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![Table 2 : New classification of childhood vasculitis [2].](profile/Francesco-Nicita/publication/231176718/figure/tbl2/AS:669582754840577@1536652377021/New-classification-of-childhood-vasculitis-2_Q320.jpg)
Central nervous system vasculitides in children may develop as a primary condition or secondary to an underlying systemic disease. Many vasculitides affect both adults and children, while some others occur almost exclusively in childhood. Patients usually present with systemic symptoms with single or multiorgan dysfunction. The involvement of centr...
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Central nervous system (CNS) involvement in Henoch-Schonlein purpura (HSP) is rare but poses diagnostic difficulties. The aim of the study was to establish the frequency of CNS involvement in HSP, to analyze its clinical characteristics and do a literature review. Medical files of patients with HSP admitted at the Department of Pediatrics, Plovdiv,...
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... Calabrese proposed and validated diagnostic criteria for PACNS in adults: an acquired neurological deficit, plus angiographic or histopathology features of angiitis within the CNS, in the absence of a systemic vasculitis or any other condition to which the angiographic or pathologic features could be secondary [22,23]. These criteria have since been adopted for childhood primary angiitis of CNS (cPACNS) by pediatric neurologists and rheumatologists [24][25][26][27]. In 2001, Lanthier reported two cases of biopsy-proven cPACNS [2]. ...
... MRI Investigation of choice to assess suspected CCV. It can demonstrate parenchymal changes (edema, infarction, and microhemorrhages), vascular abnormalities by evaluating involved vessel size, wall enhancement pattern, and outlining ancillary findings, including leptomeningeal enhancement [5,[18][19][20]. The cross-sectional (CT and MR) angiographic techniques are not reliable in the evaluation of the distal intracranial arteries. ...
... Catheter Angiography (CA): Excellent for diagnosing and assessing most vascular disorders [15,20]; however, its use is limited in children due to the invasive nature and technical difficulties. Moreover, small-vessel vasculitis may not be apparent on CA with the sensitivity and specificity in de- [1,20,29,30]. ...
... Catheter Angiography (CA): Excellent for diagnosing and assessing most vascular disorders [15,20]; however, its use is limited in children due to the invasive nature and technical difficulties. Moreover, small-vessel vasculitis may not be apparent on CA with the sensitivity and specificity in de- [1,20,29,30]. ...
Cerebral vasculitis is increasingly recognized as a common cause of pediatric arterial stroke. It can present with focal neurological deficits, psychiatric manifestations, seizures, and encephalopathy. The etiopathogenesis of childhood cerebral vasculitis (CCV) is multifactorial, making an inclusive classification challenging. In this review, we describe the common and uncommon CCV with a comprehensive discussion of etiopathogenesis, the role of various imaging modalities, and advanced techniques in diagnosing CCV. We also highlight the implications of relevant clinical, laboratory, and genetic findings to reach the final diagnosis. Based on the clinicoradiological findings, a stepwise diagnostic approach is proposed to facilitate CCV diagnosis and rule out potential mimics. Identification of key clinical manifestations, pertinent blood and cerebrospinal fluid results, and evaluation of central nervous system vessels for common and disease-specific findings will be emphasized. We discuss the role of magnetic resonance imaging, MR angiography, and vessel wall imaging as the imaging investigation of choice, and reservation of catheter angiography as a problem-solving tool. We emphasize the utility of brain and leptomeningeal biopsy for diagnosis and exclusion of imitators and masqueraders.
... Most researchers use steroids and cyclophosphamide in combination for the first 6 months of the induction period, and use methotrexate, azathioprine, or mycophenolate for prolonged maintenance therapy. 18,19 For our patient, we selected mycophenolate as an immunosuppressive agent for maintenance therapy. No obvious adverse effects were observed. ...
Introduction:
Primary angiitis of the central nervous system (PACNS) is a vasculitis confined to the CNS. A small proportion of the lesions may present as a tumor-like mass, which is rarely seen in children.
Case presentation:
A 5-year-old girl was admitted to our hospital because of an intermittent headache. Brain imaging suggested a space-occupying lesion in the right cerebral hemisphere. The final diagnosis was PACNS with a lymphocytic pattern by stereotactic brain biopsy. Her condition improved after immunotherapy.
