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Rapidly progressive dementia due to leukocytoclastic vasculitis of the central nervous system

Carolina Pires1, Henda Foreid, Cândida Barroso

  • 1Department of Neurosciences, Hospital de Santa Maria, Lisbon, Portugal. mcarolinapires@gmail.com

BMJ Case Reports
|June 9, 2012
PubMed

Insights

A 70-year-old man experienced severe cognitive decline due to leukocytoclastic vasculitis, a rare form of primary central nervous system vasculitis. Treatment with steroids led to improvement, highlighting this condition

Area of Science:

  • Neurology
  • Pathology
  • Immunology

Background:

  • Primary angiitis of the central nervous system (PACNS) is a rare condition affecting blood vessels in the brain and spinal cord.
  • PACNS can manifest with diverse neurological symptoms and histopathological findings.
  • Understanding the spectrum of PACNS is crucial for accurate diagnosis and effective treatment.

Observation:

  • A 70-year-old male presented with a 2-week history of rapidly worsening cognitive impairment.
  • Brain MRI revealed extensive white matter abnormalities in the periventricular and subcortical regions, extending to the cortex and basal ganglia.
  • Cerebral angiography did not show any abnormalities.

Findings:

  • Stereotactic brain biopsy confirmed leukocytoclastic vasculitis, characterized by inflammation of small blood vessel walls.
  • Leukocytoclastic vasculitis was identified as the underlying cause of the patient's neurological symptoms.
  • The patient showed significant clinical improvement following treatment with corticosteroids.

Implications:

  • This case expands the known histopathological spectrum of primary angiitis of the central nervous system.
  • Leukocytoclastic vasculitis should be considered in the differential diagnosis of unexplained cognitive decline and white matter lesions.
  • Prompt diagnosis and immunosuppressive therapy, such as steroids, can lead to favorable outcomes in CNS vasculitis.

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