Search

Objectives Paraneoplastic arthritis (PA) is one of the paraneoplastic syndromes. Both laboratory and clinical findings similar to rheumatological diseases can be seen. In this study we aimed to present the clinical and laboratory findings, malignancy types and pathological diagnoses of patients with paraneoplastic arthritis. Methods In a multicentre retrospective study, 92 patients with PA from the last 10 years were included. Results Patients with PA and haematological malignancies exhibited the highest ratio of lymphomas (25.6%). The most common cancer detected in patients with solid malignancy and PA was lung cancer (41.5%). All malignant patients with PA had significant anti-CCP positivity compared with the healthy control group (P  = 0.014). Conclusion Although PA is a rare condition, it can be confused with many rheumatological diseases. The most commonly involved joint is the knee joint, followed by the ankle and hand/wrist. Autoantibody negativity, high lactate dehydrogenase level and arthritis unresponsive to treatment constitute important clues for diagnosis. [ABSTRACT FROM AUTHOR]

Germ cell tumors of childhood are tumors arising from germline cells in gonadal or extragonadal locations. Extragonadal germ cell tumors are characteristically located in the midline, arising intracranially or in the mediastinum, retroperitoneum, or pelvis. These tumors are generally easily diagnosed due to typical sites of origin, characteristic imaging findings, and laboratory markers. However, germ cell tumors can be associated with unusual clinical syndromes or imaging features that can perplex the radiologist. This review will illustrate atypical imaging/clinical manifestations and complications of abdominal germ cell tumors in childhood. These features include unusual primary tumors such as multifocal primaries; local complications such as ovarian torsion or ruptured dermoid; atypical presentations of metastatic disease associated with burned-out primary tumor, growing teratoma syndrome, and gliomatosis peritonei; endocrine manifestations such as precocious puberty and hyperthyroidism; and antibody mediated paraneoplastic syndrome such as anti-N-methyl-D-aspartate-receptor antibody-mediated encephalitis. This review aims to illustrate unusual imaging features associated with the primary tumor, metastatic disease, or distant complications of abdominal germ cell tumors of childhood. [ABSTRACT FROM AUTHOR]

Paraneoplastic neurological syndromes (PNS) are a complex of neurological symptoms caused by the body’s autoimmune reaction against the tumour. The autoimmune process can affect different structures in both the peripheral and central nervous systems, thus the clinical manifestation of PNSs can be very diverse. This article presents a 53-year-old man who developed psychosis for the first time in his life. The disorder manifested with atypical clinical symptoms and a limited response to psychopharmacotherapy was observed, which raised the suspicion of an organic origin of the disorder. A detailed diagnostic workup revealed carcinoma of the right kidney. This pathology was assessed as unrelated to the mental disorder. A diagnosis of unspecified dementia was made, suspecting Lewy body dementia. After the patient had completed active oncological treatment, a significant positive dynamics in his mental status was observed during a follow-up consultation with a psychiatrist. The diagnosis of dementia was excluded. Restrospecitvely assesed, the psychiatric disorder was of secondary origin, i.e. a manifestation of paraneoplastic neurological syndrome. [ABSTRACT FROM AUTHOR]

Background and purpose: Paraneoplastic neurological autoimmunity is well described with small‐cell lung cancer, but information is limited for other neuroendocrine neoplasms (NENs). Methods: Adult patients with histopathologically confirmed non‐pulmonary NENs, neurological autoimmunity within 5 years of NEN diagnosis, and neural antibody testing performed at the Mayo Clinic Neuroimmunology Laboratory (January 2008 to March 2023) were retrospectively identified. Control sera were available from patients with NENs without neurological autoimmunity (116). Results: Thirty‐four patients were identified (median age 68 years, range 31–87). The most common primary tumor sites were pancreas (nine), skin (Merkel cell, eight), small bowel/duodenum (seven), and unknown (seven). Five patients received immune checkpoint inhibitor (ICI) therapy before symptom onset; symptoms preceded cancer diagnosis in 62.1% of non‐ICI‐treated patients. The most frequent neurological phenotypes (non‐ICI‐treated) were movement disorders (12; cerebellar ataxia in 10), dysautonomia (six), peripheral neuropathy (eight), encephalitis (four), and neuromuscular junction disorders (four). Neural antibodies were detected in 55.9% of patients studied (most common specificities: P/Q‐type voltage‐gated calcium channel [seven], muscle‐type acetylcholine receptor [three], anti‐neuronal nuclear antibody type 1 [three], and neuronal intermediate filaments [two]), but in only 6.9% of controls. Amongst patients receiving cancer or immunosuppressive therapy, 51.6% had partial or complete recovery. Outcomes were unfavorable in 48.3% (non‐ICI‐treated) and neural autoantibody positivity was associated with poor neurological outcome. Discussion: Neurological autoimmunity associated with non‐pulmonary NENs is often multifocal and can be treatment responsive, underscoring the importance of rapid recognition and early treatment. [ABSTRACT FROM AUTHOR]

Background and objectives: Dysautonomia has been associated with paraneoplastic neurological syndrome (PNS)-related mortality in anti-Hu PNS, but its frequency and spectrum remain ill-defined. We describe anti-Hu patients with dysautonomia, estimate its frequency, and compare them to patients without dysautonomia. Methods: Patients with anti-Hu antibodies diagnosed in the study centre (1990–2022) were retrospectively reviewed; those with autonomic signs and symptoms were identified. Results: Among 477 anti-Hu patients, 126 (26%) had dysautonomia (the only PNS manifestation in 7/126, 6%); gastrointestinal (82/126, 65%), cardiovascular (64/126, 51%), urogenital (24/126, 19%), pupillomotor/secretomotor (each, 11/126, 9%), and central hypoventilation (10/126, 8%). Patients with isolated CNS involvement less frequently had gastrointestinal dysautonomia than those with peripheral (alone or combined with CNS) involvement (7/23, 30% vs. 31/44, 70% vs. 37/52, 71%; P = 0.002); while more frequently central hypoventilation (7/23, 30% vs. 1/44, 2.3% vs. 2/52, 4%; P < 0.001) and/or cardiovascular alterations (18/23, 78% vs. 20/44, 45% vs. 26/52, 50%; P = 0.055). Median [95% CI] overall survival was not significantly different between patients with (37 [17; 91] months) or without dysautonomia (28 [22; 39] months; P = 0.78). Cardiovascular dysautonomia (HR: 1.57, 95% CI [1.05; 2.36]; P = 0.030) and central hypoventilation (HR: 3.51, 95% CI [1.54; 8.01]; P = 0.003) were associated with a higher risk of death, and secretomotor dysautonomia a lower risk (HR: 0.28, 95% CI [0.09; 0.89]; P = 0.032). Patients with cardiovascular dysautonomia dying ≤ 1 year from clinical onset had severe CNS (21/27, 78%), frequently brainstem (13/27, 48%), involvement. Discussion: Anti-Hu PNS dysautonomia is rarely isolated, frequently gastrointestinal, cardiovascular and urogenital. CNS dysfunction, particularly brainstem, associates with lethal cardiovascular alterations and central hypoventilation, while peripheral involvement preferentially associates with gastrointestinal or secretomotor dysautonomia, being the latest more indolent. [ABSTRACT FROM AUTHOR]

