Visual electrophysiological responses in subjects with cerebral autosomal arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)

Vincenzo Parisi, Francesco Pierelli, Alessandro Malandrini, Paola Carrera, Diego Olzi, Daniela Gregori, Rita Restuccia, Leoluca Parisi, Francesco Fattapposta

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish
Pages (from-to)1582-1588
Number of pages7
JournalClinical Neurophysiology
Volume111
Issue number9
DOIs
Publication statusPublished - Sep 1 2000

Keywords

  • CADASIL
  • Cerebral arteriopathy
  • Electroretinogram
  • Leukoencephalopathy
  • Smooth muscle cell
  • Visual evoked potential
  • Visual pathways

ASJC Scopus subject areas

  • Clinical Neurology
  • Radiology Nuclear Medicine and imaging
  • Neurology
  • Sensory Systems
  • Physiology (medical)

Fingerprint Dive into the research topics of 'Visual electrophysiological responses in subjects with cerebral autosomal arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)'. Together they form a unique fingerprint.

Cite this