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neurologic paraneoplastic

Jorge C Kattah
Background: Previous series of bilateral vestibular loss (BVL) identified numerous etiologies, but surprisingly, a cause in a significant number of cases remains unknown. In an effort to understand possible etiology and management strategies, a global effort is currently in progress. Here, I contribute my 10-year experience with both acute and chronic BVL during the 2007-2017 decade. Methods: This is a retrospective review of the charts and EMR of patients diagnosed with BVL in the last 10 years...
2018: Frontiers in Neurology
Nellihela Leel, Hemanshoo S Thakkar, David Drake, Nordeen Bouhadiba
Anti-N-methyl D-aspartate receptor (NMDAR) encephalitis is a paraneoplastic, immune-mediated encephalopathy with a known association with ovarian teratomas. We present the first case in the UK of a 7-year-old patient presenting with this condition. Having been previously fit and healthy, the patient presented with an acute onset of cognitive disturbances. The initial suspicion was that of an infective encephalopathy; however, the lumbar puncture results were negative. Electroencephalography reported diffuse background slowing, supporting a diagnosis of encephalopathy...
March 13, 2018: BMJ Case Reports
Hiroyuki Fukuda, Akira Tanaka, Yasuyuki Hirashima, Ichiro Ito
Lambert-Eaton myasthenic syndrome (LEMS) is most commonly associated with small cell lung carcinoma, while it is rarely associated with gynecological and breast carcinoma. We herein report a case of LEMS associated with synchronous double cancer, which was a combination of small cell carcinoma of the cervix and breast carcinoma. The early diagnosis and treatment of LEMS are important for achieving a good outcome. The possibility of accompanying paraneoplastic neurological syndrome must be sufficiently considered in gynecology and breast cancer patients...
March 9, 2018: Internal Medicine
A Coelho, P Brandão, M Lobo, I Lojo, A Canedo
INTRODUCTION: Major pelvic Ilio-Iliac arteriovenous fistula (AVF) is an exceedingly rare diagnosis with only a few described cases in the literature, most of them related to congenital defects or trauma. In this case report, we aim to present a case of an ilio-iliac AVF with an atypical clinical presentation. CASE REPORT: The patient is a 77-year-old women, with a relevant medical history of a temporally remote hysterectomy. She developed an exuberant unilateral right leg oedema and was diagnosed with a femoro-iliac deep vein thrombosis (DVT) and started on anticoagulation and daily use of elastic compression stockings...
March 5, 2018: Annals of Vascular Surgery
Laura Attademo, Stefano De Falco, Mario Rosanova, Marcello Esposito, Federica Mazio, Francesca Foschini, Antonio Santaniello, Giovanni Fiore, Elide Matano, Fiore Manganelli, Chiara Carlomagno
RATIONALE: Paraneoplastic limbic encephalitis (PLE) is one of the most common causes of neurologic paraneoplastic syndromes, with unclear pathogenesis. While several reports published in the last decades showed the occurrence of PLE in a variety of cancers, only a few cases have been associated with colon cancer. PATIENT CONCERNS: In February 2017, a 54-year-old man with clinical history of radically resected colon cancer started first line chemotherapy with FOLFOXIRI plus bevacizumab, after radiological diagnosis of multiple liver and bone metastases...
March 2018: Medicine (Baltimore)
Michael R Pranzatelli, Tyler J Allison, Nathan R McGee, Elizabeth D Tate
Studies of cerebrospinal fluid (CSF) γδ T cells in children are limited, especially due to the lack of control data. In adults, gamma/delta T cells (TCR-γδ) residing in the intrathecal space are sometimes involved in neuroinflammation. To evaluate the possible role of γδ T cells in pediatric neuroinflammation, we immunophenotyped CSF and blood lymphocytes using flow cytometry in a case-control study of 100 children with non-inflammatory neurologic disorders (NIND), 312 with opsoclonus-myoclonus (OMS), and 23 with other inflammatory neurologic disorders (OIND)...
February 27, 2018: Clinical and Experimental Immunology
Daniel C Wiener, Tamara B Kaplan, Carlos E Bravo-Iñiguez, Jordan Miller, Aaron L Berkowitz, Michael T Jaklitsch
Neuromyelitis optica spectrum disorders are a group of relapsing, inflammatory, demyelinating neurologic syndromes involving the central nervous system associated with antibodies against aquaporin-4. Although most commonly an idiopathic autoimmune condition, neuromyelitis optica may occur as a paraneoplastic syndrome in rare instances. We report a case of transverse myelitis caused by paraneoplastic neuromyelitis optica as the presenting clinical syndrome in a patient with esophageal adenocarcinoma.