PNS are uncommon manifestations of cancer. The current literature about these syndromes in the setting of cHL is disintegrated. A systematic literature review of all published literature was conducted. One hundred twenty-eight patients from 115 publications met the inclusion/exclusion criteria. Eight-five patients were of the NS subtype (66.4%). The most frequent clinical presentation of the PNS was CNS manifestation (25.8%). The majority of patients were diagnosed with the cHL and PNS simultaneously (42.2%). In 33.6% of patients, the lymphoma diagnosis preceded the PNS diagnosis. In 16.4% of patients, the PNS diagnosis preceded the lymphoma diagnosis. The presence of PNS antibodies was reported in 35 patients (27.3%). Age older than 18 was associated with higher prevalence of PNS. The CR rate of the lymphoma was 77.3%. The complete resolution rate of the PNS was 54.7%. Relapse of lymphoma was reported in 13 patients, and recurrence of the PNS upon relapse was reported in 10/13 patients. [ABSTRACT FROM AUTHOR]

Paraneoplastic neurological syndromes (PNSs) are an uncommon complication of cancer, affecting nearby 1/10,000 subjects with a tumour. PNSs can involve all the central and peripheral nervous systems, the muscular system, and the neuromuscular junction, causing extremely variable symptomatology. The diagnosis of the paraneoplastic disease usually precedes the clinical manifestations of cancer, making an immediate recognition of the pathology crucial to obtain a better prognosis. PNSs are autoimmune diseases caused by the expression of common antigens by the tumour and the nervous system. Specific antibodies can help clinicians diagnose them, but unfortunately, they are not always detectable. Immunosuppressive therapy and the treatment of cancer are the cornerstones of therapy for PNSs. This paper reports a case of PNSs associated with breast tumours and focuses on the most common paraneoplastic neurological syndromes. We report a case of a young female with a clinical syndrome of the occurrence of rigidity in the right lower limb with postural instability with walking supported and diplopia, with a final diagnosis of paraneoplastic cerebellar degeneration and seronegative rigid human syndrome associated with infiltrating ductal carcinoma of the breast. [ABSTRACT FROM AUTHOR]

The relentless advancement of chemotherapeutic agents has enhanced survival rates among cancer patients. However, this success comes with an increased prevalence of chemotherapy-induced neurotoxicity, which often mimics the symptoms of metastatic disease or paraneoplastic syndromes and poses a diagnostic challenge for clinicians. Imaging, particularly MRI, plays a pivotal role in unraveling this conundrum. This comprehensive review explores the MRI patterns associated with central neurotoxicities induced by various chemotherapeutic agents. Our objective is to provide radiologists and clinicians with illustrative diagrams that offer a structured approach to diagnosing these conditions. By enhancing the understanding of these distinctive MRI patterns, we aim to facilitate accurate and timely diagnosis, ultimately improving patient care in the context of evolving cancer treatments. Critical relevance statement This article describes the essential role of MRI in identifying distinct patterns of chemotherapy-induced central neurotoxicity, enabling early diagnosis and improved patient care within the field of clinical radiology. Key points • Chemotherapy-induced neurotoxicity is a growing concern for cancer patients, and MRI is a key tool in diagnosis. • This review highlights distinctive MRI patterns associated with various chemotherapy-induced neurotoxicities. • Understanding these patterns improves patient care, ensuring timely intervention and accurate diagnosis in the complex world of cancer treatment. [ABSTRACT FROM AUTHOR]

Background: Malignant neoplasms are a well-recognized global public health concern, with significant impacts on human health and quality of life. The interplay between tumors and autoimmune rheumatic diseases is complex, and the resulting tumor-associated rheumatic diseases represent a rare and intricate group of conditions that occur in the context of malignant tumors. In addition, various rheumatic diseases can arise as a consequence of oncology treatment. These diseases present with intricate clinical manifestations and pathological features, often rendering them challenging to diagnose and impacting patients' quality of life. Despite this, they have yet to be fully recognized. Methods: This article presents a literature review of published original articles and review articles concerning paraneoplastic rheumatic syndromes and rheumatic diseases associated with cancer treatment. We conducted a comprehensive literature search in PubMed, Web of Science and Google Scholar databases, excluding duplicated and irrelevant studies. In cases of duplicated research, we selected articles with higher impact factors for the review. Results: This review focuses on the clinical features, diagnosis, and treatment of paraneoplastic rheumatic diseases, as well as the pathogenesis of these diseases. Additionally, we summarize the autoimmune rheumatic diseases associated with cancer treatment. Ultimately, the goal of this review is to enhance recognition and improve the management of autoimmune rheumatic diseases related to tumors. [ABSTRACT FROM AUTHOR]

Background: Neuroblastomas are the most common extracranial solid malignancy in children with variable manifestations and complications depending on the presence of paraneoplastic syndromes. Materials and Methods: We performed a single institution retrospective cohort study of all patients less than 18 years old diagnosed with neuroblastoma or ganglioneuroblastoma between January 2002 and July 2022. Patients were identified through the pathology and cancer registry and cross-referenced with pediatric records. Patient demographics, clinical presentation, treatment, and outcomes were collected. A univariate descriptive analysis of the collected data was conducted. Results: In our study period, 130 children were diagnosed with neuroblastoma, and 15 were diagnosed with ganglioneuroblastoma. There were 12 children with a paraneoplastic syndrome identified, 8 with NBL and 4 with ganglioneuroblastoma (GNBL). The average age at diagnosis was 22 months. All but 1 underwent resection prior to treatment of paraneoplastic syndrome, and 4 children required neoadjuvant therapy. Neurological complications were the most common with 10 children (83%). The average time from symptom onset to diagnosis was 0.7 months. Eight children had complete resolution of their symptoms after treatment and resection, 2 children recently started treatment within a year, 1 had partial resolution, and 1 died during treatment. The presence of tumor-infiltrating lymphocytes occurred in 4 children with neurologic paraneoplastic syndromes. Six children had neuropil rich tumors. Conclusion: The histological profile of paraneoplastic syndromes of neuroblastoma and ganglioneuroblastoma and their treatment across a single institution can be highly variable. The presence of tumor-infiltrating lymphocytes and neuropil may have an impact on paraneoplastic pathology. [ABSTRACT FROM AUTHOR]

