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neurologic paraneoplastic

A A H Bressers, N A Goto, S Piepers, J C Regelink
BACKGROUND: Autoimmune encephalitis is a rare condition characterized by subacute development of cognitive and psychiatric symptoms. A paraneoplastic syndrome involves autoimmune encephalitis caused by classic antibodies. Although this condition is often associated with cancer, no malignancy has yet been found in 70-90% of patients at the time of diagnosis. CASE DESCRIPTION: We saw a 58-year-old male patient with fatigue, diarrhoea and weight loss. He was also experiencing hyperekplexia, personality changes and an instable gait...
2016: Nederlands Tijdschrift Voor Geneeskunde
Angela C Gauthier, Joachim M Baehring
Hashimoto's encephalopathy is a rare, imprecisely defined autoimmune neurologic syndrome associated with Hashimoto's thyroiditis that normally responds to corticosteroids. Here, we describe the case of a 55-year-old woman who presented with subacute cognitive decline and ataxia. Neoplastic, paraneoplastic, infectious, and metabolic etiologies were ruled out. Anti-TPO antibody level was markedly elevated at 966U/mL. After one month of 60mg/day of oral prednisone, she felt back to baseline and her Montreal Cognitive Assessment dramatically improved...
October 12, 2016: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
Edvina Galié, Rosaria Renna, Domenico Plantone, Andrea Pace, Mirella Marino, Bruno Jandolo, Tatiana Koudriavtseva
Morvan's syndrome (MoS) is a rare, complex neurological disorder characterized by neuromyotonia, neuropsychiatric features, dysautonomia and neuropathic pain. The majority of MoS cases have a paraneoplastic aetiology, usually occurring prior to the diagnosis of the underlying tumour and showing improvement following its treatment. The present study reports the case of a 35-year-old Caucasian male patient who was diagnosed with stage IVA thymoma. Thymectomy, lung resection, diaphragmatic pleurectomy and pericardio-phrenectomy were performed 6 months after neoadjuvant chemotherapy...
October 2016: Oncology Letters
Sajid S Qureshi, Monica Bhagat, Jay Anam, Tushar Vora
Neuroblastoma is infrequently associated with paraneoplastic syndromes. Amongst the few, opsomyoclonus (Kinsbourne syndrome) is the most common neurological paraneoplastic syndrome and diarrhea secondary to increased secretion of vasoactive intestinal peptide (Kerner-Morrison syndrome), hormonal paraneoplastic syndrome. Hypothalamic dysfunction (HD) is a rare disorder and its manifestation as a paraneoplastic syndrome of neuroblastoma is uncommonly reported. We present an interesting case of an unrelenting cervical neuroblastoma associated with HD, which posed a therapeutic challenge...
October 2016: Journal of Indian Association of Pediatric Surgeons
Takashi Inuzuka
Autoimmune encephalitis consists of limbic symptoms and signs associated with antibodies against neuronal cell-surface antigens or intracellular antigens. Some cases are known to be associated with anti-channel or anti-receptor-related molecule antibodies. Whether these cases are paraneoplastic depends on the kinds of antigens that the antibodies are produced against. Other cases due to well-characterized onco-neural antibodies are almost always paraneoplastic and are generally resistant to anti-tumor therapy and/or immunotherapy...
September 2016: Brain and Nerve, Shinkei Kenkyū No Shinpo
N Merali, M Yousuff, V Pronisceva, A Poddar
Paraneoplastic syndrome affects less than 1% of cancer patients. Diagnosis of paraneoplastic syndrome with neurological presentation requires screening for an underlying malignancy, including a complete history, physical examination and imaging studies. Treatment often results in symptom stability, rather than improvement. Paraneoplastic polymyositis can precede or instantaneously occur at diagnosis or treatment of a primary tumour, while neurological symptoms can persist even following cancer treatment. We report a rare case of metaplastic breast carcinoma with an unusual presentation of paraneoplastic polymyositis...
September 23, 2016: Annals of the Royal College of Surgeons of England
Yu Uneno, Akira Yokoyama, Yoshitaka Nishikawa, Taro Funakoshi, Yoshinao Ozaki, Ikuo Aoyama, Kiichiro Baba, Daisuke Yamaguchi, Shuko Morita, Yukiko Mori, Masashi Kanai, Hisanori Kinoshita, Takeshi Inoue, Nobukatsu Sawamoto, Riki Matsumoto, Shigemi Matsumoto, Manabu Muto
Paraneoplastic neurological syndromes (PNSs) are rare nervous system dysfunctions in cancer patients, which are primarily observed with small-cell lung cancer, gynecological cancer, and thymoma. We herein present an uncommon case of PNS in an anti-Hu antibody-positive patient with human epidermal growth factor receptor (HER)-2-positive gastric cancer (GC), who developed limbic encephalitis and a worsening cognitive function. Trastuzumab-combined chemotherapy was initiated and appeared to be partially effective for controlling the neurological symptoms and tumor volume...
2016: Internal Medicine
Boyan Fang, Andrew McKeon, Shannon R Hinson, Thomas J Kryzer, Sean J Pittock, Allen J Aksamit, Vanda A Lennon
Importance: A novel astrocytic autoantibody has been identified as a biomarker of a relapsing autoimmune meningoencephalomyelitis that is immunotherapy responsive. Seropositivity distinguishes autoimmune glial fibrillary acidic protein (GFAP) meningoencephalomyelitis from disorders commonly considered in the differential diagnosis. Objective: To describe a novel IgG autoantibody found in serum or cerebrospinal fluid that is specific for a cytosolic intermediate filament protein of astrocytes...
