2023-07-02 14:57:35 United Daily News Liu Xiuzhi The careful observation of family members and the clinical experience of doctors’ intuition, thinking and judgment are all important.
A few days ago, a 48-year-old Ms. Chen, who participated in a case discussion meeting, felt tired and had a poor appetite for two weeks, and occasionally saw double images and was hospitalized. She had seen an ophthalmologist, and the examination was normal, and she had no fever, headache, high blood pressure, or diabetes.
Ms. Chen’s physical, neurological examination, and cognitive function were normal. Blood routine, liver and kidney function, vitamin B12 concentration, immune test and tumor index were all normal, and there was no acetylcholine receptor antibody, which ruled out the possibility of myasthenia gravis.
However, the lumbar puncture showed that the white blood cells in the cerebrospinal fluid increased slightly (11/cumm), 98% of which were lymphocytes, the protein was high (68 mg/dl), and the glucose value was normal. Therefore, he was treated with viral encephalitis on the same day. One day after the intravenous injection of antiviral drugs, Ms. Chen underwent an MRI examination of the brain and spinal cord. The radiologist immediately told her that it was “autoimmune glial fibrillary acidic protein astrocytopathy” (autoimmune glial fibrillary acidic protein astrocytopathy, referred to as autoimmune GFAP astrocytopahty). Subsequent blood tests for GFAP antibodies were also positive.
The attending physician immediately gave Ms. Chen intravenous steroids. After two days of treatment, she felt better, her appetite improved, and her diplopia stopped.
Autoimmune glial fibrillary acidic protein meningoencephalomyelitis is a disease discovered by the medical team of the Mayo Clinic in the United States in 2016. Afterwards, cases were also reported in Italy, China, Japan, France, etc., but they were still rare, estimated at 0.6 cases per 100,000 population. Any age may occur, with 44-50 years old as the most, no gender or racial differences.
Glial fibrillary acidic protein is the major fibrous protein of brain astrocytes. Astrocytes not only maintain the integrity of the cerebrovascular barrier, but are also the main support cells of the brain. When neurons are damaged, astrocytes become larger and proliferate to fill the damaged area. When attacked by antibodies, damage occurs from the optic nerve to the brain, cerebellum, brainstem and spinal cord, causing meningitis, encephalitis and myelitis (meningoencephalomyelitis). The symptoms and severity of each patient are very different, including fever, headache, delirium, seizures, hand tremors, unsteady walking, anxiety, numbness in hands and feet, weakness, etc., but not every symptom will appear, so it is difficult to treat clinically. Judgment, the number of patients may also be underestimated.
The diagnosis needs to be confirmed by the positive detection of GFAP antibody in the cerebrospinal fluid or blood, as well as the typical manifestations of brain magnetic resonance imaging with contrast agent injection: multiple radial and thin line-like developing lesions, appearing around the ventricles, white matter, and basal Nuclei and meninges, or multi-nodular linear development in the spinal cord. The cause of the disease is currently unclear, and about half of the patients have cold symptoms before the onset of the disease. Treatment with steroids works well, but about 20% of cases recur.
Most of the physicians who attended the meeting had never heard of this disease, so they asked questions enthusiastically. For example, how did this brilliant radiologist make this diagnosis? That’s because she has read related papers, and when she just saw the video, she felt blessed. She really read thousands of books and used them for a while. Another example is that the patient has mild symptoms, how can he think of being admitted to the hospital? The attending physician said that the patient’s main complaints were only fatigue, poor appetite and occasional diplopia. The symptoms were not specific, and the neurological examination was normal. They were usually dealt with in the outpatient clinic, and even suspected whether there were psychological factors. However, the family members said that the patient was always cheerful, and these symptoms were obvious. Affecting her life and work, it can be seen that the careful observation of the family and the clinical experience of the doctor’s intuition, thinking and judgment are all important.
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Liu Xiuzhi
Special Physician of Taipei Veterans General Hospital National Yang Ming University Clinical Adjunct Professor
Liu Xiuzhi
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2023-07-02 06:57:35
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