Vasculitis primaria del sistema nervioso central en población pediátrica: reporte de caso y revisión de la literatura / Childhood primary vasculitis of the central nervous system: Case report and literature review

RESUMEN La vasculitis primaria del sistema nervioso central es una enfermedad infrecuente que puede afectar a adultos y población pediátrica. Puede comprometer los vasos sanguíneos cerebrales tanto de mediano o gran calibre como los de pequeño calibre y estar asociada a procesos inflamatorios, infecciosos, tumorales o ser de origen idiopático. Describimos el caso de un adolescente con deterioro neurológico focal dado por hemiplejía derecha en quien se descartaron otras causas más frecuentes de isquemia cerebral, con diagnóstico final de vasculitis primaria del sistema nervioso central asociada a infección por virus de Epstein Barr.

ABSTRACT Primary angiitis of the central nervous system is an uncommon disease that may affect adults and the paediatric population. It can involve both the medium-large and small sized cerebral blood vessels, and can be associated with inflammatory, infectious, tumour processes, or of idiopathic origin. The case is presented of an adolescent with focal neurological impairment due to right hemiplegia, in whom other more frequent causes of cerebral ischaemia were ruled out. The final diagnosis was Epstein Barr virus associated with primary angiitis of the central nervous system.

The application of MR new technique in primary angiitis of the central nervous system / 实用放射学杂志

Objective To study imaging findings of primary angiitis of the central nervous system (PACNS) on MR new techniques,and to explore the diagnostic value of MR new techniques in this disease.Methods Seventeen cases who had been clinically diagnosed as PACNS were enrolled.All the cases underwent MR regular scans [T1 WI,T2 WI,diffusion weighted imaging(DWI),enhanced-enhanced T1 WI] and 3D-posudo-continuous arterial spin labeling (3D-PCASL).Some cases were scanned by using magnetic resonance angiography (MRA),susceptibility weighted imaging (SWI) and magnetic resonance spectrum (MRS) techniques.The regional cerebral blood flow (CBF) values were achieved based on ASL.The inter-group difference of the CBF values were analyzed by independent sample t test.Results Compared to the normal site,the CBF values in the lesion site were significant lower on PACNS (P=0.000).Of 13 patients scanned by SWI,10 cases showed low signal in the lesion.Of 14 patients scanned by MRS,10 cases showed N-acetylaspartic acid (NAA) peak decrease and Cho peak increase and 2 cases showed lactic acid(Lac) peak.Conclusion 3D PCASL can detect the hemodynamic abnormalities of PACNS.SWI can improve the ability of sensibility to observe the microbleed inside the lesion.MRS can explicit the extent of destruction and repairment of neurons in the lesion.Combining with the convention MR images can improve the understanding of the disease and the diagnostic accuracy.

Primary Angiitis of the Central Nervous System Presenting Tumefactive Lesions and Small Arteriolar Ectasias

Primary angiitis of the central nervous system (PACNS) is a rare disorder of unknown etiology and a serious form of vasculitis that is limited to the brain and spinal cord. We report a case of PACNS presenting with multifocal enhancing tumefactive lesions on brain magnetic resonance imaging, and numerous small arteriolar ectasias on conventional angiography, with relapsing-remitting clinical features.

Primary Angiitis of the Central Nervous System in Children / 대한소아신경학회지

Primary angiitis of the CNS(PACNS), also known as isolated angiitis of the CNS, is an idiopathic vasculitis confined to the CNS and it primarily occurrs to middle aged adults. Neurologic manifestations include headache, focal seizure, and progressive, behavioral or multifocal neurologic impairments. A 20-month-old boy was presented with recurrent right-sided hemiplegia and loss of consciousness. Brain MRI revealed focal T2 hyperintensely enhanced lesions involving left lentiform nucleus and caudate nucleus with no mass effects. Cerebral angiogram disclosed multifocal stenosis in both middle cerebral arteries and left posterior cerebral artery. The child was treated with prednisone alone since he was diagnosed as IACNS. He was asymptomatic with a normal neurologic examination and continued on prednisone therapy. Therefore, it is concluded that children who have frequent or severe headache or focal neurologic deficits should be carefully evaluated and those meeting criteria of IACNS should be treated aggressively.

Clinical features of primary angiitis of the central nervous system / 临床神经病学杂志

Objective To investigate the clinical features of primary angiitis of the central nervous system (PACNS). Methods The clinical data of 27 patients with PACNS were analyzed. Results PACNS commonly occured in young and middli-aged adult and with acute or subacute onset. The clinical manifestations were mainly including headache,decrudescence of cortical functions,focal neurological deficits and seizures. The hypointensity signals on T1WI and T2WI were showed and could be enhanced at lesions by skull MRI. The pia vessels inflammatory lesions were revealed in brain by pathological examination. The treated with glucocorticoid and combined therapy such as anti platelet aggregation,scavenging free radicals,reducing intracranial pressure,improving microcirculation and promoting neuronic metabolism,the condition get better.Conclusions The clinical manifestation of PACNS is various. The diagnosis can be make by combining the data of clinical features,imaging and brain biopsy.The treatment with glucocorticoid can obtain better efficacy.