diagnositic and management information, including:

Answers to questions that families may frequently ask can be found at: Care Notebook may also be helpful for tracking medical history, test results, and records.

For Professionals

Infantile Spasms (NINDS)
Information about diagnosis, treatment, and current research; National Institute of Neurological Disease and Stroke.

Diagnosis and Management of Infantile Spasms (AAP)
A guide to early recognition and diagnosis of infantile spasms; American Academy of Pediatrics.

Clinician's Guide to Infantile Spasms (NORD)
Information for families that includes synonyms, signs & symptoms, causes, affected populations, related disorders, diagnosis, therapies (both standard and investigational), and support organizations; National Organization of Rare Disorders.

For Parents and Patients

Infantile Spasms (NINDS)
Information about diagnosis, treatment, and current research; National Institute of Neurological Disease and Stroke.

Infantile Spasms (American Academy of Neurology) (PDF Document 355 KB)
Information about recognition and treatment of infantile spasms from the American Academy of Neurology

Epilepsy Foundation
A national organization that provides information about epilepsy; programs to improve epilepsy treatment; materials to assist in helping people with epilepsy find jobs; activities in schools to educate the public; activities to educate policymakers; funds for research; links to find local and state resources; and news about conferences and other items of interest.

Patient Education

Let's Talk About... Infantile Spasms (Spanish & English)
Describes what spasms look like and the causes, treatments, and prognosis; Intermountain Healthcare.

Tools

Infantile Spasms Treatment Algorithm (Primary Children's Hospital, Pediatric Neurology) (PDF Document 173 KB)
An example of an algorithm for managing infantile spasms, from the Division of Pediatric Neurology, University of Utah Department of Pediatrics at Primary Children’s Hospital.

Infantile Spasms Diagnostic Algorithm (Primary Children's Hospital, Pediatric Neurology) (PDF Document 94 KB)
An example of an algorithm for diagnosing infantile spasms, from the Division of Pediatric Neurology, University of Utah Department of Pediatrics at Primary Children’s Hospital.

Services for Patients & Families Nationwide (NW)

For services not listed above, browse our Services categories or search our database.

* number of provider listings may vary by how states categorize services, whether providers are listed by organization or individual, how services are organized in the state, and other factors; Nationwide (NW) providers are generally limited to web-based services, provider locator services, and organizations that serve children from across the nation.

Studies

Infantile Spasms (Clinicaltrials.gov)
Studies looking at better understanding, diagnosing, and treating this condition; from the National Library of Medicine.

Helpful Articles

PubMed search for articles published in the last 2 years about infantile spasms

Kelley SA, Knupp KG.
Infantile Spasms-Have We Made Progress?.
Curr Neurol Neurosci Rep. 2018;18(5):27. PubMed abstract

Wheless JW, Gibson PA, Rosbeck KL, Hardin M, O'Dell C, Whittemore V, Pellock JM.
Infantile spasms (West syndrome): update and resources for pediatricians and providers to share with parents.
BMC Pediatr. 2012;12:108. PubMed abstract / Full Text

Authors & Reviewers

Initial publication: November 2013; last update/revision: November 2022
Current Authors and Reviewers:
Author: Lynne M. Kerr, MD, PhD
Reviewer: Cristina Corina Trandafir, MD, PhD
Authoring history
2020: update: Lynne M. Kerr, MD, PhDA; Audie Chris Espinoza, MDR
2013: first version: Lynne M. Kerr, MD, PhDA; Denise Morita, MDA; Paula Peterson, APRN, PNPA; Matthew Sweney, MDA
AAuthor; CAContributing Author; SASenior Author; RReviewer

Page Bibliography

Demarest ST, Shellhaas RA, Gaillard WD, Keator C, Nickels KC, Hussain SA, Loddenkemper T, Patel AD, Saneto RP, Wirrell E, Sánchez Fernández I, Chu CJ, Grinspan Z, Wusthoff CJ, Joshi S, Mohamed IS, Stafstrom CE, Stack CV, Yozawitz E, Bluvstein JS, Singh RK, Knupp KG.
The impact of hypsarrhythmia on infantile spasms treatment response: Observational cohort study from the National Infantile Spasms Consortium.
Epilepsia. 2017;58(12):2098-2103. PubMed abstract / Full Text

Dugdale, DC and Hoch, DB.
Spasmus nutans.
Medline Plus (NLM.NIH.GOV); (2009) http://www.nlm.nih.gov/medlineplus/ency/article/001409.htm.

