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

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.