Abnormal fast activity in infancy with paroxysmal downwards gaze

Area of Science:

• Neurology
• Pediatrics
• Epileptology

Background:

• Paroxysmal downwards gaze (PDG) is an uncommon clinical sign in infants.
• The association between PDG, electroencephalogram (EEG) findings, and neurodevelopmental outcomes requires further elucidation.

Purpose of the Study:

• To investigate the clinical significance of PDG in infants.
• To explore the relationship between PDG, EEG abnormalities, and the subsequent development of West syndrome.

Main Methods:

• Case series of 8 infants presenting with PDG.
• Clinical observation and video-EEG monitoring.
• Analysis of EEG for interictal spikes and abnormal fast activity (AFA), particularly in the occipital region.

Main Results:

• PDG onset ranged from 1 to 5 months corrected age.
• 7/8 infants had interictal spikes on EEG; 5/8 showed occipital AFA.
• 5/8 infants with PDG and occipital AFA later developed West syndrome.
• PDG was differentiated from subtle epileptic seizures via video-EEG monitoring.

Conclusions:

• PDG, especially when accompanied by occipital AFA on EEG, may signify occipital region damage.
• PDG with occipital AFA is proposed as a potential risk factor for West syndrome development.
• Early identification of PDG with occipital AFA may aid in predicting West syndrome.