Clinically isolated syndrome

Last updated

A clinically isolated syndrome (CIS) is a clinical situation of an individual's first neurological episode, caused by inflammation or demyelination of nerve tissue. An episode may be monofocal, in which symptoms present at a single site in the central nervous system, or multifocal, in which multiple sites exhibit symptoms. CIS with enough paraclinical evidence can be considered as a clinical stage of multiple sclerosis (MS). It can also be retrospectively diagnosed as a kind of MS when more evidence is available.

Contents

Brain lesions associated with a clinically isolated syndrome may be indicative of several neurological diseases, like multiple sclerosis (MS) or neuromyelitis optica. In order for such a diagnosis, multiple sites in the central nervous system must present lesions, typically over multiple episodes, and for which no other diagnosis is likely. A clinically definitive diagnosis of MS is made once an MRI detects lesions in the brain, consistent with those typical of MS. Other diagnostics include cerebrospinal fluid analysis and evoked response testing. [1]

Currently it is considered that the best predictor of future development of clinical multiple sclerosis is the number of T2 lesions visualized by magnetic resonance imaging during the CIS [2] and their size. [3] It is normal to evaluate diagnostic criteria against the "time to conversion to definite".

In 2001, the International Panel on the Diagnosis of multiple sclerosis issued the McDonald criteria, a revision of the previous diagnostic procedures to detect MS, known as the Poser criteria. "While maintaining the basic requirements of dissemination in time and space, the McDonald criteria provided specific guidelines for using findings on MRI and cerebrospinal fluid analysis to provide evidence of the second attack in those individuals who have had a single demyelinating episode and thereby confirm the diagnosis more quickly." [4] Further revisions were issued in 2005.

2013 revision

The 1996 definition of the clinical courses of MS (phenotypes) was updated on 2013 by an international panel (International Advisory Committee on Clinical Trials). [5]

While the main classification in 1996 was the recovery from the attacks (this clinical feature separates relapsing remitting (RR) from progressive), in the updated revision the main classification is the activity. [6]

MS courses in the new revision are divided into active and non-active, and CIS, when is active on MRI, becomes a kind of RRMS (this, of course, must be retrospectively diagnosed after the CDMS conversion) [7]

Some reviews describe CIS as "the prodromal stage of MS". [8]

CIS and conversion to MS

Before the 2010 McDonald criteria, [9] when it was not possible to prove dissemination of the lesions in space and time, the condition was called CIS and was considered outside the MS spectrum. As soon as dissemination was clear (a second lesion development) the situation was called "Conversion to MS".

The 2010 revision of the McDonald criteria allows the diagnosis of MS with only one proved lesion (CIS). Therefore, the 2013 revision of the phenotypes for the disease course, consistently, included CIS as one of the clinical phenotypes of MS. [7]

Therefore the former expression "conversion from CIS to MS", which is still in use, should be redefined consistently with the former changes, since CIS now is inside MS.[ citation needed ]

Related Research Articles

<span class="mw-page-title-main">Acute disseminated encephalomyelitis</span> Autoimmune disease

Acute disseminated encephalomyelitis (ADEM), or acute demyelinating encephalomyelitis, is a rare autoimmune disease marked by a sudden, widespread attack of inflammation in the brain and spinal cord. As well as causing the brain and spinal cord to become inflamed, ADEM also attacks the nerves of the central nervous system and damages their myelin insulation, which, as a result, destroys the white matter. The cause is often a trigger such as from viral infection or vaccinations.

<span class="mw-page-title-main">Multiple sclerosis</span> Disease that damages the myelin sheaths around nerves

Multiple sclerosis (MS) is an autoimmune disease in which the insulating covers of nerve cells in the brain and spinal cord are damaged. Being a demyelinating disease, MS disrupts the ability of parts of the nervous system to transmit signals, resulting in a range of signs and symptoms, including physical, mental, and sometimes psychiatric problems. Symptoms include double vision, vision loss, eye pain, muscle weakness, and loss of sensation or coordination. MS takes several forms, with new symptoms either occurring in isolated attacks or building up over time. In relapsing forms of MS, between attacks, symptoms may disappear completely, although some permanent neurological problems often remain, especially as the disease advances. In progressive forms of MS, bodily function slowly deteriorates once symptoms manifest and will steadily worsen if left untreated.

<span class="mw-page-title-main">Interferon beta-1a</span> Cytokine in the interferon family

Interferon beta-1a is a cytokine in the interferon family used to treat multiple sclerosis (MS). It is produced by mammalian cells, while interferon beta-1b is produced in modified E. coli. Some research indicates that interferon injections may result in an 18–38% reduction in the rate of MS relapses.

Interferon beta-1b is a cytokine in the interferon family used to treat the relapsing-remitting and secondary-progressive forms of multiple sclerosis (MS). It is approved for use after the first MS event. Closely related is interferon beta 1a, also indicated for MS, with a very similar drug profile.