Background: Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare neurological condition with paraneoplastic etiology in about 20% of cases, usually presenting before or shortly after the oncological diagnosis is established. PERM associated with anti-glycine receptor antibodies is not previously reported in a patient with bladder cancer. Case presentation: A 72-years-old Caucasian male was admitted with acute onset of dysarthria, dysphagia and trismus three years after initial surgical treatment for bladder cancer. The condition was initially diagnosed as tetanus and treated accordingly, but the diagnosis was reconsidered because of progression despite adequate treatment. Diagnostic workup on readmission revealed lung and paraaortal metastases from bladder cancer and anti-glycine receptor (anti-GlyR) antibodies both in the cerebrospinal fluid and in serum, which supplemented with the clinical presentation led to the diagnosis of PERM, presumably related to bladder cancer. The patient showed improvement and stabilization after treatment with intravenous immunoglobulin and chemotherapy against metastatic bladder cancer. Conclusion: To the best of our knowledge, this is the first reported case of anti-GlyR antibody positive PERM related to urothelial carcinoma. The symptoms mimicked tetanus, and responded to chemotherapy and immunotherapy. [ABSTRACT FROM AUTHOR]

Lung cancer is a common cause of mortality in Indian population amongst cancer causes. Risk factor identification and past medical comorbidities are predisposing factors for occurrence of carcinoma. Identification of other symptoms associated with endogenous hormone release could have positive outcome by early diagnosis of lung cancer. Method: This prospective observational study was conducted in 18-80 year patients of armed forces hospital who met the inclusion criteria. Clinical presentation, all risk factors were identified, paraneoplastic manifestations noted and managed. Results: Mean age was 61.5 years, 48 males and 12 females with associated risk factor of smoking intake identified. Cough (58%) and chest pain (18%) were main presenting symptoms. Cachexia, hypercalcemia, SIADH and superior mesenteric artery thrombosis were paraneoplastic manifestations noted. Conclusion: Delayed diagnosis due to nonspecific symptoms are responsible for worrisome outcome of lung cancer. Vigilant diagnosis of paraneoplastic manifestations can help in early diagnosis of primary carcinoma and better outcome. [ABSTRACT FROM AUTHOR]

Key Clinical Message: This case report emphasizes that we should analyze a patient's signs and symptoms as a whole rather than relying exclusively on a common pattern to diagnose the condition and indicates that thorough histological investigation and sample collection are needed to accurately diagnose this malignancy. Angiosarcoma is a rare, fatal, and poorly understood malignant tumor of vascular endothelial cells which is a challenging disease to diagnose in the clinical settings and requires early diagnosis to achieve a favorable prognosis. Paraneoplastic syndromes associated with angiosarcoma can include hypercoagulability, thrombocytopenia, anemia, fever, weight loss, and night sweats. In some cases, the paraneoplastic syndrome can be the first sign of the underlying malignancy. Here, we present a 47‐year‐old individual with angiosarcoma over the right scapula accompanied by hemoptysis and other pulmonary complaints whom at first was thought to be metastatic polmunary involvement. However, the patient's dramatic response to corticosteroids, in addition to further imaging and paraclinical studies, led us to an acute eosinophilic pneumonia (AEP) diagnosis which is an eosinophilic infiltrations of alveolar spaces. The patient received chemotherapy for angiosarcoma and radiation, since the brachial nerve network was disrupted, leaving the tumor unresectable. After 3 years of continuous follow‐up, the patient is now completely cured. [ABSTRACT FROM AUTHOR]

Background: In literature, a few reports described an association between paraneoplastic pemphigus (PNP) and Castelman's disease (CD), but no consensus have been proposed for the diagnostic-therapeutical approach. Aim of this study is to present a case report and explore the relationship between PNP and CD in pediatric patients, focusing on clinical manifestations, histopathological findings, treatment and outcome to find elements for an early diagnosis. Case presentation: We present the clinical case of a 13 years old girl with a challenging diagnosis of PNP and CD who underwent therapy at first with Rituximab and then with Siltuximab, for the control of symptoms. Conclusions: Reviewing literature, 20 clinical cases have been described in the pediatric age. Diagnosis may be challenging, requiring an average of 3 months (range from 3 weeks to 2 years). In all cases, the initial manifestations were mucocutaneous lesions, especially oral lesions with poor response to conventional treatment. Systemic symptoms may be present as well. Therapeutical approach is still discussed with no consensus. Almost all patients received corticosteroids with poor response. Other drugs including azathioprine, methotrexate, cyclosporine and monoclonal antibodies have been evaluated for the control of the disease. Further studies and experimental trials urge to define the diagnostic criteria and therapy protocol. [ABSTRACT FROM AUTHOR]

Mastocytoma is one of the most common skin tumours in dogs. It is characterised by a specific course, unpredictable behaviour, and prognosis. This tumour is dangerous because it mimics the external signs of other neoplasms, from less-threatening lipoma to skin cancer. The purpose of the study is to conduct pathomorphological verification and classification of neoplasia in dogs diagnosed during a clinical examination and to establish the features of the morphological manifestation of the skin form of mastocytoma in them. The material for the studies was obtained by excisional biopsy during surgical interventions for skin neoplasms. The diagnosis of mastocytoma was established based on the results of a clinical examination of animals and the examination of biopsy material of neoplasms using cytological and histological research methods. It was established that the share of skin neoplasia in the structure of oncological diseases of dogs was 30.12%. Among the animals with skin neoplasia that were examined, mastocytoma was diagnosed in 17.55% of dogs aged 3 to 14 years. The highest incidence rates were established in dogs aged 7 to 12 years. Most often, skin mastocytoma was diagnosed in dogs of the following breeds: Shar Pei -- 13.5%; Labrador -- 9.5%; Boxer -- 8.2%. Isolated cases of tumour formations were observed in animals of such breeds as: German shepherd, Spaniel, Central Asian Shepherd, and Pug. Depending on gender: males accounted for 52.2%, females -- 47.8%. In 50% of the examined animals, mastocytomas developed with rapid dynamics, which indicates a substantial aggressiveness of tumour growth. Clinically, signs of dehydration and gastrointestinal disorders were detected in dogs, which indicates the development of paraneoplastic syndrome. According to the results of sonography, it was established that skin mastocytomas are visualised as heterogeneous hypoechoic structures with indistinct contours and uneven edges. Based on the results of the study, new information was obtained regarding the morphological features of the manifestation and development of mastocytes in dogs. Thus, the obtained results complement and expand knowledge about the pathogenesis of mastocytoma in dogs, the frequency of spread and features of the course of this oncological pathology in a separate geographical population of Ukraine [ABSTRACT FROM AUTHOR]

