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Migrainous Infarction in a Patient With Sporadic Hemiplegic Migraine and Cystic Fibrosis: A 99mTc-HMPAO Brain SPECT Study |
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| Authors: | Valentina Mancini MD Giulio Mastria MD Viviana Frantellizzi MD Patrizia Troiani MD PhD Stefania Zampatti MD Stefania Carboni MSc Emiliano Giardina MSc PhD Rosa Campopiano MSc PhD Stefano Gambardella MD PhD Federica Turchi MD Barbara Petolicchio MD PhD Massimiliano Toscano MD PhD Mauro Liberatore MD Alessandro Viganò MD PhD Vittorio Di Piero MD PhD |
| Institution: | 1. Department of Human Neurosciences, Sapienza University of Rome, Rome, Italy;2. Department of Radiological, Oncological and Anatomopathological Sciences, Sapienza University of Rome, Rome, Italy;3. Department of Pediatrics, Sapienza University of Rome, Rome, Italy;4. Genomic Medicine Laboratory UILDM, IRCCS Santa Lucia Foundation, Rome, Italy
Molecular Genetics Center, IRCCS Neuromed, Pozzilli, Italy;5. Genomic Medicine Laboratory UILDM, IRCCS Santa Lucia Foundation, Rome, Italy;6. Genomic Medicine Laboratory UILDM, IRCCS Santa Lucia Foundation, Rome, Italy Department of Biomedicine and Prevention, University of Rome “Tor Vergata”, Rome, Italy;7. Molecular Genetics Center, IRCCS Neuromed, Pozzilli, Italy |
| Abstract: | Genetic mutations of sporadic hemiplegic migraine (SHM) are mostly unknown. SHM pathophysiology relies on cortical spreading depression (CSD), which might be responsible for ischemic brain infarction. Cystic fibrosis (CF) is caused by a monogenic mutation of the chlorine transmembrane conductance regulator (CFTR), possibly altering brain excitability. We describe the case of a patient with CF, who had a migrainous stroke during an SHM attack. A 32-year-old Caucasian male was diagnosed with CF, with heterozygotic delta F508/unknown CFTR mutation. The patient experiences bouts of coughing sometimes triggering SHM attacks with visual phosphenes, aphasia, right-sided paresthesia, and hemiparesis. He had a 48-hour hemiparesis triggered by a bout of coughing with hemoptysis, loss of consciousness, and severe hypoxia-hypercapnia. MRI demonstrated transient diffusion hyperintensity in the left frontal-parietal-occipital regions resulting in a permanent infarction in the primary motor area. Later, a brain perfusion SPECT showed persistent diffuse hypoperfusion in the territories involved in diffusion-weighted imaging alteration. Migrainous infarction, depending on the co-occurrence of 2 strictly related phenomena, CSD and hypoxia, appears to be the most plausible explanation. Brain SPECT hypoperfusion suggests a more extensive permanent neuronal loss in territories affected by aura. CF may be then a risk factor for hemiplegic migraine and stroke since bouts of coughing can facilitate brain hypoxia, triggering auras. |
| Keywords: | cortical spreading depression cystic fibrosis aura SHM CFTR |