March 2018: Annals of Thoracic Surgery
Markus Leitinger, Mihael V Varosanec, Slaven Pikija, Romana E Wass, Dave Bandke, Serge Weis, Michael Studnicka, Susanne Grinzinger, Mark R McCoy, Larissa Hauer, Johann Sellner
Immune checkpoint inhibitors are antibodies, which enhance cellular and humoral immune responses and are approved for the treatment of various tumors. Immune-related adverse events (irAE) involving different organs and systems are, however, among the side-effects. Recent reports of severe persistent neurological deficits and even fatal cases underpin the need for better understanding of the exact pathomechanisms of central nervous system (CNS) toxicity. To our knowledge, we report the first biopsy-proven case of fatal necrotizing encephalopathy after treatment with nivolumab...
2018: Frontiers in Immunology
Yuval Freifeld, Payal Kapur, Ritika Chitkara, Francesca Lee, Pravin Khemani, Aditya Bagrodia
A 44-year-old man presented with cerebellar ataxia and limbic encephalitis and was ultimately diagnosed with metastatic germ cell neoplasm resulting from a "burned out" primary testicular tumor. The patient had progressive ataxia, leading to a thorough investigation for infectious, autoimmune, metabolic, and malignant causes of acquired cerebellar ataxia that revealed no significant findings. Testicular sonography demonstrated a possible right testicular lesion that was not confirmed on radical inguinal orchiectomy...
2018: Frontiers in Neurology
Carlo Salvarani, Robert D Brown, Teresa J H Christianson, John Huston, Stephen M Ansell, Caterina Giannini, Gene G Hunder
OBJECTIVES: To record the clinical findings, response to therapy, and course of patients with primary CNS vasculitis (PCNSV) associated with lymphoma. PATIENTS AND METHODS: We reviewed the histories of 936 patients with a diagnosis of any type of vasculitis and lymphoma who were seen at the Mayo Clinic over a 32-year period. Ten patients with both PCNSV and lymphoma were identified. We compared the findings in these 10 patients with those from 158 patients with PCNSV without lymphoma seen over 29 years...
February 2, 2018: Neurology
Vito A G Ricigliano, Barbara Fossati, Lorenzo Saraceno, Michele Cavalli, Elena Bazzigaluppi, Giovanni Meola
Thymoma is a tumor originating from thymic gland, frequently manifesting with paraneoplastic neurological disorders. Its association with paraneoplastic dysautonomia is relatively uncommon. Here, we describe the challenging case of a 71 year-old female who developed subacute autonomic failure with digestive pseudo-obstruction, dysphagia, urinary tract dysfunction and orthostatic hypotension complicating an underlying extrapyramidal syndrome that had started 3 months before hospital admission. Autonomic symptoms had 2-month course and acutely worsened just before and during hospitalization...
2018: Frontiers in Neuroscience
David R Lynch, Amy Rattelle, Yi Na Dong, Kylie Roslin, Amy J Gleichman, Jessica A Panzer
In slightly more than 10 years, anti-NMDA receptor (NMDAR) encephalitis has changed from a rare paraneoplastic syndrome to the most common cause of nonviral encephalitis. It presents fulminantly with progressive psychosis, seizures, and autonomic dysfunction, leading to death if untreated. However, rapid recognition and treatment can lead to survival and a return to baseline levels of functioning in many patients. While initially associated with ovarian teratomas, it is now associated with other tumors and can reflect a postviral event...
2018: Advances in Pharmacology
Sabine Burger, Stefan Lorenzl
Paraneoplastic neurological syndromes describe a very heterogeneous group of neurological disorders. Paraneoplastic cerebellar degeneration is frequently associated with gynecological tumors, lung cancer and Hodgkin lymphoma. The following case report shows the typical course of this disease as well as the diagnostic and therapeutic challenges. Furthermore the importance of interdisciplinary cooperation between neurology, oncology and palliative care for prognosis and symptom stabilization is highlighted.
February 6, 2018: Wiener Medizinische Wochenschrift
Jyh Yung Hor, Thien Thien Lim, Mei Chih Cheng, Yuen Kang Chia, Chee Keong Wong, Su Min Lim, Chun Fai Cheah, Kenny Tan, P E Samuel Easaw, M Isabel Leite
Thymoma is associated with a wide spectrum of autoimmune paraneoplastic syndromes, though it is uncommon for multiple paraneoplastic syndromes to be present in a single individual. We report a rare case of an elderly gentleman who was found to have thymoma-associated myasthenia gravis and LGI1-encephalitis with myokymia, who presented with nephrotic syndrome (minimal change glomerulopathy) after thymectomy. The latter two paraneoplastic syndromes had manifested when prednisolone was tapered down to low dose...
