scholarly journals “When should primary angiitis of the central nervous system (PACNS) be suspected?”: literature review and proposal of a preliminary screening algorithm

2020 ◽  
Vol 41 (11) ◽  
pp. 3135-3148 ◽  
Author(s):  
Cristina Sarti ◽  
Antonella Picchioni ◽  
Roberta Telese ◽  
Marco Pasi ◽  
Ylenia Failli ◽  
...  
Keyword(s):  
Case Reports ◽  
Case Series ◽  
Neurological Deficits ◽  
Preliminary Screening ◽  
Screening Algorithm ◽  
Level Data ◽  
Primary Angiitis ◽  
The Central Nervous System ◽  
Selection Of

Abstract Background Primary angiitis of the CNS (PACNS) is a process causing variously combined neurological disturbances. Its rarity and kaleidoscopic presentation make it difficult to diagnose and even to suspect. Objective (1) To provide an up-to-date review on PACNS and (2) to create a preliminary screening algorithm based on clinical and radiological first-level data, useful to suspect PACNS and guide further investigations. Methods Review of PUBMED case series on PACNS, published from 2002 to 2017, collection of frequencies of clinical and neuroimaging features and calculation of median values. Classification of features as “major” or “minor” if frequency was higher or lower than median value. Combination of features in sets of criteria represented by all possible combinations of major and minor clinical and neuroradiological features. Application of criteria to published PACNS case reports and selection of the ones best identifying patients with definite PACNS. Results We reviewed 24 case series. “Major” clinical features were headache, stroke, cognitive impairment, focal neurological deficits; “minor” were seizures, altered consciousness, psychiatric disorders. “Major” neuroradiological features were multiple parenchymal lesions, parenchymal/meningeal contrast enhancement, magnetic resonance angiography vessel abnormalities, vessel wall enhancement; “minor” were parenchymal/subarachnoid hemorrhage, single parenchymal lesion. The selected sets of criteria able to identify all PACNS patients were (1) one clinical (major/minor) + one major neuroradiological feature; and (2) Two clinical (≥ 1 major) + one minor neuroradiological feature. Conclusion Our review provides a detailed clinical/neuroradiological picture of PACNS. The proposed algorithm should be regarded as a preliminary screening tool to move the first steps towards PACNS diagnosis that needs validation.

Sporadic Schwannomatosis: A Systematic Review Following the 2005 Consensus Statement

2018 ◽  
Vol 79 (05) ◽  
pp. 408-415 ◽  
Author(s):  
Jan Victor ◽  
Thibault Poujade ◽  
Nadine Hollevoet ◽  
Gregoire Chick
Keyword(s):  
Systematic Review ◽  
Consensus Statement ◽  
Case Reports ◽  
Case Series ◽  
Spinal Tumors ◽  
The Mean ◽  
Tumor Distribution ◽  
Key Aspects ◽  
Number Of Individuals

Aims To identify the frequency of reports of sporadic schwannomatosis, the types of patients affected, and the nerves affected. Patients and Methods We identified all case reports and case series that reported on patients with sporadic schwannomatosis according to established criteria. Results The initial search yielded 1,597 studies, of which 15 were included. A total of 38 of 55 individuals met the inclusion criteria. The mean age of the patients was 48 years; 41% were male. Thirty-three patients had peripheral nerve tumors, and 17 had spinal tumors. Twelve had tumors in both locations. Tumor distribution was unilateral in 25 of 30 cases (83.3%) and segmental (limited to one limb or five or fewer contiguous segments of the spine) in 28 of 38 cases (73.7%). Conclusion This systematic review quantified the number of individuals who meet the criteria for sporadic schwannomatosis and better described this population to facilitate the classification of neurofibromatosis in regard to the 2005 consensus statement. Unilateral or segmental distribution of nerve tumors are key aspects when dealing with multiple noncutaneous schwannomas without involvement of the vestibular nerve.

scholarly journals Primary Angiitis of the Central Nervous System: A Report of Three Cases from a Single Colombian Center

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Nicolás Coronel-Restrepo ◽  
Fabio Bonilla-Abadía ◽  
Omar A. Cortes ◽  
Jorge H. Izquierdo ◽  
Alberto M. Shinchi ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Case Reports ◽  
Clinical Manifestations ◽  
Brain Biopsy ◽  
System A ◽  
Primary Angiitis ◽  
The Central Nervous System ◽  
Systemic Manifestations ◽  
Low Incidence

The primary angiitis of the central nervous system (PACNS) is an entity with a very low incidence and prevalence. It is not clear why the inflammatory process of this entity is limited to the cerebral vasculature without systemic manifestations. 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. The vast majority of the treatment recommendations are given by series of case reports. The following paper described the clinical, imagenological, and histopathological characteristics of three Colombian patients with PACNS. The strategic therapeutic used in shown.

scholarly journals Pathogenesis and Classification of Paracocidioidomycosis: New Insights From Old Good Stuff

