Characteristics of headache in relation to the manifestation of Susac syndrome

Obelienienė, Diana; Macaitytė, Raminta; Balnytė, Renata; Pėstininkaitė, Rūta; Gleiznienė, Rymantė; Balčiūnienė, Vilma Jūratė

doi:10.1016/j.medici.2018.01.003

Cited by 12 publications

(6 citation statements)

References 14 publications

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“… 11 Migraine-like headache as a symptom of SuS could have different presentations in relation to the manifestation of SuS: exacerbation and chronification of headache just before the onset of the first symptoms of SuS, the manifestation of migraine-like headache during the first SuS episode, and an increasing frequency of headache during the course of the disease. 30 …”

Section: Resultsmentioning

confidence: 99%

Clinical Characterization and Ancillary Tests in Susac Syndrome

Wilf-Yarkoni,

Zmira,

Tolkovsky

et al. 2024

Neurol Neuroimmunol Neuroinflamm

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Susac syndrome (SuS) is an orphan microangiopathic disease characterized by a triad of encephalopathy, visual disturbances due to branch retinal artery occlusions, and sensorineuronal hearing loss. Our previous systematic review on all cases of SuS reported until 2012 allowed for a better understanding of clinical presentation and diagnostic findings. Based on these data, we suggested diagnostic criteria in 2016 to allow early diagnosis and treatment of SuS. In view of the accumulation of new SuS cases reported in the last 10 years and improved diagnostic tools, we here aimed at updating the demographic and clinical features of SuS and to review the updated ancillary tests being used for SuS diagnosis. Therefore, based on the 2016 criteria, we systematically collected and evaluated data on SuS published from January 2013 to March 2022.

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Section: Resultsmentioning

confidence: 99%

Clinical Characterization and Ancillary Tests in Susac Syndrome

Wilf-Yarkoni,

Zmira,

Tolkovsky

et al. 2024

Neurol Neuroimmunol Neuroinflamm

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“…Following exclusion of 53 items, 109 items comprising 504 cases were reviewed. A total of 53 items were labeled as “Cases—Structural” (70 individual case reports in total) 12–64 (Table ), 34 as “Cases—Nonstructural” (50 individual case reports in total) 65–98 (Table ), and 19 as “Groups” (384 cases in total) 99–117 (Table ). In addition, from two case series of three patients, 118,119 three cases were included in “Cases—Structural,” and three in “Cases—Other.” In one case, additional information was gathered from a previous report of the same case 38,120 …”

Section: Resultsmentioning

confidence: 99%

“…Six of the 53 reported cases of symptomatic MWoA fulfilled the ICHD criteria for MWoA. 73,78,119 Assumed underlying conditions were superior oblique myokymia (n = 1), aneurysm of the anterior communicating artery (n = 2), and Susac syndrome (n = 3). None of these cases fulfilled our working criteria for symptomatic migraine or possible symptomatic migraine.…”

Section: Cases-nonstructuralmentioning

confidence: 99%

Symptomatic migraine: A systematic review to establish a clinically important diagnostic entity

et al. 2021

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Objective To determine if a clinical presentation indistinguishable from migraine can occur due to an underlying condition or pathology, that is, "symptomatic migraine." Background It is currently not clear whether migraine truly can be caused by an underlying condition or pathology. Characterization of the etiology and clinical features of possible symptomatic migraine is of significant clinical importance and further may help elucidate the pathophysiology of migraine. Methods We devised operational diagnostic criteria for “symptomatic migraine” and “possible symptomatic migraine” requiring strong evidence for a causal relation between underlying cause and migraine symptoms adhering strictly to diagnostic criteria. PubMed was searched for case reports of symptomatic migraine from inception to March 2020. Only articles published in English or German were included. No restrictions were placed on study design. Relevant references in the articles were also included. Papers were systematically reviewed by two independent reviewers for detailed clinical features of migraine as well as the proposed underlying conditions and the effects of treatment of these conditions. Results Our search retrieved 1726 items. After screening, 109 papers comprising 504 cases were reviewed in detail. Eleven patients with migraine with aura (MWA) fulfilled our working criteria for symptomatic migraine, and 39 patients fulfilled our criteria for possible symptomatic migraine. The most common etiologies of symptomatic migraine were arteriovenous malformations, carotid stenosis, dissection or aneurysm, brain infarctions, meningioma, and various intra‐axial tumors. Conclusions Symptomatic MWA, indistinguishable from idiopathic MWA, may occur due to cortical lesions or microembolization. We found no clear evidence supporting the existence of symptomatic migraine without aura although we did identify possible cases. Our findings are limited by the available literature, and we suggest that prospective studies are needed.

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“…The differences between MS and SS, apart from those observed in imaging examinations, also concern the clinical image and results of laboratory tests. Headache occurs in approximately 80% of patients with SS; however, this is not a typical symptom of MS [12,49]. Moreover, headache belongs to the main red flags indicating a diagnosis other than MS [61].…”

Section: Optical Coherence Tomographymentioning

confidence: 99%

Susac’s syndrome – the crucial role of imaging tests for proper diagnosis

Roskal-Wałek¹,

Mackiewicz²,

Wypchło³

et al. 2022

Ann Agric Environ Med.

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Introduction. Susac's syndrome (SS) is a rare, autoimmune-mediated endoteliopathy characterized by a clinical triad of encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. SS is also characterized by a neuroimaging triad consisting of white matter lesions, grey matter lesions, and leptomeningeal enhancement on magnetic resonance imaging (MRI). Considering the rarity of SS, as well as certain similarity to other, more frequent neurological diseases, such as multiple sclerosis (MS), this syndrome is sometimes incorrectly diagnosed and treated.Objective. The aim of the study is to present the current state of knowledge on SS, with particular consideration for the differential diagnostics between SS and MS, using the latest available imaging techniques, such as brain MRI, optical coherence tomography (OCT), OCT angiography (OCTA) and fluorescein angiography (FA). Review methods. The major electronic databases (PubMed, Google Scholar) were searched manually in order to identify the relevant studies published on SS. Brief description of the state of knowledge. Distinguishing SS from MS is a diagnostic challenge. In the majority of cases, patients with SS do not present the complete clinical or neuroimaging triad, and a delay in making the correct diagnosis exposes the patient to the occurrence of complications, resulting from the development of the underlying disease, or/and the application of improper treatment. In the case of SS the results of brain MRI and FA are essential for making the correct diagnosis as they may reveal pathognomonic changes. Sumary. Imaging examinations, such as brain MRI, FA, and OCT complement each other, due to which the diagnosis of SS may be simpler, irrespective of the stage of the disease.

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Characteristics of headache in relation to the manifestation of Susac syndrome

Cited by 12 publications

References 14 publications

Clinical Characterization and Ancillary Tests in Susac Syndrome

Clinical Characterization and Ancillary Tests in Susac Syndrome

Symptomatic migraine: A systematic review to establish a clinically important diagnostic entity

Susac’s syndrome – the crucial role of imaging tests for proper diagnosis

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