Moyamoya syndrome: Impaired hemodynamics on ECD SPECT after EEG controlled hyperventilation

Summary

Background and purpose: Ischemic symptoms in children with Moyamoya syndrome are typically provoked by hyperventilation (HV) and are accompanied by the “re-build-up” phenomenon in EEG. The value of scintigraphic detection of HV-provoked perfusion deficits remains to be elucidated. Patients and methods: In seven children with Moyamoya syndrome regional cerebral blood flow was assessed by ^99mTc-ethyl-cysteine-dimer (ECD) single photon emission computed tomography (SPECT) after HV and under baseline conditions to identify ischemia prone regions. Results: Regional marked hypoperfusion after HV was found in all patients. Predominant perfusion deficits were detected in the frontal lobes. Conclusion: ECD SPECT is a potential tool for the preoperative evaluation of cerebral hemodynamics and for monitoring angiosurgical therapies in Moyamoya disease.

Zusammenfassung

Hintergrund und Ziel: Ischämische Symptome bei Kindern mit Moya-Moya-Erkrankung werden typischerweise durch Hyperventilation provoziert und vom Re-build-up-Phänomen im EEG begleitet. Patienten und Methoden: Bei sieben Kindern mit Moya-Moya-Syndrom wurde der regionale Blutfluss mit der ^99mTc-Ethylcysteinatdimer-( ECD-)Single-Photon-Emissionscomputertomographie (SPECT) nach Hyperventilation und unter Basisbedingungen bestimmt, um ischämiegefährdete Regionen zu identifizieren. Ergebnisse: Eine regional ausgeprägte Hypoperfusion nach Hyperventilation wurde bei allen Patienten gefunden. Vorwiegend kamen Perfusionsdefekte in den Frontallappen zur Darstellung. Schlussfolgerung: ECD-SPECT eignet sich potenziell für die präoperative Evaluation der zerebralen Hämodynamik und für die Verlaufskontrolle nach gefäßchirurgischer Therapie der Moya-Moya-Erkrankung.

Keywords

Moyamoya syndrome - ECD SPECT - EEG - Hyperventilation

Schlüsselwörter

Moya-Moya-Syndrom - ECD-SPECT - EEG - Hyperventilation

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