Autoimmunity of the nervous system

2013 ◽  
pp. 187-200
Author(s):  
Gavin P Spickett
Keyword(s):  
Multiple Sclerosis ◽  
Nervous System ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Demyelinating Diseases ◽  
Stiff Person Syndrome ◽  
Rasmussen Encephalitis ◽  
System P ◽  
Morvan’S Syndrome ◽  
Inflammatory Demyelinating Polyneuropathy ◽  
Myasthenic Syndrome

Myasthenia gravis (MG) Lambert–Eaton myasthenic syndrome (LEMS) Acquired neuromyotonia (Isaac’s disease) and stiff person syndrome (SPS) Morvan’s syndrome Rasmussen encephalitis PANDAS Paraneoplastic autoimmune neurological syndromes Demyelinating diseases 1: multiple sclerosis (MS) Demyelinating diseases 2: Guillain–Barré syndrome (GBS) and variants Demyelinating diseases 3: chronic inflammatory demyelinating polyneuropathy (CIDP), related conditions, and Devic’s syndrome (neuromyelitis optica)...

Demyelinating disorders of the central nervous system

2010 ◽  
pp. 4948-4963
Author(s):  
Siddharthan Chandran ◽  
Alastair Compston
Keyword(s):  
Multiple Sclerosis ◽  
Central Nervous System ◽  
Nervous System ◽  
Acute Disseminated Encephalomyelitis ◽  
Demyelinating Diseases ◽  
Demyelinating Disorder ◽  
System P ◽  
The Central Nervous System ◽  
Demyelinating Disorders ◽  
Brain And Spinal Cord

Clinicians suspect demyelination when episodes reflecting damage to white matter tracts within the central nervous system occur in young adults. The paucity of specific biological markers of discrete demyelinating syndromes places an emphasis on clinical phenotype—temporal and spatial patterns—when classifying demyelinating disorders. The diagnosis of multiple sclerosis, the most common demyelinating disorder, becomes probable when these symptoms and signs recur, involving different parts of the brain and spinal cord. Other important demyelinating diseases include post-infectious neurological disorders (acute disseminated encephalomyelitis), demyelination resulting from metabolic derangements (central pontine myelinosis), and inherited leucodystrophies that may present in children or in adults. Accepting differences in mechanism, presentation, and treatment, two observations can usefully be made when classifying demyelinating disorders. These are the presence or absence of inflammation, and the extent of focal vs. diffuse demyelination. Multiple sclerosis is prototypic for the former, whereas dysmyelinating disorders, such as leucodystrophies are representative of the latter....

Demyelinating disorders of the central nervous system

2020 ◽  
pp. 6026-6042
Author(s):  
Alasdair Coles ◽  
Siddharthan Chandran
Keyword(s):  
Multiple Sclerosis ◽  
Central Nervous System ◽  
Nervous System ◽  
Demyelinating Disease ◽  
Demyelinating Diseases ◽  
System P ◽  
Industrialized Nations ◽  
Distinct Disease ◽  
The Central Nervous System ◽  
Demyelinating Disorders

The common feature of all of the demyelinating diseases is that, initially at least, the oligodendrocyte-myelin unit is the primary target, with the axon comparatively spared. There are a range of causes, both acquired and inherited, which this chapter explores. Multiple sclerosis, the prototypic demyelinating disorder of the central nervous system, is the leading causing of neurological disability among young adults in many industrialized nations. In the last two decades therapies have been licensed with increasing capacity to suppress the inflammation which underlies the condition, leading to durable benefits to patients. The next most prevalent demyelinating disease is neuromyelitis optica. Originally thought to be a variant of multiple sclerosis, it is now recognized to be a distinct disease whose treatment is radically different from multiple sclerosis.

scholarly journals Balò’s concentric sclerosis in a case of cocaine-levamisole abuse

2020 ◽  
Vol 8 ◽  
pp. 2050313X2094053 ◽  
Author(s):  
Daniela Grasso ◽  
Carmela Borreggine ◽  
Giulia Castorani ◽  
Doriana Vergara ◽  
Lucia Maria Cecilia Dimitri ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Multiple Sclerosis ◽  
Central Nervous System ◽  
Nervous System ◽  
Cocaine Abuse ◽  
Hemorrhagic Stroke ◽  
Demyelinating Diseases ◽  
Resonance Imaging ◽  
Radiological Findings ◽  
Baló’S Concentric Sclerosis

