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.

Frosted branch angiitis with penetrating ocular trauma and retained intraocular foreign body

A 54-year-old male sustained ocular trauma with a projectile. Examination of the right eye revealed an intraocular foreign body (IOFB) adjacent to the optic nerve head, vitritis, vitreous hemorrhage, and translucent perivascular sheathing of the retinal vessels in all quadrants suggesting frosted branch angiitis (FBA). The patient underwent vitrectomy with removal of the IOFB and silicone oil tamponade under steroid cover. With continued use of systemic and topical steroids after surgery, complete resolution of FBA and improvement in vision were noted in a week. Prompt resolution of FBA after IOFB removal points toward a strong association between the presence of IOFB and FBA.

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.

Bilateral Frosted Branch Angiitis in Kikuchi-Fujimoto Disease

PURPOSE: A case of frosted branch angiitis in Kikuchi-Fujimoto disease is reported. CASE SUMMARY: A 33-year-old male complained of a sudden decrease in visual acuity that developed in both eyes 5 days prior. He suffered from a headache, chills, myalgia, and flank pain 1 week before. The initial best-corrected visual acuity (BCVA) was 0.1 in the right eye and 0.2 in the left eye. On slit lamp examination, no inflammatory finding was observed in the anterior chamber and vitreous body of both eyes. On fundus examination, a diffuse vascular sheathing-like frosted branch was found in the retinal vessels, and retinal hemorrhage was observed. Fluorescein angiography showed staining and leakage of dye along the vascular sheathing. Serological findings were negative, showing no evidence of an autoimmune disease or viral infection. Neck ultrasonography revealed non-tender left cervical lymph node enlargement >1 cm in diameter. Ultrasound-guided fine needle aspiration cytology showed findings compatible with Kikuchi-Fujimoto disease, including necrotic changes and pronounced karyorrhexis, plus histiocyte and lymphocyte infiltration without neutrophils. We started systemic steroid therapy. One month after treatment, the BCVA of both eyes improved to 1.0. CONCLUSIONS: In patients with frosted branch angiitis, systemic disease such as Kikuchi-Fujimoto disease should be considered.

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.

Granulomatosis eosinofílica con poliangeítis: reporte de caso yrevisión de la literatura / Eosinophilic granulomatosis with polyangiitis: case report and review of the literature

La granulomatosis eosinofílica con poliangeítis (Síndrome de Churg-Strauss) es una enfermedad vasculítica primaria poco frecuente. El diagnóstico actualmente se define a partir de la presencia de al menos cuatro de seis criterios propuestos por la Sociedad Americana de Reumatología, los cuales incluyen: asma bronquial, eosinofilia mayor que 10 por ciento, sinusitis paranasal, infiltración pulmonar, evidencia histológica de vasculitis y compromiso neurológico ya sea mono o polineuropático. En el presente artículo se reporta el caso de un paciente de 56 años con antecedentes de asma bronquial, rinitis alérgica y poliposis nasal operada, derivado a nuestro centro por cuadro de aumento de volumen doloroso en ambas extremidades inferiores, baja de peso, parestesias y debilidad muscular. Asociado a esto desarrolló lesiones purpúricas palpables cuya biopsia resultó compatible con granulomatosis eosinofílica con poliangeítis. El paciente posteriormente recibió tratamiento inmunosupresor con prednisona y un pulso de ciclofosfamida con buena respuesta clínica. Se presenta una revisión bibliográfica a propósito del caso.

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss Syndrome) is an uncommon primary vasculitis. The diagnosis is currently defined by the presence of at least four of six criteria proposed by the American College of Rheumatology, which include: asthma, eosinophilia less than 10 percent, paranasal sinusitis, pulmonary infiltration, histologic evidence of vasculitis and neurologic compromise as mono or polyneuropathy. In the present article, we report the case of a 56 year-old man with history of asthma, allergic rhinitis and operated nasal polyposis, referred to our center with painful bulking in both lower extremities, weight loss, paresthesias and muscle weakness. It also developed palpable purpura. Biopsy of skin lesions was compatible with eosinophilic granulomatosis with polyangiitis. The patient subsequently received immunosuppressive therapy with prednisone and a cyclophosphamide bolus with good clinical response. A review on the subject is also presented.

Advance in the pulmonary vasculitis in children / 中华实用儿科临床杂志

Vasculitis is divided into large,medium-sized,small vasculitis based on the size of the affect vessel.Pulmonary vasculitis may affect any vessel of the lung.It was pulmonary capillaritis if capillary vessels were involved.The common pulmonary vasculitis were granulomatosis polyangiitis,microscopic polyangiitis,allergic granulomatosis angitis,the less common pulmonary vasculitis were Schonlein-Henoch purpura,Takayasu'arteritis,Giant-cell arteritis,cryglobulinemia,polyarteritis nodosa,and Bechet'disease.The clinical features of the pulmonary vasculitis were alveolar hemorrhage,hemoptysis,anemia and pulmonary infiltration,other clinical manifestation were cough,fever,weight loss.The diagnosis of the pulmonary vasculitis was based on the clinical symptoms,signs and positive anti-neutrophil cytoplasmic antibodies or lung biopsy.Large vasculitis was diagnosised by the magnetic resonance imaging and colour Doppler ultrasonography.The glucocorticoid and immunosuppressant was used in the treatment of the vasculitis.

