Alessandra D'Amico1, Lorenzo Ugga1, Sirio Cocozza2, Sara Maria Delle Acque Giorgio1, Domenico Cicala3, Claudia Santoro4, Daniela Melis5, Giuseppe Cinalli3, Arturo Brunetti1, Sabina Pappatà6. 1. Department of Advanced Biomedical Sciences, University of Naples "Federico II", Via Pansini, 5, 80131, Naples, Italy. 2. Department of Advanced Biomedical Sciences, University of Naples "Federico II", Via Pansini, 5, 80131, Naples, Italy. sirio.cocozza@unina.it. 3. Department of Pediatric Neurosurgery, Santobono-Pausilipon Children's Hospital, Naples, Italy. 4. Referral Centre of Neurofibromatosis, Department of Woman and Child, Specialistic and General Surgery, University "Luigi Vanvitelli", Naples, Italy. 5. Department of Translational Medical Sciences, Section of Pediatrics, University of Naples "Federico II", Naples, Italy. 6. Institute of Biostructure and Bioimaging, National Research Council, Naples, Italy.
Abstract
PURPOSE: Moyamoya syndrome (MMS) is a rare intracranial arterial vasculopathy which can occur in neurofibromatosis type 1 (NF1) disease, representing a cause of cerebrovascular reserve (CVR) impairment, possibly leading to ischemic stroke. Here, we evaluated noninvasive imaging techniques used to assess CVR in MMS patients, describing clinical and imaging findings in patients affected by MMS-NF1. METHODS: Following strict inclusion and exclusion criteria, in this retrospective observational study, we evaluated imaging data of nine consecutive MMS-NF1 patients (M/F = 5/4, mean age: 12.6 ± 4.0). Subjects underwent a multimodal evaluation of cerebral vascular status, including intracranial arterial MR Angiography (MRA), MRI perfusion with dynamic susceptibility contrast (DSC) technique, and 99mTc-hexamethylpropyleneamine oxime (HMPAO) SPECT. RESULTS: In 8 out 9 patients (88.8%, 6/8 symptomatic), time-to-peak maps were correlated with the involved cerebral hemisphere, while in 6 out 9 patients (66.6%, 5/6 symptomatic), mean transit time (MTT) maps showed correspondence with the affected cerebrovascular territories. Cerebral blood flow (CBF) calculated using DSC perfusion failed to detect the hypoperfused regions instead identified by SPECT-CBF in all patients, while MTT maps overlapped with SPECT-CBF data in all cases and time-to-peak maps in 60.0%. CONCLUSIONS: Although SPECT imaging still represents the gold standard for CBF assessment, our results suggest that data obtained using DSC perfusion technique, and in particular MTT maps, might be a very useful and noninvasive tool for evaluating hemodynamic status in MMS-NF1 patients.
PURPOSE:Moyamoya syndrome (MMS) is a rare intracranial arterial vasculopathy which can occur in neurofibromatosis type 1 (NF1) disease, representing a cause of cerebrovascular reserve (CVR) impairment, possibly leading to ischemic stroke. Here, we evaluated noninvasive imaging techniques used to assess CVR in MMS patients, describing clinical and imaging findings in patients affected by MMS-NF1. METHODS: Following strict inclusion and exclusion criteria, in this retrospective observational study, we evaluated imaging data of nine consecutive MMS-NF1patients (M/F = 5/4, mean age: 12.6 ± 4.0). Subjects underwent a multimodal evaluation of cerebral vascular status, including intracranial arterial MR Angiography (MRA), MRI perfusion with dynamic susceptibility contrast (DSC) technique, and 99mTc-hexamethylpropyleneamine oxime (HMPAO) SPECT. RESULTS: In 8 out 9 patients (88.8%, 6/8 symptomatic), time-to-peak maps were correlated with the involved cerebral hemisphere, while in 6 out 9 patients (66.6%, 5/6 symptomatic), mean transit time (MTT) maps showed correspondence with the affected cerebrovascular territories. Cerebral blood flow (CBF) calculated using DSC perfusion failed to detect the hypoperfused regions instead identified by SPECT-CBF in all patients, while MTT maps overlapped with SPECT-CBF data in all cases and time-to-peak maps in 60.0%. CONCLUSIONS: Although SPECT imaging still represents the gold standard for CBF assessment, our results suggest that data obtained using DSC perfusion technique, and in particular MTT maps, might be a very useful and noninvasive tool for evaluating hemodynamic status in MMS-NF1patients.
Entities:
Keywords:
MRI; Moyamoya; Neurofibromatosis type 1; SPECT
Authors: Colin P Derdeyn; Gregory J Zipfel; Allyson R Zazulia; Patricia H Davis; Shyam Prabhakaran; Cristina S Ivan; Venkatesh Aiyagari; James R Sagar; Nancy Hantler; Lina Shinawi; John J Lee; Hussain Jafri; Robert L Grubb; J Philip Miller; Ralph G Dacey Journal: Stroke Date: 2017-03-10 Impact factor: 7.914