The imaging findings of migraine with visual aura in a CADASIL patient.

Head MRI images of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) patients during migraine attacks are rare.

Síndrome de nieve visual y su relación con la migraña / Visual snow syndrome and its relationship with migraine

Introducción El síndrome de nieve visual (SNV) es un trastorno del sistema nervioso central que implica la visión de forma constante de pequeños puntos blancos y negros en la totalidad del campo visual. Desarrollo El SNV puede presentarse desde la infancia hasta la tercera edad, siendo más frecuente en jóvenes y sin diferencia entre géneros. En sus criterios diagnósticos se incluye la presencia de nieve visual, pero también otros fenómenos visuales como palinopsia, fotofobia, nictalopía y otros fenómenos visuales persistentes. La fisiopatología del SNV es desconocida, pero se postulan como mecanismos la hiperexcitabilidad del córtex visual y una disfunción en el procesamiento visual de orden superior. La prevalencia de migraña en los pacientes con SNV es alta en comparación con la población general y cuando se presentan conjuntamente los síntomas son más severos. No se dispone de un tratamiento eficaz, pero el fármaco con mejores resultados es la lamotrigina, recomendándose únicamente en casos seleccionados con alta limitación funcional. Conclusiones El síndrome de nieve visual es una entidad poco conocida e infradiagnosticada, pero el creciente número de investigaciones durante los últimos años ha permitido definir unos criterios diagnósticos y acercarnos a su fisiopatología. Es una entidad íntimamente relacionada con la migraña, con solapamiento de síntomas y probablemente mecanismos fisiopatológicos comunes. (AU)

Introduction Visual snow syndrome (VSS) is a central nervous system disorder that consists of the constant perception of small black and white dots throughout the entire visual field. Development VSS can present from infancy to old age, with greater prevalence in the young population, and shows no difference between sexes. The diagnostic criteria include the presence of visual snow and such other visual phenomena as palinopsia, photophobia, nyctalopia, and other persistent visual phenomena. The pathophysiology of VSS is unknown, but hyperexcitability of the visual cortex and a dysfunction in higher-order visual processing are postulated as potential mechanisms. The prevalence of migraine among patients with VSS is high, compared to the general population, and symptoms are more severe in patients presenting both conditions. No effective treatment is available, but the drug with the best results is lamotrigine, which is recommended only in selected cases with severe functional limitation. Conclusions VSS is a little-known and underdiagnosed entity, but the increasing number of studies in recent years has made it possible to establish diagnostic criteria and begin studying its pathophysiology. This entity is closely related to migraine, with overlapping symptoms and probably shared pathophysiological mechanisms. (AU)

Visual migraine aura iconography: A multicentre, cross-sectional study of individuals with migraine with aura.

INTRODUCTION: Visual disturbances are the most common symptoms of migraine aura. These symptoms can be described systematically by subdividing them into elementary visual symptoms. Since visual symptoms of migraine aura are not easy to describe verbally, we developed a collection of images illustrating previously reported elementary visual symptoms. OBJECTIVES: To test a standardised visual migraine aura iconography in a large population of migraine with aura patients and to improve it based on the participants' feedback. METHODS: We created a set of images representing 25 elementary visual symptoms and a web-based survey where participants could report whether they recognised these images as part of their visual aura. Elementary visual symptoms could also be recognised via a corresponding text description or described in a free text by participants. Individuals with migraine aura recruited from four tertiary headache centres (in Switzerland, Denmark, Norway and Italy) were invited to complete the survey. RESULTS: Two hundred and fifteen participants completed the study (78.9% women, median age 36). They recognised a total of 1645 elementary visual symptoms from our predefined list. Of those, 1291 (78.4%) where recognised via standardised iconography images. A new type of elementary visual symptom was reported by one participant. CONCLUSION: Most elementary visual symptoms experienced by participants were recognised via the standardised iconography. This tool can be useful for clinical as well as research purposes.

Visual snow syndrome and its relationship with migraine.

INTRODUCTION: Visual snow syndrome (VSS) is a central nervous system disorder that consists of the constant perception of small black and white dots throughout the entire visual field. DEVELOPMENT: VSS can present from infancy to old age, with greater prevalence in the young population, and shows no difference between sexes. The diagnostic criteria include the presence of visual snow and such other visual phenomena as palinopsia, photophobia, nyctalopia, and other persistent visual phenomena. The pathophysiology of VSS is unknown, but hyperexcitability of the visual cortex and a dysfunction in higher-order visual processing are postulated as potential mechanisms. The prevalence of migraine among patients with VSS is high, compared to the general population, and symptoms are more severe in patients presenting both conditions. No effective treatment is available, but the drug with the best results is lamotrigine, which is recommended only in selected cases with severe functional limitation. CONCLUSIONS: VSS is a little-known and underdiagnosed entity, but the increasing number of studies in recent years has made it possible to establish diagnostic criteria and begin studying its pathophysiology. This entity is closely related to migraine, with overlapping symptoms and probably shared pathophysiological mechanisms.

