scholarly journals Paraneoplastic Neuropathies: What's New Since the 2004 Recommended Diagnostic Criteria

2021 ◽  
Vol 12 ◽  
Author(s):  
Marco Zoccarato ◽  
Wolfgang Grisold ◽  
Anna Grisold ◽  
Valentina Poretto ◽  
Federica Boso ◽  
...  
Keyword(s):  
Diagnostic Criteria ◽  
Cranial Nerves ◽  
Symptomatic Treatment ◽  
Cancer Therapies ◽  
Cancer Disease ◽  
Sensorimotor Polyneuropathy ◽  
Onconeural Antibodies ◽  
Immune Mediated ◽  
Advanced Stages ◽  
Paraneoplastic Neuropathy

The diagnostic criteria published by the PNS (Paraneoplastic Neurological Syndromes) Euronetwork in 2004 provided a useful classification of PNS, including paraneoplastic neuropathies. Subacute sensory neuronopathy (SSN) was the most frequently observed peripheral PNS, whereas other forms of neuropathy, as sensory polyneuropathy, sensorimotor polyneuropathy, demyelinating neuropathies, autonomic neuropathies, and focal nerve or plexus lesions, were less frequent. At the time of publication, the main focus was on onconeural antibodies, but knowledge regarding the mechanisms has since expanded. The antibodies associated with PNS are commonly classified as onconeural (intracellular) and neuronal surface antibodies (NSAbs). Since 2004, the number of antibodies and the associated tumors has increased. Knowledge has grown on the mechanisms underlying the neuropathies observed in lymphoma, paraproteinemia, and multiple myeloma. Moreover, other unrevealed mechanisms underpin sensorimotor neuropathies and late-stage neuropathies, where patients in advanced stages of cancer—often associated with weight loss—experience some mild sensorimotor neuropathy, without concomitant use of neurotoxic drugs. The spectrum of paraneoplastic neuropathies has increased to encompass motor neuropathies, small fiber neuropathies, and autonomic and nerve hyperexcitability syndromes. In addition, also focal neuropathies, as cranial nerves, plexopathies, and mononeuropathies, are considered in some cases to be of paraneoplastic origin. A key differential diagnosis for paraneoplastic neuropathy, during the course of cancer disease (the rare occurrence of a PNS), is chemotherapy-induced peripheral neuropathy (CIPN). Today, novel complications that also involve the peripheral nervous system are emerging from novel anti-cancer therapies, as targeted and immune checkpoint inhibitor (ICH) treatment. Therapeutic options are categorized into causal and symptomatic. Causal treatments anecdotally mention tumor removal. Immunomodulation is sometimes performed for immune-mediated conditions but is still far from constituting evidence. Symptomatic treatment must always be considered, consisting of both drug therapy (e.g., pain) and attempts to treat disability and neuropathic pain.

scholarly journals The incidence and pathophysiology of neurological symptoms in COVID-19

2021 ◽  
Vol 40 (4) ◽  
pp. 33-42
Author(s):  
Igor V. Litvinenko ◽  
Miroslav M. Odinak ◽  
Nikolay V. Tsygan ◽  
Aleksander V. Ryabtsev
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Rare Complication ◽  
Cranial Nerves ◽  
Neurological Symptoms ◽  
Immune Mediated ◽  
High Incidence ◽  
The Central Nervous System ◽  
Coronavirus Infection

The central nervous system seems to be quite vulnerable to SARS-CoV-2, leading to a variety of alteration pathways, high incidence and variability of the neurological symptoms of COVID-19. The COVID-19 symptoms, possibly associated with alteration to the central nervous system, include hyperthermia, shortness of breath, fatigue, headache, dizziness, dysphonia, dysphagia, hyposmia and anosmia, hypogeusia and ageusia, impairment of consciousness. The impairment of olfaction and gustation are the most common symptoms of the nervous system alteration (98% and 70%, respectively), which is most likely a consequence of the alteration of the receptors. Presumably the pathogenesis of dysphonia and dysphagia may involve neurodegenerative mechanisms or may be associated with a predominantly demyelinating alteration of the caudal cranial nerves. Pathomorphological findings in the brain of the COVID-19 patients include diffuse hypoxic and focal ischemic injuries of various sizes up to ischemic infarctions (in thrombosis of large arteries); microangiopathy; vasculitis; diapedetic and confluent hemorrhages with possible progression to hemorrhagic infarctions and rarely intracerebral hematomas. Acute cerebrovascular accident worsens the course of COVID-19 and can worsen the clinical outcome, taking into account the mechanisms of the central nervous system alteration in highly contagious coronavirus infections (SARS-CoV, MERS, SARS-CoV-2), including embolism, hypoxia, neurodegeneration, systemic inflammatory response and immune-mediated alteartion to the nervous tissue. A fairly rare complication of coronavirus infection, however, acute myelitis requires attention due to the severity of neurological disorders. The literature data show high incidence and polymorphism of the symptoms of the central nervous system alteration, as well as the important role of the cerebrovascular and neurodegenerative pathogenesis of brain alteration in COVID-19, which is taken into account in examining and treating the patients with new coronavirus infection. (1 figure, bibliography: 61 refs)

