paraneoplastic cerebellar degeneration
Introduction
Introduction to paraneoplastic cerebellar degeneration Paraneoplastic cerebellar degeneration (PCD) has been considered rare, and literature tracking reports show that about half of non-familial delayed cerebellar cortical atrophy patients will develop tumors sooner or later. Histopathological features are the disappearance of Purkinje cells, brain parenchyma. Inflammatory cell infiltration is not obvious. basic knowledge The proportion of illness: this disease is rare, the incidence rate is about 0.002% Susceptible people: no special people Mode of infection: non-infectious Complications: dementia
Cause
Paraneoplastic cerebellar degeneration
(1) Causes of the disease
The etiology is not very clear at present. The current cause of paraneoplastic lesions is autoimmune response caused by systemic or potential tumors. It has been reported that some gynecologic tumors with cerebellar degeneration have autoantibodies in serum and CSF. Anti-Yo antibody, anti-Yo antibody is a specific anti-Purkinje cell antibody (APCA), which is associated with paraneoplastic cerebellar degeneration and reproductive or gynecological tumors; anti-Hu can also occur in patients with PCD. Anti-Ri antibody, cerebellar damage, about 2/3 of patients with Hodgkin's disease PCD, confirmed by immunohistochemistry with anti-Purkinje cytoplasmic antibodies.
(two) pathogenesis
Paraneoplastic degeneration diffusely affects the cerebellum and hemisphere of the cerebellum. Its pathogenesis is the presence of antibodies that cross-react with its tumor cells and cerebellar Purkinje cells. Anti-Yo antibodies are common in PCD patients with breast cancer. Ovarian cancer or uterine cancer, anti-Yo antibody is a polyclonal IgG antibody, which specifically reacts with the cerebellar degeneration-associated antigen (CDR) in Purkinje cytoplasm in the cerebellar cortex with the participation of complement, and also with tumor Intracellular related antigens (CDRs) reacted. Immunohistochemistry showed that Purkinje cells had an endoplasmic reticulum granulocyte cytoplasmic marker. Western blot analysis of Purkinje cell protein extracts revealed that the molecular weights of the two antigens were 34 kDa and 62 kDa, respectively. In other patients with cerebellar degeneration, there is generally no anti-Yo antibody present, but one case reported anti-Yo antibody positive in non-Hodgkin's lymphoma with cerebellar degeneration, and no cerebellar degeneration in breast cancer, ovarian cancer, etc. When present, such antibodies are not present, and such antibodies are not present in cerebellar degeneration without tumors.
Prevention
Paraneoplastic cerebellar degeneration prevention
Early detection, early treatment.
Complication
Paraneoplastic cerebellar degeneration Complications dementia
Paraneoplastic damage can cause linguistic difficulties, dementia, memory impairment, pyramidal tract disease or other neuropathy if it affects other parts of the nervous system. It can also affect many tissues and organs in the body, resulting in corresponding clinical manifestations, such as joints. Inflammation, rash, endocrine dysfunction, etc.
Symptom
Symptoms of paraneoplastic cerebellar degeneration Common symptoms Mental disorders Consonant disorders Double vision Hypothyroidism Memory disorders Dementia Eyeball tremor Ataxia vertigo
1. The condition is subacute progression, progressive exacerbation, patients are bedridden in weeks or months, and neurological signs in 1/2 to 2/3 cases appear before the discovery of cancer, mainly manifested as cerebellar syndrome Such as gait and limb ataxia, dysarthria and nystagmus, etc., sometimes diplopia, dizziness, neurological hearing loss and eye movement disorders, and a few cases of emotional and mental disorders.
Symptoms of cerebellar damage can occur before or after tumor symptoms, and progress progressively within a few months, although under normal circumstances, the symptoms of cerebellar damage tend to progressively increase, but the course of disease can also be stable. It has been reported that the symptoms of cerebellar damage are alleviated in the case of treatment of the primary tumor.
Gait and limb ataxia are characteristic features of cerebellar damage. Consonant disorders exist in many cases. Ataxia of the limbs can be asymmetrical, and nystagmus is rare.
Paraneoplastic lesions, such as those involving other parts of the nervous system, can also cause language difficulties, dementia, memory impairment, pyramidal tract disease, or other neuropathy.
2. In the early stage of the disease, cerebrospinal fluid showed inflammatory changes, lymphocytes and IgG increased, and it was completely normal. Anti-Yo antibodies were found to be in-depth search for gynecological tumors, including mammography, pelvic CT, ovarian CA-125 antigen quantification, elective anesthesia Pelvic examination, curettage and repeated mammography, etc., negative examination may be laparotomy.
Anti-Purkinje cell antibodies, such as anti-Yo antibodies (ovarian and breast tumors) or anti-Tr antibodies (Hodgkin's disease); antinuclear antibodies such as anti-Hu (small cell lung cancer) and anti-Ri (breast cancer) Antibodies, sometimes detected in the blood, may have moderate lymphocytosis and elevated protein in CSF.
In general, the diagnosis of paraneoplastic cerebellar degeneration, in the case of only the manifestations of impaired nervous system without the clinical manifestations of the primary tumor, the diagnosis is very difficult, the recurrence of dysarthria and language difficulties helps Cerebellar damage caused by alcoholism or hypothyroidism is clear. Simple upper limb ataxia suggests that alcohol damage is not possible. Wernicke encephalopathy suggests that the patient may be due to malnutrition caused by cancer.
The diagnosis of paraneoplastic cerebellar degeneration is only manifested in the damage of the nervous system, but the clinical symptoms and signs of the primary tumor are very difficult. The primary tumor is not found based on the clinical manifestations and related antibody tests. Misdiagnosed before.
Examine
Examination of paraneoplastic cerebellar degeneration
1. Serum and CSF immunological specific antibody examination.
2. Regular examination of hematuria.
3. Neurological CT, MRI examination of early CT and MRI examination is normal, advanced MRI can be seen in the cerebellar white matter T2WI high signal, extensive cerebellum and brain stem atrophy.
4. Neuromuscular electrophysiological examination.
Diagnosis
Diagnosis and identification of paraneoplastic cerebellar degeneration
Anti-Yo antibodies are detected in patients with subacute cerebellar degeneration, suggesting the presence of gynecologic cancer. Anti-Hu antibodies can be detected in patients with small cell lung cancer and paraneoplastic syndrome, because anti-Hu antibodies are in the brain. Internally synthesized, the titer of this antibody in CSF is higher than that in serum, and the anti-Hu antibody specificity is lower than that of anti-Yo antibody, because anti-Hu antibody has nerves in addition to small cell lung cancer. The presence of anti-Hu antibodies can also be found in blastoma, breast cancer, and prostate cancer. In addition, in other clinical types of paraneoplastic syndromes, such as encephalomyelitis, marginal encephalitis, brainstem encephalitis, and myelitis Subacute sensory neuron disease, anterior horn cell degeneration, and Lambert-Eaton syndrome can be positive for anti-Hu antibody. Therefore, when anti-Hu antibody is present, its clinical significance needs to be analyzed.
