Childhood Primary Angiitis Of The Central Nervous System (19 Cases Report With Literature Review)

ObjectivePrimary angiitis of the central nervous system is a kind of disease who only invades central nervous system, other systems are spared. It is a rare disease of central nervous system. There are few reports about it. The etiologies and pathogenesies are not very clear. It is possible that abnormal immunity which is resulted by multiple factors mediates the inflammatory reaction. The histology which often presents with granuloma and non-granuloma vasculitis of the small vessels displays variform. Meningina and vascular parenchyma can be affected alone or simultaneously. It often accompanies small vascular haemorrhage of the brain parenchyma.PACNS can appearance variously because the vasculars which are affected are different. Headache is the main clinical manifestation of cPACNS who may be accompanied by focal and diffuse neurologic deficits. The available investigative modalities have limited sensitivities and specificities which result in diagnostic and therapeutic challenge.The prognosis mala, the patients will die without therapy. We collected 19 cases who were reported as primary angiitis of the central nervous system internally to investigate the clinical, diagnostic and therapeutic features of childhood primary angiitis of central nervous system.Materials and methods19 cases who were reported as childhood primary angiitis of the central nervous system internally were collected from July 1999 to October 2006. The cases who were choosed, 11 males and 8 females, aged from 1 to 15 years. We referred a lot of foreign resources for retroactively studying the disease by summarizing the clinical and imaging manifestations.ResultsIn these cases, 5 presented with headache, 3 language disorder and 12 dyskinesia. 2 of them would have fever, 2 diarrhea, 1 scalded, then others had no special reasons. 18 were examinated with MRI and MRA, all of whom had abnormal MRI indicated by long T₁ and long T₂ signals. Blood vessels of 15 cases were abnormal indicated by angiostenosis and vascular occulsion. There were 14 cases with affected middle cerebral artery, 1 case the posterior cerebral artery. 1 case was examined by CT and conventional angiography which indicated aneurysm of the anterior, middle and posterior cerebral artery. Abnormal blood vessels were not visible with another there cases. Only one case experienced biopsy which indicated the chronic inflammtion of the blood vessels. All of the vessels were infiltrated by lymphocyte and macrophage. None of the cases had systemic diseases, also there were no positive effects in all the examinations.ConclusionsPrimary angiitis of the central nervous syetem is a rare condition. The diagnosis remains difficult because the available investigative modalities have limited sensitivities and specificities. Cerebrospinal fluid analysis(CSF), MRI of the brain and angiography, especially MRA, are the most helpful diagnostic tests. However, brain biopsy is considered the golden standard. The diagnosis is usually based on multifocal brain parenchymal lesions seen on MRI of the brain in association with typical angiographic findings because of greate traumatic occlusion of biopsy.