Background: Paraneoplastic neurological syndromes have diverse clinical presentations and offer an opportunity for early diagnosis of malignancy and treatment. Recently, a new paraneoplastic syndrome associated with seminoma was described, consisting of rhombencephalitis with antibodies targeting the Kelch-like protein 11 (KLHL11). Questions were raised as to the spectrum of clinical symptoms and strength of association to seminoma. Methods: We present a 45-year-old man with bilateral sensorineural hearing loss, vertigo, and progressive ataxia. An extensive diagnostic workup led to the diagnosis of anti-KLHL11 paraneoplastic syndrome based on an immunofluorescence assay showing a typical pattern and a confirmatory serological assay. As a result, the patient underwent a meticulous search for an underlying seminoma. Results: Although initially, all images were interpreted as negative, a revision of the positron emission tomography-CT (PET-CT) examination identified a small mediastinal suspicious mass. The mass was resected, and pathological examination confirmed it to be an extra-testicular seminoma. Conclusions: Patients presenting with progressive sensorineural hearing loss, vertigo, and ataxia should be evaluated for KLHL11 paraneoplastic syndrome. Furthermore, we support a strong association between anti-KLH11 rhombencephalitis and an underlying seminoma and recommend a thorough search for an undiagnosed germ cell tumor in these patients. [ABSTRACT FROM AUTHOR]

Thymic tumours are rare thoracic malignancies with thymic carcinoma accounting for approximately 12% of all thymic tumours compared to thymomas which account for approximately 86%. Unlike thymomas, it is very rare for thymic carcinomas to be associated with autoimmune disorders or paraneoplastic syndromes. When these phenomena do occur, the vast majority are myasthenia gravis, pure red cell aplasia, or systemic lupus erythematous. Paraneoplastic Sjogren's syndrome is a rare complication of thymic carcinoma, with only two cases previously reported. Here we present 2 cases of patients with metastatic thymic carcinoma who developed autoimmune phenomena consistent with Sjogren's syndrome without classical symptoms prior to treatment. One patient opted for surveillance of their malignancy, while the other underwent chemoimmunotherapy with favourable results. These case reports describe two distinctive clinical presentations of a rare paraneoplastic phenomenon. [ABSTRACT FROM AUTHOR]

Seizure is a rare symptom of paraneoplastic syndrome seen in neuroblastoma without a previous history. A 4-month-old male patient who was followed up with a preliminary diagnosis of an adrenal mass in pediatric oncology was admitted to hospital with a seizure. A diagnosis of undifferentiated neuroblastoma was made with a biopsy from an adrenal mass. Seizures were resistant to anti-epileptic therapy and they were completely under control with steroids on the 4th day of treatment. Electroencephalography (EEG) disturbances disappeared and no neurologic deficit was detected. This case, which presented with isolated seizure symptoms of neuroblastoma and was treated with steroids, was a very rare presentation in which symptoms and EEG disturbances disappeared. In neuroblastoma, autoimmunity may be involved in the pathogenesis of seizures, which is a rare finding of paraneoplastic syndrome and the option of immunotherapy should be considered. [ABSTRACT FROM AUTHOR]

Paraneoplastic neurological syndromes (PNS) occur in about 1 in 300 cases of cancer. The usual mechanism is that an antigen on the cancer sets off an immune response that then cross-reacts with a nervous system antigen. The presentation is usually with a subacute progressive neurological disorder. The management of these conditions is usually of both the underlying tumor and immunomodulation to suppress the autoimmune response. There are a number of these conditions that can present to the Neuro-Ophthalmology clinic, either as a discrete condition affecting vision or eye movements or as part of a more widespread neurological disorder. This article will discuss these conditions, their management and prognosis. [ABSTRACT FROM AUTHOR]

Keywords: kidney; paraneoplastic; partial nephrectomy; RCC; small renal mass; symptoms; fever EN kidney paraneoplastic partial nephrectomy RCC small renal mass symptoms fever 434 436 3 03/24/23 20230401 NES 230401 In January 2015, a 62-year-old age woman arrived at the San Raffaele Emergency Department complaining of fever of unknown origin (FUO) and two recent syncopal episodes. Out of 3769 patients submitted to active treatment for RCC registered in our prospectively maintained database from 1987 until 2021, 46 (1.2%) had a SRM with paraneoplastic systemic symptoms. These 46 patients represented 16.6% of all the patients with systemic symptoms at presentation, and 2.8% of all the patients with a SRM. [Extracted from the article]

The symptoms of a rheumatic disease may also be a sign of a proliferative process. These include conditions that present with skin and vascular changes such as systemic sclerosis and Raynaud's phenomenon with peripheral ischaemia and ulceration. Furthermore, the less common conditions - erythromelalgia or palmar fasciitis with polyarthritis may also accompany cancer. In this article, we discuss the association of diffuse systemic sclerosis with anorectal tumor, palmar fasciitis and polyarthritis with ovarian cancer, erythromelalgia with underlying ovarian malignancy and Raynaud's phenomenon and digital ischemia associated with renal carcinoma. Based on the literature review on this topic we highlighted the importance of recognizing paraneoplastic syndromes at an early stage. This is a crucial point in the adequate management of a patient. Many of the paraneoplastic symptoms of rheumatic and other described conditions may regress with the management of the underlying malignancy. [ABSTRACT FROM AUTHOR]

Objectives: The diagnosis of early onset of myasthenia gravis in the emergency room represents a real challenge, especially when symptoms of presentation are atypical and nonspecific features. Material and methods: We described a case of 33 years old woman who went to the Emergency Department of Pescia (Pistoia, Italy) for a spontaneous intense frontal headache associated with the presence of rhinolalia and unilateral shoulder girdle disorders, initially misdiagnosed. Results: The atypical and initially mild presentation of the disease contributed to the delay in diagnosis; the progression of motor disturbances subsequently led to the definition of the clinical pattern. In particular, the patient showed AChR antibody positivity, associated with a thymoma. Conclusions: Despite being a well-known pathology and widely described in the literature, myasthenia gravis at onset is still a difficult diagnosis in cases of atypical presentation or with infrequent symptoms. [ABSTRACT FROM AUTHOR]

Paraneoplastic cerebellar ataxia is a paraneoplastic neurological syndrome (PNS) that can be the first clinical manifestation of underlying cancer. It is usually associated with onco-neuronal antibodies and has no other specific paraclinical feature. After the surgical and oncologic treatment of the primary cancer, the remaining neurological symptoms have limited therapeutic options. We describe a case of severe ataxia as the primary manifestation of ovarian cancer, with a significant clinical and paraclinical improvement of the neurological symptoms after 20 sessions of rTMS intervention. [ABSTRACT FROM AUTHOR]

