Infantile spasms

A seizure disorder of infancy and early childhood with the onset predominantly in the first year of life of myoclonic seizures, hypsarrhythmia (abnormal, chaotic electroencephalogram), and mental retardation. The spasms are sudden, brief contractions of one or more muscle groups, and may be followed by a longer (less than 10 seconds) tonic phase. Most often the spasms occur in clusters during which the intensity or the frequency of the spasms may increase progressively to a peak, decline, or cease. The clusters tend to occur soon after arousal from sleep. They are not a feature of falling asleep. The spasms usually involve the muscles of the neck, trunk, and extremities. Neurological abnormalities other than seizures and retardation — such as cerebral atrophy, congenital abnormalities and hydrocephalus — are commonly reported among children with infantile spasms. Some patients may be treated successfully with either ACTH (adrenocorticotrophic hormone) or prednisone. Newer antiepileptic medications, such as vigabatrin, and occasionally surgical resection of a seizure focus which triggers the spasms, may be useful in selected patients. Infantile spasms represent one of the most devastating seizure disorders affecting infants. Spontaneous cessation of spasms occurs in most patients with increasing age. Overall, however, the intellectual prognosis for patients is generally poor because a large number of these babies have neurological impairment prior to the onset of spasms. Many go on to have different types of seizures in later childhood. The disease is sometimes called West syndrome.

* * *

(salaam attacks)

a rare but serious form of epilepsy that usually begins between three and eight months of age. The spasms are involuntary flexing movements of the arms, legs, neck, and trunk; each spasm lasts 1-3 seconds and is associated with flushing of the face, and runs of spasms occur over a period of several minutes. They may occur many times in one day. The baby fails to respond to human contact and development is usually profoundly slowed. An EEG pattern of hypsarrhythmia is usual and diagnostic. Diagnosis may be delayed as spasms are sometimes confused with colic. Immediate recognition and treatment with antiepileptic medication, corticosteroids, or ACTH offers a chance of arresting the disease, but outcome depends primarily on the nature of the underlying brain abnormality.

* * *

infantile massive spasms a syndrome of severe myoclonus appearing in the first 18 months of life and associated with general cerebral deterioration; it is marked by severe flexion spasms of the head, neck, and trunk and extension of the limbs. Called also jackknife seizures or spasms;salaam convulsions, seizures, or spasms; and West syndrome.