Abstract:
:We report a 4-year-old child with severe semi-lobar holoprosencephaly (HPE) not expected to survive after birth. Magnetic resonance imaging (MRI) revealed agenesis of the corpus callosum, absence of the third ventricle, fused thalami and basal ganglia. To investigate sensory function, visual, auditory and somatosensory evoked potential and imaging studies were carried out. The visual response evoked by human face stimuli evoked larger responses over the left side of the holosphere as compared to responses evoked by checkerboard pattern, while auditory evoked potentials were evident over the frontal regions to both pure tones and speech stimuli. No consistent scalp somatosensory evoked potentials were evident. This case demonstrates that electrophysiological measures are able to identify and quantify sensory processing not expected to be present based on the anatomical presentation of the cortex in a child with severe HPE.
journal_name
Neurocasejournal_title
Neurocaseauthors
Liasis A,Hildebrand D,Clark C,Katz X,Gunny R,Stieltjes B,Taylor Ddoi
10.1080/13554790802631936subject
Has Abstractpub_date
2009-01-01 00:00:00pages
110-8issue
2eissn
1355-4794issn
1465-3656pii
907910127journal_volume
15pub_type
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