Conclusion:
Pediatricians should consider the possibility of PACNS when encountering intracranial tumor-like lesions. Early diagnosis of tumor-like PACNS and prompt immunotherapy could improve the long-term prognosis and avoid surgery.
... Calabrese proposed and validated diagnostic criteria for PACNS in adults: an acquired neurological de cit, plus angiographic or histopathology features of angiitis within the CNS, in the absence of a systemic vasculitis or any other condition to which the angiographic or pathologic features could be secondary. [22,23] These criteria have since been adopted for childhood primary angiitis of CNS (cPACNS) by pediatric neurologists and rheumatologists [24][25][26][27]. In 2001, Lanthier [2] reported two cases of biopsy-proven cPACNS. ...
Background: Primary CNS angiitis of childhood (cPACNS) is an autoimmune process of CNS vascular structure. It is considered a novel and challenging clinical problem in pediatric neurology and rheumatology. This study discusses as a significant case series study of cPACNS.
Methods: From 1994 to 2021 at pediatric rheumatology clinic, patients <18 years of age were enrolled this study with diagnosis of cPACNS if they had: a clinical symptoms compatible with primary CNS vasculitis, and MRA findings demonstrating arterial stenosis and or aneurism that are not attributable to other disease and background.
Results: There were 22 patients with mean age 10 years, 12 patients (54%) were female. The mean delayed time to diagnosis was 4 years. The most common neurologic symptoms were headache (88%) then seizure and mental disorder in (45%). Fever was in (54%) and positive ANA result in (31%) patients. 14 patients (63 %) showed abnormality in both MCA and ACA, whereas PCA showed this abnormity in 36% (8) patients. Most of patients 86 %( 19) had normal EEG findings. Five patients (22%) had severe and permanent neurological damage and sequel.
Conclusion: Although cPACNS seems to be a rare and unfamiliar disorder, however it can be conceptualized much in the same way as pediatric ischemic disorder, and it should be considered in any patient with unexplained headache, seizure and mental disorder.
... Henoch-Schönlein purpura (HSP) is associated with IgA1-dominant immune deposits in small blood vessels (predominantly capillaries, venules, or arterioles). Therefore, the CHCC 2012 replaced the eponym "Henoch-Schönlein purpura" with IgA vasculitis to better define the pathophysiological features observed in this condition [1][2][3][4][5][6]. ...
In this study, our purpose is to evaluate cochlear and vestibular function in juveniles with IgA vasculitis using audiometry, distortion product otoacoustic emissions, and cervical vestibular evoked myogenic potential (cVEMP) tests. Forty children diagnosed with IgA vasculitis from the pediatry clinic and 40 age- and sex-matched healthy children were evaluated with distortion product otoacoustic emissions, audiometry, and cVEMP test in a tertiary hospital. The audiometry average values for both ears of the IgA vasculitis group and the control subjects were compared, and as a result, median 4.7-dB sensorineural hearing loss (SHL) was found for the IgA vasculitis group compared to the control group at 250 Hz and it was statistically significant (p < 0.001). An average of 6.4-dB SHL was detected at 8000 Hz (p < 0.001). There was a statistically significant difference among IgA vasculitis and control groups regarding measurement results of average p1-n1 latency time of both ears (0.9 ms (ms) increase, p = 0.035). In IgA vasculitis patients, the median amplitude difference of both ears’ average p1 n1 was found to be 5.6 mV, statistically significantly decreased compared to the control group (p = 0.003).
Conclusion: This study, firstly in literature, demonstrated that IgA vasculitis may have association with hearing loss and vestibular dysfunction in children. We think this might be due to autoimmune mechanisms.What is Known:
• Ig A vasculitis is a leukocytoclastic vasculitis with unknown etiology, involving the skin, joints, gastrointestinal system, kidneys, and rarely other organs.
• No study has been reported for the cochlear and vestibular association of Ig A vasculitis in current literature.
What is New:
• This study demonstrated that Ig A vasculitis may have association with hearing loss in children.