A Case of Migraine Aura Status in a Migraine Patient With Typical Aura Without Headache.

Migraine aura status (MAS) is a rare complication of migraine and is listed in the appendix of the International Classification of Headache, Third Edition. MAS occurs in migraine with aura (MwA) patients, with at least three auras occurring over a period of three days. We describe a case of MAS associated with a patient who had a migraine with a typical aura without headache (TAWH). She has experienced only visual auras, including periods of MAS complications, and has not experienced headaches or other auras, including sensory, speech, and motor. The patient is a 45-year-old woman. She had discomfort with visual aura without a headache every two to three months. The visual aura began to gradually darken a portion of the right side of the visual field binocularly, which gradually expanded around it and was surrounded by iridescent flashes running in a zig-zag pattern to the periphery. The area of the visual field abnormality gradually decreased and disappeared after approximately 15 minutes. One day, she had an aura once after dinner, and when she awoke without any trigger after going to bed, the aura appeared and repeated approximately three times. She experienced three to four auras the next day and the day after. In the six-month follow-up period, there was no further recurrence of MAS. Most previous reports of MAS have been associated with typical MwA, and there have been few other reports of MAS associated with TAWH as far as we could investigate.

A Case of Reversible Posterior Leukoencephalopathy Syndrome (PRES) With a History of Migraine and Onset With Initial Visual Aura and Migraine-Like Headache, With a Significant Response to Lasmiditan: A Case Report.

Posterior reversible encephalopathy syndrome (PRES) is a neurological disease that presents with various neurological symptoms and is often accompanied by elevated blood pressure at onset. Neuroimaging, especially magnetic resonance imaging (MRI), often shows a characteristic parieto-occipital pattern with a symmetrical distribution of changes, reflecting vasogenic edema. Hypertension and endothelial cell damage are the most common causes of PRES. An association between migraine and endothelial cell damage has been suggested, but the relationship between migraine and PRES is unknown. Reports on PRES triggered by migraines are scarce. We report a case of PRES in a 59-year-old woman with migraine without aura. At the onset, the patient experienced a first-ever visual aura and a migraine-like headache. In this case, it was also difficult to distinguish between PRES headache and headache caused by a pre-existing migraine; however, lasmiditan, an acute migraine treatment without vasoconstrictive properties, was remarkably effective for headaches.

Visual Aura in Non-Migraine Headaches: A Population Study.

BACKGROUND: Visual aura (VA) occurs mostly in migraine with aura (MA), but some case studies have reported aura in non-migraine headaches. Thus, information of VA in non-migraine headaches is scarce. Aim of this study was to investigate the prevalence and impact of VA in non-migraine headache and compare it with that of migraine headache. METHODS: This study was a nationwide population-based study. We used an internet-based headache diagnosis questionnaire to diagnose headache, and various modules to evaluate clinical features and comorbidities of participants with headache. We defined migraine headache as migraine and probable migraine (PM), whereas non-migraine headache was defined as a headache but not migraine or PM. VA was defined as a self-reporting VA rating scale score ≥ 3. RESULTS: Of the 3,030 participants, 1,431 (47.2%) and 507 (16.7%) had non-migraine headache and migraine headache, respectively. VA prevalence was much lower in the non-migraine headache group than in the migraine headache group (14.5% [207/1,431] vs. 26.0% [132/507], P < 0.001). In subjects with non-migraine headache, those with VA had a markedly higher number of headache days per 30 days (median [25th-75th percentiles]: 2.0 [1.0-5.0] vs. 2.0 [1.0-3.0], P < 0.001), and headache-related disability (6.0 [3.0-16.0] vs. 2.0 [0.0-7.0], P < 0.001) than those without VA. VA prevalence did not differ significantly according to age and sex. CONCLUSION: Non-migraine headache with VA patients had more severe symptoms than those without VA. These findings may improve the understanding of VA and the management of individuals with non-migraine headache.

Evaluating migraine with typical aura with neuroimaging.

