Background: Hypsarrhythmia is generally associated with infantile spasms, a combination referred to as West syndrome. It is debatable whether hypsarrhythmia is usefully regarded as a form of non-convulsive status epilepticus (NCSE).
Summary points: The earliest English language description of hypsarrhythmia reported an almost continuous EEG pattern, although later studies showed a degree of state dependence. Its principal features are very high amplitude and irregular slow waves with superimposed multifocal epileptiform discharges. Paroxysms of spasms are clearly overt seizure events, and there are variable EEG patterns associated with this ictus. There remains a debate about the definitional boundaries of hypsarrhythmia, and about the defining characteristics of NCSE. There is evidence that hypsarrhythmia is an age-dependent EEG pattern that evolves, sometimes independently of clinical features. Frequently, hypsarrhythmia is associated with delay in or regression of neuro-developmental skills, and recent studies have reported that a longer lead time to diagnosis and effective treatment is associated with poorer long-term neuro-developmental outcomes. Recent consensus definitions and classifications of NCSE have suggested boundaries that permit inclusion of hypsarrhythmia as an EEG pattern of NCSE. In practice, adopting the idea that hypsarrhythmia is a form of NCSE might lead to earlier appropriate investigation of infants with subtle developmental delay or regression, hence avoiding treatment delays and potentially preserving developmental potential.