Introduction
Optic neuromyelitis(neuro-optic myelitis, NOM) is an acute or subacute demyelinating lesion that occurs simultaneously or sequentially in the optic nerve and spinal cord. Devic (1894) reviewed 16 cases and one of his deaths, describing the clinical features of NOM as monocular or binocular blindness with acute or subacute onset, with several days or weeks before or after Transverse or ascending myelitis, which was later called Devic disease or Devic syndrome.
Cause
(1) Causes of the disease
The etiology and pathogenesis of NOM are still unclear. NOM has long been considered a clinical subtype of MS. Caucasians have ethnic susceptibility to MS, mainly brain stem lesions; non-whites are susceptible to NOM, with optic nerve and spinal cord lesions being the most common. This may be related to genetic quality and ethnic differences.
NOM is a serious monophasic course disease, but many cases are relapsing courses.
(two) pathogenesis
Acute MS can show the involvement of the optic nerve and the spinal cord. About 25% of MS patients have sudden posterior optic neuritis as the initial symptom. The relationship between NOM and MS remains to be elucidated. Wingerchuk et al (1999) described the disease spectrum, clinical index events (in the case of optic neuritis and myelitis), CSF and serology, MRI features and long-term disease assessment in 71 patients with NOM, and found clinical course of NOM, cerebrospinal fluid And neuroimaging features are different from MS.
The pathological changes of NOM are demyelination, sclerosing plaque and necrosis, with perivascular inflammatory cell infiltration. Unlike the classic MS, the lesion mainly involves the optic nerve, optic chiasm and spinal cord (thoracic and cervical segments), destructive lesions are obvious, spinal cord necrosis and eventually cavity formation, glial cell proliferation is not significant. Necrosis may reflect the severity of the inflammatory process and is not the essence of the disease.
symptom
1. The age of onset is 5 to 60 years old. 21 to 41 years old, there are many children, both men and women can be affected. Acute transverse or disseminated myelitis and bilateral optic neuritis (ON) are characteristic features of this disease, which occur continuously in a short period of time, leading to paraplegia and blindness, and the disease progresses rapidly. Relief - recurrence.
2. Patients with acute optic neuritis have partial or total loss of monocular vision within a few hours or days. Some patients have intraorbital pain one or two days before vision loss, eye movement or compression, and optic discitis or posterior Optic neuritis. Symptoms reached a peak within 1 to 2 months of subacute onset. A small number of patients with chronic onset, vision loss progressed steadily within a few months, progressively worse.
3. Acute transverse myelitis is an acute progressive inflammatory demyelinating disease of the spinal cord. It has been confirmed that most of them are MS manifestations, which are monophasic or chronic multiphasic recurrence. Common clinical disseminated myelitis, signs of asymmetry and incompleteness, rapid (Hours or Days) progression of light paraplegia, bilateral Babinski sign, trunk sensory dysfunction plane and sphincter dysfunction. Acute myelitis with Lhermitte's sign, paroxysmal tonic spasm and radiculopathy can be seen in about 1/3 of recurrent patients, but patients with single-phase disease usually rarely occur.
4. Most NOM patients have a single-phase course, 70% of the cases have paraplegia within a few days, and about half of the patients are blinded by the affected eye. A small number of patients have a recurrent disease course, about 1/3 of which have paraplegia. About 1/4 of visual acuity is involved. The clinical event interval is several months to six months. In the next 3 years, isolated ON and myelitis can be recurred multiple times.
Diagnostic basis: According to the patient's acute transverse or disseminated myelitis, and the clinical manifestations of simultaneous or sequential optic neuritis on both sides, combined with MRI showed optic nerve and spinal cord lesions, visual evoked potential abnormalities, increased CSF-IgG index and appearance Oligoclonal bands and the like can make a clinical diagnosis.
diagnosis
1. Early eye symptoms are easily confused with simple posterior optic neuritis. ON often damages one eye. This disease is often affected by both eyes, and there is spinal cord lesion or obvious relief-recurrence.
2. MS can express the clinical model of NOM, CSF and MRI examinations are quite discriminative. NOM CSF-MNC> 50 × 106 / L or neutrophilia is more common, MS is rare; more than 90% of MS can see oligoclonal bands, NOM is not common. Head MRI is normal in the early stage of NOM, recurrent-remission type MS often has typical lesions; NOM spinal cord longitudinal fusion lesions more than 3 vertebral segments, common spinal cord swelling and tendon enhancement, MS spinal cord lesions rarely exceed 1 spinal segment.
3. Subacute spinal optic neuropathy is more common in children, first with abdominal pain, diarrhea and other symptoms, mainly sympathetic paresthesia, more no convulsions, no recurrence, no significant changes in CSF.
complication
Depending on the condition, or the extent of the disease, the symptoms and signs that appear may be manifestations of the disease itself or as complications (see Clinical Performance). In addition, attention should be paid to secondary lung infections, urinary tract infections, hemorrhoids, and falls caused by decreased vision.
treatment
(a) treatment
1. Methylprednisolone(Methylprednisolone) high-dose shock therapy can accelerate the recovery of episodes such as ON, and terminate or shorten the deterioration of NOM. 500 ~ 1000mg / d, intravenous drip, for 3 to 5 days; afterwards with large dosesPrednisoneoral. It should be noted that oral prednisone alone may increase the risk of new onset of ON.
2. Clinical trials have shown that about half of patients with ineffective corticosteroid treatment can improve symptoms by plasma exchange.
(two) prognosis
The clinical manifestations of NOM are more serious than those of MS, and NOM is often aggravated by a series of episodes. Relapsing NOM has a poor prognosis. Most patients have a stepwise progression, with severe disability such as blind or paraplegia, and one third of patients die of respiratory failure, which is not common in MS.
prevention
There is no effective prevention method for autoimmune diseases, and prevention of infection, cold, and induced factors such as cold or hot is the focus of prevention and treatment; prevention and treatment of complications is also an important part of clinical medical care.
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