Important clues to teen NIID were discovered
Important clues to teen NIID were discovered
September 18, 2018 Source: Health News
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Intranuclear inclusion disease (NIID) is a rare neurodegenerative disease that is difficult to diagnose. The neurology clinical research group of the First Affiliated Hospital of Chongqing Medical University found that encephalitis-like episodes are rare clinical manifestations in adolescent NIID patients; skin biopsy is an effective auxiliary examination method for suspected adolescent NIID patients. Related papers in this study were recently published online in the American Medical Association Neurology.
Associate Professor Xiao Fei of the research group introduced that since NIID was first reported in 1968, it has only reported hundreds of cases worldwide, and it has only gradually attracted the attention of neurologists in the last 10 years. The clinical manifestations of the disease are complex and the diagnosis is very difficult. The research team reported that 19-year-old young men were admitted to hospital with acute onset fever, headache, convulsions and encephalitis-like symptoms of nausea and vomiting. A detailed examination of his medical history found that there were three similar encephalitis-like episodes in the past 21 months, and that the patient began to experience a slow-moving mental decline 6 years ago, gradually losing weight. Two years ago, patients began to experience muscle atrophy and urinary retention. Magnetic resonance imaging revealed significant brain atrophy and leukoencephalopathy-like changes. After careful clinical, electrophysiological, imaging, comprehensive metabolic and genetic screening, the research team ruled out other diseases, and finally used the upper arm of the arm for biopsy, immunofluorescence and electron microscopy in the patient's skin, sweat gland cells, fat cells and Intranuclear inclusion bodies were found in fibroblasts, confirming that the patient was adolescent NIID.
It is reported that in recent years, it has been reported that adult NIID patients are diagnosed by skin biopsy in East Asian population. However, for adolescent NIID patients, there have been no reports of successful inclusion of intranuclear inclusions in dermal dermal cells in the world. The achievement of this research is of great significance for further optimizing the diagnostic process of adolescent NIID and even adolescent neurodegenerative diseases, researching pathogenesis and finding treatment methods. (Special correspondent Zhang Libin correspondent Yin Lei)
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