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32693004: Demyelination (morphologic abnormality)

Status: current, Not sufficiently defined by necessary conditions definition status (core metadata concept). Date: 31-Jan 2002. Module: SNOMED CT core

Descriptions:

Id Description Lang Type Status Case? Module
54573012 Demyelination en Synonym (core metadata concept) Active Entire term case insensitive (core metadata concept) SNOMED CT core
54575017 Myelinolysis en Synonym (core metadata concept) Active Entire term case insensitive (core metadata concept) SNOMED CT core
54576016 Myelinoclasis en Synonym (core metadata concept) Active Entire term case insensitive (core metadata concept) SNOMED CT core
764006015 Demyelination (morphologic abnormality) en Fully specified name Active Entire term case insensitive (core metadata concept) SNOMED CT core

2 descendants. Search Descendants:

Expanded Value Set

Outbound Relationships Type Target Active Characteristic Refinability Group Values
Demyelination Is a dégénérescence false Inferred relationship Some
Demyelination Is a Degenerative abnormality true Inferred relationship Some
Inbound Relationships Type Active Source Characteristic Refinability Group
Chronic inflammatory demyelinating polyradiculoneuropathy Associated morphology False Demyelination Inferred relationship Some 2
Subacute inflammatory demyelinating polyradiculoneuropathy Associated morphology False Demyelination Inferred relationship Some 2
polyneuropathie démyélinisante inflammatoire chronique Associated morphology False Demyelination Inferred relationship Some 2
Peripheral demyelinating neuropathy (disorder) Associated morphology True Demyelination Inferred relationship Some 1
Guillain-Barré syndrome Associated morphology True Demyelination Inferred relationship Some 1
A new leukoencephalopathy, the CACH syndrome (Childhood Ataxia with Central nervous system Hypomyelination) or VWM (Vanishing White Matter) was identified on clinical and MRI criteria. Classically, this disease is characterized by onset between 2 and 5 years of age, with a cerebello-spastic syndrome exacerbated by episodes of fever or head trauma leading to death after 5 to 10 years of disease evolution, a diffuse involvement of the white matter on cerebral MRI with a CSF-like signal intensity (cavitation), a recessive autosomal mode of inheritance, neuropathologic findings consistent with a cavitating orthochromatic leukodystrophy with increased number of oligodendrocytes with sometimes foamy aspect. Associated morphology False Demyelination Inferred relationship Some 1
Demyelinating sensorimotor neuropathy Associated morphology True Demyelination Inferred relationship Some 1
Subacute inflammatory demyelinating polyradiculoneuropathy Associated morphology True Demyelination Inferred relationship Some 2
Chronic inflammatory demyelinating polyradiculoneuropathy Associated morphology True Demyelination Inferred relationship Some 2
A first neurologic episode caused by inflammation/demyelination of one or more central nervous system sites that lasts at least 24 hours. Associated morphology False Demyelination Inferred relationship Some 1
A new leukoencephalopathy, the CACH syndrome (Childhood Ataxia with Central nervous system Hypomyelination) or VWM (Vanishing White Matter) was identified on clinical and MRI criteria. Classically, this disease is characterized by onset between 2 and 5 years of age, with a cerebello-spastic syndrome exacerbated by episodes of fever or head trauma leading to death after 5 to 10 years of disease evolution, a diffuse involvement of the white matter on cerebral MRI with a CSF-like signal intensity (cavitation), a recessive autosomal mode of inheritance, neuropathologic findings consistent with a cavitating orthochromatic leukodystrophy with increased number of oligodendrocytes with sometimes foamy aspect. Associated morphology False Demyelination Inferred relationship Some 1
A first neurologic episode caused by inflammation/demyelination of one or more central nervous system sites that lasts at least 24 hours. Associated morphology True Demyelination Inferred relationship Some 1
polyneuropathie inflammatoire démyélinisante aigüe Associated morphology False Demyelination Inferred relationship Some 1
Acute disseminated encephalomyelitis following infectious disease (disorder) Associated morphology True Demyelination Inferred relationship Some 6
