Infantile Spasms Information Page

Infantile Spasms Information Page


What research is being done?

The NINDS supports broad and varied programs of research on epilepsy and other seizure disorders aimed at discovering new ways to prevent, diagnose, and treat these disorders.  Studies looking at the cellular, mlolecular, and genetic bases of epilepsy may lead to new therapies.  In one project, scientists are investigating whether insulin growth factor-1, which has been shown to have neuroprotective aspects in some studies, can reduce the serious side effects of vigabatrin in order to develop a new combination therapy for infantile epilepsy.
Epilepsy

×
What research is being done?

The NINDS supports broad and varied programs of research on epilepsy and other seizure disorders aimed at discovering new ways to prevent, diagnose, and treat these disorders.  Studies looking at the cellular, mlolecular, and genetic bases of epilepsy may lead to new therapies.  In one project, scientists are investigating whether insulin growth factor-1, which has been shown to have neuroprotective aspects in some studies, can reduce the serious side effects of vigabatrin in order to develop a new combination therapy for infantile epilepsy.
Epilepsy

The NINDS supports broad and varied programs of research on epilepsy and other seizure disorders aimed at discovering new ways to prevent, diagnose, and treat these disorders.  Studies looking at the cellular, mlolecular, and genetic bases of epilepsy may lead to new therapies.  In one project, scientists are investigating whether insulin growth factor-1, which has been shown to have neuroprotective aspects in some studies, can reduce the serious side effects of vigabatrin in order to develop a new combination therapy for infantile epilepsy.
Epilepsy


Definition
Definition
Treatment
Treatment
Prognosis
Prognosis
Clinical Trials
Clinical Trials
Organizations
Organizations
Publications
Publications
Definition
Definition

An epileptic spasm is a specific type of seizure seen in an epilepsy syndrome of infancy and childhood often called West Syndrome.  These are more commonly called infantile spasms (IS) since they are seen most often in the first year of life.  West Syndrome/IS is characterized by epileptic spasms, developmental problems, and a specific brain wave pattern on electroencephalography (EEG) testing called hypsarrhythmia.  The onset is usually in the first year of life, typically between 4-8 months.  The seizures often look like a sudden bending forward of the body with stiffening of the arms and legs lasting for 1-2 seconds; some children arch their backs as they extend their arms and legs.  Spasms tend to occur upon awakening and often occur in multiple clusters and hundreds of seizures per day.  Most children, but not all, will have EEG readings of  hypsarrhythmia.  Infantile spasms usually stop by age five, but may be replaced by other seizure types. Many underlying disorders, such as birth injury, metabolic disorders, and genetic disorders can give rise to IS, making it important to identify the underlying cause.  In some children, no cause can be found.

×
Definition

An epileptic spasm is a specific type of seizure seen in an epilepsy syndrome of infancy and childhood often called West Syndrome.  These are more commonly called infantile spasms (IS) since they are seen most often in the first year of life.  West Syndrome/IS is characterized by epileptic spasms, developmental problems, and a specific brain wave pattern on electroencephalography (EEG) testing called hypsarrhythmia.  The onset is usually in the first year of life, typically between 4-8 months.  The seizures often look like a sudden bending forward of the body with stiffening of the arms and legs lasting for 1-2 seconds; some children arch their backs as they extend their arms and legs.  Spasms tend to occur upon awakening and often occur in multiple clusters and hundreds of seizures per day.  Most children, but not all, will have EEG readings of  hypsarrhythmia.  Infantile spasms usually stop by age five, but may be replaced by other seizure types. Many underlying disorders, such as birth injury, metabolic disorders, and genetic disorders can give rise to IS, making it important to identify the underlying cause.  In some children, no cause can be found.

Treatment
Treatment

Standard first-line therapies for IS include several forms of hormonal therapy (including adrenocorticotrophic hormone [ACTH] or prednisolone) or the anti-seizure medication vigabatrin.  These treatments are high effective but have serious side effects and shold be administered in consulataton with a pediatric neurologist.   The goalof treatment for infantile spasms is for the seizures to stop and the EEG to improve (hypsarrhythmia should resolve).  Some children have spasms as the result of brain lesions, and surgical removal of these lesions may result in improvement.  When standard treatments do not lead to improvement, other options such as the ketogenic diet and anti-seizure medications are considered.  Regardless of the specific treatment chosen, it is critical to begin therapy as soon as possible.  Ongoing IS (and hypsarrhythmia) have the potential to adversely impact all aspects of brain development. 

