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Related Experiment Videos

White matter dementia in CADASIL.

C M Filley1, L L Thompson, C I Sze

  • 1Department of Neurology, UCHSC, and Denver Veterans Affairs Medical Center, CO 80262, USA.

Journal of the Neurological Sciences
|June 17, 1999
PubMed
Summary

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) can present as a prolonged neurobehavioral disorder. This case highlights CADASIL

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Area of Science:

  • Neurology
  • Neuroscience
  • Genetics

Background:

  • Cerebral white matter disorders are often linked to significant neurobehavioral deficits.
  • Understanding the diverse clinical manifestations of leukoencephalopathies is crucial for accurate diagnosis.

Observation:

  • A 62-year-old man presented with a 25-year history of progressive personality changes, psychosis, mood disorder, and dementia.
  • Neurologic examination revealed abulia, memory retrieval impairment, and preserved language with minimal motor deficits.
  • Neuropsychological testing indicated attention deficits, cognitive slowing, impaired learning, and perseveration.

Findings:

  • Brain MRI showed extensive leukoencephalopathy.
  • Brain biopsy revealed white matter pallor and arteriolar hyaline narrowing.

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  • Genetic analysis identified a NOTCH3 gene mutation, confirming cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL).

    • Implications:

    • CADASIL can manifest primarily as a long-term, isolated neurobehavioral disorder.
    • The dementia associated with CADASIL resembles other white matter dementias.
    • This case underscores the importance of considering CADASIL in the differential diagnosis of progressive white matter dementias.