Sensory function in severe semilobar holoprosencephaly.

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

Neurocase

journal_title

Neurocase

authors

Liasis A,Hildebrand D,Clark C,Katz X,Gunny R,Stieltjes B,Taylor D

doi

10.1080/13554790802631936

subject

Has Abstract

pub_date

2009-01-01 00:00:00

pages

110-8

issue

2

eissn

1355-4794

issn

1465-3656

pii

907910127

journal_volume

15

pub_type

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