• This study also demonstrated that Ig A vasculitis may have association with vestibular dysfunction in children.
... İmmünglobulin A vasküliti (IgAV;Henoch-Schönlein purpura) pediatrik yaş grubunda vaskülitler arasında en sık görülenidir. Kompleman komponentlerinin ve immünglobulin A (IgA) içeren immün komplekslerin küçük damar duvarlarında depolanması ile karakterize sistemik lökositoklastik bir vaskülittir (1)(2)(3). Daha çok deri, eklem, böbrek ve gastrointestinal sistem tutulumu ön plandadır fakat akciğer, beyin ve skrotum gibi diğer organlar da IgAV seyri esnasında tutulabilmektedir (1,4). Genellikle 5-15 yaş grubunda görülen, trombositopenik olmayan purpuranın en sık sebebidir. ...
Amaç: Bu çalışma çocukluk çağında en sık görülen vaskülit olan Henoch schönlein purpurasıyla iç Anadolu yöresinde takip edilen hastaların klinik özelliklerini, laboratuvar değerlerini ve bunlar arasındaki istatistiksel ilişkiyi ortaya koymak amacıyla yapıldı.
Gereç ve Yöntem: Bu retrospektif çalışmada Ekim 2016 ile Eylül 2018 tarihleri asında Selçuk Üniversitesi Tıp Fakültesi Çocuk Sağlığı ve Hastalıkları Anabilim Dalı Çocuk Romatoloji polikliniğine, Çocuk acil servisine, Çocuk Sağlığı polikliniğine ve Çocuk Nefroloji polikliniğine başvurup HSP tanısı alan 103 hastanın verileri, otomasyon epikriz kayıtları ve poliklinik dosyalarının incelenmesi ile değerlendirildi. Yaşın, anne-baba yaşının, cinsiyetin, tanı aldığı mevsimin, vücut ağırlığının, boyun, laboratuvar değerlerinin sistem tutulumlarının, nüks ve semptomlar üzerine etkileri araştırıldı.
Bulgular: Çalışmamıza dâhil edilen hastaların erkek/kız oranı yapılan çalışmalara benzer şekilde 1,34 olarak bulundu. Hastalar yaş gruplarına göre 10 yaşında büyük ve 10 yaşından küçük olacak şekilde 2 gruba ayrıldı. Başvuru mevsimleri incelendiğinde sonbahar ve kış aylarında tanı alan hasta sayılarının ilkbahar ve yaz aylarına göre belirgin fazla olduğu görüldü. Sistem tutulumu açısından dağılım incelendiğinde hastaların tamamında cilt tutulumu, %66’sında kas iskelet sistemi tutulumu, %51’inde GİS tutulumu, %15,5’inde böbrek tutulumu ve %8,7’sinde skrotal tutulum olduğu tespit edildi.
Sonuç: Daha önce yapılan çocukluk çağı HSP çalışmaları ile benzer şekilde büyük yaş ve WBC yüksekliği ile böbrek tutulumu arasında istatistiksel olarak anlamlı bir ilişki bulundu.
... HSV is generally well progressed, does not leave sequelae, and lasts approximately 4-6 weeks, yet relapse may occur. Many systemic ndings of HSV such as gastrointestinal, joints, skin, and kidney involvements have been reported before [1][2][3][4][5][6]. ...
Purpose: In this study, our purpose is to evaluate cochlear and vestibular function in juveniles with HSV using audiometry, DPOAEs, and cVEMP tests.
Methods: 40 children diagnosed with HSV from the pediatry clinic and 40 age and sex-matched healthy children were evaluated with distortion product otoacoustic emissions, audiometry, and cervical vestibular evoked myogenic potential (cVEMP) test in a tertiary hospital.
Results: The audiometry average values for both ears of HSV group and the control subjects were compared, and as a result, median 4.7 dB sensorineural hearing loss (SHL) was found for HSV group compared to control group at 250 Hz and it was statistically significant (p <0.001). An average of 6.4 dB SHL was detected at 8000 Hz (p <0.001). There was a statistically significant difference among HSV and control group regarding measurement results of average p1-n1 latency time of both ears (0.9 milliseconds (ms) increase, P = 0.035). In HSV patients, the median amplitude difference of both ears' average p1 n1 was found to be 5,6 millivolt, statistically significantly decreased compared to the control group (p = 0.003).