2020 ◽  
Author(s):  
Gil Benard ◽  
Gilda Del Negro
Keyword(s):  
Natural History ◽  
Case Reports ◽  
Case Series ◽  
Current View ◽  
Clinical Classification ◽  
Chronic Form

Abstract Different classifications of paracoccidioidomycosis emerged since its discovery in 1908, culminating in the proposition of a simplified and consensual one in 1987. However, by revisiting these classifications, case-reports or case-series from which the authors based their own, we found many patients who never truly fitted, neither in the 1987’s classification nor in the correspondent natural history/pathogenesis view. Herein, the concepts of paracoccidioidomycosis-infection, primary pulmonary paracoccidioidomycosis (PP-PCM) and other subclinical forms of PCM are reassessed. A classification is proposed to encompass all these subtle but distinct outcomes. We suggest a continuum between the PP-PCM and the overt chronic form of disease, and not the current view of quiescent foci, frozen in time and suddenly reactivated for unknown reasons. Failure to fully resolve the infection since its initial stages is a conceivable hypothesis for the chronic form. The proposed clinical classification might offer new insights to better characterize and manage PCM patients.

scholarly journals Implications of COVID-19 in high burden countries for HIV/TB: A systematic review of evidence

2020 ◽  
Author(s):  
Jacques L Tamuzi ◽  
Ayele T Birhanu ◽  
Constance S Shumba ◽  
Olatunji Adetokunboh ◽  
Jeannine Uwimana-Nico ◽  
...  
Keyword(s):  
Clinical Management ◽  
Observational Studies ◽  
Case Reports ◽  
Case Series ◽  
People Living With Hiv ◽  
Related Field ◽  
High Burden ◽  
Screening Algorithm ◽  
Clinical Trials Registry ◽  
Living With Hiv

Abstract Background The triple burden of COVID-19, tuberculosis and human immunodeficiency virus is one of the major global health challenges of the 21 st century. In high burden HIV/TB countries, the spread of COVID-19 among people living with HIV is a well-founded concern. A thorough understanding of HIV/TB and COVID-19 pandemics is important as the three diseases interact. This may clarify HIV/TB/COVID-19 as a newly related field and play an important role in the present and future management of the co-infections. However, several gaps are remaining in the knowledge of the burden of COVID-19 on patients with TB and HIV, the diagnosis, and management of these patients. Objectives The study was conducted to review different studies on SARS-CoV, MERS-CoV or COVID-19 associated with HIV/TB co-infection or only TB and to understand the interactions between HIV, TB and COVID-19 and its implications on the burden of the COVID-19 among HIV/TB co-infected or TB patients, screening algorithm and clinical management. Methods We conducted electronic search of potential eligible studies published in English in the Cochrane Controlled Register of Trials, PubMed, Medrxiv, Google scholar and Clinical Trials Registry databases. We included case studies, case series and observational studies published between January, 2002 and March, 2020 in which SARS-CoV, MERS-CoV and COVID-19 co-infected to HIV/TB or TB were managed in adult patients. We screened titles, abstracts and full articles for eligibility. As we anticipated heterogeneity in the literature, results were reported narratively. Main results After removing 69 duplicates, 24 out of 246 articles were assessed for eligibility, of which 9 studies were included for qualitative analysis. Among them, we included two case reports, four case series, one case-control and two retrospective observational studies. The studies have shown that TB may occur during or after SARS-CoV. In terms of severity, the proportion of severe/critical SARS, MERS and COVID cases with TB co-infection was higher than in patients with mild/moderate stages (P= 0.0008). Conclusion SARS/MERS-CoV/COVID-19 associated to HIV/TB or TB subjects had a higher risk of developing severe/critical than mild/moderate SARS/MERS-CoV/COVID-19. Diagnostic algorithms and clinical management were suggested for efficiently improving COVID-19/HIV/TB co-infections outcomes.

scholarly journals Pai syndrome: a review

2020 ◽  
Vol 36 (11) ◽  
pp. 2635-2640
Author(s):  
Francesca Olivero ◽  
Thomas Foiadelli ◽  
Sabino Luzzi ◽  
Gian Luigi Marseglia ◽  
Salvatore Savasta
Keyword(s):  
Corpus Callosum ◽  
Cleft Lip ◽  
Case Reports ◽  
Case Series ◽  
Rare Condition ◽  
Frontonasal Dysplasia ◽  
Pubmed Database ◽  
Median Cleft ◽  
The Central Nervous System

Abstract Background Pai syndrome is a rare idiopathic developmental condition characterized by midline craniofacial abnormalities. It was originally described as the presence of a median cleft lip, cutaneous polyps of the nasal mucosa and face, and midline lipomas of the central nervous system, mostly at the corpus callosum. However, there is great phenotypical variability and these characteristics are rarely all present at once. Objective The aim of this review was to analyze the available evidence regarding Pai syndrome in order to better delineate this rare condition and its features. Methods We analyzed the PubMed database using the words “Pai syndrome”, “frontonasal dysplasia”, “cleft lip”, “nasal polyp”, “facial polyp”, and “corpus callosum lipoma”, including reviews, case reports and case series. Conclusion There is no consensus regarding the diagnostic criteria of Pai syndrome up to date. It is usually diagnosed at birth, and its incidence is often underestimated. At present, the etiology of Pai syndrome is unknown. Several hypotheses regarding its genetic background have been made; however, there are not enough data yet to elucidate this point. An improved awareness could help in diagnosing the condition and performing the necessary investigations. These patients should have a multidisciplinary follow-up.

