neuromyelitis optica

Introduction

Introduction to 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. basic knowledge The proportion of illness: 0.0003% Susceptible people: no special people Mode of infection: non-infectious Complications: urinary tract infections acne

Cause

Cause of optic neuromyelitis

Genetic quality (30%):

The etiology and pathogenesis 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. Optic nerve and spinal cord damage are most common, which may be related to genetic quality and ethnic differences.

NOM is a serious single-phase disease, but many cases have a recurrent course.

Pathogenesis

Acute MS can be associated with optic nerve and spinal cord. About 25% of MS patients have initial symptoms of sudden posterior optic neuritis. The relationship between NOM and MS remains to be elucidated. Wingerchuk et al. (1999) describe the disease spectrum of 71 patients with NOM. The characteristics of clinical index events (optical events and optic neuritis), CSF and serology, MRI features and long-term disease assessment, found that the clinical course of NOM, cerebrospinal fluid and neuroimaging characteristics are different from MS.

The pathological changes of NOM are demyelination, sclerosing plaque and necrosis, and perivascular inflammatory cell infiltration. Unlike classic MS, the lesion mainly involves the optic nerve, optic chiasm and spinal cord (thoracic and cervical segments), and 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, not the nature of the disease.

Prevention

Optic neuromyelitis prevention

There is no effective prevention method for autoimmune diseases, and prevention of infection, cold and cold or hot is the key to prevention and treatment; prevention and treatment of complications is also an important part of clinical medical care.

Complication

Optic neuromyelitis complications Complications, urinary tract infection, acne

With the development of the disease, or the degree of disease, the symptoms and signs may be manifested by the disease itself, or as a complication (see clinical manifestations). In addition, secondary lung infections, urinary tract infections, hemorrhoids, and decreased vision should be noted. Caused by a bruise and so on.

Symptom

Common symptoms of optic nerve myelitis Symptoms of spinal cord transection damage to the spinal cord intermittent breaks of the sputum-like movements, spatial function impairment, sensory dysfunction, sphincter dysfunction, posterior optic neuritis, reading small characters and difficulty in color discrimination

1. The age of onset is 5 to 60 years old, 21 to 41 years old, and there are many children, both men and women can have the disease, acute transverse or disseminated myelitis and bilateral optic neuritis (optic neuritis, ON) It is a characteristic manifestation of this disease, which occurs continuously in a short period of time, leading to paraplegia and blindness. The disease progresses rapidly and may have remission-relapse.

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 In optic neuritis, the symptoms of the subacute onset reached a peak within 1 to 2 months, and a small number of patients developed chronic onset. The visual loss progressed steadily within a few months and progressively worsened.

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. The clinical common disseminated myelitis is characterized by asymmetry and Incompleteness, rapid paraplegia (hours or days) of progression, bilateral Babinski sign, torso sensory dysfunction plane and sphincter dysfunction, acute myelitis with Lhermitte sign, paroxysmal tonic spasm and radiculopathy It can be seen in about 1/3 of patients with recurrent disease, but patients with single-phase disease usually rarely occur.

4. Most patients with NOM are in a single-phase course, 70% of the cases have paraplegia within a few days, about half of the patients are blinded by the affected eye, and a small number of patients are recurrent, of which about 1/3 have paraplegia. About 1/4 of vision is involved, clinical The interval between the events is several months to six months, and the isolated ON and myelitis can be recurred multiple times in the next three years.

Examine

Examination of optic neuromyelitis

The increase of CSF-MNC was more significant than that of MS, 73% of single-phase disease and 82% of patients with recurrence, MNC>5×10 6 /L, about 1/3 of single-phase and recurrent patients with MNC>50×10 6 /L The increase of CSF protein is more obvious in the recurrence type than in the single phase.

Spinal MRI showed that 88% of recurrent spinal fusion lesions had more than 3 spinal segments, usually 6 to 10 segments, and spinal cord swelling and tendon enhancement were more common.

Diagnosis

Diagnosis and differentiation of optic neuromyelitis

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.

Differential 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 differentiated. NOM CSF-MNC>50×10 6 /L or neutrophilia is more common, MS is rare; more than 90% of MS can be seen Clonal 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 longitudinal fusion lesions more than 3 vertebral segments, common spinal cord swelling and tendon enhancement, MS spinal cord lesions rarely exceed 1 spine segment.

3. Subacute spinal optic neuropathy is more common in children, first with abdominal pain, diarrhea and other symptoms, mainly sympathetic dysfunction, more sputum, no recurrence, no significant changes in CSF.

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