Aims: To evaluate the role of high-resolution Intracranial Vessel Wall Imaging (VWI) to detect disease activity in a case of Susac Syndrome (SS). Material and Methods: A 40-year-old woman with a confirmed diagnosis of SS under specific therapy for this disease, received two brain 3T magnetic resonance imaging (MRI) scan 6 months apart. In the second one the sequence Proton Density Weighted Image (PDWI), before and after gadolinium, was included in order to explore the presence of inflammation of vessel walls. Results: Despite conventional MRI did not show new lesions, PDWI revealed enhancement of an arteriola with the "tram track" sign, a doble linear enhancement attributable to inflamed arteriolar walls; as a collateral finding, an enhancement of right inner ear structures was observed. In addition, retinal fluorescein angiogram (FA) performed a few days later showed new arterial wall hyperfluorescence (AWH) of retinal arterioles, confirming persisting inflammatory activity despite treatment. Discussion: SS is a rare immunological disorder, that at pathological level appears based on immune-mediated inflammation occlusion affect- ing small vessels of retinal, inner ear and brain small vessels. Lesions of the corpus callosum, the so-called "snowball lesions" considered highly suggestive of SS, are usually interpreted as microinfarcts due to occlusion of tiny arterioles. One additional supportive finding of small vessel occlusion, i.e. evidence of wall’s inflammation of small brain arterioles, was highlighted in this case by high resolution VWI sequences. Although not specific of SS, this sign could support SS diagnosis in the early phase when clinical and/or neuroimaging triad are incomplete, allowing anticipation of treatment. Furthermore, in the present case, an abnormal enhancement of the inner ear observed by this MRI sequence, anticipated an additional cardinal diagnostic element. Current available markers of sub- optimal therapeutic response are: presence of new lesions or persistent active enhanced lesions on standard MRI, new visual field defect and evidence of AWH on FA. In addition, these data suggests that evaluation of small vessels inflammation through High resolution VWI might add valuable information, particularly in central nervous system predominant SS and that stability of brain lesion load is not sufficient to establish an absent disease activity. Concordant results with FA, the most accurate test to evaluate disease activity in SS, also validate accuracy of this MRI marker. Conclusions: The proper use of VWI could represent a supportive tool in diagnostic and mainly therapeutic management of SS.
Magnetic Resonance intracranial Vessel Wall Imaging (VWI) in Susac Syndrome: potential relevance for diagnosis and therapeutic management / Antonio Lotti, Alice Mariottini, Alessandro Barilaro, Giovanna Carlucci, Lorenzo Vannozzi, Enrico Fainardi, Luca Massacesi.. - In: NEUROLOGICAL SCIENCES. - ISSN 1590-1874. - ELETTRONICO. - 43 (Suppl 1):(2022), pp. 71-72. [10.1007/s10072-022-06531-9]
Magnetic Resonance intracranial Vessel Wall Imaging (VWI) in Susac Syndrome: potential relevance for diagnosis and therapeutic management.
Antonio Lotti
;Alice Mariottini;Giovanna Carlucci;Enrico Fainardi;Luca Massacesi.
2022
Abstract
Aims: To evaluate the role of high-resolution Intracranial Vessel Wall Imaging (VWI) to detect disease activity in a case of Susac Syndrome (SS). Material and Methods: A 40-year-old woman with a confirmed diagnosis of SS under specific therapy for this disease, received two brain 3T magnetic resonance imaging (MRI) scan 6 months apart. In the second one the sequence Proton Density Weighted Image (PDWI), before and after gadolinium, was included in order to explore the presence of inflammation of vessel walls. Results: Despite conventional MRI did not show new lesions, PDWI revealed enhancement of an arteriola with the "tram track" sign, a doble linear enhancement attributable to inflamed arteriolar walls; as a collateral finding, an enhancement of right inner ear structures was observed. In addition, retinal fluorescein angiogram (FA) performed a few days later showed new arterial wall hyperfluorescence (AWH) of retinal arterioles, confirming persisting inflammatory activity despite treatment. Discussion: SS is a rare immunological disorder, that at pathological level appears based on immune-mediated inflammation occlusion affect- ing small vessels of retinal, inner ear and brain small vessels. Lesions of the corpus callosum, the so-called "snowball lesions" considered highly suggestive of SS, are usually interpreted as microinfarcts due to occlusion of tiny arterioles. One additional supportive finding of small vessel occlusion, i.e. evidence of wall’s inflammation of small brain arterioles, was highlighted in this case by high resolution VWI sequences. Although not specific of SS, this sign could support SS diagnosis in the early phase when clinical and/or neuroimaging triad are incomplete, allowing anticipation of treatment. Furthermore, in the present case, an abnormal enhancement of the inner ear observed by this MRI sequence, anticipated an additional cardinal diagnostic element. Current available markers of sub- optimal therapeutic response are: presence of new lesions or persistent active enhanced lesions on standard MRI, new visual field defect and evidence of AWH on FA. In addition, these data suggests that evaluation of small vessels inflammation through High resolution VWI might add valuable information, particularly in central nervous system predominant SS and that stability of brain lesion load is not sufficient to establish an absent disease activity. Concordant results with FA, the most accurate test to evaluate disease activity in SS, also validate accuracy of this MRI marker. Conclusions: The proper use of VWI could represent a supportive tool in diagnostic and mainly therapeutic management of SS.I documenti in FLORE sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.