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.

Vasculitis primaria del sistema nervioso central, un reto diagnóstico. Reporte de caso / Primary vasculitis of the central nervous system, a diagnostic challenge. Case report

RESUMEN La angeítis primaria del sistema es una rara entidad, en la cual hay destrucción y compromiso inflamatorio de los vasos del sistema nervioso central. Con frecuencia, es subdiagnosticada por sus múltiples formas de presentación clínica y por los hallazgos en neuroimágenes que pueden simular otras patologías, lo que retrasa el diagnóstico y el manejo oportuno. Este tipo de vasculitis es muy poco comprendida y no existen suficientes estudios en la actualidad respecto a su fisiopatología y manejo. Los marcadores inflamatorios suelen ser normales, con alteración del LCR en un 80-90 % de los casos y la arteriografía cerebral puede mostrar un patrón típico descrito como áreas alternantes de dilatación y estenosis; sin embargo, con muy baja sensibilidad. Ante una alta sospecha clínica y ausencia de pruebas diagnósticas no invasivas, el diagnóstico se realiza a través de biopsia de las lesiones cerebrales y meninges. Finalmente, el tratamiento se basa en combinación de corticoides y ciclofosfamida, aunque se carece de estudios controlados que lo soporten.

SUMMARY Primary angiitis of the central nervous system is a rare condition in which there is destruction and inflammatory involvement of the vessels of the central nervous system. It is often underdiagnosed given its multiple forms of clinical presentation and neuroimaging findings that can mimic other pathologies delaying diagnosis and management. This type of vasculitis is very poorly understood and there are not enough studies at present regarding the pathophysiology and management. Inflammatory markers are usually normal with abnormal CSF in 80-90 %% of cases and cerebral arteriography may show a typical pattern described as in "Rosario" however with very low sensitivity. Given the persistence of high clinical and absence of non-invasive diagnostic tests suspect the final diagnosis is made by biopsy of brain lesions and meninges. Finally, treatment is based on a combination of corticosteroids and cyclophosphamide however has not controlled studies support it.

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.

Intracerebral hemorrhage with a favorable outcome in a patient with childhood primary angiitis of the central nervous system / Hemorragia intracerebral com evolução favorável em paciente com angiíte primária do sistema nervoso central juvenil

ABSTRACT Childhood primary angiitis of the central nervous system (cPACNS) is a rare inflammatory brain disease of unknown etiology. Of note, brain hemorrhage has been rarely reported in cPACNS patients, generally associated with a delayed clinical diagnosis, or with a diagnosis only at necropsy. We present the case of a boy with cPACNS that previously suffered an ischemic stroke. At the age of 7 years and 10 months, he presented a sudden and severe headache, vomiting and reduction in consciousness level (Glasgow coma scale 7), requiring prompt tracheal intubation. Brain computed tomography demonstrated intraparenchymal hematoma in the right parieto-occipital lobe and a small focus of bleeding in the right frontal lobe, vasogenic edema, herniation of the uncus and a 10 mm deviation to the left from the midline. C-reactive protein (9.2 mg/dL) and von Willebrand factor (vWF) antigen (202%) were elevated. Decompressive craniotomy was performed and methylprednisolone and cyclophosphamide were administered. One week later, the patient had left hemiparesis without other sequelae. Importantly, motor deficits have been improving progressively. Our case reinforces the inclusion of this vasculitis as a differential diagnosis in children and adolescents with CNS hemorrhage.

RESUMO Angiíte primária do sistema nervoso central juvenil (APSNCJ) é uma doença inflamatória cerebral rara e de etiologia desconhecida. Hemorragia cerebral tem sido raramente reportada em pacientes com APSNCJ, geralmente associada com atraso diagnóstico, ou com um diagnóstico somente por necrópsia. Relata-se um caso de um paciente do gênero masculino com APSNCJ e que previamente sofreu um acidente vascular cerebral isquêmico. Aos 7 anos e 10 meses de idade, o menino apresentou subitamente cefaleia intensa, vômitos e redução do nível de consciência (escala de coma de Glasgow 7), requerendo imediata intubação traqueal. Uma tomografia computadorizada cerebral demonstrou hematoma intraparenquimatoso no lobo parieto-occipital direito e um pequeno foco de sangramento no lobo frontal direito, edema vasogênico, herniação do úncus e um desvio de 10 mm da linha média para a esquerda. A proteína C-reativa (9.2 mg/dL) e o fator antígeno de von Willebrand (202%) estavam elevados. Foi realizada uma craniotomia descompressiva, seguida pela administração de metilprednisolona e ciclofosfamida. Transcorrida uma semana, o paciente apresentava hemiparesia esquerda, sem outras sequelas. É digno de nota que o déficit motor tem melhorado progressivamente. Nosso caso reforça a inclusão dessa vasculite como diagnóstico diferencial em crianças e adolescentes com hemorragia do sistema nervoso central.

A case report: primary CNS angiitis.

The primary angiitis of the central nervous system (PACNS) is an entity with a very low incidence and prevalence. It affects small and medium sized arteries of the brain parenchyma, spinal cord and leptomeniges resulting in CNS dysfunction. It is defined by inflammation of the cerebral vasculature without angiitis of other organ. Its clinical manifestations are very heterogeneous and make clinical diagnosis difficult. In most cases, a brain biopsy is required. Only the clinical suspicion and the ability to recognize the possible clinical and imagenological patterns of presentation make an accurate diagnosis possible. We hereby report a case of Primary angiitis of CNS in 35 year old right handed male who presented with ischemic stroke with Left Hemiplegia. The diagnosis of PACNS was made after ruling out most of the causes of secondary CNS angiitis.

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.

Benign angiopathy of central nervous system.

Benign angiopathy of central nervous system (BACNS) is rare in children. We report a seven-year-old boy presenting with sudden severe headache and progressive external ophthalmoplegia. Magnetic resonance angiography (MRA) showed diffuse segmental narrowing of major cerebral arteries. Following a course of glucocorticoid, there was complete resolution of vascular lesions and follow-up MRA did not show any evidence of new lesions.

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.