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Infantile Spasms (West Syndrome)

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An epileptic syndrome characterized by the triad of infantile spasms, hypsarrhythmia, and arrest of psychomotor development at seizure onset. The majority present between 3-12 months of age, with spasms consisting of combinations of brief flexor or extensor movements of the head, trunk, and limbs. The condition is divided into two forms: cryptogenic (idiopathic) and symptomatic (secondary to a known disease process such as intrauterine infections; nervous system abnormalities; BRAIN DISEASES, METABOLIC, INBORN; prematurity; perinatal asphyxia; TUBEROUS SCLEROSIS; etc.). (From Menkes, Textbook of Child Neurology, 5th ed, pp744-8)

Also Known As:

West Syndrome; Lightning Attack; Infantile Spasm; Spasmus Nutans; Spasms, Infantile; Cryptogenic West Syndrome; Lightning Attacks; Salaam Attacks; Symptomatic West Syndrome; Attack, Lightning; Attacks, Lightning; Attacks, Salaam; Cryptogenic Infantile Spasm; Hypsarrhythmias; Infantile Spasm, Cryptogenic; Infantile Spasm, Symptomatic; Infantile Spasms, Cryptogenic; Infantile Spasms, Symptomatic; Jackknife Seizure; Nodding Spasms; Seizure, Jackknife; Seizures, Jackknife; Seizures, Salaam; Spasm, Cryptogenic Infantile; Spasm, Nodding; Spasm, Symptomatic Infantile; Spasms, Cryptogenic Infantile; Spasms, Nodding; Spasms, Symptomatic Infantile; Symptomatic Infantile Spasm; Syndrome, Cryptogenic West; Syndrome, Symptomatic West; Syndrome, West; West Syndrome, Cryptogenic; West Syndrome, Symptomatic; Cryptogenic Infantile Spasms; Hypsarrhythmia; Jackknife Seizures; Nodding Spasm; Salaam Seizures; Symptomatic Infantile Spasms

Networked: 1132 relevant articles (162 outcomes, 147 trials/studies)

Relationship Network

Disease Context: Research Results

Nervous System Diseases: 6158
- Central Nervous System Diseases: 1649
  - Brain Diseases: 2114
    - Epilepsy: 22953
      - Generalized Epilepsy: 1060
        Infantile Spasms: 1132
        Lennox-Gastaut type Epileptic encephalopathy: 740
        Norrie disease: 153
        X-linked infantile spasm syndrome: 20
        PEHO syndrome: 19
        1 benign familial infantile Convulsions: 10
        corpus callosum dysgenesis and cleft lip-palate Microcephaly: 1

Related Diseases

1.	Seizures (Seizure)
2.	Infantile Spasms (West Syndrome)
3.	Tuberous Sclerosis (Bourneville's Disease)
4.	Epilepsy (Aura)
5.	Spasm (Spasms)

Experts

1.	Zou, Li-Ping: 15 articles (12/2015 - 11/2006)
2.	Mytinger, John R: 9 articles (09/2015 - 08/2010)
3.	Dulac, Olivier: 9 articles (01/2015 - 03/2003)
4.	Kossoff, Eric H: 9 articles (09/2013 - 05/2002)
5.	Snead, O Carter: 8 articles (08/2015 - 01/2002)
6.	Galanopoulou, Aristea S: 8 articles (07/2015 - 08/2011)
7.	Chiron, Catherine: 8 articles (01/2015 - 03/2003)
8.	Yang, Guang: 7 articles (08/2015 - 02/2011)
9.	Haginoya, Kazuhiro: 7 articles (06/2015 - 07/2009)
10.	Oguni, Hirokazu: 7 articles (01/2015 - 05/2005)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Infantile Spasms:

