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Evaluation of the Cognitive Performance of Hypertensive Patients with Silent Cerebrovascular Lesions
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Cognitive dysfunction and brain atrophy in Susac syndrome.

Stéphanie Machado1,2, Eric Jouvent1,2,3, Isabelle Klein4

  • 1Department of Neurology, APHP, Lariboisière Hospital, F-75475, Paris, France.

Journal of Neurology
|December 13, 2019
PubMed
Summary

Susac syndrome causes cognitive impairment, particularly affecting processing speed. Despite significant brain and corpus callosum atrophy, this study found no direct link to cognitive changes, raising questions about underlying mechanisms.

Keywords:
Brain atrophyCorpus callosumMild cognitive impairmentProcessing speed slowingSusac syndrome

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Area of Science:

  • Neuroscience
  • Neurology
  • Cerebral Small Vessel Disease

Background:

  • Susac syndrome is a rare cerebral small vessel disease.
  • It can lead to significant cognitive impairment in patients.
  • This study investigates cognitive deficits and brain atrophy in Susac syndrome.

Purpose of the Study:

  • To evaluate processing speed, executive dysfunction, and apathy in Susac syndrome patients.
  • To assess the relationship between cognitive alterations and whole brain and callosal atrophy.
  • To understand the progression of cognitive and volumetric changes over time.

Main Methods:

  • Prospective observational cohort study of clinically stable Susac syndrome patients.
  • Standardized brain MRI and a specialized neuropsychological battery were used.
  • Brain volume and corpus callosum area were measured using 3D-T1 sequences and analyzed with linear regression.

Main Results:

  • Nineteen patients (mean age 37.5 years) were followed for 2.6 years.
  • Significant processing speed slowing was observed, despite largely preserved global cognitive function (MoCA scores).
  • Striking brain and corpus callosum atrophy was evident, but no correlation was found with cognitive performance.

Conclusions:

  • Susac syndrome patients exhibit notable processing speed deficits.
  • Prominent and evolving brain and corpus callosum atrophy does not correlate with observed cognitive alterations.
  • The mechanisms driving cognitive changes in Susac syndrome require further investigation.