Hemophagocytic lymphohistiocytosis (HLH) is a rare, elusive, and life-threatening condition that is characterized by the pathologic and uncontrolled secondary activation of the cytotoxic T-cells, natural killer cells (NK-cells), and macrophages of the innate immune system. This condition can develop in sporadic or familial contexts associated with hematological malignancies, as a paraneoplastic syndrome, or linked to an infection related to immune system deficiency. This leads to the systemic inflammation responsible for the overall clinical manifestations. Diagnosis should be thorough, and treatment should be initiated as soon as possible. In the current manuscript, we focus on classifying the HLH spectrum, describing the pathophysiology and the tools needed to search for and correctly identify HLH, and the current therapeutic opportunities. We also present the first case of a multiple myeloma patient that developed HLH following therapy with the ixazomib-lenalidomide-dexamethasone protocol. [ABSTRACT FROM AUTHOR]

Background: Paraneoplastic neurological syndromes (PNS) affecting the CNS (central nervous system) are rare, presenting in less than 1% of all those with cancer. The pathogenesis of paraneoplastic neurological syndromes is not fully understood, but it is presumed to result from an immune attack on the underlying malignancy. The presence of different types of onconeural antibodies may occur in different tumors and can lead to different clinical manifestations, making the early detection of cancers challenging. Aim: An evaluation of [18F]FDG PET/CT in neoplastic tumor detection in patients with paraneoplastic neurological syndromes having negative or unremarkable results of conventional radiological imaging. Methods: Among all patients diagnosed with paraneoplastic neurological syndromes in the Neurology Department in 2016–2020, 15 patients with unremarkable conventional radiological findings who underwent [18F]FDG PET/CT were included in the study. Results: [18F]FDG PET/CT enabled localization of suspected malignancy in 53% (8 of 15) of PNS cases with previous unremarkable conventional radiological findings. Conclusion: [18F]FDG PET/CT may be considered as a useful tool for neoplastic tumor detection in patients with paraneoplastic neurological syndromes, accelerating the diagnostic process and enabling faster initiation of appropriate treatment. [ABSTRACT FROM AUTHOR]

Adrenal cortical carcinoma is a rare and aggressive cancer with poor prognosis. Cases usually present with signs and symptoms of excessive hormone production. Hyperglycemia and Cushing syndrome are common, but tumor-associated hypoglycemia due to paraneoplastic secretion of insulin-like growth factor-2 (termed Anderson's syndrome) is uncommon. Given the rarity of adrenal cortical carcinoma, diagnosis and management of associated complications is challenging. In this study, we present a case of metastatic adrenal cortical carcinoma with a myriad of hormonal abnormalities. We will also briefly review literature regarding genetic association, pathophysiology, treatment options, and prognosis. [ABSTRACT FROM AUTHOR]

The article provides information on a case of a 65-year-old female with stage 4 invasive ductal carcinoma of the breast who developed carcinoma en cuirasse and systemic sclerosis as skin manifestations. The patient's condition presented challenges in determining the underlying cause, with possibilities including direct skin infiltration of tumor cells, paraneoplastic phenomenon, or chemotherapy-induced scleroderma.

Paraneoplastic syndromes of the nervous system are rare disorders, strongly linked with lung cancer. This report aims to explore the challenges in the diagnosis and treatment of a patient with early-stage small cell lung cancer and neurological manifestations as initial clinical symptoms. [ABSTRACT FROM AUTHOR]

Background: Paraneoplastic neurological syndromes constitute rare neurological complications of malignant disease, manifesting in <1% of patients with cancer. Opsoclonus-myoclonus syndrome (OMS) presents with chaotic ocular saccades (opsoclonus), spontaneous muscular jerking (myoclonus) that may be accompanied by ataxia, strabismus, aphasia, or mutism. Its paraneoplastic variant in the adult is most commonly associated with small-cell lung cancer, followed by breast cancer. Importantly, neurological symptoms usually precede the diagnosis of breast cancer and tend to recure after its treatment. Case presentation: A 43-year-old premenopausal Caucasian woman with a medical history of hypertension was admitted following an episode of focal seizure. This progressed to generalised tonic-clonic seizures and she was subsequently loaded with phenytoin, valproate, and levetiracetam. Initial workup included whole body CT scan, viral and autoimmune serology. The CT scan revealed an enhancing right axillary lymph node, which in combination with Anti-Ri antibody positivity raised the spectre of paraneoplastic OMS. MRI of the head revealed subtle nonspecific white matter signal change within the centrum semiovale without any mass lesions, while MRI of the spine was unremarkable. An uncomplicated right mastectomy and axillary lymph node clearance was performed: histopathology revealed a 9-mm, grade 2, oestrogen receptor-positive, progesterone receptor-negative (ER8, PR0), Her2-negative invasive ductal carcinoma, and 4/6 positive lymph nodes (T1b N2 M0). Two months later, she was readmitted with vertigo, diplopia, facial weakness, and ataxia, setting the diagnosis anti-Ri syndrome recurrence. MDT recommended mammogram and ultrasound of the left breast, which were normal. Subsequently, four months after initial discharge, she suffered another neurological recurrence; due to concomitant abdominal pain, PET-CT was performed demonstrating a hypermetabolic right ovarian focus. Bilateral salpingo-oophorectomy was performed as per gynaecology MDT and final histology showed normal tubes and ovaries. She has remained on remission since then, with a negative annual mammogram follow-up. Conclusions: In conclusion, we report a case of OMS associated with breast cancer anti-Ri onconeural antibody. Its manifestations preceded the diagnosis of malignancy and it persisted after cancer treatment, underlining the importance for high clinical suspicion in cases of classical paraneoplastic neurological syndromes as well as the need for long-term clinical follow-up. [ABSTRACT FROM AUTHOR]

Paraneoplastic neurological syndromes (PNS) are rare presentations of an underlying oncological disease and more unusual during an oncological disease. They most likely present in small-cell lung carcinomas and thymomas, but present in <1% of the gynecological neoplasms. Acknowledging the pathophysiology is essential for management, explaining its clinical presentation, and future research. We present a patient with an underlying gynecological cancer that during her disease developed a PNS with an unusual autoantibody (anti-CV2/CRMP5) mediating the disease. We report a case of a 62-year-old female diagnosed with ovarian cancer who in the course of her disease developed neurological symptoms associated with cerebellar degeneration. After ruling out differential diagnoses such as metastases, a PNS was suspected and studied, in which anti-CV2/CRMP5 antibodies were positive. With her clinical presentation, radiological features, autoantibody positivity on cerebrospinal fluid, and an underlying oncological disease, cerebellar degeneration was diagnosed. The pathophysiology of PNS is not fully understood; therefore, its diagnosis and management are complex. Diagnosis is based on clinical presentation and specific antibodies associated. Unfortunately, patients have a bad prognosis and diminished quality of life, and therefore a multidisciplinary approach is needed. It is important to mention that the presentation of PNS does not mandatorily appear before the diagnosis of cancer, and multiple cases have been reported in which patients with an underlying oncological disease develop these syndromes. As medical oncologists and neurologists, we must consider and study these syndromes as a possible etiology in cases with an underlying cancer who develop neurological symptoms in the course of their disease. [ABSTRACT FROM AUTHOR]

