Migrainous Infarction in a Patient With Sporadic Hemiplegic Migraine and Cystic Fibrosis

A 99mTc-HMPAO Brain SPECT Study

Valentina Mancini, Giulio Mastria, Viviana Frantellizzi, Patrizia Troiani, Stefania Zampatti, Stefania Carboni, Emiliano Giardina, Rosa Campopiano, Stefano Gambardella, Federica Turchi, Barbara Petolicchio, Massimiliano Toscano, Mauro Liberatore, Alessandro Viganò, Vittorio Di Piero

Research output: Contribution to journalArticle

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.

Original languageEnglish
Pages (from-to)253-258
Number of pages6
JournalHeadache
Volume59
Issue number2
DOIs
Publication statusPublished - Feb 1 2019

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Technetium Tc 99m Exametazime
Single-Photon Emission-Computed Tomography
Cystic Fibrosis
Infarction
Cortical Spreading Depression
Brain
Paresis
Mutation
Epilepsy
Phosphenes
Stroke
Brain Infarction
Brain Hypoxia
Occipital Lobe
Parietal Lobe
Unconsciousness
Hemoptysis
Hypercapnia
Paresthesia
Aphasia

Keywords

  • aura
  • CFTR
  • cortical spreading depression
  • cystic fibrosis
  • SHM

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

Cite this

Mancini, V., Mastria, G., Frantellizzi, V., Troiani, P., Zampatti, S., Carboni, S., ... Di Piero, V. (2019). Migrainous Infarction in a Patient With Sporadic Hemiplegic Migraine and Cystic Fibrosis: A 99mTc-HMPAO Brain SPECT Study. Headache, 59(2), 253-258. https://doi.org/10.1111/head.13472

Migrainous Infarction in a Patient With Sporadic Hemiplegic Migraine and Cystic Fibrosis : A 99mTc-HMPAO Brain SPECT Study. / Mancini, Valentina; Mastria, Giulio; Frantellizzi, Viviana; Troiani, Patrizia; Zampatti, Stefania; Carboni, Stefania; Giardina, Emiliano; Campopiano, Rosa; Gambardella, Stefano; Turchi, Federica; Petolicchio, Barbara; Toscano, Massimiliano; Liberatore, Mauro; Viganò, Alessandro; Di Piero, Vittorio.

In: Headache, Vol. 59, No. 2, 01.02.2019, p. 253-258.

Research output: Contribution to journalArticle

Mancini, V, Mastria, G, Frantellizzi, V, Troiani, P, Zampatti, S, Carboni, S, Giardina, E, Campopiano, R, Gambardella, S, Turchi, F, Petolicchio, B, Toscano, M, Liberatore, M, Viganò, A & Di Piero, V 2019, 'Migrainous Infarction in a Patient With Sporadic Hemiplegic Migraine and Cystic Fibrosis: A 99mTc-HMPAO Brain SPECT Study', Headache, vol. 59, no. 2, pp. 253-258. https://doi.org/10.1111/head.13472
Mancini, Valentina ; Mastria, Giulio ; Frantellizzi, Viviana ; Troiani, Patrizia ; Zampatti, Stefania ; Carboni, Stefania ; Giardina, Emiliano ; Campopiano, Rosa ; Gambardella, Stefano ; Turchi, Federica ; Petolicchio, Barbara ; Toscano, Massimiliano ; Liberatore, Mauro ; Viganò, Alessandro ; Di Piero, Vittorio. / Migrainous Infarction in a Patient With Sporadic Hemiplegic Migraine and Cystic Fibrosis : A 99mTc-HMPAO Brain SPECT Study. In: Headache. 2019 ; Vol. 59, No. 2. pp. 253-258.
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