Contributions
Abstract: P263
Type: Poster
Abstract Category: Clinical aspects of MS - Diagnosis and differential diagnosis
Introduction: Susac Syndrome (SuS) is an infrequent autoimmune endotheliopathy. While the disease affects brain, inner ear and retina, clinical manifestations can not be concomitant. Brain lesions could require differential diagnosis with multiple sclerosis (MS). Because SuS is a disabling disease, early detection is essential to provide adequate immunosupressive treatment.
Objectives: The aim of this research is to analyze clinical presentation, ancillary methods, differential diagnoses, evolution and treatment in patient with SuS.
Methods: A retrospective review was carried out in 6 adult patients with SuS from January 2007 to April 2016 in an Argentinian center.
Results: The middle age at diagnosis was 36 years old (range 22-52) with male dominance (M/F:5/1). The clinical triad was complete in 50% of cases. 3 patients just suffered visual and encephalic disturbances but one of them presented asintomathic unilateral lower tones hypoacusia in audiometry (AT). Fluorescein angiography (FA) showed abnormalities in all cases. The cognitive evaluation showed multidomain compromise in 4 cases.
Regarding MRI findings, all patients presented typical corpus callosum images known as “snowball lesions” and periventricular lesions. Three cases had fornix microinfractions, 4 cases had yuxtacortical involvement and 2 cases presented infrantentorial infractions and lesions with contrast enhancement.
For acute treatment: 5 patients received treatment with corticosteroids (CE) and 4 of them in combination with IV immunoglobulins. Cyclophosphamide and mycophenolate mofetil were employed for 5 patients as maintenance strategy. One patient received rituximab and 1 patient received azathioprine. All cases presented at least 1 relapse (range: 1-3).
One patient did not complete the follow-up.
Conclusions: Typical SuS clinical triad could not be present from the beginning, MRI, FA and audiometry should be performed as soon as SuS is suspected to detect subclinical compromise.
It is important to suspect SuS as differential diagnosis for MS, through corpus callosum typical lesions and fornix microinfractions in MRI, typical FA and AT findings.
Treatment must be as aggressive as necessary to prevent permanent disability.
Disclosure:
Mariano Marrodan has nothing to disclose related to this abstract.
Jorge Correale has nothing to disclose related to this Abstract.
Lucas Alessandro has nothing to disclose related to this Abstract.
Mariela Amaya has nothing to disclose related to this Abstract.
Alejandro Köhler has nothing to disclose related to this Abstract.
Marcela Fiol has nothing to disclose related to this Abstract.
Abstract: P263
Type: Poster
Abstract Category: Clinical aspects of MS - Diagnosis and differential diagnosis
Introduction: Susac Syndrome (SuS) is an infrequent autoimmune endotheliopathy. While the disease affects brain, inner ear and retina, clinical manifestations can not be concomitant. Brain lesions could require differential diagnosis with multiple sclerosis (MS). Because SuS is a disabling disease, early detection is essential to provide adequate immunosupressive treatment.
Objectives: The aim of this research is to analyze clinical presentation, ancillary methods, differential diagnoses, evolution and treatment in patient with SuS.
Methods: A retrospective review was carried out in 6 adult patients with SuS from January 2007 to April 2016 in an Argentinian center.
Results: The middle age at diagnosis was 36 years old (range 22-52) with male dominance (M/F:5/1). The clinical triad was complete in 50% of cases. 3 patients just suffered visual and encephalic disturbances but one of them presented asintomathic unilateral lower tones hypoacusia in audiometry (AT). Fluorescein angiography (FA) showed abnormalities in all cases. The cognitive evaluation showed multidomain compromise in 4 cases.
Regarding MRI findings, all patients presented typical corpus callosum images known as “snowball lesions” and periventricular lesions. Three cases had fornix microinfractions, 4 cases had yuxtacortical involvement and 2 cases presented infrantentorial infractions and lesions with contrast enhancement.
For acute treatment: 5 patients received treatment with corticosteroids (CE) and 4 of them in combination with IV immunoglobulins. Cyclophosphamide and mycophenolate mofetil were employed for 5 patients as maintenance strategy. One patient received rituximab and 1 patient received azathioprine. All cases presented at least 1 relapse (range: 1-3).
One patient did not complete the follow-up.
Conclusions: Typical SuS clinical triad could not be present from the beginning, MRI, FA and audiometry should be performed as soon as SuS is suspected to detect subclinical compromise.
It is important to suspect SuS as differential diagnosis for MS, through corpus callosum typical lesions and fornix microinfractions in MRI, typical FA and AT findings.
Treatment must be as aggressive as necessary to prevent permanent disability.
Disclosure:
Mariano Marrodan has nothing to disclose related to this abstract.
Jorge Correale has nothing to disclose related to this Abstract.
Lucas Alessandro has nothing to disclose related to this Abstract.
Mariela Amaya has nothing to disclose related to this Abstract.
Alejandro Köhler has nothing to disclose related to this Abstract.
Marcela Fiol has nothing to disclose related to this Abstract.