Conclusion: This study, firstly in literature, demonstrated that HSV may cause hearing loss and vestibular dysfunction in children. We think this might be due to autoimmune mechanisms.
... First, some autoantibodies triggered by KWD could persist in vivo for years, which in turn leads to some autoantibodymediated diseases or systemic autoimmune diseases so that seizure disorders or epilepsy could only be sentinel disorders of these diseases (46,47). Second, given that medium-sized vessel vasculitis is the bedrock of KWD, it is reasonable to infer that human brain, an organ rich in vessels, is subject to cryptogenic changes in structures, which contribute to epilepsy later in life (48)(49)(50). Although none has been proven, both are possible. ...
Background: Kawasaki disease is a common vasculitis of childhood in East Asia. The complications following Kawasaki disease mostly included cardiovascular sequelae; non-cardiac complications have been reported but less studied. This study investigated potential epilepsy following Kawasaki disease in Taiwanese children.
Objectives: Through National Health Insurance Research Database, we retrospectively analyzed the data of children aged <18 years with clinically diagnosed Kawasaki disease from January 1, 2000 to December 31, 2012 in Taiwan. These patients were followed up to estimate the incidence of epilepsy in the Kawasaki cohort in comparison with that in the non-Kawasaki cohort in Taiwan.
Results: A total of 8,463 and 33,872 patients in the Kawasaki and non-Kawasaki cohorts were included in the study, respectively. Of the total eligible study subjects, 61.1% were boys and 38.9% were girls; most patients with newly diagnosed Kawasaki disease were aged <5 years [88.1%]. Patients with Kawasaki disease showed a higher incidence rate [47.98 vs. 27.45 every 100,000 person years] and significantly higher risk [adjusted hazard ratio = 1.66, 95% confidence interval = 1.13–2.44] of epilepsy than those without the disease. Additionally, female sex [adjusted hazard ratio = 2.30, 95% confidence interval = 1.31–4.04] and age <5 years [adjusted hazard ratio = 1.82, 95% confidence interval = 1.22–2.72] showed a significantly higher risk of epilepsy in the Kawasaki cohort.
Conclusion: Results revealed a higher incidence rate and significant risk of epilepsy in Taiwanese children with Kawasaki disease than in those without the disease. Therefore, children diagnosed with Kawasaki disease are recommended follow-up as they have a high risk of epilepsy and seizure disorders.
... MRI brain with angiography can identify cerebral vasculitis with reasonable accuracy, hence included as primary modality in the evaluation [3] . Various treatment modalities have been used like IVIG, steroids, immunosuppressive agents to treat cerebral vasculitis and to prevent its long term complications [4] . We discuss here our experience and difficulties in treating a rare staphylococcal meningitis with CNS vasculitis. ...
... 81 There are no pathognomonic MRI findings in angiography-negative PACNS, underscoring the diagnostic limitations of neuroimaging and the need for pathologic correlation. 82 Lesions are usually multifocal, not restricted to a cerebral vessel territory, can be bilateral or unilateral, symmetric or asymmetric, and involve gray matter, white matter, or both. 83 MRI in subacute infarction typically shows hyperintense signal conforming to a laminar pattern on T1-weighted images without contrast, which reflects the resorptive phase of liquefactive cortical necrosis. ...
Vascular injury is increasingly recognized as an important cause of mortality and morbidity in children (29 days to 18 years of age). Since vascular brain injury in children appears to be less common than in adults, the index of suspicion for vascular brain injury is usually lower. In this review article, we describe frequent and rare conditions underlying pediatric stroke including cardioembolic, viral, autoimmune, post-traumatic, and genetic etiologies. Furthermore, we provide a neuroimaging correlate for clinical mimics of pediatric stroke. This review highlights the role of multimodal noninvasive neuroimaging in the early diagnosis of pediatric stroke, providing a problem-solving approach to the differential diagnosis for the neuroradiologist, emergency room physician, and neurologist.
