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Radiologically isolated syndrome

December 18, 2023

Radiologically isolated syndrome (RIS) is a clinical situation in which a person has white matter lesions suggestive of multiple sclerosis (MS), as shown on an MRI scan that was done for reasons unrelated to MS symptoms. The nerve lesions in these people show dissemination in space with an otherwise normal neurological examination and without historical accounts of typical MS symptoms.[1]

MRI findings that are consistent with multiple sclerosis have been observed in healthy people who underwent MRI scanning, and 50% go on to develop symptomatic MS, sometimes with a primary progressive course.[2][3] This condition was first characterized in 2009.[4]

Diagnosis edit

The criteria for an RIS diagnosis are as follows:[5][4][6]

  1. The presence of incidental MRI findings in the CNS white matter:
    1. Ovoid and well-circumscribed homogeneous foci, with or without involvement of the corpus callosum
    2. T2 hyperintensities larger than 3 mm in diameter, which fulfill at least 3 of the 4 Barkhof MRI criteria[7] for DIS
    3. The CNS abnormalities are not consistent with a vascular condition
  2. No historical accounts of clinical symptoms consistent with neurological dysfunction.
  3. MRI anomalies do not account for apparent impairment in social, occupational, or generalized areas of functioning.
  4. MRI anomalies are not due to substance abuse, such as recreational drug use, toxic exposure, or a prior known medical condition.
  5. Exclusion of a differential diagnosis of leukoaraiosis, or extensive white matter pathology excluding the corpus callosum.
  6. MRI anomalies of the CNS are not accounted for by another disease.

Discovery edit

RIS is discovered when an MRI scan is performed for other reasons. The most common symptom that led to the incidental discovery of RIS is headache.[5] Other common reasons are trauma, psychiatric disorders, and endocrinological disorders.[5]

Management edit

Currently, routine clinical follow-up and MRI neuroimaging surveillance is the standard by which patients are observed.[4] While treatment of MS disease modifying therapies have been given to some individuals with RIS, the majority opt for active surveillance and the appearance of clinical symptoms before commencing treatment,[5] as treatment is considered controversial.[8]

Prognosis edit

In a 5 year study, clinical events, which refers to the first symptoms of exacerbations, clinical attacks, flare ups, or severe symptoms, indicative of MS, appeared in 34% of individuals.[9] Of those who developed symptoms, 9.6% fulfilled criteria for primary progressive multiple sclerosis (PPMS).[9]

Epidemiology edit

Due to the incidental nature of RIS, exact figures on prevalence is unknown, though it has been suggested that RIS is the most common type of asymptomatic MS.[10] The prevalence may be higher in relatives of patients with MS.[11] One study, at a university hospital that is located in a high region of MS disease incidence, put the disease prevalence at approximately 1 in 2000.[12] An earlier study in 1961 of 15,644 autopsies found 12 cases (0.08%) of unexpected MS findings without a previous history of MS symptoms.[5][13] The mean age of first indication of RIS from 451 patients is 37.2 years.[9]

RIS in children edit

Though rare, some children that have had MRI scans for reasons unrelated to MS have shown signs of RIS. The most common reason for an initial MRI in these children was a headache. The first occurrence of a clinical event characteristic of MS in nearly half of the children examined was 2 years, though in a majority of cases, 'radiologic evolution', i.e. the increase in the number of size of lesions as detected in subsequent MRI, developed after one year. The presence of oligoclonal bands in the CSF and spinal cord lesions were associated with an increased risk of a first clinical event characteristic of MS. It was found that children with RIS had a substantial risk of subsequent clinical symptoms and/or radiologic evolution.[6]

Research directions edit

Calls have been made for longer prospective studies, tracking the development of potential disease progression over a longer period of time are warranted. This would ensure criteria in RIS is satisfactory and whether consideration should be given to treating individuals with RIS on current MS medication.[5][7]

RIS has been linked to prodromal multiple sclerosis.[14]