Acute disseminated encephalomyelitis following infectious disease (disorder) Associated morphology False Demyelination Inferred relationship Some 9
Subcortical leukoencephalopathy Associated morphology True Demyelination Inferred relationship Some 3
Acute disseminated encephalomyelitis Associated morphology True Demyelination Inferred relationship Some 2
Acute hemorrhagic leukoencephalitis Associated morphology False Demyelination Inferred relationship Some 5
Acute disseminated encephalomyelitis following infectious disease (disorder) Associated morphology True Demyelination Inferred relationship Some 1
Leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation Associated morphology False Demyelination Inferred relationship Some 3
Multifocal clinically isolated syndrome Associated morphology True Demyelination Inferred relationship Some 1
Monofocal clinically isolated syndrome Associated morphology True Demyelination Inferred relationship Some 1
Progressive multifocal leucoencephalopathy co-occurrent with human immunodeficiency virus infection Associated morphology False Demyelination Inferred relationship Some 4
Demyelinating disease of central nervous system co-occurrent with human immunodeficiency virus infection Associated morphology True Demyelination Inferred relationship Some 2
A rare pure motor axonal form of Guillain-Barré syndrome (GBS). Associated morphology True Demyelination Inferred relationship Some 3
A rare chronic immune-mediated polyneuropathy characterized by a progressive disabling neuropathy with marked gait disturbance primarily due to sensory ataxia with concurrent cranial neuropathies (internal or external ophthalmoplegia, dysphagia, dysarthria, or facial weakness) and anti-disialosyl IgM antibodies. Associated morphology True Demyelination Inferred relationship Some 4
A demyelinating polyneuropathy characterized clinically by sensory ataxia, tremor, paresthesia, and impaired gait. Associated morphology True Demyelination Inferred relationship Some 1
A rare motor-sensory, axonal form of Guillain-Barré syndrome (GBS). Associated morphology True Demyelination Inferred relationship Some 3
A rare inflammatory neuropathy belonging to the clinical spectrum of Guillain-Barré syndrome (GBS). Associated morphology True Demyelination Inferred relationship Some 3
This syndrome is characterized by the association of sensorineural hearing impairment and peripheral neuropathy. Associated morphology True Demyelination Inferred relationship Some 4
White matter disease (disorder) Associated morphology True Demyelination Inferred relationship Some 1
Epilepsy co-occurrent and due to demyelinating disorder (disorder) Associated morphology True Demyelination Inferred relationship Some 4
Progressive relapsing multiple sclerosis (disorder) Associated morphology True Demyelination Inferred relationship Some 1
Demyelination of central nervous system due to Behcet disease (disorder) Associated morphology True Demyelination Inferred relationship Some 2
Demyelination of central nervous system co-occurrent and due to mitochondrial disease (disorder) Associated morphology True Demyelination Inferred relationship Some 2
Demyelination of central nervous system co-occurrent and due to systemic lupus erythematosus (disorder) Associated morphology True Demyelination Inferred relationship Some 4
Demyelination of central nervous system co-occurrent and due to Sjogren disease (disorder) Associated morphology True Demyelination Inferred relationship Some 5
Demyelination of central nervous system co-occurrent and due to neurosarcoidosis (disorder) Associated morphology True Demyelination Inferred relationship Some 6
Multiple sclerosis-ichthyosis-factor VIII deficiency syndrome is characterized by the association of multiple sclerosis with lamellar ichthyosis and hematological anomalies (beta thalassemia minor and a quantitative deficit of factor VIII-von Willebrand complex). Other clinical manifestations may include eye involvement (optic atrophy, diplopia), neuromuscular involvement (ataxia, pyramidal syndrome, gait disturbance) and sensory disorder. There have been no further descriptions in the literature since 1992. Associated morphology True Demyelination Inferred relationship Some 5
White matter disorder due to nutritional deficiency (disorder) Associated morphology True Demyelination Inferred relationship Some 2
Dementia co-occurrent and due to progressive multifocal leukoencephalopathy (disorder) Associated morphology True Demyelination Inferred relationship Some 2
