Inflammatory demyelinating diseases of the central nervous system include at least four diseases (note that vaccination, viral infections, and poisoning can also cause inflammatory demyelinating changes), including isolated syndrome, acute disseminated encephalomyelitis, multiple sclerosis, and optic neuromyelitis optica. It can be assumed that the first two diseases are the early stages of the latter two, but what exactly is it of both? It can only be gradually clarified with time extension, disease progression, and relevant auxiliary examinations. Because the pathogenesis of the latter two is different, the treatment and prognosis are also different and should be taken seriously.
Definition of isolated syndrome: It is a multifactorial clinical syndrome.
1, single or multiple inflammatory demyelinating lesions in the CNS.
2, no clinical history of CNS demyelinating disease (i.e., no optic neuritis, transverse myelitis, hemispheric and brainstem-related syndromes).
3. absence of manifestations of encephalopathy that cannot be explained by fever (absence of consciousness such as somnolence and behavior such as delirium and agitation changes).
4. MRI features that do not meet the diagnostic criteria for multiple sclerosis.
Acute disseminated encephalomyelitis definition: a multifactorial clinical syndrome.
1, first multiple CNS inflammatory demyelinating events
2, encephalopathy that cannot be explained by fever (see 3 above)
3, no new clinical or MRI changes after R3 months of onset
4, In the acute phase, there are abnormalities on MRI
5. The typical features of MRI of the brain are.
(1) The lesion mainly involves the white matter of the brain and is diffuse, poorly defined, and >1-2 cm in extent.
(2) T1 low signal changes in the white matter are rare.
(3) Deep gray matter (e.g., thalamus, basal ganglia) can be damaged.
A reoccurrence of non-febrile encephalopathy >3 months apart with symptoms, signs and MRI changes corresponding to a new or old lesion is called multitemporal acute disseminated encephalomyelitis; encephalopathy >3 episodes, possibly multiple sclerosis or optic neuromyelitis optica, is no longer called multitemporal acute disseminated encephalomyelitis.
Multiple sclerosis defined as.
1, R2 non-encephalopathy episodes of clinical CNS inflammatory demyelinating changes, >30 days apart, at >1 site.
2, one episode of non-encephalopathy presenting with typical multiple sclerosis manifestations meeting the 2010 revised McDonald MRI spatial multiple diagnostic criteria; follow-up MRI showing at least 1 new enhancing or non-enhancing lesion meeting the multiple sclerosis criteria for temporal multiple episodes.
3, non-encephalitic clinical event 3-4 months after the onset of acute disseminated encephalomyelitis, with MRI showing new lesions meeting the 2010 revised McDonald criteria for spatial multiplicity.
4, First, single, acute event that does not meet the criteria for acute disseminated encephalomyelitis, while MRI is consistent with the 2010 revised Mcdonald criteria for temporal and spatial multiplicity.
Optic neuromyelitis optica.
1, optic neuritis.
2, acute myelitis.
3, At least 2 of the following 3 supporting criteria.
(1) spinal cord MRI showing lesion length >3 vertebral segments
(2) MRI of the brain does not meet the diagnostic criteria for multiple sclerosis
(3) Positive serum anti-water channel protein-4 IgG