Hypsarrhythmia paroxysm intensities that initiate and render physical and mental retardation irreversible in West syndrome.

The present study would like to investigate the effect of hypsarrhythmia paroxysms on neurological examination findings. This study enrolled 48 children with normal cranial magnetic resonance imaging (cMRI) findings who were previously untreated with adrenocorticotropic hormone (ACTH) and had no history of asphyxia or anoxia at birth, no underlying disease, and no history of head trauma or central nervous system infection. In these children, duration of treatment delay (DTD) was calculated, HPs in NREM sleep were counted, and neurological examination findings were identified. During the study, sometimes the 'countable hypsarrhythmia paroxysms index' (cHPI) and sometimes the 'durational hypsarrhythmia paroxysm index (dHPI)' was estimated. The onset of neurological examination findings, the onset of physical and mental retardation and the time when physical and mental retardation became irreversible were investigated. The children were stratified into 5 groups based on DTD and attempts were made to prevent the recurrence of aborted ISs by ACTH treatment. I- When 'cHPI = 4/min'; mild growth retardation may be noticed. II- When 'cHPI = 6/min' and 'dHPI = 25%'; reduced eye tracking and reduced movement may be observed. III- When 'cHPI = 8/min' and 'dHPI >32%'; reduced eye tracking and reduced movement become evident, and neurological examination findings are reversible. IV- When 'dHPI = 45%'; partially permanent, mild motor and psychiatric sequelae develop. V- When 'dHPI >49%'; neurological examination findings become irreversible. VI- No relationship was found between neurological examination findings the early onset of ISs. WS cannot be treated unless DCHP is elucidated.

Citation

Attila Altunel, Aynur Muduroglu-Kirmizibekmez, Ozlem Altunel, Ali Sever, Ihsan Kara. Hypsarrhythmia paroxysm intensities that initiate and render physical and mental retardation irreversible in West syndrome. The International journal of neuroscience. 2022 May 24:1-9

PMID: 35574641

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