Go CY, Mackay MT, Weiss SK, Stephens D, Adams-Webber T, Ashwal S, Snead OC 3rd.
Evidence-based guideline update: medical treatment of infantile spasms. Report of the Guideline Development Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society.
Neurology. 2012;78(24):1974-80. PubMed abstract / Full Text

Howell KB, Freeman JL, Mackay MT, Fahey MC, Archer J, Berkovic SF, Chan E, Dabscheck G, Eggers S, Hayman M, Holberton J, Hunt RW, Jacobs SE, Kornberg AJ, Leventer RJ, Mandelstam S, McMahon JM, Mefford HC, Panetta J, Riseley J, Rodriguez-Casero V, Ryan MM, Schneider AL, Smith LJ, Stark Z, Wong F, Yiu EM, Scheffer IE, Harvey AS.
The severe epilepsy syndromes of infancy: A population-based study.
Epilepsia. 2021;62(2):358-370. PubMed abstract

Hussain SA, Lay J, Cheng E, Weng J, Sankar R, Baca CB.
Recognition of Infantile Spasms Is Often Delayed: The ASSIST Study.
J Pediatr. 2017;190:215-221.e1. PubMed abstract

Kelley SA, Knupp KG.
Infantile Spasms-Have We Made Progress?.
Curr Neurol Neurosci Rep. 2018;18(5):27. PubMed abstract

Kossoff EH, Dorward JL.
The modified Atkins diet.
Epilepsia. 2008;49 Suppl 8:37-41. PubMed abstract

Li S, Zhong X, Hong S, Li T, Jiang L.
Prednisolone/prednisone as adrenocorticotropic hormone alternative for infantile spasms: a meta-analysis of randomized controlled trials.
Dev Med Child Neurol. 2020;62(5):575-580. PubMed abstract

Mackay MT, Weiss SK, Adams-Webber T, Ashwal S, Stephens D, Ballaban-Gill K, Baram TZ, Duchowny M, Hirtz D, Pellock JM, Shields WD, Shinnar S, Wyllie E, Snead OC 3rd.
Practice parameter: medical treatment of infantile spasms: report of the American Academy of Neurology and the Child Neurology Society.
Neurology. 2004;62(10):1668-81. PubMed abstract / Full Text

Nasuti G, Temple VA.
The risks and benefits of snow sports for people with disabilities: a review of the literature.
Int J Rehabil Res. 2010;33(3):193-8. PubMed abstract

O'Callaghan FJ, Lux AL, Darke K, Edwards SW, Hancock E, Johnson AL, Kennedy CR, Newton RW, Verity CM, Osborne JP.
The effect of lead time to treatment and of age of onset on developmental outcome at 4 years in infantile spasms: evidence from the United Kingdom Infantile Spasms Study.
Epilepsia. 2011;52(7):1359-64. PubMed abstract

Paciorkowski AR, Thio LL, Dobyns WB.
Genetic and biologic classification of infantile spasms.
Pediatr Neurol. 2011;45(6):355-67. PubMed abstract / Full Text

Pellock JM, Hrachovy R, Shinnar S, Baram TZ, Bettis D, Dlugos DJ, Gaillard WD, Gibson PA, Holmes GL, Nordl DR, O'Dell C, Shields WD, Trevathan E, Wheless JW.
Infantile spasms: a U.S. consensus report.
Epilepsia. 2010;51(10):2175-89. PubMed abstract
Although evidence-based guidelines were published in 2004, many questions remained about the diagnosis, evaluation, and management of infantile spasms. This article develops consensus guidelines regarding some of those questions.

Peltzer B, Alonso WD, Porter BE.
Topiramate and adrenocorticotropic hormone (ACTH) as initial treatment for infantile spasms.
J Child Neurol. 2009;24(4):400-5. PubMed abstract / Full Text

Prezioso G, Carlone G, Zaccara G, Verrotti A.
Efficacy of ketogenic diet for infantile spasms: A systematic review.
Acta Neurol Scand. 2018;137(1):4-11. PubMed abstract

Riikonen R.
Infantile Spasms: Outcome in Clinical Studies.
Pediatr Neurol. 2020;108:54-64. PubMed abstract

Samueli S, Dressler A, Gröppel G, Scholl T, Feucht M.
Everolimus in infants with tuberous sclerosis complex-related West syndrome: First results from a single-center prospective observational study.
Epilepsia. 2018. PubMed abstract

Song JM, Hahn J, Kim SH, Chang MJ.
Efficacy of Treatments for Infantile Spasms: A Systematic Review.
Clin Neuropharmacol. 2017;40(2):63-84. PubMed abstract

Wheless JW, Gibson PA, Rosbeck KL, Hardin M, O'Dell C, Whittemore V, Pellock JM.
Infantile spasms (West syndrome): update and resources for pediatricians and providers to share with parents.
BMC Pediatr. 2012;12:108. PubMed abstract / Full Text

Willmore LJ, Abelson MB, Ben-Menachem E, Pellock JM, Shields WD.
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