1.	Adrenocorticotropic Hormone (ACTH)FDA Link 01/01/2002 - "The efficacy of ACTH, particularly in high doses, for rapid and complete elimination of infantile spasms (IS) has been demonstrated in prospective controlled studies. " 09/05/2008 - "The polymorphisms of the MC2R promoter might be one important factor that influences the efficacy of ACTH therapy on infantile spasms." 03/01/2015 - "Given the importance of rapid remission, clinicians should consider adding or changing treatment if infantile spasms do not resolve within two weeks of adrenocorticotropic hormone initiation. " 01/01/2005 - "To minimize adverse effects and to get good efficacy of ACTH therapy against West syndrome, we tried a new 2-steps therapeutic protocol consisting of the shortened ACTH therapy and the additional ACTH therapy. " 09/01/2013 - "Adrenocorticotrophic hormone (ACTH) therapy in infantile spasms (IS): current evidence for its superior therapeutic efficacy."
2.	Vigabatrin FDA Link 03/01/2012 - "Clinical studies have shown that vigabatrin is superior to placebo in decreasing the frequency of infantile spasms. " 05/01/2010 - "Vigabatrin is particularly effective for the treatment of patients with West syndrome; however, the patients need to be regularly monitored for the development of peripheral visual field defect. " 03/01/2008 - "Vigabatrin has proved to be effective against infantile spasms due to TSC. " 11/01/2003 - "This is a particular problem as vigabatrin is effective against infantile spasms. " 09/01/2002 - "Vigabatrin is an effective, well-tolerated treatment for infantile spasms. "
3.	Lennox-Gastaut type Epileptic encephalopathyIBA 11/01/2001 - "TRH is safe in children and effective in some cases of West syndrome and Lennox-Gastaut syndrome. " 06/01/2015 - "There is a great need for safe and effective therapies for treatment of infantile spasms (IS) and Lennox-Gastaut syndrome (LGS). " 12/01/2004 - "However, only 28% of patients with West syndrome or Lennox-Gastaut syndrome showed improvement. " 01/01/2012 - "In the study of 887 new born infants with prenatal and perinatal risk factors for brain damage, 11 children with West syndrome that progressed into Lennox-Gastaut syndrome and another 4 children with Lennox-Gastaut syndrome that had not been preceded by West syndrome were found. " 08/01/2009 - "This study examines factors influencing the evolution of West syndrome to Lennox-Gastaut syndrome. "
4.	topiramate (Topamax)FDA LinkGeneric 05/01/2006 - "Low-dose topiramate is effective in the treatment of infantile spasms." 03/01/2008 - "Topiramate proved to be an effective and safe monotherapy and add-on therapy in patients with infantile spasms younger than 1 year." 11/01/2006 - "Topiramate proves to be an effective and safe first-choice drug not only as adjunctive but also as monotherapy of infantile spasms in children younger than 2 years." 08/01/2015 - "The aim of this study was to determine the rate of infantile spasm remission with topiramate at our institution. " 08/01/2015 - "Remission of infantile spasms with topiramate was uncommon and no patient experienced persistent electroclinical remission. "
5.	Valproic Acid (Valproate, Semisodium)FDA LinkGeneric 11/01/2003 - "Valproate has also been used in the treatment of infantile spasms, with an efficacy of approximately 25 - 40%. " 06/01/2002 - "Remission of West syndrome associated with valproate hepatotoxicity." 01/01/1985 - "Although valproic acid (VPA) is used to treat infantile spasms, VPA's efficacy in infantile spasms has not been determined in a controlled study. " 01/01/2014 - "Encephalopathy in an infant with infantile spasms: possible role of valproate toxicity." 01/01/2014 - "The present case discusses a 5-year-old child with GDD associated with infantile spasms treated with sodium valproate. "
6.	zonisamide (Zonegran)FDA LinkGeneric 12/01/2009 - "Although further study of zonisamide is warranted, high-dose zonisamide can be effective and safe in some infants with newly diagnosed West syndrome." 09/01/2000 - "We determined that zonisamide was effective in three epileptic infants with hypsarrhythmia. " 05/01/1999 - "[Efficacy of zonisamide in West syndrome]." 04/01/1999 - "We determined the efficacy of and tolerability to zonisamide (ZNS) in newly diagnosed patients with infantile spasms. " 09/01/1997 - "We determined the short-term efficacy of zonisamide (ZNS) monotherapy in newly diagnosed patients with infantile spasms (IS). "
7.	lamotrigine (Lamictal)FDA LinkGeneric 05/01/2013 - "Lamotrigine effectively controlled the infantile spasms and improved the abnormal EEG findings. " 09/01/2002 - "Low-dose lamotrigine in West syndrome." 11/12/1994 - "Lamotrigine in infantile spasms." 07/01/2002 - "Lamotrigine is a useful and well tolerated drug for partial seizures and infantile spasms in infants <1 year of age. " 01/01/2000 - "Vigabatrin and lamotrigine are being used as first line drugs for infantile spasms and Lennox Gestaut syndrome (LGS) respectively. "
8.	Anticonvulsants (Antiepileptic Drugs)IBA 04/01/1983 - "Some anticonvulsants are also effective in treating infantile spasms, and modern research has explained many actions of these agents as well. " 10/01/2002 - "These new antiepileptic drugs represent a potential nonhormonal approach for infantile spasms, but additional studies are needed to verify their efficacy and tolerability. " 12/01/2014 - "The most frequent epileptic syndrome associated with DS is West syndrome, with good response to specific antiepileptics. " 07/01/2014 - "The patient developed infantile spasms at 4 months of age, and these were refractory to multiple antiepileptic drugs. " 04/01/2014 - "efficacy, doses, side effects, treatment in specific aetiological subtypes of infantile spasms), and other antiepileptic drugs (i.e. "
9.	felbamate (Felbatol)FDA Link 03/01/1995 - "Controlled studies are needed to firmly establish that felbamate is both safe and effective for the treatment of infantile spasms. " 10/01/1997 - "Felbamate for refractory infantile spasms." 03/01/1996 - "The use of felbamate to treat infantile spasms." 03/01/1995 - "The use of felbamate to treat infantile spasms." 03/01/1995 - "Based on the initial successful use of felbamate for infantile spasms in an infant with tuberous sclerosis, three additional infants with infantile spasms of different etiologies who had failed conventional therapies were treated with felbamate. "
10.	Vitamin B 6IBA 11/01/2009 - "Vitamin B6 (VB6) is used frequently as one of the first-choice drug for the treatment of West syndrome (WS) in Japan. " 11/01/2009 - "[Reappraisal of vitamin B6 therapy for West syndrome]." 11/01/2001 - "High-dose vitamin B(6) treatment is useful as a first line agent in treating West syndrome." 11/01/2001 - "Approximately 10-30% of patients with West syndrome respond to high-dose vitamin B(6) treatment. " 11/01/2001 - "High-dose vitamin B(6) treatment in West syndrome."

