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Atypical Young-onset Dementia in Cerebral Thromboangiitis Obliterans: A Case Report

Eun-Joo Kim1, Na-Yeon Jung2, Myung Jun Lee1

  • 1Department of Neurology, Pusan National University Hospital, Pusan National University School of Medicine and Medical Research Institute.

Alzheimer Disease and Associated Disorders
|October 1, 2021
PubMed

Insights

Cerebral thromboangiitis obliterans (CTAO) can present as a rare cause of young-onset dementia (YOD). Postmortem findings confirmed CTAO in a 41-year-old man with atypical YOD symptoms.

Area of Science:

  • Neurology
  • Pathology

Background:

  • Young-onset dementia (YOD) presents before age 45 and has a wide range of potential causes.
  • Cerebral thromboangiitis obliterans (CTAO) is a rare condition that can lead to vascular dementia.

Observation:

  • A 41-year-old man exhibited atypical symptoms suggestive of YOD.
  • Comprehensive antemortem investigations, including clinical, laboratory, neuroimaging, and genetic assessments, were inconclusive.
  • Postmortem neuropathological examination revealed cortical sickle-shaped granular atrophy due to remote infarcts and microinfarcts, primarily in the frontal and parietal lobes.

Findings:

  • The neuropathological findings confirmed a diagnosis of cerebral thromboangiitis obliterans (CTAO).
  • CTAO was identified as the underlying cause of vascular dementia in this YOD case.

Implications:

  • CTAO should be considered in the differential diagnosis of YOD, particularly in patients with a history of heavy smoking.
  • Symmetric watershed-territory damage on neuroimaging may suggest CTAO in YOD patients.
  • This case highlights the importance of considering rare vascular etiologies in unexplained YOD.

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