scholarly journals Diagnostic Criteria for Primary Autoimmune Cerebellar Ataxia—Guidelines from an International Task Force on Immune-Mediated Cerebellar Ataxias

2020 ◽  
Vol 19 (4) ◽  
pp. 605-610 ◽  
Author(s):  
Marios Hadjivassiliou ◽  
Francesc Graus ◽  
Jerome Honnorat ◽  
Sven Jarius ◽  
Maarten Titulaer ◽  
...  
Keyword(s):  
Diagnostic Criteria ◽  
Cerebellar Ataxia ◽  
Task Force ◽  
Immune Mediated ◽  
Autoimmune Cerebellar Ataxia ◽  
Cerebellar Ataxias

Surgical correction of severe dorsal tracheal membrane redundancy in a Belgian shepherd dog

2018 ◽  
Vol 6 (3) ◽  
pp. e000644
Author(s):  
Vlad Focsaneanu ◽  
Pieter Nelissen ◽  
Simon Tappin
Keyword(s):  
Weight Loss ◽  
Treatment Option ◽  
Chronic Cough ◽  
Exercise Tolerance ◽  
Symptomatic Treatment ◽  
Surgical Correction ◽  
Exercise Intolerance ◽  
Tracheal Lumen ◽  
Inspiratory Stridor ◽  
Immune Mediated

A two-year-old working Belgian shepherd dog was referred for diagnosis and management of a chronic cough, exercise intolerance and inspiratory stridor. A diagnosis of severe dorsal tracheal membrane redundancy was made based on radiography and tracheoscopy. Vomiting, weight loss and lethargy were also reported at initial presentation presumed secondary to dietary indiscretion, which was supported by the identification of undigested bones in the stomach on radiographs and ultrasound; these signs resolved with symptomatic treatment. The patient had previously been diagnosed with primary immune-mediated polyarthritis and had responded well to immunomodulatory medication. To correct the narrowing of the tracheal lumen, the dorsal tracheal membrane was plicated surgically, improving the tracheal diameter postoperatively. Twelve weeks later the dog had returned to full exercise, and the handler reported excellent exercise tolerance and no cough. Plication of excessive dorsal tracheal membrane is one possible treatment option for dogs with severe dorsal tracheal membrane redundancy.

scholarly journals Severe Fever with Thrombocytopenia Syndrome Virus Infection Associated with Hemophagocytic Lymphohistiocytosis as Poor Prognostic Factor

2017 ◽  
Vol 4 (suppl_1) ◽  
pp. S319-S319
Author(s):  
Sunmin Park ◽  
Juwon Kim ◽  
Hyo Youl Kim ◽  
Young Uh ◽  
Young Keun Kim
Keyword(s):  
Diagnostic Criteria ◽  
Clinical Features ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Inflammatory Diseases ◽  
Clinical Feature ◽  
Poor Prognostic Factor ◽  
Immune Mediated ◽  
Life Threatening ◽  
Christian Hospital ◽  
Severe Fever

Abstract Background Severe fever with thrombocytopenia (SFTS) is an emerging infectious disease caused by a novel bunyavirus designated SFTS virus (SFTSV) with a high fatality rate. Hemophagocytic lymphohistiocytosis (HLH) is an immune-mediated life-threatening disease triggered by infections, neoplasms and noninfectious inflammatory diseases. A few HLH associated with SFTSV were reported. According to the diagnostic criteria of HLH, 11 patients with SFTS were reviewed. Methods During last 2 years (2015–2016), 11 SFTS patients were diagnosed at the Wonju Severance Christian Hospital, Yonsei University Wonju College of Medicine, Wonju, South Korea. Clinical features were analyzed using diagnostic criteria of 2004-HLH trial. We described if the prognosis of SFTSV-infected patients was associated with clinical features of HLH. Results Of 11 patients, four patients were fulfillled the diagnostic criteria of 2004-HLH trial (five of eight criteria). Two patients were fulfilled the four criteria. Five patients were fulfilled three or less criteria. Three of six patients who fulfilled four or more criteria were died. There was no mortality in five patients who fulfilled three or less criteria. Hemophagocytosis in bone marrow (BM) was observed in all six patients who were taken BM study. Conclusion In SFTS, HLH was severe clinical feature and it might be associated with poor prognosis. Disclosures All authors: No reported disclosures.