<span class="mw-page-title-main">McDonald criteria</span>

The McDonald criteria are diagnostic criteria for multiple sclerosis (MS). These criteria are named after neurologist W. Ian McDonald who directed an international panel in association with the National Multiple Sclerosis Society (NMSS) of America and recommended revised diagnostic criteria for MS in April 2001. These new criteria intended to replace the Poser criteria and the older Schumacher criteria. They have undergone revisions in 2005, 2010 and 2017.

<span class="mw-page-title-main">Lesional demyelinations of the central nervous system</span>

Multiple sclerosis and other demyelinating diseases of the central nervous system (CNS) produce lesions and glial scars or scleroses. They present different shapes and histological findings according to the underlying condition that produces them.

Inflammatory demyelinating diseases (IDDs), sometimes called Idiopathic (IIDDs) due to the unknown etiology of some of them, are a heterogenous group of demyelinating diseases - conditions that cause damage to myelin, the protective sheath of nerve fibers - that occur against the background of an acute or chronic inflammatory process. IDDs share characteristics with and are often grouped together under Multiple Sclerosis. They are sometimes considered different diseases from Multiple Sclerosis, but considered by others to form a spectrum differing only in terms of chronicity, severity, and clinical course.

<span class="mw-page-title-main">Marburg acute multiple sclerosis</span> Medical condition

Marburg acute multiple sclerosis, also known as Marburg multiple sclerosis or acute fulminant multiple sclerosis, is considered one of the multiple sclerosis borderline diseases, which is a collection of diseases classified by some as MS variants and by others as different diseases. Other diseases in this group are neuromyelitis optica (NMO), Balo concentric sclerosis, and Schilder's disease. The graver course is one form of malignant multiple sclerosis, with patients reaching a significant level of disability in less than five years from their first symptoms, often in a matter of months.

<span class="mw-page-title-main">Signs and symptoms of multiple sclerosis</span> Neurological signs and symptoms

The signs and symptoms of multiple sclerosis (MS) encompass a wide range of neurological and physical manifestations, including vision problems, muscle weakness, coordination difficulties, and cognitive impairment, varying significantly in severity and progression among individuals.

Research in multiple sclerosis may find new pathways to interact with the disease, improve function, curtail attacks, or limit the progression of the underlying disease. Many treatments already in clinical trials involve drugs that are used in other diseases or medications that have not been designed specifically for multiple sclerosis. There are also trials involving the combination of drugs that are already in use for multiple sclerosis. Finally, there are also many basic investigations that try to understand the disease better and in the future may help to find new treatments.

<span class="mw-page-title-main">Tumefactive multiple sclerosis</span> Medical condition

Tumefactive multiple sclerosis is a condition in which the central nervous system of a person has multiple demyelinating lesions with atypical characteristics for those of standard multiple sclerosis (MS). It is called tumefactive as the lesions are "tumor-like" and they mimic tumors clinically, radiologically and sometimes pathologically.

Fred D. Lublin is an American neurologist and an authority on the treatment of multiple sclerosis. Along with colleagues at the National Multiple Sclerosis Society, his work redefined the clinical course definitions of MS.

Poser criteria are diagnostic criteria for multiple sclerosis (MS). They replaced the older Schumacher criteria, and are now considered obsolete as McDonald criteria have superseded them. Nevertheless, some of the concepts introduced have remained in MS research, like CDMS, and newer criteria are often calibrated against them. The criteria were unveiled in the Annals of Neurology in 1983 by a team led by Dr. Charles M. Poser.

Schumacher criteria are diagnostic criteria that were previously used for identifying multiple sclerosis (MS). Multiple sclerosis, understood as a central nervous system (CNS) condition, can be difficult to diagnose since its signs and symptoms may be similar to other medical problems. Medical organizations have created diagnostic criteria to ease and standardize the diagnostic process especially in the first stages of the disease. Schumacher criteria were the first internationally recognized criteria for diagnosis, and introduced concepts still in use, as CDMS.

Malignant multiple sclerosis is used to describe MS patients who reach significant level of disability in a short period of time. Malignant MS cases are not common, less than 5% of patients with MS experience this type of progression.

<span class="mw-page-title-main">Diagnosis of multiple sclerosis</span>

Current standards for diagnosing multiple sclerosis (MS) are based on the 2018 revision of McDonald criteria. They rely on MRI detection of demyelinating lesions in the CNS, which are distributed in space (DIS) and in time (DIT). It is also a requirement that any possible known disease that produces demyelinating lesions is ruled out before applying McDonald's criteria.

Clinical Electrophysiological Testing is based on techniques derived from electrophysiology used for the clinical diagnosis of patients. There are many processes that occur in the body which produce electrical signals that can be detected. Depending on the location and the source of these signals, distinct methods and techniques have been developed to properly target them.

<span class="mw-page-title-main">Pathology of multiple sclerosis</span> Pathologic overview

Multiple sclerosis (MS) can be pathologically defined as the presence of distributed glial scars (scleroses) in the central nervous system that must show dissemination in time (DIT) and in space (DIS) to be considered MS lesions.