×
Treatment

Standard first-line therapies for IS include several forms of hormonal therapy (including adrenocorticotrophic hormone [ACTH] or prednisolone) or the anti-seizure medication vigabatrin.  These treatments are high effective but have serious side effects and shold be administered in consulataton with a pediatric neurologist.   The goalof treatment for infantile spasms is for the seizures to stop and the EEG to improve (hypsarrhythmia should resolve).  Some children have spasms as the result of brain lesions, and surgical removal of these lesions may result in improvement.  When standard treatments do not lead to improvement, other options such as the ketogenic diet and anti-seizure medications are considered.  Regardless of the specific treatment chosen, it is critical to begin therapy as soon as possible.  Ongoing IS (and hypsarrhythmia) have the potential to adversely impact all aspects of brain development. 

Definition
Definition

An epileptic spasm is a specific type of seizure seen in an epilepsy syndrome of infancy and childhood often called West Syndrome.  These are more commonly called infantile spasms (IS) since they are seen most often in the first year of life.  West Syndrome/IS is characterized by epileptic spasms, developmental problems, and a specific brain wave pattern on electroencephalography (EEG) testing called hypsarrhythmia.  The onset is usually in the first year of life, typically between 4-8 months.  The seizures often look like a sudden bending forward of the body with stiffening of the arms and legs lasting for 1-2 seconds; some children arch their backs as they extend their arms and legs.  Spasms tend to occur upon awakening and often occur in multiple clusters and hundreds of seizures per day.  Most children, but not all, will have EEG readings of  hypsarrhythmia.  Infantile spasms usually stop by age five, but may be replaced by other seizure types. Many underlying disorders, such as birth injury, metabolic disorders, and genetic disorders can give rise to IS, making it important to identify the underlying cause.  In some children, no cause can be found.

Treatment
Treatment

Standard first-line therapies for IS include several forms of hormonal therapy (including adrenocorticotrophic hormone [ACTH] or prednisolone) or the anti-seizure medication vigabatrin.  These treatments are high effective but have serious side effects and shold be administered in consulataton with a pediatric neurologist.   The goalof treatment for infantile spasms is for the seizures to stop and the EEG to improve (hypsarrhythmia should resolve).  Some children have spasms as the result of brain lesions, and surgical removal of these lesions may result in improvement.  When standard treatments do not lead to improvement, other options such as the ketogenic diet and anti-seizure medications are considered.  Regardless of the specific treatment chosen, it is critical to begin therapy as soon as possible.  Ongoing IS (and hypsarrhythmia) have the potential to adversely impact all aspects of brain development. 

Prognosis
Prognosis

The prognosis for children with IS is largely dependent on the underlying cause.  The intellectual prognosis for children with IS is generally poor because many babies with IS have neurological impairment prior to the onset of spasms.   Children who have rapid initiation of treatment, normal development prior to infantile spasms, and no identifiable cause may do well.  IS usually resolves by mid-childhood, but more than half of the children with IS will develop other types of seizures such as Lennox-Gastaut syndrome, an epileptic disorder of later childhood.  In addition, children with IS are at a higher risk for autism.  Shorter duration between the onset of IS and initiation of standard treatment appears to lead to an improved outcome; therefore early recognition of the seizures and early treatment are essential.

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The prognosis for children with IS is largely dependent on the underlying cause.  The intellectual prognosis for children with IS is generally poor because many babies with IS have neurological impairment prior to the onset of spasms.   Children who have rapid initiation of treatment, normal development prior to infantile spasms, and no identifiable cause may do well.  IS usually resolves by mid-childhood, but more than half of the children with IS will develop other types of seizures such as Lennox-Gastaut syndrome, an epileptic disorder of later childhood.  In addition, children with IS are at a higher risk for autism.  Shorter duration between the onset of IS and initiation of standard treatment appears to lead to an improved outcome; therefore early recognition of the seizures and early treatment are essential.