Demyelination due to systemic vasculitis (disorder) Associated morphology True Demyelination Inferred relationship Some 2
Peripheral demyelinating neuropathy-central dysmyelinating leucodystrophy-Waardenburg syndrome-Hirschsprung disease (PCWH) is a systemic disease characterised by the association of the features of Waardenburg-Shah syndrome (WSS) with neurological features of variable severity. Associated morphology True Demyelination Inferred relationship Some 1
Marburg acute multiple sclerosis is a rare variant of multiple sclerosis characterized by a rapidly progressive, aggressive form of multiple sclerosis with numerous large multifocal demyelinating lesions in deep white matter on cerebral MRI that usually leads to severe disability or death within weeks to months without remission. A relapsing form of multiple sclerosis is observed in surviving patients. Associated morphology True Demyelination Inferred relationship Some 1
A rare variant of Guillain-Barré syndrome characterized by acute onset monophasic sensory neuropathy with diminished or absent tendon reflexes, loss of proprioception, positive Romberg sign and nerve conduction features of demyelination. It presents several weeks after acute infection with paresthesias, ataxia and neuropathic pain. Associated morphology True Demyelination Inferred relationship Some 2
Paraparetic variant of Guillain-Barré syndrome is a rare variant of Guillain-Barré syndrome characterized by isolated leg weakness, areflexia and radicular leg pain that may simulate a cauda equina or spinal cord syndrome. The arms, ocular, facial, and oropharyngeal muscles are spared, and sphincteric function is normal. Associated morphology True Demyelination Inferred relationship Some 2
Leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation Associated morphology True Demyelination Inferred relationship Some 1
Multiple sclerosis-ichthyosis-factor VIII deficiency syndrome is characterized by the association of multiple sclerosis with lamellar ichthyosis and hematological anomalies (beta thalassemia minor and a quantitative deficit of factor VIII-von Willebrand complex). Other clinical manifestations may include eye involvement (optic atrophy, diplopia), neuromuscular involvement (ataxia, pyramidal syndrome, gait disturbance) and sensory disorder. There have been no further descriptions in the literature since 1992. Associated morphology True Demyelination Inferred relationship Some 1
Progressive multifocal leucoencephalopathy co-occurrent with human immunodeficiency virus infection Associated morphology True Demyelination Inferred relationship Some 1
Radiologically isolated syndrome Associated morphology True Demyelination Inferred relationship Some 1
A rare, acquired, demyelinating neuropathy disease characterized by acute, symmetric, monophasic sensory neuropathy without motor involvement, typically manifesting with numbness in the distal lower limbs which progressively extends to all the limb, tingling sensation in the distal lower limbs, generalized areflexia, and unsteady gait, as well as clumsiness of the upper limbs, pseudoathetosis and loss of vibration sense. Associated morphology True Demyelination Inferred relationship Some 2
Pharyngeal-cervical-brachial variant of Guillain-Barré syndrome is a rare, acquired peripheral neuropathy disease characterized by rapidly progressive oropharyngeal (facial palsy, dysarthria) and cervicobrachial weakness, associated with upper limb weakness and hypo/areflexia, in the absence of ophthalmoplegia, ataxia, altered consciousness, and prominent lower limb weakness. The presence of monospecific IgG anti-GT1a antibodies is associated. Associated morphology True Demyelination Inferred relationship Some 2
Acute disseminated encephalomyelitis following infectious disease (disorder) Associated morphology False Demyelination Inferred relationship Some 7
Neuromyelitis optica Associated morphology True Demyelination Inferred relationship Some 1
Acute hemorrhagic leukoencephalitis Associated morphology False Demyelination Inferred relationship Some 1
Marburg acute multiple sclerosis is a rare variant of multiple sclerosis characterized by a rapidly progressive, aggressive form of multiple sclerosis with numerous large multifocal demyelinating lesions in deep white matter on cerebral MRI that usually leads to severe disability or death within weeks to months without remission. A relapsing form of multiple sclerosis is observed in surviving patients. Associated morphology True Demyelination Inferred relationship Some 3