Etymology edit

The acronym RIS was coined in 2009 by Okuda and colleagues.[4] Siva and colleagues suggested an alternate name, radiologically uncovered asymptomatic possible inflammatory-demyelinating disease (RAPIDD).[5][15]

References edit

  1. ^ Labiano-Fontcuberta, Andrés; Benito-León, Julián (October 2016). "Radiologically isolated syndrome: An update on a rare entity". Multiple Sclerosis (Houndmills, Basingstoke, England). 22 (12): 1514–1521. doi:10.1177/1352458516653666. ISSN 1477-0970. PMID 27288053. S2CID 31847038.
  2. ^ Reich, Daniel S; Lucchinetti, Claudia F.; Calabresi, Peter A (January 2018). "Multiple Sclerosis". The New England Journal of Medicine. 378 (2): 169–180. doi:10.1056/NEJMra1401483. PMC 6942519. PMID 29320652.
  3. ^ Kantarci, Orhun H.; Lebrun, Christine; Siva, Aksel; Keegan, Mark B.; Azevedo, Christina J.; Inglese, Matilde; Tintoré, Mar; Newton, Braeden D.; Durand-Dubief, Francoise (February 2016). "Primary Progressive Multiple Sclerosis Evolving From Radiologically Isolated Syndrome". Annals of Neurology. 79 (2): 288–294. doi:10.1002/ana.24564. hdl:11365/1003841. ISSN 1531-8249. PMID 26599831. S2CID 19868714.
  4. ^ a b c d Okuda, D. T.; Mowry, E. M.; Beheshtian, A.; Waubant, E.; Baranzini, S. E.; Goodin, D. S.; Hauser, S. L.; Pelletier, D. (2009-03-03). "Incidental MRI anomalies suggestive of multiple sclerosis: the radiologically isolated syndrome". Neurology. 72 (9): 800–805. doi:10.1212/01.wnl.0000335764.14513.1a. ISSN 1526-632X. PMID 19073949. S2CID 9981947.
  5. ^ a b c d e f g Granberg, Tobias; Martola, Juha; Kristoffersen-Wiberg, Maria; Aspelin, Peter; Fredrikson, Sten (March 2013). "Radiologically isolated syndrome--incidental magnetic resonance imaging findings suggestive of multiple sclerosis, a systematic review". Multiple Sclerosis. 19 (3): 271–280. doi:10.1177/1352458512451943. ISSN 1477-0970. PMID 22760099. S2CID 24307387.
  6. ^ a b Makhani, Naila; Lebrun, Christine; Siva, Aksel; Brassat, David; Dallière, Clarisse Carra; Seze, Jérôme de; Du, Wei; Dubief, Françoise Durand; Kantarci, Orhun (2017-11-01). "Radiologically isolated syndrome in children: Clinical and radiologic outcomes". Neurology: Neuroimmunology & Neuroinflammation. 4 (6): e395. doi:10.1212/NXI.0000000000000395. ISSN 2332-7812. PMC 5614726. PMID 28959703.
  7. ^ a b Leahy, Hannah; Center, University of Massachusetts Memorial Medical; Garg, Neeta (2013). "Radiologically Isolated Syndrome: An Overview". Neurological Bulletin. 5 (1): 22–26. doi:10.7191/neurol_bull.2013.1044.
