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Figure.  Visual Snow Resembling the View of a Badly Tuned Analog Television
Visual Snow Resembling the View of a Badly Tuned Analog Television
1.
Schankin  CJ, Maniyar  FH, Digre  KB, Goadsby  PJ.  ‘Visual snow’: a disorder distinct from persistent migraine aura.  Brain. 2014;137(pt 5):1419-1428. doi:10.1093/brain/awu050PubMedGoogle ScholarCrossref
2.
Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders, 3rd edition. Cephalalgia. 2018;38(1):1-211.
3.
Schankin  CJ, Maniyar  FH, Sprenger  T, Chou  DE, Eller  M, Goadsby  PJ.  The relation between migraine, typical migraine aura and “visual snow”.  Headache. 2014;54(6):957-966. doi:10.1111/head.12378PubMedGoogle ScholarCrossref
4.
Goadsby  PJ, Holland  PR, Martins-Oliveira  M, Hoffmann  J, Schankin  C, Akerman  S.  Pathophysiology of migraine: a disorder of sensory processing.  Physiol Rev. 2017;97(2):553-622. doi:10.1152/physrev.00034.2015PubMedGoogle ScholarCrossref
5.
Boulloche  N, Denuelle  M, Payoux  P, Fabre  N, Trotter  Y, Géraud  G.  Photophobia in migraine: an interictal PET study of cortical hyperexcitability and its modulation by pain.  J Neurol Neurosurg Psychiatry. 2010;81(9):978-984. doi:10.1136/jnnp.2009.190223PubMedGoogle ScholarCrossref
6.
Hadjikhani  N, Sanchez Del Rio  M, Wu  O,  et al.  Mechanisms of migraine aura revealed by functional MRI in human visual cortex.  Proc Natl Acad Sci U S A. 2001;98(8):4687-4692. doi:10.1073/pnas.071582498PubMedGoogle ScholarCrossref
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    Research Letter
    November 25, 2019

    Episodic Visual Snow Associated With Migraine Attacks

    Author Affiliations
    • 1Department of Neurology, Inselspital, University Hospital Bern, University of Bern, Bern, Switzerland
    JAMA Neurol. 2020;77(3):392-393. doi:10.1001/jamaneurol.2019.4050

    Visual snow syndrome (VSS) is a debilitating disorder characterized by continuous visual snow (VS), ie, tiny flickering dots in the entire visual field resembling the view of a badly tuned analog television (Figure), plus additional visual symptoms, such as photophobia and palinopsia. There is a high comorbidity with migraine and migraine aura.1 To our knowledge, this is the first report of patients with an episodic form of VS (eVS), strictly co-occurring with migraine attacks.

    Methods

    Between January 2016 and December 2017, we saw 3 patients with eVS and 1934 patients with migraine at our tertiary outpatient headache center. Diagnoses were made according to the International Headache Society International Classification of Headache Disorders-32 and our previous work,1 except for the requirement of permanence for VS. The case series was approved by the Cantonal Ethics Committee Bern (Req-2017-00698) with waiver of written informed consent based on general consent given by all patients.

    Results

    The 3 patients presented initially for headache as chief complaint. They denied VS outside migraine attacks and did not report additional visual symptoms suggestive of VSS during headache except for photophobia. Neurological examination and magnetic resonance imaging results were normal.

    Patients experienced black and white (2 patients) or black and yellow (1 patient) eVS during migraine attacks, nonfluctuating in distribution and severity. Episodes lasted from less than 2 minutes before and during the attack in 1 patient to during the entire migraine attack in 2 patients.

    Discussion

    Three patients report episodes of VS exclusively at the beginning or during migraine attacks. The description was identical and matched the definition of VS in VSS except for not being continuous.1,2 In the syndrome-defining study,1 only patients with continuous VS were included, impeding the identification of an episodic form. Based on the present case series, we propose to distinguish between VSS, a debilitating disorder characterized by continuous VS and additional visual symptoms persisting over years, and eVS, an uncommon self-limiting symptom during migraine attacks.