scholarly journals Polyphenols as Modulator of Oxidative Stress in Cancer Disease: New Therapeutic Strategies

2016 ◽  
Vol 2016 ◽  
pp. 1-17 ◽  
Author(s):  
Anna Maria Mileo ◽  
Stefania Miccadei
Keyword(s):  
Oxidative Stress ◽  
Genome Instability ◽  
Antioxidant Activities ◽  
Therapeutic Strategies ◽  
Cellular Growth ◽  
Cancer Therapies ◽  
Cancer Disease ◽  
Antioxidant Agents ◽  
Cytotoxic Treatment ◽  
Therapeutic Tools

Cancer onset and progression have been linked to oxidative stress by increasing DNA mutations or inducing DNA damage, genome instability, and cell proliferation and therefore antioxidant agents could interfere with carcinogenesis. It is well known that conventional radio-/chemotherapies influence tumour outcome through ROS modulation. Since these antitumour treatments have important side effects, the challenge is to develop new anticancer therapeutic strategies more effective and less toxic for patients. To this purpose, many natural polyphenols have emerged as very promising anticancer bioactive compounds. Beside their well-known antioxidant activities, several polyphenols target epigenetic processes involved in cancer development through the modulation of oxidative stress. An alternative strategy to the cytotoxic treatment is an approach leading to cytostasis through the induction of therapy-induced senescence. Many anticancer polyphenols cause cellular growth arrest through the induction of a ROS-dependent premature senescence and are considered promising antitumour therapeutic tools. Furthermore, one of the most innovative and interesting topics is the evaluation of efficacy of prooxidant therapies on cancer stem cells (CSCs). Several ROS inducers-polyphenols can impact CSCs metabolisms and self-renewal related pathways. Natural polyphenol roles, mainly in chemoprevention and cancer therapies, are described and discussed in the light of the current literature data.

scholarly journals Characterization of the first symptoms of multiple sclerosis in a Brazilian center: cross-sectional study

2017 ◽  
Vol 135 (3) ◽  
pp. 222-225 ◽  
Author(s):  
Vitor Breseghello Cavenaghi ◽  
Fernanda Martinho Dobrianskyj ◽  
Guilherme Sciascia do Olival ◽  
Rafael Paternò Castello Dias Carneiro ◽  
Charles Peter Tilbery
Keyword(s):  
Multiple Sclerosis ◽  
Medical Center ◽  
Cranial Nerves ◽  
Cross Sectional Study ◽  
Common Symptom ◽  
Cross Sectional ◽  
Immune Mediated ◽  
Initial Symptoms ◽  
Mean Time

ABSTRACT CONTEXT AND OBJECTIVE: Multiple sclerosis (MS) is a chronic, immune-mediated and degenerative central nervous system (CNS) disease with well-established diagnostic criteria. Treatment can modify the course of the disease. The objective of this study was to describe the initial symptoms of multiple sclerosis in a Brazilian medical center. DESIGN AND SETTING: Descriptive study, conducted in a Brazilian reference center for multiple sclerosis treatment. METHODS: Data on 299 patients with confirmed diagnoses of MS were included in the study. Their medical files were evaluated and the data were analyzed. RESULTS: The most common symptom involved the cranial nerves (50.83%) and unifocal manifestation was presented by the majority of this population (73.91%). The mean time between the first symptom and the diagnosis was 2.84 years. Unifocal symptoms correlated with longer time taken to establish the diagnosis, with an average of 3.20 years, while for multifocal symptoms the average time taken for the diagnosis was 1.85 years. Unifocal onset was related to greater diagnostic difficulty. CONCLUSIONS: MS is a heterogeneous disease and its initial clinical manifestation is very variable.

