Demyelinating syndrome
Michael Y. Wang, Andrea L. Strayer, Odette A. Harris, Cathy M. Rosenberg, Praveen V. Mummaneni in Handbook of Neurosurgery, Neurology, and Spinal Medicine for Nurses and Advanced Practice Health Professionals, 2017
Two other disease courses have been described that were both present in our clinical case. These are disease courses seen prior to the diagnosis of CDMS. Clinically Isolated Syndrome (CIS): CIS is the first clinical presentation of RRMS and usually presents as a syndrome: unilateral optic neuritis, transverse myelitis, or a brainstem attack (Miller et al., 2005). Patients with CIS and an abnormal brain MRI are likely to develop clinically definite MS (CDMS) (Fisniku et al., 2008).Radiologically Isolated Syndrome (RIS): May be considered a presymptomatic phase of MS, usually an incidental image finding highly suggestive of an inflammatory demyelinating disease, in the absence of clinical signs and symptoms of MS. Patients should be closely monitored (Lublin et al., 2014).
Neurological Disease
John S. Axford, Chris A. O'Callaghan in Medicine for Finals and Beyond, 2023
In practical terms, the first step is to establish whether a given neurological impairment is likely due to inflammation within the nervous system of the kind seen in MS. The history is usually of onset over a few days to a few weeks, with associated abnormalities on examination and characteristic lesions on brain or spinal cord MRI. CSF may show modest elevation of white cells, along with oligoclonal bands. With no history of previous events, this is a so-called clinically isolated syndrome. If, however, there is dissemination of inflammation in space (affecting different parts of the nervous system, either clinically or by MRI) and time (either from the history, or seeing lesions of different ages at MRI), or if there is a clinically isolated syndrome with dissemination in space (two of periventricular, cortical/juxtacortical, infratentorial brain or spinal cord) and CSF oligoclonal bands, then a diagnosis of MS can be made.
Oxidative Stress and Inflammation
Abhai Kumar, Debasis Bagchi in Antioxidants and Functional Foods for Neurodegenerative Disorders, 2021
MS is a common chronic inflammatory and neurodegenerative disease of the CNS (Sethi et al., 2009). It is often characterized by the accumulation of focal plaques or demyelinating lesions throughout the CNS, infiltration of immune cells, progressive axonal loss, and neuronal death. It impairs the sensory, motor, and cognitive abilities (Di Bari et al., 2017). Currently, four phenotypes of MS depending on the severity of progression have been described. In the initial phase, characterized by clinically isolated syndrome and the relapsing-remitting MS (RRMS) caused by neuroinflammation and myelin sheath damage, patients undergo reversible episodes of neurological deficits or relapses followed by long periods of remission. In the later stages or primary progressive MS (PPMS), the disease progresses from the onset with no or occasionally minor remissions and improvements. Over time, the disease advances to the secondary progressive MS (SPMS) stage where gradual worsening of the neurological deficits and clinical disabilities occur (Filippi et al., 2018; Macaron and Ontaneda, 2019).
Sequencing of disease-modifying therapies for relapsing–remitting multiple sclerosis: a theoretical approach to optimizing treatment
Published in Current Medical Research and Opinion, 2018
Francois Grand’Maison, Michael Yeung, Sarah A. Morrow, Liesly Lee, Francois Emond, Brian J. Ward, Pierre Laneuville, Robyn Schecter
Clinically isolated syndrome (CIS) is the first neurologic attack caused by inflammation or demyelination in the central nervous system (CNS). Individuals who experience CIS may or may not go on to develop MS. At 2 years, the risk of conversion to clinically definite MS and development of subsequent brain lesions in subjects with CIS was reduced in the range of 44% to 50% by IFNs and GA11. Platform DMTs such as IFNs and GA are indicated for subjects who have experienced a first attack and are at a high risk of recurrent attacks. IFNs and GA primarily influence the balance of pro-inflammatory and anti-inflammatory signaling pathways in RRMS; however, the specific MoA remains poorly understood12. In subjects with RRMS, IFNs and GA have reduced annualized relapse rate (ARR) in the range of 18% to 36% versus placebo13. Minocycline (not approved) appears to have similar efficacy14. Of the available oral drugs and monoclonal antibodies, only teriflunomide has been tested in CIS and demonstrated significant benefits versus placebo at both 7 mg and 14 mg doses; however, it is not approved for this indication15.
Glial cells as therapeutic targets in progressive multiple sclerosis
Published in Expert Review of Neurotherapeutics, 2019
Brandon Wilbanks, LJ Maher, Moses Rodriguez
MS diagnosis is based on McDonald criteria, last updated in 2017, which combines consideration of several clinical factors [5]. These include the number of clinically defined attacks, number of gadolinium-enhancing lesions observed by magnetic resonance imagining (MRI), spacial dissemination of lesions, temporal dissemination of clinical attacks, presence of oligoclonal bands in cerebrospinal fluid (CSF), and others. Disease is categorized among one of four subtypes: 1) clinically isolated syndrome (CIS), 2) relapsing-remitting (RRMS), 3) secondary progressive (SPMS), or 4) primary progressive (PPMS) [6]. CIS is the most recently defined MS disease course, characterized by an early presentation of disease that has not yet disseminated. Subsequent development of disease over time leads to further classification among the remaining three types, with over 80% of cases categorized as RRMS. This most common form is manifested as a series of onset and remission of symptoms, but approximately 90% of patients develop into SPMS as disease course begins to worsen. Finally, about 10% of patients are diagnosed with PPMS, which is identified by continuous worsening of disease following the onset of symptoms. In instances of SPMS or PPMS, demyelination is not reversed, and symptoms will compound with worsening disability.
Understanding and managing autonomic dysfunction in persons with multiple sclerosis
Published in Expert Review of Neurotherapeutics, 2021
Ivan Adamec, Magdalena Krbot Skorić, Mario Habek
One of the most important characteristics of early stages of relapsing remitting MS is that symptoms may fluctuate over time. Similarly changes in cardiovascular AD in pwMS occur during the course of the disease. In a group of 121 people diagnosed with clinically isolated syndrome followed over two years 47.3% had either worsening or improvement in sympathetic adrenergic function while in 90% of people with clinically isolated syndrome there was no change in parasympathetic nervous system tests [24]. The total number of T2 lesions was found to be an independent predictor for worsening of symptomatic dysautonomia. There is also a difference in cardiovascular AD burden in patients with relapsing remitting and progressive types of MS. Patients with progressive types of MS have a higher autonomic symptom burden and have more pronounced AD on formal testing [25,26]. It appears that there is a two-way interaction between AD and MS activity as autonomic symptom burden seems to predict disease activity in early multiple sclerosis [27]. Furthermore, patients with clinically isolated syndrome who have PoTS convert earlier to clinically definite MS [28].
Related Knowledge Centers
- Central Nervous System
- Inflammation
- Lesion
- Neurology
- Nerve
- Signs & Symptoms
- Multiple Sclerosis
- Retrospective Diagnosis
- Neuromyelitis Optica Spectrum Disorder
- Medical Diagnosis