scholarly journals PD-1-inhibitor pembrolizumab for treatment of progressive multifocal leukoencephalopathy

2021 ◽  
Vol 14 ◽  
pp. 175628642199368
Author(s):  
Nora Möhn ◽  
Mike P. Wattjes ◽  
Ortwin Adams ◽  
Sandra Nay ◽  
Daria Tkachenko ◽  
...  
Keyword(s):  
Progressive Multifocal Leukoencephalopathy ◽  
Case Series ◽  
Neurological Deficits ◽  
Drug Induced ◽  
Fluid Analysis ◽  
Brain Infection ◽  
Causal Therapy ◽  
The Central Nervous System ◽  
Checkpoint Inhibitor Therapy ◽  
Promising Treatment

The reactivation of human JC polyoma virus (JCPyV) results in lytic infection of oligodendrocytes and neuronal cells. The corresponding clinical picture is called progressive multifocal leukoencephalopathy (PML) and results mostly from a disease-related or drug-induced immunosuppression. The opportunistic brain infection leads to a progressive demyelination of multiple areas of the central nervous system. Patients can present with various neurological deficits ranging from slight motoric symptoms to marked aphasia or reduced vigilance. Currently, there is no effective causal therapy for PML. Survival depends on the ability to achieve timely immune reconstitution. If the immune system cannot be restored, PML progresses rapidly and often ends fatally within months. Recently, some evidence for positive response has been reported in patients treated with immune checkpoint inhibitor therapy. Here, we provide a case series of three PML patients with underlying hematological malignancies who were treated with anti-PD-1-antibody pembrolizumab at Hannover Medical School. All patients received an extensive diagnostic follow-up including cerebrospinal fluid analysis, brain imaging, and lymphocyte-phenotyping via flow cytometry. Our patients had very different outcomes, with the only patient showing a specific anti-JCPyV immune response in the sense of an increased JCPyV antibody index clearly benefiting most from the treatment. Our results partly support the hypothesis that anti-PD-1 therapy may represent a promising treatment option for patients with PML. However, there is a current lack of pre-therapeutic stratification regarding the therapeutic response rates. Before larger studies can be initiated to further evaluate the efficacy of anti-PD-1 antibodies in PML, it is imperative to develop a reliable strategy for selecting suitable patients.

Organ system effects and reinfection of COVID-19: A systematic review

2021 ◽  
Vol 9 (1) ◽  
pp. 6-6
Author(s):  
Inderbir Padda ◽  
Nimrat Khehra ◽  
Urooj Jaferi ◽  
Dina Mosabbeh ◽  
Harshan Atwal ◽  
...  
Keyword(s):  
Case Reports ◽  
Meta Analysis ◽  
Case Series ◽  
Kidney Injury ◽  
Multiple Organ ◽  
Organ System ◽  
Fulminant Myocarditis ◽  
Preventative Measures ◽  
Organ Systems ◽  
The Central Nervous System

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and its effects on the organ systems have been summarized in recent literature with predominant pulmonary characteristics as a hallmark of the COVID-19 virus. Considering its accelerated appearance from Wuhan, China, in December 2019, extrapulmonary effects have been reported globally of SARS-CoV-2 involving the central nervous system (CNS), cardiovascular, gastrointestinal, renal, and hematologic systems; thus, the potential mechanisms, pathophysiology, clinical characteristics, management, outcome, and case reports per organ system are summarized in depth. The authors interpreted articles composed of case reports, case-series, meta-analysis, cohort studies, retrospective studies, and narrative reviews focusing on COVID-19 confirmed cases and their effects on the organ systems. Prevalent clinical organ system complexities include pneumonia, acute respiratory distress syndrome (ARDS), pulmonary hypertension, pulmonary embolism, hypertension, cardiac arrhythmias, myocarditis progressing to fulminant myocarditis, anorexia, nausea, vomiting, diarrhea, liver dysfunction, encephalopathy, encephalitis, meningitis, intracerebral hemorrhage, acute kidney injury, and hypercoagulability causing stroke and disseminated intravascular coagulation (DIC). This comprehensive literature review article will help clinicians and researchers gain insight about SARS-CoV-2 and its diverse effects on multiple organ systems involved, therefore help implement prospective management and preventative measures.

scholarly journals Primary angiitis of the central nervous system in children - case series

2011 ◽  
Vol 9 (S1) ◽  
Author(s):  
V Krakovská ◽  
R Stibitzová ◽  
T Pískovský ◽  
Z Vrábelová ◽  
M Macků ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Case Series ◽  
Primary Angiitis ◽  
The Central Nervous System
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