Related Experiment Videos
Infarctions and non-invasive diagnosis in moyamoya disease: two case reports
C Weiller1, W Müllges, M Leibold
1Department of Neurology, Neuroradiology, Rhenish Westphalian Technical University Aachen, Fed. Rep. of Germany.
Insights
Adult Moyamoya disease (MMD) involves arterial stenosis and reduced blood flow, leading to potential infarctions. Noninvasive imaging like TCD and MRI aids in diagnosing MMD and understanding its hemodynamic effects.
Area of Science:
- Neurology
- Radiology
- Vascular Medicine
Background:
- Moyamoya disease (MMD) is a rare cerebrovascular disorder characterized by progressive stenosis of the internal carotid arteries and the circle of Willis.
- Adult MMD presents with diverse neurological symptoms, often including ischemic or hemorrhagic strokes.
Observation:
- Two adult patients (31-year-old female, 32-year-old male) diagnosed with Moyamoya disease (MMD) exhibited bilateral carotid siphon (C1), middle cerebral artery (M1), and anterior cerebral artery (A1) stenoses.
- Transcranial Doppler sonography (TCD) and angio Magnetic Resonance Imaging (angio-MRI) confirmed stenoses. Proton and T1-weighted MRI sequences visualized a 'rete mirabile' of dilated lenticulostriate arteries in the female patient.
- CO2-dependent vasomotor reactivity was reduced bilaterally and exhausted in the right middle cerebral artery territory in both patients. Single-photon emission computed tomography (SPECT) revealed significantly reduced cerebral perfusion reserve in the anterior region of the male patient.
Findings:
- Both patients presented with bilateral small subcortical infarctions in the corona radiata.
- These infarctions are suggested to be hemodynamically induced, low-flow infarctions, consistent with compromised cerebral perfusion.
- Reduced CO2-dependent vasomotor reactivity and diminished cerebral perfusion reserve highlight the critical hemodynamic impairment in these MMD patients.
Implications:
- Transcranial Doppler sonography (TCD) and Magnetic Resonance Imaging (MRI) are valuable noninvasive tools for the preliminary diagnosis of Moyamoya disease.
- Understanding the hemodynamic consequences, such as reduced cerebral perfusion reserve and vasomotor reactivity, is crucial for managing MMD.
- The findings underscore the potential for hemodynamically driven, low-flow infarctions in adult Moyamoya disease, emphasizing the need for early diagnosis and intervention.
Abstract:
An 31-year-old female and a 32-year-old male had clinical signs and angiographical confirmation of adult Moyamoya disease (MMD). Bilateral carotid siphon (C1), middle cerebral artery (M1), and anterior cerebral artery (A1) stenoses were diagnosed by means of transcranial Doppler sonography (TCD) and visualized during angio Magnetic Resonance Imaging (angio-MRI). In the woman, a 'rete mirabile' of dilated and tortuous lenticulostriate arteries could be visualized during proton weighted and T1-weighted MRI sequences. CO2-dependent vasomotor reactivity was bilaterally reduced and completely exhausted in the territory of right middle cerebral artery in both patients. Cerebral perfusion reserve, defined as the ratio of cerebral blood flow (CBF) to cerebral blood volume (CBV) was assessed by SPECT, and was found to be dramatically reduced in the anterior region of the male patient. Both patients had bilateral small subcortical infarctions in the corona radiata. TCD and MRI are important noninvasive techniques for a preliminary diagnosis of Moyamoya disease. Infarctions in Moyamoya disease may be hemodynamically produced low-flow infarctions.