MELANOTIC NONPSAMMOMATOUS TRIGEMINAL SCHWANNOMA AS THE FIRST MANIFESTATION OF CARNEY COMPLEX

Neurosurgery ◽  
2006 ◽  
Vol 59 (6) ◽  
pp. E1334-E1335 ◽  
Author(s):  
Carmen A. Carrasco ◽  
David Rojas-Salazar ◽  
Renato Chiorino ◽  
Juan C. Venega ◽  
Nelson Wohllk
Keyword(s):  
Diagnostic Criteria ◽  
Cranial Nerves ◽  
Cardiac Myxoma ◽  
Carney Complex ◽  
Regulatory Subunit ◽  
Nerve Sheath Tumor ◽  
Psammoma Bodies ◽  
Middle Fossa Approach ◽  
Melanotic Schwannoma ◽  
The Right

Abstract OBJECTIVE Melanotic schwannoma is a rare neoplasm, classifiable as a peripheral nerve sheath tumor, and differentiated from a typical schwannoma by heavy pigmentation. Psammoma bodies can be visualized in more than 50% of melanotic schwannomas. Half of patients with such “psammomatous melanotic schwannomas” have Carney complex, a dominantly transmitted autosomal disorder. Most recently, the tumor suppressor gene, PRKAR1A, coding for the Type 1α regulatory subunit of protein kinase A was found to be mutated in approximately half of the known Carney complex families. Although cranial schwannomas have been described in patients with Carney complex, their numbers are too small to be considered a definite part of the syndrome. Furthermore, only melanotic schwannomas with psammoma bodies are included as diagnostic criteria for Carney complex. The objective of this report is to communicate a case of trigeminal nonpsammomatous melanotic schwannoma as the first manifestation of Carney complex. CLINICAL PRESENTATION A 34-year-old woman presented with odontalgia, right V3hypoesthesia, V2paresthesia, and diplopia. Magnetic resonance imaging scans of the brain revealed a small tumor with homogenous contrast in the right trigeminal pathway. INTERVENTION We performed an extradural approach to the right cavernous sinus by a middle fossa approach. The lateral wall was opened between the cranial nerves, and a soft and black tumor was resected in a piecemeal fashion. Histology and immunohistochemical analysis of the tumor were compatible with melanotic schwannoma, but no psammomatous bodies were identified. Endocrine evaluation showed that this patient's symptoms fulfilled the diagnostic criteria of Carney complex, with lentiginosis, multiple breast ductal adenomas, multiple hypoechoic nodules on thyroid ultrasonography, and a 4 × 5-cm asymptomatic atrial cardiac myxoma, which was removed 15 days after the neurosurgery. Three months later, a recurrence of melanotic schwannoma was identified. Molecular analyses of genomic and somatic deoxyribonucleic acid from the patient found a 578 to 579delTG mutation of PRKAR1A. CONCLUSION We present the unusual case of a nonpsammomatous trigeminal melanotic schwannoma associated with Carney complex, with confirmed PRKAR1Agene mutation. Our case highlights that neurosurgeons, in the presence of a melanotic schwannoma, should be aware of the features of the Carney complex because, in such cases, pre- and postoperative management is significantly affected. We also postulate that the absence of psammoma bodies or cranial localization do not exclude this diagnosis.

scholarly journals Exosomes and Brain Metastases: A Review on Their Role and Potential Applications

2021 ◽  
Vol 22 (19) ◽  
pp. 10899
Author(s):  
Filipa D. Oliveira ◽  
Miguel A. R. B. Castanho ◽  
Vera Neves
Keyword(s):  
Brain Metastases ◽  
Intercellular Communication ◽  
Therapeutic Strategies ◽  
Cancer Therapies ◽  
Life Prognosis ◽  
Potential Applications ◽  
Advanced Stages ◽  
Promising Application

Brain metastases (BM) are a frequent complication in patients with advanced stages of cancer, associated with impairment of the neurological function, quality of life, prognosis, and survival. BM treatment consists of a combination of the available cancer therapies, such as surgery, radiotherapy, chemotherapy, immunotherapy and targeted therapies. Even so, cancer patients with BM are still linked to poor prognosis, with overall survival being reported as 12 months or less. Intercellular communication has a pivotal role in the development of metastases, therefore, it has been extensively studied not only to better understand the metastization process, but also to further develop new therapeutic strategies. Exosomes have emerged as key players in intercellular communication being potential therapeutic targets, drug delivery systems (DDS) or biomarkers. In this Review, we focus on the role of these extracellular vesicles (EVs) in BM formation and their promising application in the development of new BM therapeutic strategies.