  8. ^ Yamout, B.; Khawajah, M. Al (2017-10-01). "Radiologically isolated syndrome and multiple sclerosis". Multiple Sclerosis and Related Disorders. 17: 234–237. doi:10.1016/j.msard.2017.08.016. ISSN 2211-0348. PMID 29055465.
  9. ^ a b c Okuda, Darin T.; Siva, Aksel; Kantarci, Orhun; Inglese, Matilde; Katz, Ilana; Tutuncu, Melih; Keegan, B. Mark; Donlon, Stacy; Hua, Le H. (2014-03-05). "Radiologically Isolated Syndrome: 5-Year Risk for an Initial Clinical Event". PLOS ONE. 9 (3): e90509. Bibcode:2014PLoSO...990509O. doi:10.1371/journal.pone.0090509. ISSN 1932-6203. PMC 3943959. PMID 24598783.
  10. ^ Siva, Aksel (2013-12-01). "Asymptomatic MS". Clinical Neurology and Neurosurgery. 115: S1–S5. doi:10.1016/j.clineuro.2013.09.012. ISSN 0303-8467. PMID 24321147. S2CID 29282376.
  11. ^ Gabelic, T.; Ramasamy, D. P.; Weinstock-Guttman, B.; Hagemeier, J.; Kennedy, C.; Melia, R.; Hojnacki, D.; Ramanathan, M.; Zivadinov, R. (2014-01-01). "Prevalence of Radiologically Isolated Syndrome and White Matter Signal Abnormalities in Healthy Relatives of Patients with Multiple Sclerosis". American Journal of Neuroradiology. 35 (1): 106–112. doi:10.3174/ajnr.A3653. ISSN 0195-6108. PMC 7966501. PMID 23886745.
  12. ^ Granberg, Tobias; Martola, Juha; Aspelin, Peter; Kristoffersen-Wiberg, Maria; Fredrikson, Sten (2013-11-01). "Radiologically isolated syndrome: an uncommon finding at a university clinic in a high-prevalence region for multiple sclerosis". BMJ Open. 3 (11): e003531. doi:10.1136/bmjopen-2013-003531. ISSN 2044-6055. PMC 3822304. PMID 24189079.
  13. ^ Georgi W. Multiple sclerosis. Anatomopathological findings of multiple sclerosis in diseases not clinically diagnosed. Schweiz Med Wochenschr 1961; 91: 605–607. (German)
  14. ^ Tremlett, Helen; Okuda, Darin T.; Lebrun-Frenay, Christine (2021). "The multiple sclerosis prodrome is just unspecific symptoms in radiologically isolated syndrome patients – No". Multiple Sclerosis Journal. 27 (12): 1824–1826. doi:10.1177/13524585211035951. PMC 8521363. PMID 34494923.
  15. ^ Siva, A.; Saip, S.; Altintas, A.; Jacob, A.; Keegan, B.M.; Kantarci, O.H. (2009). "Multiple sclerosis risk in radiologically uncovered asymptomatic possible inflammatory-demyelinating disease". Multiple Sclerosis Journal. 15 (8): 918–927. doi:10.1177/1352458509106214. PMID 19667020. S2CID 20746782.

