Visual function in infants with West syndrome: correlation with EEG patterns.
OBJECTIVE
Several studies have reported behavioral and electrophysiological evidence of visual impairment during the active stage of West syndrome. The underlying mechanisms are, however, poorly understood, and little has been reported about the correlation between visual impairment, EEG patterns, and brain lesions. The aim of the study was to assess visual function at the onset of spasm and 2 months thereafter and relate visual findings to brain lesions and EEG features.


METHODS
Twenty-five infants with West syndrome were enrolled and studied with (a) a full clinical assessment including a battery of tests specifically designed to assess visual function, (b) a video-polygraphic study, and (c) brain magnetic resonance imaging (MRI). Besides brain neuroimaging and EEG comparison with visual function, an intra-EEG analysis was performed to investigate the possible relation of EEG patterns to fluctuating visual behavior (fixation and following).


RESULTS
Twenty-two children had at least one abnormal result on one or more of the tests assessing visual function at T0. Visual impairment at the spasm onset was related to the sleep disorganization rather than to the hypsarrhythmic pattern in awake EEG. After 2 months, both EEG features become significantly linked to visual function. Visual function improved in several cases after 2 months, in parallel with the seizure regression. No relation was found between EEG patterns and fluctuating visual behavior.


CONCLUSIONS
The study supplies new evidence of the involvement of visual function in West syndrome. The presence of abnormal visual findings in infants without lesions on brain MRI suggests that visual abnormalities are due not only to brain injury but also to epileptic disorder per se. New insight is also provided into the possible mechanisms underlying clinical and EEG abnormalities.
Randò
Teresa
T
Division of Child Neurology and Psychiatry, Catholic University, Rome, Italy.
Bancale
Adina
A
Baranello
Giovanni
G
Bini
Margherita
M
De Belvis
Anton Giulio
AG
Epifanio
Roberta
R
Frisone
Maria Flavia
MF
Guzzetta
Andrea
A
La Torre
Giuseppe
G
Ricci
Daniela
D
Signorini
Sabrina
S
Tinelli
Francesca
F
Biagioni
Enrico
E
Veggiotti
Pierangelo
P
Mercuri
Eugenio
E
Fazzi
Elisa
E
Cioni
Giovanni
G
Guzzetta
Francesco
F
D001921:Brain / Q000503:physiopathology*; D001927:Brain Diseases / Q000175:diagnosis* / Q000503:physiopathology; D002540:Cerebral Cortex / Q000503:physiopathology; D004569:Electroencephalography / Q000706:statistics & numerical data*; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D012890:Sleep / Q000502:physiology; D013036:Spasms, Infantile / Q000175:diagnosis* / Q000503:physiopathology; D014741:Video Recording; D014786:Vision Disorders / Q000175:diagnosis* / Q000503:physiopathology; D014787:Vision Tests