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Recurrent leukoencephalopathy with microhemorrhages: gradient-echo MRI study diagnostic value in CNS primary angiitis

M Arias1, Xr Osorio, D Dapena

  • 1Department of Neurology, Complexo Hospitalario, Universitario, Santiago de Compostela, Spain. mariasg@meditex.es

Multiple Sclerosis (Houndmills, Basingstoke, England)
|August 30, 2008
PubMed

Insights

Diagnosing primary angiitis of the central nervous system (PACNS) is challenging. T2*-MRI findings of petechial hemorrhages may aid early PACNS diagnosis in patients with compatible clinical symptoms.

Area of Science:

  • Neurology
  • Neuroimaging
  • Immunology

Background:

  • Primary angiitis of the central nervous system (PACNS) presents diagnostic challenges due to varied clinical symptoms and nonspecific neuroimaging findings.
  • Early and accurate diagnosis is crucial for effective treatment and improved patient outcomes.

Observation:

  • A young patient exhibited a recurrent encephalopathic clinical course.
  • Initial MRI revealed white matter lesions suggestive of demyelination, which responded to immunotherapy (steroids and immunoglobulins).
  • Persistent cortical-subcortical petechial hypointense lesions (microhemorrhages) were noted on echo gradient MRI (T2*-MRI).

Findings:

  • Cerebral biopsy confirmed the diagnosis of PACNS.
  • Treatment with intravenous cyclophosphamide resulted in sustained remission for over 18 months.
  • T2*-weighted imaging (T2*-WI) revealed permanent microhemorrhages, a potentially key diagnostic indicator.

Implications:

  • The presence of petechial hemorrhages on T2*-WI may serve as an important early diagnostic marker for PACNS in patients with suggestive clinical presentations.
  • This finding could potentially streamline the diagnostic pathway, reducing delays in initiating appropriate immunosuppressive therapy.
  • Further research validating T2*-MRI for early PACNS detection is warranted.

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