Limbic encephalitis with antibodies against adenylate kinase 5 (AK5) has been difficult to characterize because of its rarity. In this study, we identified 10 new cases and reviewed 16 previously reported patients, investigating clinical features, IgG subclasses, human leucocyte antigen and CSF proteomic profiles. Patients with anti-AK5 limbic encephalitis were mostly male (20/26, 76.9%) with a median age of 66 years (range 48-94). The predominant symptom was severe episodic amnesia in all patients, and this was frequently associated with depression (17/25, 68.0%). Weight loss, asthenia and anorexia were also highly characteristic, being present in 11/25 (44.0%) patients. Although epilepsy was always lacking at disease onset, seizures developed later in a subset of patients (4/25, 16.0%). All patients presented CSF abnormalities, such as pleocytosis (18/25, 72.0%), oligoclonal bands (18/25, 72.0%) and increased Tau (11/14, 78.6%). Temporal lobe hyperintensities were almost always present at disease onset (23/26, 88.5%), evolving nearly invariably towards severe atrophy in subsequent MRIs (17/19, 89.5%). This finding was in line with a poor response to immunotherapy, with only 5/25 (20.0%) patients responding. IgG1 was the predominant subclass, being the most frequently detected and the one with the highest titres in nine CSF-serum paired samples. A temporal biopsy from one of our new cases showed massive lymphocytic infiltrates dominated by both CD4+ and CT8+ T cells, intense granzyme B expression and abundant macrophages/microglia. Human leucocyte antigen (HLA) analysis in 11 patients showed a striking association with HLA-B*08:01 [7/11, 63.6%; odds ratio (OR) = 13.4, 95% confidence interval (CI): 3.8-47.4], C*07:01 (8/11, 72.7%; OR = 11.0, 95% CI: 2.9-42.5), DRB1*03:01 (8/11, 72.7%; OR = 14.4, 95% CI: 3.7-55.7), DQB1*02:01 (8/11, 72.7%; OR = 13.5, 95% CI: 3.5-52.0) and DQA1*05:01 (8/11, 72.7%; OR = 14.4, 95% CI: 3.7-55.7) alleles, which formed the extended haplotype B8-C7-DR3-DQ2 in 6/11 (54.5%) patients (OR = 16.5, 95% CI: 4.8-57.1). Finally, we compared the CSF proteomic profile of five anti-AK5 patients with that of 40 control subjects and 10 cases with other more common non-paraneoplastic limbic encephalitis (five with antibodies against leucine-rich glioma inactivated 1 and five against contactin-associated protein-like 2), as well as 10 cases with paraneoplastic neurological syndromes (five with antibodies against Yo and five against Ma2). These comparisons revealed 31 and seven significantly upregulated proteins in anti-AK5 limbic encephalitis, respectively mapping to apoptosis pathways and innate/adaptive immune responses. These findings suggest that the clinical manifestations of anti-AK5 limbic encephalitis result from a distinct T cell-mediated pathogenesis, with major cytotoxicity-induced apoptosis leading to a prompt and aggressive neuronal loss, likely explaining the poor prognosis and response to immunotherapy. [ABSTRACT FROM AUTHOR]

Background: Paraneoplastic syndrome is a distant effect caused by malignant tumors, which is related to the production of cellular immune response. The nervous system is the most common involved system of paraneoplastic syndrome. It is easy to be misdiagnosed. Lung cancer is the most common cancer relating to paraneoplastic neurological syndrome (PNS). Method: This study retrospectively analyzed clinical data of patients with the combination of PNS and lung cancer between January 2005 and March 2021 at Qilu Hospital of Shandong University, China. Results: A total of 111 patients were diagnosed with lung cancer complicated with PNS. A total of 95 (85.6%) cases had neurological symptoms as the first symptom. Sixty‐three cases had the pathological results. A total of 43 (68.3%) of small cell lung cancer (SCLC) were diagnosed. PNS patients diagnosed with SCLC included peripheral neuropathy (15 cases, 34.9%). PNS patients diagnosed with non‐small cell lung cancer (NSCLC) included peripheral neuropathy (6 cases, 30%) and limbic encephalitis (6 cases, 30%). Anti‐Hu is popular in patients with SCLC (12 cases, 42.9%) and NSCLC (6 cases, 40%). Conclusions: Most patients with PNS had neurological symptoms as the first symptom. It was more common in males. It had a higher incidence in SCLC. Peripheral neuropathy was the most common PNS associated with SCLC, followed by Lambert‐Eaton syndrome. Peripheral neuropathy and limbic encephalitis were the most common PNS associated with NSCLC. Anti‐Hu is the most common antibodies both in SCLC and NSCLC. Tumor markers do not have significant difference between different pathological types. [ABSTRACT FROM AUTHOR]

Primary Sjögren syndrome (SS) is a chronic inflammatory systemic autoimmune disease with a high risk of malignancy development, namely, lymphoproliferative neoplasms. Few studies also reported a high risk of solid cancers; however, the coexistence of primary SS and pancreatic cancer has been rarely described. In this paper, we aim to describe a case of a 59-year-old woman who was an active smoker with sicca symptoms and symmetrical polyarthritis and was diagnosed with primary SS two years before the development of metastatic pancreatic adenocarcinoma. Despite institution of chemotherapy, the patient succumbed to the malignancy. Besides that, we explore the link between primary SS and solid cancers including the main predictors of malignancy and the role of primary SS as a paraneoplastic syndrome. Patients with primary SS should be closely monitored for malignancy, not only for hematological cancer, but also for solid tumors. Further research is necessary to understand which are the predictors of cancer proliferation in primary SS patients. [ABSTRACT FROM AUTHOR]

Renal cell carcinoma (RCC) accounts for most renal malignancies. This article, the second in a three-part series, addresses how renal masses are classified, signs and symptoms of RCC, medical treatments for RCC, and priority nursing interventions for patients with RCC. [ABSTRACT FROM AUTHOR]

Polyneuropathy, organomegaly, endocrinopathy, M protein elevation, and skin changes (POEMS) syndrome is one of the rare paraneoplastic disorders of the multiorgan association whose initialism describes the principal clinical features as polyneuropathy, organomegaly, endocrinopathy, M-protein elevation, and skin changes caused by an underlying plasma cell disorder, one of the major diagnostic criteria for which is the presence of an osseous lesion. We present a case of POEMS syndrome with nearly all signs and symptoms on clinical and hematological examinations, which was further investigated with MRI of the whole spine and 18F-fluorodeoxyglucose positron emission tomography/computed tomography scan, as the latter is a very useful imaging modality for evaluation of any paraneoplastic syndromes, including myeloproliferative disorders such as plasmacytoma or multiple myeloma, and lymphoproliferative or other malignancies. [ABSTRACT FROM AUTHOR]

