Clinically isolated syndrome

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.

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] 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."^[3] 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).

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

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)^[4]

References

↑ "Clinically Isolated Syndrome (CIS)". Library. Retrieved 2007-10-27.
↑ Cramer SP, Modvig S, Simonsen HJ, Frederiksen JL, Larsson HB. Permeability of the blood-brain barrier predicts conversion from optic neuritis to multiple sclerosis. Brain. 2015 Jul 17. pii: awv203. doi:10.1093/brain/awv203 PMID 26187333
↑ McDonald, WI; Compston, A; Edan, G; Goodkin, D; Hartung, HP; Lublin, FD; McFarland, HF; Paty, DW; Polman, CH (2001). "McDonald criteria". Annals of Neurology. 50 (1): 121–127. doi:10.1002/ana.1032. PMID 11456302.
↑ Lublin; et al. (Jul 2014). "revisions". Neurology. 83 (3): 278–86. doi:10.1212/WNL.0000000000000560. PMID 24871874.

External links

Multiple sclerosis and other demyelinating diseases of CNS (G35–G37, 340–341)

Signs and symptoms	Ataxia Depression Diplopia Dysarthria Dysphagia Fatigue Incontinence Neurological fatigue Nystagmus Optic neuritis Pain Uhthoff's phenomenon Dawson's fingers

Diagnosis and evolution following	Diagnostic criteria for MS McDonald criteria Poser criteria EDSS Clinically isolated syndrome

Investigation	Pathophysiology Experimental autoimmune encephalomyelitis Multiple sclerosis drug pipeline Multiple sclerosis research

Approved Treatment	Interferon beta 1b Glatiramer acetate Interferon beta 1a Mitoxantrone Natalizumab Fingolimod Teriflunomide Dimethyl fumarate Alemtuzumab Daclizumab

Borderline forms	Acute disseminated encephalomyelitis Balo concentric sclerosis Neuromyelitis optica Marburg multiple sclerosis Schilder's disease Tumefactive multiple sclerosis (Autoimmune PNS diseases like Guillain-Barré syndrome and CIDP are normally set apart) AntiMOG associated encephalomyelitis

Other	List of people with multiple sclerosis List of multiple sclerosis organizations

Pathology of the nervous system, primarily CNS (G04–G47, 323–349)

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Brain	Encephalitis Viral encephalitis Herpesviral encephalitis Limbic encephalitis Encephalitis lethargica Cavernous sinus thrombosis Brain abscess Amoebic

Spinal cord	Myelitis: Poliomyelitis Demyelinating disease Transverse myelitis Tropical spastic paraparesis Epidural abscess

Both/either	Encephalomyelitis Acute disseminated Myalgic Meningoencephalitis

Brain/
encephalopathy

Degenerative

Extrapyramidal and movement disorders	Basal ganglia disease Parkinsonism PD Postencephalitic NMS PKAN Tauopathy PSP Striatonigral degeneration Hemiballismus HD OA Dyskinesia Dystonia Status dystonicus Spasmodic torticollis Meige's Blepharospasm Athetosis Chorea Choreoathetosis Myoclonus Myoclonic epilepsy Akathisia Tremor Essential tremor Intention tremor Restless legs Stiff person

Dementia	Tauopathy Alzheimer's Early-onset Primary progressive aphasia Frontotemporal dementia/Frontotemporal lobar degeneration Pick's Dementia with Lewy bodies Posterior cortical atrophy Vascular dementia

Mitochondrial disease	Leigh disease

Demyelinating

Episodic/
paroxysmal

Seizure/epilepsy	Focal Generalised Status epilepticus Myoclonic epilepsy

Headache	Migraine Familial hemiplegic Cluster Tension

Cerebrovascular	TIA Amaurosis fugax Transient global amnesia Acute aphasia Stroke MCA ACA PCA Foville's Millard–Gubler Lateral medullary Weber's Lacunar stroke

Sleep disorders	Insomnia Hypersomnia Sleep apnea Obstructive Congenital central hypoventilation syndrome Narcolepsy Cataplexy Kleine–Levin Circadian rhythm sleep disorder Advanced sleep phase disorder Delayed sleep phase disorder Non-24-hour sleep–wake disorder Jet lag

CSF

Other

Spinal cord/
myelopathy

Both/either

Degenerative

SA	Friedreich's ataxia Ataxia telangiectasia

MND	UMN only: Primary lateral sclerosis Pseudobulbar palsy Hereditary spastic paraplegia LMN only: Distal hereditary motor neuronopathies Spinal muscular atrophies SMA SMAX1 SMAX2 DSMA1 Congenital DSMA SMA-PCH SMA-LED SMA-PME Progressive muscular atrophy Progressive bulbar palsy Fazio–Londe Infantile progressive bulbar palsy both: Amyotrophic lateral sclerosis

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