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REVIEW PAPER
Susac’s syndrome – the crucial role of imaging tests for proper diagnosis
1
Department of Ophthalmology, Voivodeship Regional Hospital, Kielce, Poland
2
Jan Kochanowski University, Kielce, Poland
3
Department of Vitreoretinal Surgery, Medical University, Lublin, Poland
4
Department of Imaging Diagnostics, Voivodeship Regional Hospital, Kielce, Poland
5
Ophthalmology Clinic Boni Fratres Lodziensis, Poland
Corresponding author
Joanna Roskal-Wałek
Department of Ophthalmology, Voivodeship Regional Hospital, Grunwaldzka 45, 25-736, Kielce, Poland
Department of Ophthalmology, Voivodeship Regional Hospital, Grunwaldzka 45, 25-736, Kielce, Poland
Ann Agric Environ Med. 2022;29(2):190-200
KEYWORDS
magnetic resonance imagingoptical coherence tomographyfluorescein angiographymultiple sclerosisSusac‘s syndrome
TOPICS
Biological agents posing occupational risk in agriculture, forestry, food industry and wood industry and diseases caused by these agents (zoonoses, allergic and immunotoxic diseases)Prevention of occupational diseases in agriculture, forestry, food industry and wood industry
ABSTRACT
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.
Summary:
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.
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.
Summary:
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.
ACKNOWLEDGEMENTS
Project financed under the program the Minister of Education
and Science called “Regional Initiative of Excellence” in the
years 2019–2022, project no. 024/RID/2018/19, amount of
financing 11 999 000,00 PLN.
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