Susac Syndrome: A differential diagnosis of white matter lesions

Marrodán, Mariano; Correale, Jorge; Alessandro, Lucas; Amaya, Mariela; Fracaro, María Eugenia; Kohler, Alejandro; Fiol, Marcela

doi:10.1016/j.msard.2017.04.007

Cited by 20 publications

(6 citation statements)

References 18 publications

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“…However, they are not specific as they have been reported in callosal lesions of other etiologies. 8,11 This observation has been supported by radiological studies on schizophrenia, where neuropsychiatric symptoms have been associated with splenium of CC structural abnormalities (reduced size or lower diffusion tensor imaging anisotropy) 12 or agenesis. 13 This highlights the important role of CC in regulation of cognitive, affective, and self-regulative behaviors and can explain the neuropsychiatric manifestations in patients with CLOCCs.…”

Section: Discussionmentioning

confidence: 77%

Cytotoxic lesions of the corpus callosum during the course of ketotic hyperglycemia revealing type I diabetes: A case report

Scoppettuolo,

Sinkunaite,

Topciu

et al. 2023

SAGE Open Medical Case Reports

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Cytotoxic lesions of the corpus callosum are lesions secondary to different medical conditions. Radiologically, lesions are identified on magnetic resonance imaging as a hyperintense signal on diffusion-weighted imaging and decreased apparent diffusion coefficient values of the splenium of corpus callosum. Signal changes are reversible in almost totality of the cases. Previous cases of cytotoxic lesions of the corpus callosums have been associated with several metabolic disturbances, but ketotic hyperglycemia has never been reported. We here discussed the case of 28-year-old patient with complex visual hallucinations presenting with cytotoxic lesions of the corpus callosums and type I diabetes. Treatment of hyperglycemia was followed by full clinical recovery and complete regression of the radiological abnormalities at 3-month follow-up. Elevated levels of circulating pro-inflammatory mediators associated with ketotic hyperglycemia in type I diabetes support an implication of cytokines in the pathophysiology of the cytotoxic lesions of the corpus callosums.

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Section: Discussionmentioning

confidence: 77%

Cytotoxic lesions of the corpus callosum during the course of ketotic hyperglycemia revealing type I diabetes: A case report

Scoppettuolo,

Sinkunaite,

Topciu

et al. 2023

SAGE Open Medical Case Reports

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“…It is worth remembering that the lesions visible in MRI in SuS may imitate those present in multiple sclerosis (MS) or acute disseminated encephalomyelitis (ADEM). However, involvement of the grey matter and the presence of lesions in the fibres of the central part of the corpus callosum with relative sparing of the peripheral fibres, allows differentiating SuS from MS and ADEM [11]. In FA, BRAO is observed, which in the acute form of the disease may occur with pseudoemboli known as Gass plaques, present in the central segments of the retinal arterioles, accompanied by characteristic vascular staining and fluorescein leakage at sites remote from the actual BRAO.…”

Section: Discussionmentioning

confidence: 99%

Susac syndrome as a multidisciplinary diagnostic challenge

Sapko¹,

Jamróz-Wiśniewska²,

Woźniak³

et al. 2021

Ann Agric Environ Med.

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“…Es importante tener en cuenta el tiempo entre que se presenta un síntoma y otro porque puede demorarse hasta cinco meses en presentarse la triada patognomónica, es decir, se presenta en solo 13 % de los casos. (20,21)…”

Section: Sistema Visualunclassified

“…(34) Pese al esquema de tratamiento hay casos en los que el paciente no responden a la terapéutica por lo que se considera como alternativa la indicación de rituximab administrando con dosis de inicio de 1 000 mg continuando con la misma dosis luego de dos semanas, con intervalos de 4 a 6 meses y de 6 a 12 meses. (20,34) Debido a que no existen ensayos específicos acerca del manejo terapéutico las directrices del tratamiento para esta enfermedad siguen siendo limitadas. Esto justifica el gran reto que representa dado que que los órganos implicados se alteran de manera inalterable con gran facilidad.…”

Section: Inmunomoduladoresunclassified

Management of Susac Syndrome

Chamba

Garzón

Jiménez

et al. 2022

Salud, Ciencia y Tecnología

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Introduction: Susac syndrome is a disease with autoimmune microangiopathic etiology of vascular endothelia that causes occlusion of arterial branches. Due to its high diagnostic complexity, it cannot be detected at the onset of the syndrome because most patients do not present the characteristic triad at a certain time, they start their picture with neurological manifestations which implies several differential diagnoses. Aim: To describe the correct management of Susac Syndrome based on the main diagnostic criteria and the efficacy of pharmacological treatment. Methods: A literature review study of articles from 2017 to 2022 on Susac syndrome was conducted in the following databases: Scopus, SciELO, Pubmed, Cochrane and Lilacs. Results: This disease is characterized by the presence of a pathognomonic triad of encephalopathy, sensorineural hearing loss and retinal arterial occlusion. Consequently, it is vital to use different diagnostic methods: laboratory data, imaging studies and audiometry. Treatment regimens have been proposed, establishing corticosteroids and immunomodulators as the first line of treatment. When considering pharmacological treatments, not only the severity of SS must be contemplate but also the presence of comorbidities and characteristics according to each patient in order to define the appropriate pharmacological scheme for each patient. Conclusions: Audiometric examinations are considered fundamental for the diagnosis since sensorineural hearing loss can be evidenced in low and medium frequencies, it is important to perform surgical interventions with the purpose of avoiding the progression of the syndrome.

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Susac Syndrome: A differential diagnosis of white matter lesions

Cited by 20 publications

References 18 publications

Cytotoxic lesions of the corpus callosum during the course of ketotic hyperglycemia revealing type I diabetes: A case report

Cytotoxic lesions of the corpus callosum during the course of ketotic hyperglycemia revealing type I diabetes: A case report

Susac syndrome as a multidisciplinary diagnostic challenge

Management of Susac Syndrome

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