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Infantile spasms (salaam seizures) are seizures characterized by sudden flexion of the arms, forward flexion of the trunk, extension of the legs, and hypsarrhythmia on EEG.
Infantile spasms last a few seconds and can recur many times a day. They usually manifest in children < 1 yr. Seizures may resolve spontaneously by about age 5 yr but are often replaced by other types of seizures.
Pathophysiology is unknown; however, infantile spasms may reflect abnormal interactions between the cortex and brain stem.
Usually, infantile spasms occur in infants with serious brain disorders and developmental abnormalities that often have already been recognized. These disorders may include injuries to the brain during the perinatal period, metabolic disorders, and brain malformations. Tuberous sclerosis (see Tuberous Sclerosis) is a common cause; prognosis is sometimes better when seizures are caused by this disorder than when seizures have other identifiable causes. Sometimes the cause cannot be identified.
Spasms begin with a sudden, rapid, tonic contraction of the trunk and limbs, sometimes for several seconds. Spasms range from subtle head nodding to contraction of the whole body. They involve flexion, extension, or, more often, both (mixed). The spasms usually occur in clusters, often several dozens, in close succession; they occur typically after children wake up and occasionally during sleep. Sometimes at first, they are mistaken for startles.
Developmental defects are usually present. In the first stages of the disorder, developmental regression can occur (eg, children may stop smiling or lose the ability to sit up or roll over).
Rate of premature death ranges from 5 to 31% and is related to the etiology of the infantile spasms.
Previous history (eg, neonatal hypoxic-ischemic encephalopathy) and/or symptoms and signs suggest the diagnosis in some children. Physical and neurologic examinations are done, but often no pathognomonic findings are identified except in tuberous sclerosis.
Waking and sleep EEG is done to confirm the diagnosis and check for specific abnormalities. Typically, the interictal pattern is hypsarrhythmic (chaotic, high-voltage polymorphic delta and theta waves with superimposed multifocal spike discharges). Multiple variations (eg, focal or asymmetric hypsarrhythmia) are possible. The ictal pattern is usually a sudden, marked and diffuse attenuation of electrical activity.
Neuroimaging, preferably MRI, is done if it has not already recently been done.
Tests to determine the cause if it is not clear from neuroimaging or the previous history may include
Infantile spasms are not responsive to typical anticonvulsants. The most effective treatment is ACTH. Both high-dose (150 IU/m 2 ) ACTH and low-dose (20 IU/m 2 ) ACTH, given daily IM, have been used, and evidence that the higher doses work better is not conclusive; however, generally, if low-dose therapy has not stopped spasms within 2 wk, higher doses are used. ACTH therapy is typically continued at the effective dose for 2 to 3 wk and then tapered off over 6 to 9 wk.
Vigabatrin is the only anticonvulsant with proven efficacy; it is the drug of choice when the spasms are caused by tuberous sclerosis and is often used in children with an established preexisting serious brain injury or malformation. Dosage of vigabatrin is 25 mg/kg po bid, increased gradually up to 75 mg/kg bid if needed. There is insufficient evidence that any other anticonvulsants and the ketogenic diet are effective.
Corticosteroids (eg, prednisone 2 mg/kg/day) is sometimes given for 4 to 7 wk as an alternative to ACTH.
In some patients with resistant spasms, focal cortical resection can eliminate seizures.
There is evidence that the more quickly effective therapy is initiated, the better the neurodevelopmental outcome, particularly when no cause is identified.
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