Abstract
Infantile spasms remain the most challenging of the epileptic encephalopathies of
childhood. Infantile spasms are classified as an epileptic encephalopathy, as the
adverse cognitive and behavioral burden of the condition is out of proportion to the
burden one would expect from the underlying etiology or the accompanying magnetic
resonance imaging. The ictal and interictal electroencephalographic (EEG) activity
is presumed to contribute to the progressive cerebral dysfunction. In many of these
children, the underlying etiology also contributes to the severe mental subnormality
and autistic behavior. Though it is the syndromic approach that guides the pediatric
epileptologist, it is best to keep in mind that one syndrome may evolve into another
in infancy and early childhood. A baby with Ohtahara syndrome may, after 2 to 7 months,
begin to have spasms. Lennox-Gastaut syndrome with its typical seizure types and EEG
may evolve in a child with infantile spasms.
The unique modalities used in the treatment of infantile spasms make early recognition
important. It is, however, also of paramount importance to make an etiological diagnosis
as the underlying etiology may be eminently treatable. The treating physician cannot
abandon them as wholly “intractable” epilepsy. The excellent response to treatment
in the few who just cannot be defined or accurately predicted drives the physician
to exercise his brain. Use of the two well-accepted modalities of treatment; vigabatrin
and adrenocorticotrophic hormone singly or in combination, oral steroids in high dose,
ketogenic diet, the conventional antiepileptic medications, and strategies to target
the basic cause have been tried out by various clinicians. Here, we have made an attempt
to collate evidence and describe the progress in the management of infantile spasms.
Keywords
infantile spasms - adrenocorticotrophic hormone - vigabatrin