Objective: To provide an up-to-date narrative literature review of imaging in migraine with typical aura, as a means to understand better migraine subtypes and aura biology. Background: Characterizing subtypes of migraine with typical aura and appreciating possible biological differences between migraine with and without aura, are important to understanding the neurobiology of aura and trying to advance personalized therapeutics in this area through imaging biomarkers. One means of doing this over recent years has been the use of increasingly advanced neuroimaging techniques. Methods: We conducted a literature review of neuroimaging studies in migraine with aura, using a PubMed search for terms 'imaging migraine', 'aura imaging', 'migraine with aura imaging', 'migraine functional imaging' and 'migraine structural imaging'. We collated the findings of the main studies, excluding small case reports and series with n < 6, and have summarized these and their implications for better understanding of aura mechanisms. Results: Aura is likely mediated by widespread brain dysfunction in areas involving, but not limited to, visual cortex, somatosensory and insular cortex, and thalamus. Higher brain excitability in response to sensory stimulation and altered resting-state functional connectivity in migraine sufferers with aura could have a genetic component. Pure visual aura compared to visual aura with other sensory or speech symptoms as well, may involve different functional reorganization of brain networks and additional mitochondrial dysfunction mediating more aura symptoms. Conclusion: There is a suggestion of at least some distinct neurobiological differences between migraine with and without aura, despite the shared phenotypic similarity in headache and other migraine-associated symptoms. It is clear from the vast majority of aura phenotypes being visual that there is a particular predisposition of the occipital cortex to aura mechanisms. Why this is the case, along with the relationships between cortical spreading depression and headache, and the reasons why aura does not consistently present in affected individuals, are all important research questions for the future.

Evaluation of the choroidal vascular index and choroidal changes in migraine subgroups.

PURPOSE: To investigate binarized choroidal structural parameters, retinal nerve fiber layer (RNFL) thickness, and retina changes duringattack-free periods in patients with migraine using enhanced depth imaging optic coherence tomography (EDI-OCT), and compare patients with migraine with aura and without aura (MwA and MoA, respectively) and aura subgroups (visual aura, non-visual aura), with age and sex-matched healthy subjects. METHOD: This observational, prospective study included 102 patients with migraine and 36 healthy controls. Central macular thickness (CMT), retinal nerve fiber layer (RNFL), ganglion cell layer (GCL), and choroidal thickness (CT) were measured using a Spectralis OCT device. The choroid vascularity index (CVI) was evaluated using the Image-J software. CVI was calculated as the proportion of the luminal area (LA) to the total choroidal area (TCA). RNFL, CMT, CT, and CVI measurements were compared statistically. RESULTS: Choroidal thickness at 1500 µm temporal of the fovea was found to be statistically significantly thinner in the MwA and MoA groups compared with the control group (p ≤ 0.01). There was a significant difference in the subfoveal CT values of the MwA and control groups (p < 0.05). The mean RNFL thickness of patients with migraine with visual aura was found to be statistically significantly thinner than in the migraine group with non-visual aura (98.73 ± 8.4 and 109.4 ± 16.8) (p < 0.05). There were no statistically significant differences between the RNFL CMT, GCC, and CVI values in the MwA, MoA, and control groups (p > 0.05). CONCLUSION: We found that the choroidal thickness was significantly decreased in patients with migraine, especially in the MwA group. In the visual aura subgroup, the mean RNFL thickness was significantly decreased compared with the non-visual aura subgroup.

Elucidating the visual phenomena in epilepsy: A mini review.

Epilepsy is one of the most recognizable neurological diseases, globally. Epilepsy may be accompanied by various complications, including vision impairments, which may severely impact one's quality of life. These visual phenomena may occur in the preictal, ictal and/or postictal periods of seizures. Examples of epilepsy associated visual phenomena include visual aura, visual hallucinations, transient visual loss and amaurosis (blindness). These ophthalmologic signs/symptoms of epilepsy may be temporary or permanent and may vary depending of the type of epilepsy and location of the seizure foci (occipital or temporal lobe). Some visual phenomena may even be utilized to diagnose the epilepsy type, although solely depending on visual symptoms for diagnosis may lead to mistreatment. Some antiseizure medications (ASMs) may also contribute to certain visual disturbances, thereby impacting its therapeutic efficiency for patients with epilepsy (PWE). Although the development of visual comorbidities has been observed diversely among PWE, there may still be a lack of understanding on their relevance and manifestation in epilepsy, which may contribute to the rate of misdiagnosis and the current scarcity in therapeutic relieve. Therefore, this mini narrative review aimed to discuss the common epilepsy associated visual phenomena, based on the available literature. This review also showcased the relationship between the type of visual complications and the site of seizure onset, as well as compared the visual phenomena between occipital lobe epilepsy and temporal lobe epilepsy. Evaluation of these findings may be crucial in reducing the risk of permanent seizure/epilepsy related vision deficits among PWE.