December 18, 2023
radiologically, isolated, syndrome, clinical, situation, which, person, white, matter, lesions, suggestive, multiple, sclerosis, shown, scan, that, done, reasons, unrelated, symptoms, nerve, lesions, these, people, show, dissemination, space, with, otherwise, . Radiologically isolated syndrome RIS is a clinical situation in which a person has white matter lesions suggestive of multiple sclerosis MS as shown on an MRI scan that was done for reasons unrelated to MS symptoms The nerve lesions in these people show dissemination in space with an otherwise normal neurological examination and without historical accounts of typical MS symptoms 1 MRI findings that are consistent with multiple sclerosis have been observed in healthy people who underwent MRI scanning and 50 go on to develop symptomatic MS sometimes with a primary progressive course 2 3 This condition was first characterized in 2009 4 Contents 1 Diagnosis 1 1 Discovery 2 Management 3 Prognosis 4 Epidemiology 5 RIS in children 6 Research directions 7 Etymology 8 ReferencesDiagnosis editThe criteria for an RIS diagnosis are as follows 5 4 6 The presence of incidental MRI findings in the CNS white matter Ovoid and well circumscribed homogeneous foci with or without involvement of the corpus callosum T2 hyperintensities larger than 3 mm in diameter which fulfill at least 3 of the 4 Barkhof MRI criteria 7 for DIS The CNS abnormalities are not consistent with a vascular condition No historical accounts of clinical symptoms consistent with neurological dysfunction MRI anomalies do not account for apparent impairment in social occupational or generalized areas of functioning MRI anomalies are not due to substance abuse such as recreational drug use toxic exposure or a prior known medical condition Exclusion of a differential diagnosis of leukoaraiosis or extensive white matter pathology excluding the corpus callosum MRI anomalies of the CNS are not accounted for by another disease Discovery edit RIS is discovered when an MRI scan is performed for other reasons The most common symptom that led to the incidental discovery of RIS is headache 5 Other common reasons are trauma psychiatric disorders and endocrinological disorders 5 Management editCurrently routine clinical follow up and MRI neuroimaging surveillance is the standard by which patients are observed 4 While treatment of MS disease modifying therapies have been given to some individuals with RIS the majority opt for active surveillance and the appearance of clinical symptoms before commencing treatment 5 as treatment is considered controversial 8 Prognosis editIn a 5 year study clinical events which refers to the first symptoms of exacerbations clinical attacks flare ups or severe symptoms indicative of MS appeared in 34 of individuals 9 Of those who developed symptoms 9 6 fulfilled criteria for primary progressive multiple sclerosis PPMS 9 Epidemiology editDue to the incidental nature of RIS exact figures on prevalence is unknown though it has been suggested that RIS is the most common type of asymptomatic MS 10 The prevalence may be higher in relatives of patients with MS 11 One study at a university hospital that is located in a high region of MS disease incidence put the disease prevalence at approximately 1 in 2000 12 An earlier study in 1961 of 15 644 autopsies found 12 cases 0 08 of unexpected MS findings without a previous history of MS symptoms 5 13 The mean age of first indication of RIS from 451 patients is 37 2 years 9 RIS in children editThough rare some children that have had MRI scans for reasons unrelated to MS have shown signs of RIS The most common reason for an initial MRI in these children was a headache The first occurrence of a clinical event characteristic of MS in nearly half of the children examined was 2 years though in a majority of cases radiologic evolution i e the increase in the number of size of lesions as detected in subsequent MRI developed after one year The presence of oligoclonal bands in the CSF and spinal cord lesions were associated with an increased risk of a first clinical event characteristic of MS It was found that children with RIS had a substantial risk of subsequent clinical symptoms and or radiologic evolution 6 Research directions editCalls have been made for longer prospective studies tracking the development of potential disease progression over a longer period of time are warranted This would ensure criteria in RIS is satisfactory and whether consideration should be given to treating individuals with RIS on current MS medication 5 7 RIS has