Symptomatic characteristics
① Acute or subacute onset; ② The clinical symptoms are severe, but the imaging lesions are relatively small; ③ MRI of spinal cord showed that the focus was multicentric, the central canal of spinal cord was not obviously dilated, and the focus was patchy enhanced or not enhanced; Sometimes MRI examination of the head finds paraventricular or brainstem lesions; ④ The effect of hormone therapy is obvious; ⑤ There is a history of optic neuritis or myelitis in the past, and MRI follow-up of spinal cord is of great value to suspicious patients. The most common clinical disease is multiple sclerosis.
According to the damage degree of myelin sheath itself or other tissue structures (bystander effect), it can be divided into primary and secondary. Myeloslysis refers to the destruction of normal myelin, while myelination disorder refers to the abnormality of enzymes needed for myelin metabolism, also known as leukodystrophy. There are also classification methods of inflammatory demyelination and non-inflammatory demyelination.
1.Etiological classification 1.Sehaumburg According to the etiology, Sehaumburg divides demyelination into five categories: viral, immune, hereditary (myelination), toxic (or nutritional) and traumatic.
2.Olekmj is effective in treating autoimmune diseases, acute disseminated encephalomyelitis, acute hemorrhagic leukoencephalitis, multiple sclerosis, infection, progressive multifocal leukoencephalopathy, toxic (or metabolic), carbon monoxide poisoning, vitamin B ~ (12) deficiency, mercury poisoning (Minamata disease) and alcohol (or tobacco). Marcea's Fava-Bigger nano-syndrome, hypoxia, radiation or vascular injury, Binswanger's disease, inherited diseases of myelin metabolism, adrenal leukodystrophy, metachromatic leukodystrophy, krabbe's disease, Alexander's disease, canavan-Van disease, bogart-Bertrand disease, Pelizza-Merzbach disease, phenylketonuria, etc.
3. Allen classification (Allen ⅳ. Neuropathology in Greenfield, edward arnold. London, 1992: 447-520).
(1) Acute disseminated encephalomyelitis: classic (infectious, after vaccination, idiopathic) and hyperacute (acute hemorrhagic leukoencephalitis).
(2) Multiple sclerosis: classic type (Charcot type), acute type (Marburg type), extensive type (Schilder type), centripetal type (Balo type) and neuromyelitis optica (Devic type).
Multiple sclerosis is the most common demyelinating disease in clinic. Because of multifocal sclerosis, the clinical symptoms are complex and mixed type is not uncommon. Early symptoms usually manifest as an attack-remission process. Sometimes patients will automatically recover (or basically recover) without treatment, and then with the delay of time, the disease will enter an irreversible stage, thus causing acute attacks of multiple sclerosis. Patients may have systemic symptoms, including visual impairment, bladder-rectum and sexual dysfunction, and motor symptoms (such as muscle weakness and spasm). ), sensory symptoms (such as numbness, irritability, false feeling, etc. ), brain symptoms (such as tremor and ataxia) and other symptoms (such as fatigue, cognitive impairment and mental syndrome). ). According to the lesion site, it is generally divided into the following four types.
1. Spinal cord type mainly involves lateral bundle and posterior bundle. When a single large plaque or multiple plaques are fused, one side or a certain segment of the spinal cord can be injured, which shows semi-transverse or transverse spinal cord injury. Patients often complain of back pain first, followed by central paralysis of lower limbs, sensory impairment below the damage level, urinary retention and impotence. When the posterior bundle of cervical spinal cord is damaged, the patient's head flexion can cause radiation shock-like numbness or pain from the upper back to the lower limbs, which is Lhermitt sign. Tonic spasms and painful seizures that spontaneously and briefly spread to one side or both sides from a certain part of the trunk and limbs can also occur, which are called tonic painful spasms.
2. Optic nerve spinal cord type is also called optic neuromyelitis and Devic disease. The first symptom of this type can be optic nerve and chiasma injury, or spinal cord injury, which can be separated by months or even years. It is not uncommon for both to be damaged at the same time. The onset can be acute or slow. Patients with optic nerve injury show pain during eye movement, decreased vision or complete blindness, normal or pale optic disc, and frequent binocular injury. Optic chiasma lesions are mainly visual field defects. Patients with optic disc inflammation have obvious optic disc edema besides decreased vision. The manifestations of spinal cord injury are the same as those of spinal cord type.
3. Brain stem cerebellar type is characterized by dizziness, diplopia, nystagmus, unclear pronunciation, central or peripheral facial paralysis, pseudobulbar paralysis or paralysis, cross paralysis or hemiplegia, exercise ataxia, limb tremor and dance.
4. Brain types are mainly characterized by hemiplegia, aphasia and mental disorders, such as emotional instability, involuntary crying, paranoia, stupor and mental retardation, which are rare in clinic.