Case report: An EEG captured case of migralepsy/migraine aura-triggered seizures.

Introduction: Migraine and epilepsy are common chronic neurological disorders presenting with paroxysmal attacks of transient cerebral dysfunction, followed by subsequent return to baseline between episodes. The term "migralepsy" has been proposed to define migraine-triggered epileptic seizures classified by the ICHD-III as a complication of migraine with an aura. Case: A 55-year-old man with a 30-year history of migraine without aura presented with a new onset left parietal pain accompanied by visual disturbances occurring up to 20 times per day. His visual distortions included kaleidoscopic vision, flashes of shadows, and a right superior quadrantanopia lasting 20 min. He described discrete 2-min episodes of scintillating scotomas in his right visual field. Ictal EEG demonstrated a left occipital onset focal aware seizure with his clinical symptoms. The patient was started on valproic Acid and has remained asymptomatic. Discussion: The diagnostic criteria as set out by the ICHD-III for migralepsy and other syndromes with migrainous and ictal features remain confusing for practitioners as there is much overlap in clinical manifestations of these entities. EEG should be obtained when ictal features are noted among patients presenting with headache.

Aura Type and Outcome After Anterior Temporal Lobectomy.

OBJECTIVE: Temporal lobe epilepsy (TLE) is one of the most common causes of medically refractory focal epilepsy. Anterior temporal lobectomy (ATL) leads to improved seizure control in patients with medically refractory TLE. Various auras are associated with TLE; however, the relationships between aura type and outcome after ATL are poorly understood. Our objective was to investigate the associations among clinical features, aura type, and seizure outcome after ATL. METHODS: The records of patients who underwent ATL between 1993 and 2016 at a single institution (N = 174) were retrospectively reviewed. Demographic and clinical variables were compared among aura types using analysis of variance and logistic regression analysis. A multiple regression analysis was conducted to determine whether aura type predicted seizure outcome after ATL. RESULTS: Mesial temporal sclerosis (MTS) on magnetic resonance imaging inversely correlated with cephalic auras (P = 0.0090). Affective auras (P = 0.014) and somatosensory auras (P = 0.021) were correlated with findings of MTS on pathology, whereas this finding was inversely correlated with the presence of auditory auras (P = 0.0056). On multiple regression analysis, predictors of worse seizure outcome after ATL were cephalic auras (P = 0.0048), gustatory auras (P = 0.029), visual auras (P = 0.049), and tonic-clonic seizures (P = 0.047). Fewer preoperative antiepileptic medications (P = 0.0032), and presence of multiple auras (P = 0.011) were associated with better outcome. CONCLUSIONS: Cephalic auras, gustatory auras, and visual auras were associated with worse seizure outcome after ATL.

Jean-Martin Charcot (1825-1893) and the "Alice in Wonderland syndrome".

The "Alice in Wonderland syndrome" (AIWS) is a neurological disorder characterized by altered body schema perception, visual, or somesthetic symptoms, which is frequently associated with migraine. In this article, we present the earliest known description of symptoms attributable to AIWS in the medical literature. During a lecture held on November 22, 1887, at the Salpêtrière, Jean-Martin Charcot (1825-1893) examined a patient with somesthetic symptoms (partial macrosomatognosia) in the context of migraine with aura. Although this condition was not known at the time, Charcot tried to provide an accurate semiological and nosographic framework of this case, attributing the complex of symptoms to migraine with aura and epilepsy with sensory symptoms. With intellectual honesty and clinical prudence, Charcot correctly pointed to a disturbance in the excitability of cortical areas responsible for processing and perceiving sensory stimuli.

Migrainous infarction of the eye: Two cases of monocular ischemic complications associated with retinal migraine.

