Download Infantile Spasms: Fact Sheet1

PRACTICE AID
Infantile Spasms: Fact Sheet1
This Practice Aid has been provided as a quick reference to help learners apply the information to their daily
practice and care of patients. It is part of the activity “Infantile Spasms (West Syndrome): What Is the Current
State of Identification, Diagnosis, and Management?” which can be found in its entirety at
www.peerviewpress.com/y/r338
Characteristic Features of Infantile Spasms (West Syndrome): Triad
Epileptic spasms
Characteristic
Features of
Infantile Spasms
Hypsarrhythmic
EEGs
Mental
retardation
Alternative Names for West Syndrome
Massive spasms
Salaam seizures
Flexion spasms
Jackknife seizures
Massive myoclonic jerks
Infantile myoclonic seizures
Blitz-Nick-Salaam Krämpfe (German)
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PRACTICE AID
Infantile Spasms: Fact Sheet1
(Cont'd)
Classification of Infantile Spasms
Description
Type
Flexor
Flexion of the neck, trunk, arms, and legs; spasms of the muscles of
the upper limbs resulting in either adduction of the arms or a
self-hugging motion, or adduction of the arms to either side of the
head with the arms flexed at the elbow
Extensor
A predominance of extensor muscle contractions, which produce
abrupt extension of the neck and trunk, along with extensor
abduction or adduction of the arms, legs, or both
Mixed flexorextensor
Flexion of the neck, trunk, and arms and extension of the legs, or
flexion of the legs and extension of the arms with varying degrees
of flexion of the neck and trunk
Asymmetric
Occasionally occur, in which the infant maintains a “fencing” posture
Infantile spasms may also be associated with autonomic dysfunction:
Lacrimation
Flushing
Pupillary
dilation
Autonomic
Dysfunction
Sweating
Changes in respiratory
Pallor
and heart rate
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PRACTICE AID
Infantile Spasms: Fact Sheet1
(Cont'd)
Clinical Presentation
•
Infantile spasms are very brief, and the casual observer may miss single
seizures.
•
Infantile spasms frequently occur in clusters.
•
The number of seizures per cluster varies considerably. Some clusters have
as many as 150 seizures. The number of clusters per day also varies. Some
patients have as many as 60 clusters per day. Clusters can occur during sleep
or shortly after awakening. Crying or irritability during or after a flurry of
spasms is commonly observed. Infantile spasms are frequently associated
with developmental delay or regression, but this delay may not be present
at onset.
•
Infantile spasms are commonly associated with strikingly abnormal EEGs.
The most common interictal abnormality is hypsarrhythmia.
EEGs: electroencephalograms.
1. Infantile Spasms. http://www.epilepsy.com/information/professionals/about-epilepsy-seizures/overview-epilepsy-syndromes/infantile-spasms. Accessed July 17, 2014.
Material presented within this CME-certified activity is supported by independent educational grants subject to written agreements. No endorsement of approved/unapproved products or uses is
made or implied by coverage of these products or uses in this activity. No responsibility is taken for errors or omissions. The information presented is solely intended for general education purposes,
and it is not intended to discuss any specific product.
Download a copy of this Practice Aid at: www.peerviewpress.com/y/r338
PRACTICE AID
Treating Infantile Spasms
This Practice Aid has been provided as a quick reference to help learners apply the information to their daily
practice and care of patients. It is part of the activity “Infantile Spasms (West Syndrome): What Is the Current
State of Identification, Diagnosis, and Management?” which can be found in its entirety at
www.peerviewpress.com/y/r338.
FDA-Approved Treatment Options
Agent
Description
Dosage/Administration
ACTH1
Repository
corticotropin
injection
80 U/mL
150 U/m2 divided into
twice-daily intramuscular
injections of 75 U/m2; after
2 weeks of treatment,
dosing should be gradually
tapered and discontinued
over a 2-week period
Vigabatrin2
Antiepileptic Initiate therapy at
drug
50 mg/kg/day orally given
in two divided doses,
increasing total daily
dose per instructions
to a maximum of
150 mg/kg/day given
in two divided doses
Potential AEs
•
•
•
•
•
•
•
•
•
•
•
•
•
•
AEs similar to those associated
with corticosteroids
Increased risk of infection
Cushingoid features
Cardiac hypertrophy
Hypertension
Irritability
Bilateral concentric visual field
constriction
Sedation/somnolence
Irritability
Insomnia
Constipation
Lethargy
Decreased appetite
Hypotonia
Other Steroid Therapies
Prednisolone
Dexamethasone
Methylprednisolone
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PRACTICE AID
Treating Infantile Spasms
(Cont'd)
Other Treatmentsa
Anti-seizure medications
•
•
•
•
•
•
Levetiracetam
Nitrazepam
Sulthiame
Topiramate
Valproic acid
Zonisamide
Combination therapies
•
•
•
•
ACTH + hydrocortisone
ACTH + magnesium sulfate
ACTH + valproic acid
ACTH + vigabatrin
Novel therapies
•
•
IV immunoglobulin
Thyrotropin-releasing hormone
Ketogenic diet
Vitamin B6
a
There is not enough evidence to know if these treatments help treat infantile spasms.
ACTH: adrenocorticotropic hormone.
1. H.P. Acthar Gel (repository corticotropin injection) Prescribing Information. http://www.acthar.com/pdf/Acthar-PI.pdf. Accessed July 17, 2014.
2. Sabril (vigabatrin) Prescribing Information. http://www.lundbeck.com/upload/us/files/pdf/Products/Sabril_PI_US_EN.pdf. Accessed July 17, 2014.
Material presented within this CME-certified activity is supported by independent educational grants subject to written agreements. No endorsement of approved/unapproved products or uses is
made or implied by coverage of these products or uses in this activity. No responsibility is taken for errors or omissions. The information presented is solely intended for general education purposes,
and it is not intended to discuss any specific product.
Download a copy of this Practice Aid at: www.peerviewpress.com/y/r338