Demyelination
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Demyelination is incorrectly often equated to multiple sclerosis, whereas in reality it is a generic pathological term simply describing, as the word suggests, the loss of normal myelin around axons in the central nervous system. This should be distinguished from dysmyelination where the formation of normal myelin is absent.
Primary
Primary demyelinating disorders include:
-
clinically isolated syndrome (CIS)
- first symptomatic episode which may or may not progress to MS
-
multiple sclerosis (MS)
- can only be diagnosed if the McDonald diagnostic criteria (or accepted alternative) are met
- variants include
- tumefactive multiple sclerosis
- acute malignant Marburg type
- neuromyelitis optica (Devic disease)
- Schilder type (diffuse cerebral sclerosis)
- Balo concentric sclerosis (BCS)
-
acute disseminated encephalomyelitis (ADEM)
- monophasic usually post viral acute demyelination
- often considered a secondary form of demyelination
- transverse myelitis
- chronic inflammatory demyelinating polyneuropathy (CIDP)
- Guillain
-Barre Syndrome-Barré syndrome
Infective
- progressive multifocal leukoencephalopathy (PML)
- HIV encephalitis / HIV dementia complex / HIV associated myelopathy
- progressive rubella panencephalitis
In most cases, toxic and metabolic disease which affect the white matter are considered separately but for the sake of completeness, they are listed below.
Toxic
- osmotic demyelination syndrome
- posterior reversible encephalopathy syndrome (PRES)
- chemotherapy
Metabolic/genetic
- leukodystrophies
- sometimes classified as leukodystrohpies 2
Ischaemic
Although not referred to as demyelination many processes which cause ischaemia lead to demyelination:
- deep white matter ischaemia
- radiotherapy changes
-<li>can only be diagnosed if <a href="/articles/mcdonald-diagnostic-criteria-for-multiple-sclerosis-2017-current">McDonald diagnostic criteria</a> (or accepted alternative) are met</li>- +<li>can only be diagnosed if the <a href="/articles/mcdonald-diagnostic-criteria-for-multiple-sclerosis-2017-current">McDonald diagnostic criteria</a> (or accepted alternative) are met</li>
-<a href="/articles/chronic-inflammatory-demyelinating-polyneuropathy-cipd">chronic inflammatory demyelinating polyneuropathy</a> (CIDP)</li>-<li><a href="/articles/guillain-barre-syndrome-2">Guillain-Barre Syndrome</a></li>- +<a href="/articles/chronic-inflammatory-demyelinating-polyneuropathy">chronic inflammatory demyelinating polyneuropathy</a> (CIDP)</li>
- +<li><a href="/articles/guillain-barre-syndrome-2">Guillain-Barré syndrome</a></li>
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Case 53: solitary splenium lesion
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