BACKGROUND: Retinal migraine is defined by fully reversible monocular visual phenomena. We present two cases that were complicated by permanent monocular vision deficits. CASES: A 57-year-old man with history of retinal migraine experienced persistent monocular vision loss after one stereotypical retinal migraine, progressing to finger-count vision over 4 days. He developed paracentral acute middle maculopathy that progressed to central retinal artery occlusion. A 27-year-old man with history of retinal migraine presented with persistent right eye superotemporal scotoma after a retinal migraine. Relative afferent pupillary defect and superotemporal visual field defect were noted, consistent with ischemic optic neuropathy. CONCLUSION: Retinal migraine can complicate with permanent monocular visual loss, suggesting potential migrainous infarction of the retina or optic nerve. A thorough cerebrovascular evaluation must be completed, which was unrevealing in our cases. Acute and preventive migraine therapy may be considered in retinal migraine patients, to mitigate rare but potentially permanent visual loss.

Not All Cases of Visual Snows are Benign: Mimics of Visual Snow Syndrome.

Visual snow syndrome (VSS) is a clinical disorder characterized by pan-field visual disturbance. It is a diagnosis of exclusion since its pathophysiology remains unknown. Excluding other mimics is of great significance since some serious pathologies can have secondary visual snow (VS) as an initial presentation. Delayed or incorrect diagnosis of these VSS mimics may lead to permanent vision loss or even death. The purpose of this review is to help physicians distinguish VSS mimics promptly to avoid bad outcomes. The authors performed a PubMed literature search of articles, case reports, and reviews describing VS symptoms in patients with underlying diseases other than VSS. The red flags of secondary VS symptoms were highlighted, such as new-onset or intermittent VS, unilateral or quadrant VS, and accompanied ocular or neurological deficits. There are four main categories of VSS mimics, ie, including neurological disorders, ocular pathologies, drug-related VS, and other systemic diseases. The physicians could largely exclude most etiologies based on history taking, ophthalmologic and neurologic examinations, and neuroimaging. Further research in VS should carefully define and unify the inclusion and exclusion criteria of this disorder and investigate these secondary VS conditions and their pathogenesis.

Occipital bending in migraine with visual aura.

OBJECTIVE: To analyze occipital bending (OB) frequency in patients with migraine with visual aura compared with those without aura. BACKGROUND: A unique type of asymmetry in the human brain in which one occipital pole crosses the midline and bends over the other pole is called OB. OB frequency has been shown to be related to major psychiatric diseases. Hence, it may suggest more than an anatomical variation. Structural differences in the brain have been demonstrated but unequivocally between patients with migraine with aura and without aura. OB is newly recognized, and we aimed to evaluate its frequency among patients with migraine. METHODS: For this retrospective cohort study, we reviewed our records from 2016 to 2021 from a database of the outpatient headache clinic of Koç University Hospital. RESULTS: We found 84 patients with migraine who fulfilled diagnostic criteria for migraine with aura and migraine without aura and also had cranial magnetic resonance imaging. The median age of the population was 40 (IQR, 32-52). The female-to-male ratio of participants was 2:1. A quarter of the patients had visual aura. The prevalence of OB in patients with migraine in our retrospective study was 33.3% (28/84). Between our study groups, OB was significantly higher in patients with migraine with visual aura (57.1%, 12 out of 21 patients) than in those without aura (25.4%, 16 out of 63), (odds ratio 3.9 (95% confidence interval 1.4 to 11.0), p = 0.015). CONCLUSION: OB frequency is two times higher in patients with migraine with visual aura. It may have pathophysiological implications.

Aura Mapping: Where Vision and Somatosensation Meet.

While migraine auras are most frequently visual, somatosensory auras are also relatively common. Both are characterized by the spread of activation across a cortical region containing a spatial mapping of the sensory (retinal or skin) surface. When both aura types occur within a single migraine episode, they may offer an insight into the neural mechanism which underlies them. Could they both be initiated by a single neural event, or do the timing and laterality relationships between them demand multiple triggers? The observations reported here were carried out 25 years ago by a group of six individuals with migraine with aura. They timed, described and mapped their visual and somatosensory auras as they were in progress. Twenty-nine episode reports are summarized here. The temporal relationship between the onset of the two auras was quite variable within and across participants. Various forms of the cortical spreading depression hypothesis of migraine aura are evaluated in terms of whether they can account for the timing, pattern of symptom spread and laterality of the recorded auras.

Transient and Recurrent Vision Loss in a High-Altitude Porter from Pakistan on a Polish Winter Karakoram Expedition.

Visual sensations appear in most migraine auras, but binocular blindness is uncommon. We described a case of multiple transient losses of vision in a man on a winter expedition to K2. His symptoms were later diagnosed as recurrent visual auras without pain. Sojourns at altitude can induce migraine attack; therefore, susceptible individuals should avoid factors that might provoke migraines at high altitude, such as improper acclimatization, dehydration and an inadequate sleep regime.