Most cases of skin lesions related to COVID vaccines are related to mRNA ones.1,3 From our perspective, this is the first study presenting a case of erythema gyratum repens (EGR) secondary to COVID vaccine. We would like to thank Dra Fernanda Gonçalves Moya from the University of São Paulo (São Paulo - SP, Brazil) for the Immunohistochemistry study. [Extracted from the article]

Introduction: Limbic encephalitis is an autoimmune neurologic disorder, often of paraneoplastic origin, that seldom complicates prostatic tumors. The nonspecificity of symptoms makes the diagnosis sometimes difficult to establish. Prognosis is essentially determined by comorbidities and sensorineural and cognitive sequelae.Clinical Case: A 66-year-old Caucasian patient known to have prostatic small-cell neuroendocrine adenocarcinoma under hormonal therapy developed complex partial epileptic seizures associated with rapidly aggravating severe memory impairment. The tripod of autoimmune limbic encephalitis diagnosis was based on the clinical aspect of brain's functional deterioration, electroencephalography aspect, and γ-aminobutyric acid type B anti-receptor antibody positivity. Clinical, diagnostic, and therapeutic management as well as evolutionary risks were further analyzed.Conclusion: Limbic encephalitis is an extremely rare presentation of neurologic paraneoplastic syndromes. A better knowledge of this entity would help better manage diagnostic and therapeutic difficulties and reduce the risk of possible sequelae. [ABSTRACT FROM AUTHOR]

Anti-amphiphysin antibody-induced encephalitis is an immune-mediated inflammatory disease of the brain caused by onconeuronal antibodies. Amphiphysin antibodies are associated with various paraneoplastic neurological syndromes and tumours. The absence of a particular clinical pattern and pathognomonic features makes diagnosing paraneoplastic-autoimmune encephalitis complicated, especially when a malignancy is not initially apparent. Detection of specific antibodies in serum and cerebrospinal fluid remains the main diagnostic tool. Up to this date, no cases with epileptic seizures as one of the clinical manifestations of anti-amphiphysin encephalitis have been reported. In this article, we present a rare clinical case of a 54-year-old man diagnosed with autoimmune-paraneoplastic encephalitis caused by antibodies against amphiphysin which manifested with atypical clinical symptoms such as epileptic seizures, visual and perception impairment, paraesthesia, and elevated CSF protein levels with no detectable tumour. [ABSTRACT FROM AUTHOR]

Approximately 20% of renal cell carcinoma (RCC) is diagnosed because of paraneoplastic manifestations. RCC has been associated with a large variety of paraneoplastic syndromes (PNS), but it is rarely associated with PNS vasculitis. We present a case of a previously healthy male who presented with systemic vasculitis; bitemporal headaches, diplopia, polyarthritis, palpable purpura, tongue lesion, peri-orbital edema, scleritis, chondritis and constitutional symptoms. He was subsequently found to have oligometastatic RCC. Both his primary lesion and site of oligometastasis were treated with stereotactic radiotherapy (SBRT) and resulted in the resolution of his vasculitis, as well as sustained oncologic response. This is the first case to demonstrate that effective sustained treatment for PNS vasculitis due to oligometastatic RCC is possible with SBRT. [ABSTRACT FROM AUTHOR]

Background: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory disorder with unclear causes. Paraneoplastic etiology may be a cause. We report a case of CLIPPERS with parotid carcinoma.Case Presentation: A 54-year-old man with a history of lymphoma was hospitalized with a pontocerebellar syndrome. Brain MRI revealed that the pons and cerebellum were "peppered" with punctate and curvilinear enhancement lesions that supported the diagnosis of CLIPPERS. The relapse of lymphoma was excluded by a further cerebellum biopsy revealing predominantly CD3+ T cells in white matter. The patient was relieved after pulse therapy with intravenous methylprednisolone and a large dose of corticosteroids, but he complained of a worsening gait problem when corticosteroids were tapered to a lower dose. Although the clinical symptoms gradually improved again by increasing the dosage of corticosteroids with Azathioprine, the patient still had a slight unsteady gait during follow-up. At the 7-month follow-up, a parotid mass was detected by MRI and was verified as carcinoma by biopsy. After resection of parotid carcinoma, the residual symptoms and previous MRI lesions disappeared, and no relapse occurred.Conclusions: CLIPPERS may not be a distinct nosologic entity but an overlapping diagnosis with other diseases. Some cases of CLIPPERS might be a subtype of paraneoplastic neurological syndromes (PNS) due to the similar mechanism of antibody-mediated encephalitis. Tumor screening and serum paraneoplastic autoantibody tests are recommended for patients with CLIPPERS, especially for those who relapse when corticosteroids treatment is stopped or tapered. [ABSTRACT FROM AUTHOR]

CV2/CRMP5 is the most common antibody accompaniment of paraneoplastic choreoathetosis. We present a case of paraneoplastic choreothetosis with associated cerebellar dysfunction, peripheral neuropathy, and likely dysautonomia. Our patient developed a movement disorder after a cardiopulmonary arrest, which unfortunately masked the true etiology of his symptoms. He was later found to have extensive stage small cell lung cancer, with further evaluation revealing seropositivity for anti-CV2 antibodies. Choreoathetosis is a known sequelae of hypoxic-ischemic brain injury, but clinicians should continue to keep an open mind. The utility of immunotherapy is unclear in these circumstances and many physicians adopt a symptom-based approach. [ABSTRACT FROM AUTHOR]

Neurological paraneoplastic syndromes are exceedingly rare, and often difficult to recognize clinically. Paraneoplastic achalasia is a condition characterized by new-onset dysphagia that is unrelated to tumor burden, most often due to the development of auto-immune antibodies targeting esophageal tissue. Due to the rarity of this condition, diagnosis is often delayed, leading to increased time to treatment. Here we report a case of a rare paraneoplastic achalasia in a female child with EBV + Hodgkin lymphoma (HL), review literature describing paraneoplastic achalasia, and discuss treatment strategies for improving clinical outcome in these patients. [ABSTRACT FROM AUTHOR]

Raynaud's phenomenon (RP) is characterized by episodes of vasospasm affecting the hands and feet. Paraneoplastic RP, as a single presenting symptom is rarely seen in cases of ovarian cancer (OC), and thus may lead to misdiagnosis. We present a case of paraneoplastic RP in a patient with high‐grade serous OC. A 66‐year‐old female presented with dyspnea and bilateral peripheral cyanosis involving her fingers. CA125 was elevated (423 U/mL). CT revealed a pleural effusion on the left side, suspicious omental lesions and ascites. Omental biopsy and pleural cytology demonstrated high‐grade serous OC. Neoadjuvant chemotherapy (carboplatin/paclitaxel) resulted in objective improvement in finger ischemia and complete regression of vasospastic features. However, the patient's disease was refractory to post‐surgical treatment and eventually she deceased of multiple organ failure. To conclude, RP may be a presenting symptom of OC. It is important to determine the underlying disease and develop an effective treatment strategy. [ABSTRACT FROM AUTHOR]