Prognosis
Prognosis

The prognosis for children with IS is largely dependent on the underlying cause.  The intellectual prognosis for children with IS is generally poor because many babies with IS have neurological impairment prior to the onset of spasms.   Children who have rapid initiation of treatment, normal development prior to infantile spasms, and no identifiable cause may do well.  IS usually resolves by mid-childhood, but more than half of the children with IS will develop other types of seizures such as Lennox-Gastaut syndrome, an epileptic disorder of later childhood.  In addition, children with IS are at a higher risk for autism.  Shorter duration between the onset of IS and initiation of standard treatment appears to lead to an improved outcome; therefore early recognition of the seizures and early treatment are essential.

Definition

An epileptic spasm is a specific type of seizure seen in an epilepsy syndrome of infancy and childhood often called West Syndrome.  These are more commonly called infantile spasms (IS) since they are seen most often in the first year of life.  West Syndrome/IS is characterized by epileptic spasms, developmental problems, and a specific brain wave pattern on electroencephalography (EEG) testing called hypsarrhythmia.  The onset is usually in the first year of life, typically between 4-8 months.  The seizures often look like a sudden bending forward of the body with stiffening of the arms and legs lasting for 1-2 seconds; some children arch their backs as they extend their arms and legs.  Spasms tend to occur upon awakening and often occur in multiple clusters and hundreds of seizures per day.  Most children, but not all, will have EEG readings of  hypsarrhythmia.  Infantile spasms usually stop by age five, but may be replaced by other seizure types. Many underlying disorders, such as birth injury, metabolic disorders, and genetic disorders can give rise to IS, making it important to identify the underlying cause.  In some children, no cause can be found.

Treatment

Standard first-line therapies for IS include several forms of hormonal therapy (including adrenocorticotrophic hormone [ACTH] or prednisolone) or the anti-seizure medication vigabatrin.  These treatments are high effective but have serious side effects and shold be administered in consulataton with a pediatric neurologist.   The goalof treatment for infantile spasms is for the seizures to stop and the EEG to improve (hypsarrhythmia should resolve).  Some children have spasms as the result of brain lesions, and surgical removal of these lesions may result in improvement.  When standard treatments do not lead to improvement, other options such as the ketogenic diet and anti-seizure medications are considered.  Regardless of the specific treatment chosen, it is critical to begin therapy as soon as possible.  Ongoing IS (and hypsarrhythmia) have the potential to adversely impact all aspects of brain development. 

Prognosis

The prognosis for children with IS is largely dependent on the underlying cause.  The intellectual prognosis for children with IS is generally poor because many babies with IS have neurological impairment prior to the onset of spasms.   Children who have rapid initiation of treatment, normal development prior to infantile spasms, and no identifiable cause may do well.  IS usually resolves by mid-childhood, but more than half of the children with IS will develop other types of seizures such as Lennox-Gastaut syndrome, an epileptic disorder of later childhood.  In addition, children with IS are at a higher risk for autism.  Shorter duration between the onset of IS and initiation of standard treatment appears to lead to an improved outcome; therefore early recognition of the seizures and early treatment are essential.

What research is being done?

The NINDS supports broad and varied programs of research on epilepsy and other seizure disorders aimed at discovering new ways to prevent, diagnose, and treat these disorders.  Studies looking at the cellular, mlolecular, and genetic bases of epilepsy may lead to new therapies.  In one project, scientists are investigating whether insulin growth factor-1, which has been shown to have neuroprotective aspects in some studies, can reduce the serious side effects of vigabatrin in order to develop a new combination therapy for infantile epilepsy.
Epilepsy

Patient Organizations
Child Neurology Foundation
201 Chicago Avenue, Suite 200
Minneapolis
MN
Minneapolis, MN 55415
Tel: 612-928-6325
Epilepsy Foundation
8301 Professional Place East, Suite 200
Landover
MD
Landover, MD 20785-2353
Tel: 301-459-3700; 800-EFA-1000 (332-1000)
Intractable Childhood Epilepsy Alliance
PO Box 365
6360 Shallowford Road
Lewisville
NC
Lewisville, NC 27023
Tel: 336-946-1570
National Organization for Rare Disorders (NORD)
55 Kenosia Avenue
Danbury
CT
Danbury, CT 06810
Tel: 203-744-0100; Voice Mail: 800-999-NORD (6673)
Publications

Information booklet on seizures, seizure disorders, and epilepsy compiled by the National Institute of Neurological Disorders and Stroke (NINDS).

Myoclonus fact sheet compiled by the National Institute of Neurological Disorders and Stroke (NINDS).

Patient Organizations