January 30, 2018: Journal of Neuroimmunology
Alberto Vogrig, Bastien Joubert, Francois Ducray, Laure Thomas, Cristina Izquierdo, Kévin Decaestecker, Olivier Martinaud, Emmanuel Gerardin, Sylvie Grand, Jérome Honnorat
OBJECTIVE: To identify the clinical and radiological features that should raise suspicion for the autoimmune encephalitis (AE)-like presentation of glioblastoma. METHODS: This is an observational, retrospective case series of patients referred to the French National Reference Center on Paraneoplastic Neurological Diseases for suspected AE (possible, probable or definite, using the 2016 criteria) who later received a final diagnosis of glioblastoma according to 2016 WHO criteria...
January 30, 2018: Journal of Neurology
Shuai Miao, Shaohua Liao, Heng Li, Bing Niu, Huaiqiang Hu, Ying Qian, Hongwei Guo, Bingzhen Cao
OBJECTIVE: To analyze the clinical features, diagnostic strategies and therapeutic methods associated with paraneoplastic neurological syndromes (PNS). METHODS: A retrospective study of PNS was performed at a single center in Shandong, East China. The medical records and follow-up data of 28 PNS patients were intensively reviewed between February 2011 and December 2014. RESULTS: A total of 28 patients diagnosed with PNS were included in this study...
January 22, 2018: International Journal of Neuroscience
Katalin Böröcz, Zsófia Hayden, Viktória Mészáros, Zsuzsanna Csizmadia, Kornélia Farkas, Zoltán Kellermayer, Péter Balogh, Ferenc Nagy, Tímea Berki
INTRODUCTION: The role of autoimmune responses against central nervous system (CNS) antigens in encephalitis presenting with non-classified neurologic or psychiatric symptoms has been appreciated in the past decade. Paraneoplastic limbic encephalitis has a poor prognosis and is most commonly associated with lung, ovarium, and testicular neoplasms, leading to immune reactions against intracellular antigens (anti-Hu/ANNA1, anti-Ri/ANNA2, anti-CV2/CRMP5 and anti-Ma2/Ta). In contrast, the recently described autoimmune encephalitis subtypes present with a broad spectrum of symptoms, respond to autoimmune therapies well and usually associate with autoantibodies against neuronal cell surface receptors (NMDAR, GABABR, AMPAR) or synaptic proteins (LGI1, CASPR2)...
January 2018: Orvosi Hetilap
D De Simoni, R Höftberger
BACKGROUND: Paraneoplastic neurological syndromes (PNNS) are remote effects of a tumor and mediated by an altered immune reaction. In the last ten years, the spectrum of PNNS has changed profoundly with the discovery of a new category of neurological diseases that are associated with antibodies against surface or synaptic antigens. In contrast to classical PNNS, patients with surface receptor autoimmunity are often highly responsive to immunotherapy. OBJECTIVES: This article provides an update on the most relevant PNNS, focusing on specific syndromes associated with antibodies against classical onconeuronal antigens as well as surface and synaptic proteins...
January 11, 2018: Der Internist
G S Chowdhary, Malav Jhala
Tumours may produce growth factors and cytokines responsible for signs and symptoms distant to the primary or metastatic site. This may be the first sign of a malignancy and its recognition may be critical for early cancer detection. Moreover, proper diagnosis spares the patient of extensive and expensive search for an alternate cause of the neurological dysfunction. In neurological paraneoplastic syndromes like Lambert Eaton Myasthenic syndrome associated with small cell lung cancer, evidence of autoimmunity against presynaptic neuro-muscular junction by anti voltage gated calcium channel anti bodies is well documented...
September 2017: Journal of the Association of Physicians of India
Assim A Alfadda, Hicham Benabdelkamel, Afshan Masood, Anwar A Jammah, Aishah A Ekhzaimy
Thyroid hormone is a potent stimulator of metabolism, playing a critical role in regulating energy expenditure and in key physiological mechanisms, such as growth and development. Although administration of thyroid hormone in the form of levo thyroxine (l-thyroxine) has been used to treat hypothyroidism for many years, the precise molecular basis of its physiological actions remains uncertain. Our objective was to define the changes in circulating protein levels that characterize alterations in thyroid hormone status...
January 1, 2018: International Journal of Molecular Sciences
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