Delirium as presentation of late-onset and relapsing Susac syndrome.

Betend, Romain; Humm, Andrea Monika; Medlin, Friedrich (2017). Delirium as presentation of late-onset and relapsing Susac syndrome. BMJ case reports, 2017 BMJ Publishing Group 10.1136/bcr-2017-220494

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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.

Item Type:

Journal Article (Further Contribution)


04 Faculty of Medicine > Department of Head Organs and Neurology (DKNS) > Clinic of Neurology

UniBE Contributor:

Humm, Andrea Monika


600 Technology > 610 Medicine & health




BMJ Publishing Group




Stefanie Hetzenecker

Date Deposited:

19 Feb 2018 14:09

Last Modified:

19 Feb 2018 14:09

Publisher DOI:


PubMed ID:


Uncontrolled Keywords:

Delirium Immunology Neurology Retina


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