scholarly journals Tolosa-Hunt syndrome: is it really necessary to show granuloma? - The report of eight cases

2017 ◽  
Vol 74 (3) ◽  
pp. 287-293 ◽  
Author(s):  
Ana Podgorac ◽  
Jasna Zidverc-Trajkovic ◽  
Zagorka Jovanovic ◽  
Aleksandar Ristic ◽  
Aleksandra Radojicic ◽  
...  
Keyword(s):  
Diagnostic Criteria ◽  
Cranial Nerve ◽  
Clinical Presentation ◽  
Correct Diagnosis ◽  
Cranial Nerves ◽  
Granulomatous Inflammation ◽  
Diagnostic Procedures ◽  
Hunt Syndrome ◽  
Number Of Patients ◽  
Significant Difference

Introduction. Tolosa?Hunt syndrome (THS) is a rare entity, characterized by unilateral orbital pain associated with paresis of one or more of the oculomotor cranial nerves and caused by a granulomatous inflammation in the cavernous sinus, superior orbital fissure or orbit. The low prevalence of THS with a broad spectrum of other disorders that could cause painful ophtalmoplegia resulted in a stricter diagnostic criteria of THS in the latest edition of the International Classification of Headache Disorders. Current criteria require demonstration of granuloma by magnetic resonance imaging or biopsy. The diagnosis could be difficult and the initiation of treatment delayed due to a high variablity of clinical presentation of TSH. Reducing the number of patients that, based on clinical presentation, could be classified as having THS, but do not fullfil all diagnostic criteria further complicates establishing of correct diagnosis. Case report. Hereby we presented eight patients diagnosed with and treated for THS. Inspite the exclusion of other causes of painful ophtalmoplegia, granuloma could not be demonstrated in a half of patients. Clinical presentation of THS in patients with and without shown granuloma, did not significantly differ concerning headache characteristics (localization, intensity, quality, duration preceding cranial nerve palsy, response to steroids), the affected cranial nerve, disease course and response to the treatment, as well as types of diagnostic procedures that were performed in ruling out other diseases from the extensive differential diagnosis of painful ophthalmoplegia. Conclusion. There is no significant difference between the THS patients with and without demonstrated granuloma.

scholarly journals Clinical and Immunological Profile of Mixed Connective Tissue Disease and a Comparison of Four Diagnostic Criteria

2020 ◽  
Vol 2020 ◽  
pp. 1-6 ◽  
Author(s):  
Kevin John John ◽  
Mohammad Sadiq ◽  
Tina George ◽  
Karthik Gunasekaran ◽  
Nirmal Francis ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Diagnostic Criteria ◽  
Connective Tissue Disease ◽  
Lupus Erythematosus ◽  
Mixed Connective Tissue Disease ◽  
Cross Sectional Study ◽  
Cross Sectional ◽  
Immunological Profile ◽  
Immune Mediated ◽  
Organ Systems

Mixed connective tissue disease (MCTD) was initially described as a chronic immune-mediated disease with overlapping features of systemic lupus erythematosus, scleroderma, and polymyositis. We conducted a cross-sectional study to describe the clinical and immunological profile of patients with MCTD and to compare the four diagnostic criteria, namely, Sharp, Kasukawa, Alarcón-Segovia, and Khan criteria. A total of 291 patients who were admitted from June 2007 to June 2017 and fulfilled the inclusion criteria were included in the study. A clinical diagnosis of MCTD was made in 111 patients, of whom 103 (92.8%) were women. The mean age at presentation was 39.3 years (SD±11.6). The most common organ systems that were involved were musculoskeletal system (95.5%), skin and mucosa (78.4%), and the gastrointestinal and hepatobiliary systems (56%). The maximum sensitivity was for the Kasukawa criteria with a sensitivity of 77.5% (95% CI 68.4-84.6) and specificity of 92.2% (95% CI 87-95.5). The Kahn criteria and Alarcón-Segovia criteria had the maximum specificity; the Alarcón-Segovia criteria had a sensitivity of 69.4% (95% CI 59.8-77.6) and a specificity of 99.4% (95% CI 96.5-99.9), while the Kahn criteria had a sensitivity of 52.3% (95% CI 42.6-61.7) and a specificity of 99.4% (95% CI 96.5-99.9). The sensitivity and specificity of Sharp criteria were 57.7% (95% CI 47.9-66.87) and 90% (95% CI 84.4-93.8), respectively.

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