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Delirium as presentation of late-onset and relapsing Susac syndrome
  1. Romain Betend1,
  2. Andrea M Humm1,2,
  3. Friedrich Medlin1,3
  1. 1Department of Internal Medicine, Unit of Neurology, HFR Fribourg Hopital Cantonal, Fribourg, Switzerland
  2. 2Department of Neurology, Inselspital Universitatsspital Bern, Bern, Switzerland
  3. 3Department of Clinical Neurosciences, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland
  1. Correspondence to Dr Romain Betend, romain_betend{at}


A 67-year-old patient, only known for bilateral presbycusis, presented with subacute onset of delirium. Clinical examination showed multifocal neurological deficits, all together suggesting subcortical frontal dysfunction together with cerebellar and corpus callosum involvement.

Cerebral MRI demonstrated supratentorial and infratentorial subcortical and periventricular T2-hyperintense lesions with cerebellar gadolinium enhancement and multiple central lesions of the corpus callosum (snowball lesions). The diagnosis of Susac syndrome was made and the patient treated with intravenous methylprednisolone, followed by a prednisone maintenance dose over 8 weeks. After a clinical improvement, a relapse was noticed during corticosteroid tapering. The patient was again treated with intravenous methylprednisolone followed by a prednisone maintenance therapy with simultaneous introduction of mycophenolate mofetil acid and one cycle of intravenous rituximab. The patient recovered rapidly. At 11-month follow-up, only mild executive dysfunction and persistent right postural tremor was noted, MRI showed partial regression of subcortical and juxtacortical lesions.

  • Neurology
  • Immunology
  • Retina
  • Delirium
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  • Contributors RB: Study concept and design, analysis and interpretation of data, drafting of the manuscript. AMH: Critical revision of the manuscript. FM: Study concept and design, data collection and interpretation, drafting and critical revision of the manuscript, supervised the study.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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