Progressive multiple sclerosis Associated morphology True Demyelination Inferred relationship Some 2
Acute hemorrhagic leukoencephalitis Associated morphology True Demyelination Inferred relationship Some 7
Van Bogaert's sclerosing leukoencephalitis Associated morphology True Demyelination Inferred relationship Some 1
A rare, localized variant of Guillain-Barré syndrome characterized by rapidly progressive bilateral facial nerve palsy, distal paresthesias, and minimal or no motor weakness. Deep tendon reflexes are usually diminished or absent but can be present or even exaggerated in rare cases. CSF analysis may reveal albuminocytologic dissociation. Nerve conduction velocity studies often show demyelinating type of neuropathy, although axonal polyneuropathy has been also described. Associated morphology True Demyelination Inferred relationship Some 1
Osmotic demyelination syndrome (disorder) Associated morphology True Demyelination Inferred relationship Some 1
Pediatric multiple sclerosis (MS) is a rare multiple sclerosis variant characterized by the onset of multiple sclerosis (i.e. one or multiple episodes of clinical CNS symptoms consistent with acquired CNS demyelination, with radiologically proven dissemination of inflammatory lesions in space and time, following exclusion of other disorders) before the age of 18 years old. Pediatric MS patients present a predominantly relapsing-remitting course with first attack usually consisting of optic neuritis, transverse myelitis, acute disseminated encephalomyelitis and monofocal or polyfocal neurological deficits. A high burden of T2-hyperintense lesions on initial MRI, primarily of the supratentorial region and/or of the cervical spinal cord, has been reported. Associated morphology True Demyelination Inferred relationship Some 1
Myelin oligodendrocyte glycoprotein antibody-associated disease Associated morphology True Demyelination Inferred relationship Some 1
Brachial variant of chronic immune demyelinating polyradiculoneuropathy (disorder) Associated morphology True Demyelination Inferred relationship Some 1
Demyelination of central nervous system due to Whipple disease (disorder) Associated morphology True Demyelination Inferred relationship Some 1
Demyelination of central nervous system due to Lyme borreliosis (disorder) Associated morphology True Demyelination Inferred relationship Some 1
Autonomic disorder due to multiple sclerosis Associated morphology True Demyelination Inferred relationship Some 3
Chronic inflammatory demyelinating disease of peripheral nervous system and central nervous system (disorder) Associated morphology True Demyelination Inferred relationship Some 1
Clinically isolated syndrome of brainstem (disorder) Associated morphology True Demyelination Inferred relationship Some 1
Clinically isolated syndrome of cerebellum Associated morphology True Demyelination Inferred relationship Some 1
Chronic inflammatory demyelinating disease of peripheral nervous system and central nervous system (disorder) Associated morphology True Demyelination Inferred relationship Some 5
Acquired demyelinating disorder of peripheral nerve (disorder) Associated morphology True Demyelination Inferred relationship Some 1
White matter disease due to microglioma (disorder) Associated morphology True Demyelination Inferred relationship Some 1
White matter disease due to anti-phospholipid syndrome (disorder) Associated morphology True Demyelination Inferred relationship Some 1
Fisher's syndrome Associated morphology True Demyelination Inferred relationship Some 7
A rare genetic neurological disorder with characteristics of childhood to adolescence onset of progressive demyelination occurring in episodes, sensorimotor polyneuropathy, and hearing loss. Disease progression and severity is variable. In general, in an increasing and decreasing course, patients eventually develop respiratory insufficiency, loss of motor skills and ambulation, ataxia, and cognitive decline. Vision problems and skin rashes are commonly reported. Associated morphology True Demyelination Inferred relationship Some 1
Posterior cord syndrome due to multiple sclerosis (disorder) Associated morphology True Demyelination Inferred relationship Some 1
Brown-Séquard syndrome due to multiple sclerosis Associated morphology True Demyelination Inferred relationship Some 1

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