TY - JOUR
T1 - Visual electrophysiological responses in subjects with cerebral autosomal arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)
AU - Parisi, Vincenzo
AU - Pierelli, Francesco
AU - Malandrini, Alessandro
AU - Carrera, Paola
AU - Olzi, Diego
AU - Gregori, Daniela
AU - Restuccia, Rita
AU - Parisi, Leoluca
AU - Fattapposta, Francesco
PY - 2000/9/1
Y1 - 2000/9/1
N2 - Objectives: To evaluate visual electrophysiological responses in subjects with cerebral autosomal arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). Methods: Three subjects (one male and two females, mean age 55.3 ± 2.9 years) belonging to an Italian family already diagnosed with CADASIL through clinicopathological and genetic studies and 14 control subjects (6 males and 8 females, mean age 52.7 ± 3.6 years) were enrolled in the study. Flash electroretinogram (ERG), oscillatory potentials (OPs) and simultaneous recordings of pattern electroretinogram (PERG) and visual evoked potentials (VEPs) were assessed in all 3 subjects with CADASIL and age-matched controls. Results: Subjects with CADASIL showed: reduced ERG, OP and PERG (N35-P50, P50-N95) amplitudes with respect to our normal limits; delayed PERG (N35, P50) and VEP (P100) implicit times when compared with our normal limits; and VEP (N75-P100) amplitudes and retinocortical times within our normal limits. Conclusions: Subjects with CADASIL present a dysfunction in the outer, middle and innermost retinal layers when the index of neural conduction in the postretinal visual pathways is normal. The delay in visual cortical responses observed in subjects with CADASIL may be ascribable to retinal impairment with a possible functional sparing of the postretinal visual structures. (C) 2000 Elsevier Science Ireland Ltd.
AB - Objectives: To evaluate visual electrophysiological responses in subjects with cerebral autosomal arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). Methods: Three subjects (one male and two females, mean age 55.3 ± 2.9 years) belonging to an Italian family already diagnosed with CADASIL through clinicopathological and genetic studies and 14 control subjects (6 males and 8 females, mean age 52.7 ± 3.6 years) were enrolled in the study. Flash electroretinogram (ERG), oscillatory potentials (OPs) and simultaneous recordings of pattern electroretinogram (PERG) and visual evoked potentials (VEPs) were assessed in all 3 subjects with CADASIL and age-matched controls. Results: Subjects with CADASIL showed: reduced ERG, OP and PERG (N35-P50, P50-N95) amplitudes with respect to our normal limits; delayed PERG (N35, P50) and VEP (P100) implicit times when compared with our normal limits; and VEP (N75-P100) amplitudes and retinocortical times within our normal limits. Conclusions: Subjects with CADASIL present a dysfunction in the outer, middle and innermost retinal layers when the index of neural conduction in the postretinal visual pathways is normal. The delay in visual cortical responses observed in subjects with CADASIL may be ascribable to retinal impairment with a possible functional sparing of the postretinal visual structures. (C) 2000 Elsevier Science Ireland Ltd.
KW - CADASIL
KW - Cerebral arteriopathy
KW - Electroretinogram
KW - Leukoencephalopathy
KW - Smooth muscle cell
KW - Visual evoked potential
KW - Visual pathways
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U2 - 10.1016/S1388-2457(00)00366-7
DO - 10.1016/S1388-2457(00)00366-7
M3 - Article
C2 - 10964068
AN - SCOPUS:0034284321
VL - 111
SP - 1582
EP - 1588
JO - Clinical Neurophysiology
JF - Clinical Neurophysiology
SN - 1388-2457
IS - 9
ER -