been linked to prodromal multiple sclerosis 14 Etymology editThe acronym RIS was coined in 2009 by Okuda and colleagues 4 Siva and colleagues suggested an alternate name radiologically uncovered asymptomatic possible inflammatory demyelinating disease RAPIDD 5 15 References edit Labiano Fontcuberta Andres Benito Leon Julian October 2016 Radiologically isolated syndrome An update on a rare entity Multiple Sclerosis Houndmills Basingstoke England 22 12 1514 1521 doi 10 1177 1352458516653666 ISSN 1477 0970 PMID 27288053 S2CID 31847038 Reich Daniel S Lucchinetti Claudia F Calabresi Peter A January 2018 Multiple Sclerosis The New England Journal of Medicine 378 2 169 180 doi 10 1056 NEJMra1401483 PMC 6942519 PMID 29320652 Kantarci Orhun H Lebrun Christine Siva Aksel Keegan Mark B Azevedo Christina J Inglese Matilde Tintore Mar Newton Braeden D Durand Dubief Francoise February 2016 Primary Progressive Multiple Sclerosis Evolving From Radiologically Isolated Syndrome Annals of Neurology 79 2 288 294 doi 10 1002 ana 24564 hdl 11365 1003841 ISSN 1531 8249 PMID 26599831 S2CID 19868714 a b c d Okuda D T Mowry E M Beheshtian A Waubant E Baranzini S E Goodin D S Hauser S L Pelletier D 2009 03 03 Incidental MRI anomalies suggestive of multiple sclerosis the radiologically isolated syndrome Neurology 72 9 800 805 doi 10 1212 01 wnl 0000335764 14513 1a ISSN 1526 632X PMID 19073949 S2CID 9981947 a b c d e f g Granberg Tobias Martola Juha Kristoffersen Wiberg Maria Aspelin Peter Fredrikson Sten March 2013 Radiologically isolated syndrome incidental magnetic resonance imaging findings suggestive of multiple sclerosis a systematic review Multiple Sclerosis 19 3 271 280 doi 10 1177 1352458512451943 ISSN 1477 0970 PMID 22760099 S2CID 24307387 a b Makhani Naila Lebrun Christine Siva Aksel Brassat David Dalliere Clarisse Carra Seze Jerome de Du Wei Dubief Francoise Durand Kantarci Orhun 2017 11 01 Radiologically isolated syndrome in children Clinical and radiologic outcomes Neurology Neuroimmunology amp Neuroinflammation 4 6 e395 doi 10 1212 NXI 0000000000000395 ISSN 2332 7812 PMC 5614726 PMID 28959703 a b Leahy Hannah Center University of Massachusetts Memorial Medical Garg Neeta 2013 Radiologically Isolated Syndrome An Overview Neurological Bulletin 5 1 22 26 doi 10 7191 neurol bull 2013 1044 Yamout B Khawajah M Al 2017 10 01 Radiologically isolated syndrome and multiple sclerosis Multiple Sclerosis and Related Disorders 17 234 237 doi 10 1016 j msard 2017 08 016 ISSN 2211 0348 PMID 29055465 a b c Okuda Darin T Siva Aksel Kantarci Orhun Inglese Matilde Katz Ilana Tutuncu Melih Keegan B Mark Donlon Stacy Hua Le H 2014 03 05 Radiologically Isolated Syndrome 5 Year Risk for an Initial Clinical Event PLOS ONE 9 3 e90509 Bibcode 2014PLoSO 990509O doi 10 1371 journal pone 0090509 ISSN 1932 6203 PMC 3943959 PMID 24598783 Siva Aksel 2013 12 01 Asymptomatic MS Clinical Neurology and Neurosurgery 115 S1 S5 doi 10 1016 j clineuro 2013 09 012 ISSN 0303 8467 PMID 24321147 S2CID 29282376 Gabelic T Ramasamy D P Weinstock Guttman B Hagemeier J Kennedy C Melia R Hojnacki D Ramanathan M Zivadinov R 2014 01 01 Prevalence of Radiologically Isolated Syndrome and White Matter Signal Abnormalities in Healthy Relatives of Patients with Multiple Sclerosis American Journal of Neuroradiology 35 1 106 112 doi 10 3174 ajnr A3653 ISSN 0195 6108 PMC 7966501 PMID 23886745 Granberg Tobias Martola Juha Aspelin Peter Kristoffersen Wiberg Maria Fredrikson Sten 2013 11 01 Radiologically isolated syndrome an uncommon finding at a university clinic in a high prevalence region for multiple sclerosis BMJ Open 3 11 e003531 doi 10 1136 bmjopen 2013 003531 ISSN 2044 6055 PMC 3822304 PMID 24189079 Georgi W Multiple sclerosis Anatomopathological findings of multiple sclerosis in diseases not clinically diagnosed Schweiz Med Wochenschr 1961 91 605 607 German Tremlett Helen Okuda Darin T Lebrun Frenay Christine 2021 The multiple sclerosis prodrome is just unspecific symptoms in radiologically isolated syndrome patients No Multiple Sclerosis Journal 27 12 1824 1826 doi 10 1177 13524585211035951 PMC 8521363 PMID 34494923 Siva A Saip S Altintas A Jacob A Keegan B M Kantarci O H 2009 Multiple sclerosis risk in radiologically uncovered asymptomatic possible inflammatory demyelinating disease Multiple Sclerosis Journal 15 8 918 927 doi 10 1177 1352458509106214 PMID 19667020 S2CID 20746782 Retrieved from https en wikipedia org w index php title Radiologically isolated syndrome amp oldid 1136202671, wikipedia, wiki, book, books, library,

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