Frosted Branch Angiitis as Ocular Manifestation of Behcet's Disease: Unusual Case Report and Literature Review

We report an unusual case of unilateral frosted branch angiitis associated with Behcet's disease, including a review of previously reported cases. A 39-year-old male with history of recurrent oral and genital ulcers presented with visual loss in his left eye. Fundus findings demonstrated occlusive retinal vasculitis resembling acute frosted branch angiitis. Laboratory examinations including viral markers revealed no abnormal findings except positive HLA-B51. The patient was treated with systemic steroid and cyclosporine. Six months after presentation, new oral ulcers and pseudofolliculitis appeared, and he was diagnosed with Behcet's disease following rheumatology consultation. During follow-up, there was no change in visual acuity of hand movement, and disc neovascularization developed even after complete panretinal photocoagulation. Ocular manifestations of Behcet's disease can present as unilateral frosted branch angiitis, and may consecutively involve in both eyes. Early immunosuppressive treatment is recommended.

An Unusual Presentation of Churg-Strauss Disease (Allergic Angiitis and Granulomatosis).

We present a case of a 45-year-old female with history of four days of fever and rash with swelling over both limbs diagnosed on postmortem autopsy as Churg-Strauss disease and further give a brief overview of the disease.

Frosted branch angiitis, neuroretinitis as initial ocular manifestation in Behçet disease.

Behçet disease is an idiopathic, multisystem disorder characterized by recurrent episodes of orogenital ulceration and vasculitis of the veins and arteries of all calibers. Ocular involvement may affect the conjunctiva, sclera, uveal tract, vitreous, blood vessels, and retina. Many theories have pointed toward an autoimmune response behind its pathogenesis, which may be triggered by exposure to an infectious agent. Frosted branch angiitis is characterized by vascular inflammation, sheathing, retinal edema, and retinal hemorrhages. The disease may be idiopathic in a majority of the cases or may be associated with ocular and systemic pathology. Association between Behηet disease, Frosted branch angiitis, and neuroretinitis is not reported in literature. This uncommon combination reflects the varied systemic and ocular manifestations in Behηet disease, especially in patients who are not diagnosed and treated in time. We hereby report a case of bilateral frosted branch angiitis and neuroretinitis in a young male from Middle-east, suffering from Behçet disease.

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.

Effectiveness of Goshajinkigan on the Cold Sensation and Dysthesia in A Case of Nodular Multiple Angiitis / 日本東洋医学雑誌

A 70 year-old male with nodular multiple angiitis visited our outpatient clinic because of cold sensation and dysthesia. Although the vascular disease went into remission with steroid and immunosuppressant use, dysthesia continued. An improvement in subjective symptoms was evaluated with a visual analogue scale, after Gosha jinki-gan extract was administered on the basis of Kampo diagnosis. Furthermore, this beneficial effect increased when Syuchibushi matsu administration was added. Thus far, there are no such reports on Goshajinkigan as being useful for nodular multiple angiitis. Although the direct effect was not seen on the peripheral nerve conduction velocity, Goshajinkigan might be useful for the treatment of subjective cold sensation and dysthesia symptoms, in patients with nodular multiple angiitis. The mechanism of the above effect is considered to be similar to that in diabetic peripheral neuropathy.

A Case of Primary Angiitis of the Central Nervous System: A Case Presentation

Primary angiitis of the central nervous system (PACNS) is a rare idiopathic disorder with recurrent vasculitis restricted to the central nervous system (CNS) and involves small-sized (and at time medium-sized) blood vessels. The typical clinical manifestations include multifocal strokes or encephalopathy, often associated with severe headaches. There are very few pediatric cases with PACNS reported in the literature. The diagnosis of PACNS is mainly based on its clinical features, demonstration of angiitis on angiography or biopsy, and exclusion of secondary causes of angiitis. A high index of suspicion is necessary for the diagnosis. Though the prognosis is generally poor, use of steroids and immunosuppressive therapy has been useful in achieving successful remission in many patients. We report the case of a patient with PACNS presenting recurrent headaches for 5 days.

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.

Frosted Branch Angiitis

PURPOSE: Frosted branch angiitis is a rare retinal disease with the characteristic sheathing in the retinal vessels. We report a typical case of this disease. METHODS: A thirty-year-old woman complained of sudden decrease of visual acuity developed in both eyes three days ago. Visual acuity was 0.4 with several inflammatory cells in the anterior chambers of both eyes. There were diffuse vascular sheathing like frosted branch in both retinas. Fluorescein angiogram showed staining and leakage of dye along the vascular sheathing. RESULTS: Visual acuity recovered to 1.0 and retinal findings returned normal 2 weeks after treatment with the low dose of systemic steroid. There was no recurrence during follow-up period. CONCLUSIONS: Typical frosted branch angiitis occurs suddenly in both eyes of the young, and it responds well to treatment with the low dose of systemic steroid.

A Case of Acute Fulminant Neuropathy in Patient with Allergic Granulomatosis and Angiitis / 대한류마티스학회지

No abstract available.

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.