Therapies and Procedures

1.	Ketogenic Diet 05/01/2002 - "Efficacy of the ketogenic diet for infantile spasms." 09/01/2013 - "The aim of this study was to provide additional evidences on prognostic factors for infantile spasms and the possible role of a ketogenic diet. " 04/01/2011 - "To compare the prognoses between short-term (8 months) and conventional long-term (> 2 years) trials involving patients with refractory infantile spasms who successfully completed the ketogenic diet (KD). " 10/01/2006 - "This study sought to evaluate the efficacy, tolerability, and safety of a ketogenic diet (KD) in the treatment of infantile spasms (IS), incorporating a revised protocol based on our previous experience with KD. " 05/01/2002 - "The objective of this study was to determine whether the ketogenic diet is safe, well-tolerated, and efficacious in the treatment of infantile spasms. "
2.	Carbohydrate-Restricted Diet (Low Carbohydrate Diet) 01/01/2012 - "The modified Atkins diet was found to be effective and well tolerated in children with refractory infantile spasms (ClinicalTrials.gov identifier: NCT01006811)." 01/01/2012 - "Use of the modified Atkins diet in infantile spasms refractory to first-line treatment." 08/01/2015 - "Trial of Modified Atkins Diet for other cases of infantile spasm with similar SCN2A mutations is worthwhile pursuing." 01/01/2012 - "This prospective, open label, uncontrolled study was performed to evaluate the efficacy and tolerability of the modified Atkins diet in children with refractory infantile spasms. " 08/01/2015 - "SCN2A mutation in a Chinese boy with infantile spasm - response to Modified Atkins Diet."
3.	Hemispherectomy 11/01/1999 - "The postoperative development of nonverbal communication was studied in 29 children, aged 18.2 (SD = 11.54) months, who underwent multilobar resection or hemispherectomy for intractable symptomatic infantile spasms (IS). " 07/01/1993 - "Twenty-three infants and children underwent cortical resection (n = 15) or hemispherectomy (n = 8) for intractable infantile spasms. " 01/01/1994 - "Multilobar resection or hemispherectomy for patients with infantile spasms associated with CDLs and for patients with hemimegalencephaly are often associated with dramatic improvement in seizure control. " 12/01/2011 - "The outcome of hemispherectomy in pediatric patients is good for those with refractory epilepsies, such as West syndrome, Lennox-Gastaut syndrome, epileptic encephalopathy with continuous spikes and waves during slow sleep, and startle epilepsy arising from a hemispheric lesion associated with hemiplegia."
4.	Drug Therapy (Chemotherapy) 01/01/2013 - "All randomised controlled trials (RCTs) of the administration of drug therapy to patients with infantile spasms. " 01/01/2008 - "All randomised controlled trials of the administration of drug therapy to patients with infantile spasms. " 04/01/2014 - "The aim of this article is to review recent developments in the pharmacotherapy of infantile spasms and to highlight the practical implications of the latest research. " 04/01/2014 - "Recent advances in the pharmacotherapy of infantile spasms." 01/01/2005 - "Taking into consideration the psychokinetic retardation occurring in the West Syndrome because of prenatal, perinatal or postnatal disturbances apart from pharmacotherapy an intensive psychokinetic stimulation based on plasticity of the brain should be stressed out. "
5.	Prenatal Care 11/01/2001 - "The present study examined whether changes in the incidence of West syndrome (WS) could be used to evaluate changes in the quality of prenatal care over time. " 03/01/1995 - "Little can be done to reduce the incidence of infantile spasms, but every effort should be made to reduce the number of SGA infants by good prenatal care. "