Background Paraneoplastic cerebellar degeneration (PCD) is a clinical syndrome in which cerebellar dysfunction is associated with identifiable or occult cancer but without the direct involvement of the nervous system by cancer.1,2 Typically, the disorder evolves sub-acutely, causes severe immune-mediated pan cerebellar dysfunction, and is associated with extensive purkinje cell loss, sometimes accompanied by meningeal and deep cerebellar nuclear inflammatory infiltrates.1 It has been linked to elevated anti-yo antibody serum titre in affected patients. It is most commonly associated with breast and other gynaecological cancers and often presents in patients prior to their cancer diagnosis. Hence, an aggressive search for an underlying malignancy would confirm the diagnosis and provide a basis for treatment, which is what this case series intends to highlight. Materials and method We present three cases of patients (aged 50-70 years) presenting with cerebellar features; truncal and/or limb ataxia, dysarthria and nystagmus. All of them have a rise in anti-yo antibody titre, as well as a positive oligoclonal band in the CSF, raising a strong suspicion of PCD.3,4 Their preliminary CT TAP and MRI brain were unyielding. However, the high index of suspicion prompted a positron emission tomographic (PET) scan, which identified the origin of the malignancy as breast in the first two cases and fallopian tube in the third case (metastatic deposits in the pelvic side wall were seen here on the PET scan necessitating an exploratory laparotomy). All the cancers were confirmed on histology. Results All three patients received intravenous immunoglobulin (IVIg) as part of their treatment. Of the two breast cancer cases, one was a grade 3 non-invasive DCIS, hence mastectomy was offered in addition. Unfortunately, none of the patients improved despite treatment at the time this information was collected, although there was no long-term follow up. Conclusion The case series demonstrated the need for a high index of suspicion of underlying malignancy in patients presenting with features of PCD and the need to pursue it aggressively. This would not only help to confirm the diagnosis but would aid in management. However, further studies are needed to explore alternative treatment options. [ABSTRACT FROM AUTHOR]

Stiff-person syndrome (SPS) is a rare disorder seen in approximately one in one million people. Although it is rare, the symptoms and findings of a typical case should paint a clear clinical picture for those who are familiar with the disease. The primary findings in SPS include progressive axial muscle rigidity as well as muscle spasms. These symptoms most commonly occur in the setting of antibodies against Glutamic Acid Decarboxylase (GAD), the rate-limiting enzyme in the production of Gamma-Aminobutyric Acid (GABA), which is the primary inhibitory enzyme in the central nervous system. Here, we report the case of a 65-year-old African-American female with a past medical history of hypothyroidism, anxiety, and depression with psychotic features who presented with axial muscle rigidity and lactic acidosis. She had been symptomatic for several months and reported extensive workups performed at two previous hospitals without a definitive diagnosis. A complete neurological and musculoskeletal investigation yielded no positive findings except for the presence of GAD antibodies. The patient was treated with diazepam, tizanidine, and Intravenous Immunoglobulin (IVIG) with significant improvement, thus solidifying the diagnosis of SPS, a rare autoimmune and/or paraneoplastic syndrome. [ABSTRACT FROM AUTHOR]

Anti-leucine-rich glioma inactivated 1 ( anti-LGI 1) encephalitis is a rare autoimmune limbic encephalitis. As the clinical presentation of this disease is similar to other types of encephalitis most often associated with paraneoplastic process or endocrine disorder, a thorough testing for oncological or endocrine disease is necessary to correctly differentiate the diagnosis and administer appropriate treatment. We present a clinical case of a 63-year-old female patient treated in the Neurology Department of the Hospital of Lithuanian University of Health Sciences Kaunas Clinics for impairment of consciousness, probably of epileptic origin. After identifying memory problems, focal impaired awareness seizures, cognitive dysfunction, and typical brain magnetic resonance imaging changes, the diagnosis of limbic encephalitis was suspected. A thorough testing allowed to exclude paraneoplastic processes or endocrine syndromes, and the results of the cerebrospinal fluid antibody panel confirmed the diagnosis of anti-LGil antibody autoimmune encephalitis. Further treatment using glucocorticoids and intravenous immunoglobulin gave good results. [ABSTRACT FROM AUTHOR]

Background: Paraneoplastic Neurological Syndromes (PNSs) are a heterogeneous group of immune-mediated disorders that often precede tumor diagnosis. There are few systematic studies on the spectrum and follow-up of PNSs. Objective: To analyze the clinical spectrum, associated tumors, antibody profile, outcomes, and prognostic predictors in a cohort of PNSs admitted in a tertiary care center. Methods: This retrospective study included 97 patients (2008-2019). PNSs were further classified as "classical," "nonclassical," "definite," and "possible." Clinical profile, diagnostic strategies, therapeutic options, and predictors of outcomes were identified. Results: The median age was 54 years (range 17--81). Thirty-nine (40.2%) had classical PNS, and 58 (59.8%) had nonclassical PNS, 74 (76.3%) had "Definite" PNS while 23 (23.7%) had "Possible" PNS. Cerebellar degeneration, peripheral neuropathy, and encephalopathy were the three most common neurological syndromes. Tumors were diagnosed in 66 (68%) patients; Lung cancer was the most common primary tumor. Antibodies were positive in 52 (53.6%). Anti-Yo antibody and anti-Ma2 antibody were the most common antibodies. The majority (57.7%) received immunotherapy in addition to definitive treatment for the tumor. A good outcome was seen in 53 (54.6%). Factors associated with good outcome were: early diagnosis, mRS <3 at presentation, absence of metastatic disease, and adjuvant immunotherapy. Conclusion: A high index of clinical suspicion is essential for early diagnosis and prompt management of PNS, especially the nonclassical syndromes. Multimodality diagnostic imaging techniques and antibody profiling play a crucial role in the diagnosis. A favorable prognosis can be expected with the judicious use of immunotherapy and definitive treatment of malignancy. [ABSTRACT FROM AUTHOR]

In malignant and rheumatological diseases, there is often an overlap of signs and symptoms that requires that neoplasms be considered as a differential diagnosis. When musculoskeletal symptoms are the first manifestation of disease, diagnosis may be difficult because, at the time of the initial presentation, obvious signs of malignancy may be absent. (B) Immunohistochemical characterization of the tumour revealed strong reactivity in the majority of tumour cells with ALK (immunoperoxidase stain). [Extracted from the article]