Baló’s concentric sclerosis is a rare variant of multiple sclerosis. It belongs to the group of primary inflammatory central nervous system demyelinating diseases having no clear etiology. Peculiar radiological findings on magnetic resonance imaging are alternating rings of demyelinated and myelinated axons resembling an “onion bulb.” We report on a case of a patient with cocaine abuse who presented with Balò’s-like acute multifocal leukoencephalopathy supported by histological and radiological findings. The abuse of cocaine and its most frequent adulterant, levamisole, may induce ischemic or hemorrhagic stroke and metabolic or multifocal inflammatory leukoencephalopathy. Only a few studies described levamisole-induced leukoencephalopathy mimicking Balò round lesions. Nevertheless, it has not yet been established the correlation between them; it might also be possible that the cocaine/levamisole addiction represents just a coincidence in some of those patients affected by Balò sclerosis disease.

scholarly journals Anti-Neurofascin 155 Antibody-Positive Chronic Inflammatory Demyelinating Polyneuropathy/Combined Central and Peripheral Demyelination: Strategies for Diagnosis and Treatment Based on the Disease Mechanism

2021 ◽  
Vol 12 ◽  
Author(s):  
Jun-ichi Kira
Keyword(s):  
Nervous System ◽  
Demyelinating Disease ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Cranial Nerves ◽  
Human Leukocyte ◽  
Demyelinating Polyneuropathy ◽  
Macrophage Infiltration ◽  
Spinal Root ◽  
Inflammatory Demyelinating Polyneuropathy ◽  
Demyelinating Lesions

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated demyelinating disease of the peripheral nervous system (PNS). A small number of CIDP patients harbors autoantibodies against nodal/paranodal proteins, such as neurofascin 155 (NF155), contactin 1, and contactin-associated protein 1. In most cases, the predominant immunoglobulin (IgG) subclass is IgG4. Node/paranode antibody-positive CIDP demonstrates distinct features compared with antibody-negative CIDP, including a poor response to intravenous immunoglobulin. The neuropathology of biopsied sural nerve shows Schwann cell terminal loop detachment from axons without macrophage infiltration or inflammation. This is partly attributable to IgG4, which blocks protein–protein interactions without inducing inflammation. Anti-NF155 antibody-positive (NF155+) CIDP is unique because of the high frequency of subclinical demyelinating lesions in the central nervous system (CNS). This is probably because NF155 coexists in the PNS and CNS. Such cases showing demyelinating lesions in both the CNS and PNS are now termed combined central and peripheral demyelination (CCPD). NF155+ CIDP/CCPD commonly presents hypertrophy of spinal nerve roots and cranial nerves, such as trigeminal and oculomotor nerves, and extremely high levels of cerebrospinal fluid (CSF) protein, which indicates nerve root inflammation. In the CSF, the CXCL8/IL8, IL13, TNFα, CCL11/eotaxin, CCL2/MCP1, and IFNγ levels are significantly higher and the IL1β, IL1ra, and GCSF levels are significantly lower in NF155+ CIDP than in non-inflammatory neurological diseases. Even compared with anti-NF155 antibody-negative (NF155−) CIDP, the CXCL8/IL8 and IL13 levels are significantly higher and the IL1β and IL1ra levels are significantly lower than those in NF155+ CIDP. Canonical discriminant analysis revealed NF155+ and NF155− CIDP to be separable with IL4, IL10, and IL13, the three most significant discriminators, all of which are required for IgG4 class switching. Therefore, upregulation of both Th2 and Th1 cytokines and downregulation of macrophage-related cytokines are characteristic of NF155+ CIDP, which explains spinal root inflammation and the lack of macrophage infiltration in the sural nerves. All Japanese patients with NF155+ CIDP/CCPD have one of two specific human leukocyte antigen (HLA) haplotypes, which results in a significantly higher prevalence of HLA-DRB1*15:01-DQB1*06:02 compared with healthy Japanese controls. This indicates an involvement of specific HLA class II molecules and relevant T cells in addition to IgG4 anti-NF155 antibodies in the mechanism underlying IgG4 NF155+ CIDP/CCPD.

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