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.

Brenda Banwell is Chief of the Division of Neurology and Co-Director of the Neuroscience Center, and Professor of Neurology at Children's Hospital of Philadelphia and holder of the Grace R. Loeb Endowed Chair in Neurosciences. She also holds the title of Professor of Pediatrics and Neurology at the Perelman School of Medicine at the University of Pennsylvania.

References

  1. ↑ "Clinically Isolated Syndrome (CIS)". Library. Archived from the original on 2007-12-06. Retrieved 2007-10-27.
  2. ↑ Cramer, Stig P; Modvig, Signe; Simonsen, Helle J; Frederiksen, Jette L; Larsson, Henrik B. W (2015). "Permeability of the blood–brain barrier predicts conversion from optic neuritis to multiple sclerosis". Brain. 138 (9): 2571–83. doi:10.1093/brain/awv203. PMC   4547053 . PMID   26187333.
  3. ↑ Crimi, Alessandro; Commowick, Olivier; Maarouf, Adil; Barillot, Christian (2014). "Predictive value of imaging markers at multiple sclerosis disease onset based on gadolinium-and USPIO-enhanced MRI and machine learning". PLOS ONE. 9 (4): e93024. Bibcode:2014PLoSO...993024C. doi: 10.1371/journal.pone.0093024 . PMC   3972197 . PMID   24691080.
  4. ↑ McDonald, W. Ian; Compston, Alistair; Edan, Gilles; Goodkin, Donald; Hartung, Hans-Peter; Lublin, Fred D; McFarland, Henry F; Paty, Donald W; Polman, Chris H; Reingold, Stephen C; Sandberg-Wollheim, Magnhild; Sibley, William; Thompson, Alan; Van Den Noort, Stanley; Weinshenker, Brian Y; Wolinsky, Jerry S (2001). "Recommended diagnostic criteria for multiple sclerosis: Guidelines from the international panel on the diagnosis of multiple sclerosis". Annals of Neurology. 50 (1): 121–7. CiteSeerX   10.1.1.466.5368 . doi:10.1002/ana.1032. PMID   11456302. S2CID   13870943.
  5. ↑ Lublin, FD (Jul 15, 2014). "Defining the clinical course of multiple sclerosis: the 2013 revisions". Neurology. 83 (3): 278–286. doi: 10.1212/WNL.0000000000000560 . PMC   4117366 . PMID   24871874.
  6. ↑ Lublin, FD (Jul 15, 2014). "Defining the clinical course of multiple sclerosis: the 2013 revisions". Neurology. 83 (3): 278–286. doi: 10.1212/WNL.0000000000000560 . PMC   4117366 . PMID   24871874.
  7. 1 2 Lublin, F. D; Reingold, S. C; Cohen, J. A; Cutter, G. R; Sorensen, P. S; Thompson, A. J; Wolinsky, J. S; Balcer, L. J; Banwell, B; Barkhof, F; Bebo, B; Calabresi, P. A; Clanet, M; Comi, G; Fox, R. J; Freedman, M. S; Goodman, A. D; Inglese, M; Kappos, L; Kieseier, B. C; Lincoln, J. A; Lubetzki, C; Miller, A. E; Montalban, X; O'Connor, P. W; Petkau, J; Pozzilli, C; Rudick, R. A; Sormani, M. P; et al. (2014). "Defining the clinical course of multiple sclerosis: The 2013 revisions". Neurology. 83 (3): 278–86. doi:10.1212/WNL.0000000000000560. PMC   4117366 . PMID   24871874.
  8. ↑ Dupont, Anne-Claire; Largeau, Bérenger; Santiago Ribeiro, Maria; Guilloteau, Denis; Tronel, Claire; Arlicot, Nicolas (2017). "Translocator Protein-18 kDa (TSPO) Positron Emission Tomography (PET) Imaging and Its Clinical Impact in Neurodegenerative Diseases". International Journal of Molecular Sciences. 18 (4): 785. doi: 10.3390/ijms18040785 . PMC   5412369 . PMID   28387722.
  9. ↑ Polman, Chris H; Reingold, Stephen C; Banwell, Brenda; Clanet, Michel; Cohen, Jeffrey A; Filippi, Massimo; Fujihara, Kazuo; Havrdova, Eva; Hutchinson, Michael; Kappos, Ludwig; Lublin, Fred D; Montalban, Xavier; O'Connor, Paul; Sandberg-Wollheim, Magnhild; Thompson, Alan J; Waubant, Emmanuelle; Weinshenker, Brian; Wolinsky, Jerry S (2011). "Diagnostic criteria for multiple sclerosis: 2010 Revisions to the McDonald criteria". Annals of Neurology. 69 (2): 292–302. doi:10.1002/ana.22366. PMC   3084507 . PMID   21387374.
This page is based on this Wikipedia article
Text is available under the CC BY-SA 4.0 license; additional terms may apply.
Images, videos and audio are available under their respective licenses.