Paraneoplastic strabismus ophthalmoclonus-myoclonus
Introduction
Paraneoplastic strabismic eye myoclonus - myoclonus Paraneoplasicopsoclonus-myoclnia (POM) is a disorganized, arrhythmetic, and rapidly changing eyeball abnormality syndrome that is unrelated to the direction of gaze. It is often associated with myoclonus. presence. basic knowledge The proportion of illness: 0.005% Susceptible people: more common in children and adults Mode of infection: non-infectious Complications: neuroblastoma
Cause
Paraneoplastic strabismic eye myoclonus - myoclonus etiology
(1) Causes of the disease
It is currently preferred that the cause is an autoimmune response caused by systemic or potential tumors.
(two) pathogenesis
The pathogenesis of this disease is currently unclear, and the autoimmune mechanism plays a role. Anti-Ri antibodies are present in the serum and cerebrospinal fluid of some patients with breast cancer, although the role of this antibody in the disease is not very Clear, but when the patient has paraneoplastic strabismus and cerebellar ataxia, the presence of anti-Ri antibodies suggests the potential for breast cancer. Some people believe that the lesion is located in the central network of the pons Within the present, because the disease responds well to corticosteroids, it is speculated that corticosteroids may inhibit antibody-mediated autoimmune responses to the central nervous system; or modify and regulate certain neurotransmitters.
There is no specific pathological change in this disease, and the focal lesions of eye myoclonus remain unclear. The pathological changes and symptoms of this disease are not consistent. The pathological manifestations of some patients are not obvious, but the pathological changes of most patients are The diffuse loss of Purkinje cells in the cerebellum, the loss of neurons in the lower olive nucleus, mild inflammatory reaction, etc., some pathological data show that inflammatory cells infiltrate around the small blood vessels in the brain, and mononuclear cell infiltration is the main cause. Cerebellum, brainstem, pia mates, etc.
Prevention
Paraneoplastic strabismic eye myoclonus - myoclonus prevention
Early detection, early treatment.
Complication
Paraneoplastic strabismic eye myoclonus - myoclonus complications Complications neuroblastoma
There are clinical manifestations of primary site tumors, children often have neuroblastoma; adults can be associated with a variety of tumors.
Symptom
Paraneoplastic strabismic eye myoclonus - myoclonus symptoms common symptoms involuntary movement cerebellar ataxia dysarthria vertigo myoclonus gait instability
Paraneoplastic strabismic eye myoclonus - myoclonus is a disordered, arrhythmia-free, rapidly changing eyeball abnormality syndrome that is unrelated to the direction of gaze, often associated with myoclonus.
Ocular myoclonus (opsoclonus) is derived from Polish and is the meaning of eyeball dancing. It can occur in both children and adults. The average age of onset of children is 18 months. Women are slightly more than men. Acute onset is more common. Adult patients are ill. Different ages, the disease is mostly subacute, progressing for several weeks, and some cases are acute onset or slow progression.
The disease is subacute onset, the clinical symptoms are involuntary, non-arrhytic, high-amplitude saccade movement without fixed direction, accompanied by blinking movements, when the eyes are closed or sleeping, the above movement still exists; When the eyeball tracks or fixes the eyeball, the above-mentioned involuntary movement is aggravated, and the multi-directional eyeball has a large arrhythmia, which is non-arbitrary and non-cooperative, sometimes accompanied by extensive myoclonus, often accompanied by cerebellar syndrome and brainstem. Extensive damage is a characteristic manifestation of this disease. POM generally shows volatility. Children with neuroblastoma can develop POM, low muscle tone and irritability, and strabismic eye palsy can appear as a single symptom. Can be used as a special symptom with other symptoms, such as ataxia, dysarthria, gait instability and so on.
Children often have neuroblastoma, and adults can be associated with a variety of tumors. Early identification of strabismic cerebral palsy is a very important clue to the existence of neuroblastoma. About 20% of adult patients may have malignant tumors. The most common is lung cancer, followed by breast cancer, cervical cancer, ovarian cancer, lymphoma, thyroid cancer or bladder cancer. In adult patients, the symptoms are often associated with cerebellar ataxia, dysarthria, myoclonus, Dizziness and brain parenchymal lesions occur in combination.
There may be anti-Yo antibodies in the serum and cerebrospinal fluid of the patient, as well as the presence of anti-Ri antibodies, which can respond to the nuclear proteins of all central nervous system neurons, not to the proteins in the neuronal cytoplasm. In response, the anti-Ri antibody specifically reacts with proteins having a relative molecular weight of 53 to 61 kDa and 79 to 84 kDa isolated from human neurons and nucleus, which is different from the anti-Hu antibody.
Anti-Ri antibodies have specificity. Anti-Ri antibodies cannot be detected in patients with non-paraneoplastic strabismic cerebral palsy. Sometimes anti-Ri antibodies are not detected in a few tumors, and false negative results are observed. The presence of the -Ri antibody can be used as an indication for strabismic ocular palsy complicated with cancer, and can also be used as an indicator of prognosis, because the patient's clinical symptoms and antibodies in blood and CSF after tumor treatment or corticosteroid treatment The titer can be improved at the same time.
CT scans often have no abnormal findings. MRI examinations sometimes show abnormal signals in the brainstem. CSF examination reveals mild leukopenia and mild protein content.
Excision of primary tumors and the use of corticosteroids (ACTH) or corticosteroids can improve clinical symptoms. About half of the patients with cerebral palsy or other symptoms can disappear. Natural remission has been reported, but it is very rare. Symptomatic treatment is available. Clonazepam (clodroxetine).
Examine
Paraneoplastic strabismic eye myoclonus - myoclonus examination
1. CSF examination revealed mild leukopenia and mild protein content.
2. Anti-Yo antibodies may exist in serum and cerebrospinal fluid, and anti-Ri antibodies may also exist. The presence of anti-Ri antibodies may be used as an indication for strabismic ocular palsy complicated with cancer and prognosis.
3. CT scans often have no abnormal findings.
4. MRI examination sometimes shows abnormal signals in the brain stem.
Diagnosis
Diagnosis and differentiation of paraneoplastic strabismus eye myoclonus-myoclonus
Mainly based on the patient's clinical manifestations and related antibody tests, systemic examination did not find cancer, need to be reviewed regularly, cerebrospinal fluid and electrophysiological examination is helpful for diagnosis, serum or CSF-specific autoantibodies can confirm paraneoplastic syndrome (paraneoplastic syndrome, PNS) and suggest potential tumor properties.
Pay attention to the identification of primary diseases of the nervous system.
