Differential Diagnosis and Related Syndromes

Abstract

The ictal events (i.e., epileptic spasms) that are typically associated with West syndrome have characteristic features that, if recognized, usually permit this disorder to be identified with a reasonable degree of certainty by the physician (see Chapter 4). However, the short duration of the events, their often sporadic occurrence, and their sometimes subtle motor expression not uncommonly make it very difficult, or even impossible, for observers to accurately determine such features. Consequently, parents, and other observers, including physicians, not infrequently confuse these ictal behaviors with other phenomena including certain normal movements, particular abnormal behaviors associated with other medical disorders, and other types of epilepsy. In one large study, a correct diagnosis of infantile spasms was made at the time of the original medical evaluation in only 11% of the cases (Bellman, 1983). In fact, the correct diagnosis of infantile spasms is sometimes not made for some time, perhaps months, after onset of the disorder. In contrast, the occurrence of normal events, or certain more benign medical conditions, may falsely be identified as infantile spasms, possibly leading to unwarranted medical procedures and/or treatment. In this chapter, the differential diagnosis of infantile spasms is considered, and the clinical features which best differentiate this disorder are discussed. Knowledge of the features which best delineate these other conditions will often permit the early clinical diagnosis of infantile spasms, although in many cases it will be necessary to use EEG/video monitoring techniques in order to obtain definitive information.