    The relationship between migraine and VSS is still unresolved.3 Although the severity of VS in VSS does not fluctuate in parallel to the migraine cycle,1 the strict co-occurrence of eVS and migraine reported here epitomizes a close proximity. This is in agreement with the clinical picture of migraine being a disorder of sensory processing4 and VSS being a disorder of visual processing1 and also with neuroimaging showing overlapping dysfunctional areas in the visual association cortex.3,5

    Episodic VS is uncommon given that only 3 of 1934 patients with migraine (<0.2%) were identified despite asking routinely for visual symptoms. It was remarkably linked to migraine attacks and occurred without the additional symptoms found in VSS.1 In the first patient, the occurrence prior to headache attacks might suggest an aura phenomenon. However, the history of migraine without aura in all patients, the brief duration in 1 patient and long duration in 2 patients, the affection of the entire visual field, and the lack of directed movement speak against a cortical spreading depressionlike mechanism6 and thus against eVS being an aura symptom. In clinical practice, a detailed history in patients reporting visual flickering is therefore necessary to differentiate aura from eVS. This is important because the diagnosis of aura might have implications for patient guidance on contraception or timing of triptan intake.

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    Article Information

    Corresponding Author: Christoph J. Schankin, MD, Department of Neurology, Inselspital, Bern University Hospital, University of Bern, Freiburgstrasse, CH-3010 Bern, Switzerland (christoph.schankin@insel.ch).

    Accepted for Publication: September 26, 2019.

    Published Online: November 25, 2019. doi:10.1001/jamaneurol.2019.4050

    Author Contributions: Dr Schankin had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.

    Concept and design: Hodak, Schankin.

    Acquisition, analysis, or interpretation of data: All authors.

    Drafting of the manuscript: Hodak, Schankin.

    Critical revision of the manuscript for important intellectual content: All authors.

    Statistical analysis: Hodak, Schankin.

    Obtained funding: Schankin.

    Administrative, technical, or material support: Hodak.

    Supervision: Bassetti, Schankin.

    Conflict of Interest Disclosures: Dr Fischer reports grants from the Swiss National Science Foundation, Swiss Heart Foundation, and Medtronic and served as a consultant for Stryker, Medtronic, and CSL Behring outside the submitted work. Dr Schankin reports grants from Swiss Heart Foundation outside the submitted work; personal fees from Novartis, Eli Lilly and Company, Almirall, and Allergan outside the submitted work; and personal fees and nonfinancial support from Teva Pharmaceuticals and Amgen outside the submitted work. No other disclosures were reported.

    Funding/Support: This work was supported by Deutsche Migräne-und Kopfschmerzgesellschaft, Eye on Vision Foundation, and Baasch-Medicus Foundation.

    Role of the Funder/Sponsor: The funders had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication.

    References
    1.
    Schankin  CJ, Maniyar  FH, Digre  KB, Goadsby  PJ.  ‘Visual snow’: a disorder distinct from persistent migraine aura.  Brain. 2014;137(pt 5):1419-1428. doi:10.1093/brain/awu050PubMedGoogle ScholarCrossref
    2.
    Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders, 3rd edition. Cephalalgia. 2018;38(1):1-211.
    3.
    Schankin  CJ, Maniyar  FH, Sprenger  T, Chou  DE, Eller  M, Goadsby  PJ.  The relation between migraine, typical migraine aura and “visual snow”.  Headache. 2014;54(6):957-966. doi:10.1111/head.12378PubMedGoogle ScholarCrossref
    4.
    Goadsby  PJ, Holland  PR, Martins-Oliveira  M, Hoffmann  J, Schankin  C, Akerman  S.  Pathophysiology of migraine: a disorder of sensory processing.  Physiol Rev. 2017;97(2):553-622. doi:10.1152/physrev.00034.2015PubMedGoogle ScholarCrossref
    5.
    Boulloche  N, Denuelle  M, Payoux  P, Fabre  N, Trotter  Y, Géraud  G.  Photophobia in migraine: an interictal PET study of cortical hyperexcitability and its modulation by pain.  J Neurol Neurosurg Psychiatry. 2010;81(9):978-984. doi:10.1136/jnnp.2009.190223PubMedGoogle ScholarCrossref
    6.
    Hadjikhani  N, Sanchez Del Rio  M, Wu  O,  et al.  Mechanisms of migraine aura revealed by functional MRI in human visual cortex.  Proc Natl Acad Sci U S A. 2001;98(8):4687-4692. doi:10.1073/pnas.071582498PubMedGoogle ScholarCrossref
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