September 12, 2016: JAMA Neurology
Daiki Yamanaka, Takashi Kawano, Hiroki Tateiwa, Hideki Iwata, Fabricio M Locatelli, Masataka Yokoyama
BACKGROUND: Anti-N-methyl-d-aspartate receptor (NMDA-R) encephalitis is a recently identified but increasingly recognized autoimmune paraneoplastic disease. Because these patients present complex neuropsychiatric symptoms due to NMDA-R dysfunction, the optimal methods of sedation/anesthesia remain controversial. Here, we present animal experiment data, along with a related case report, implying the safe and effective use of dexmedetomidine in patients with anti-NMDA-R encephalitis. FINDINGS: (1) Animal experiment: in order to investigate whether dexmedetomidine may interfere with NMDA-R activity, an NMDA antagonist (MK-801) model in rats was used to simulate anti-NMDA-R encephalitis...
2016: SpringerPlus
Lidia M Yshii, Christina M Gebauer, Béatrice Pignolet, Emilie Mauré, Clémence Quériault, Mandy Pierau, Hiromitsu Saito, Noboru Suzuki, Monika Brunner-Weinzierl, Jan Bauer, Roland Liblau
CTLA4 is an inhibitory regulator of immune responses. Therapeutic CTLA4 blockade enhances T cell responses against cancer and provides striking clinical results against advanced melanoma. However, this therapy is associated with immune-related adverse events. Paraneoplastic neurologic disorders are immune-mediated neurological diseases that develop in the setting of malignancy. The target onconeural antigens are expressed physiologically by neurons, and aberrantly by certain tumour cells. These tumour-associated antigens can be presented to T cells, generating an antigen-specific immune response that leads to autoimmunity within the nervous system...
September 6, 2016: Brain: a Journal of Neurology
Marie Angélique De Scheerder, Sylvie Rottey, An Mariman, Marleen Praet, Dirk Vogelaers
We describe a 43-year-old patient with subacute appearance of neurological and atypical complaints of anergia, anorexia and weight loss six months earlier. In spite of several admissions in different hospitals, no underlying somatic cause could be found and he was admitted to a psychiatric hospital with a tentative diagnosis of major depressive disorder. Subsequently, he was referred to the unit of medically unexplained physical symptoms within the department of general internal medicine for assessment by the psychiatrist, involved in this programme...
September 2, 2016: Acta Clinica Belgica
Nao Nagata, Takuma Miyoshi, Yuzo Otake, Hitomi Suzuki, Yumiko Kagawa, Tetsushi Yamagami, Mitsuhiro Irie
Neurological signs and serum acetylcholine receptor antibody (AChR-Ab) levels before, and after thymectomy were monitored in a 6-year-old male cat with acquired Myasthenia Gravis (MG) as a paraneoplastic syndrome of thymoma. Soon after surgery, the neurological symptoms relapsed, and the cholinesterase inhibitor was administered to control them. The AChR-Ab levels increased postoperatively until 90 days after surgery. This is the first report on long term measurements of serum AChR-Ab levels in a cat with MG...
September 3, 2016: Journal of Veterinary Medical Science
Zeynep Özözen Ayas, Dilcan Kotan, Yeşim Güzey Aras
INTRODUCTION: Autoimmune neurological syndrome is a group of disorders caused by cancer affecting nervous system by different immunological mechanisms. In this study, we aim to study the clinical symptoms, cerebrospinal fluid (CSF) findings, autoantibody tests, computed tomography (CT), magnetic resonance imaging (MRI) signs and treatment outcome of patients with autoimmune syndromes. METHODS: In this study, 7 patients (4 male, 3 female) diagnosed with autoimmune neurological syndrome were retrospectively examined...
October 6, 2016: Neuroscience Letters
Sverre Georg Sæther, Morten Schou, Winfried Stoecker, Bianca Teegen, Kathrin Borowski, Arne Vaaler, Daniel Kondziella, Solveig Klæbo Reitan
Paraneoplastic neurological disorders associated with onconeural antibodies often appear with neuropsychiatric symptoms. To study the prevalence of onconeural antibodies in patients admitted to acute psychiatric inpatient care, the serum of 585 such patients was tested for antibodies targeting MOG, GLRA1B, DPPX, GRM1, GRM5, DNER, Yo, ZIC4, GAD67, amphiphysin, CV2, Hu, Ri, Ma2, and recoverin. Only one sample was positive (antirecoverin IgG). The present findings suggest that serum onconeural antibody positivity is rare among patients acutely admitted for inpatient psychiatric care...
August 31, 2016: Journal of Neuropsychiatry and Clinical Neurosciences
Waqar Waheed, James Boyd, Farrah Khan, Sharon L Mount, Neil M Borden, Rup Tandan
Patients with Purkinje cell cytoplasmic autoantibody type 2 (PCA-2) and collapsin response-mediator protein-5 (CRMP-5) autoantibody can present with multifocal elements of encephalomyeloneuropathy. Except for an anecdotal report, case descriptions of paraneoplastic small fibre neuropathy are lacking. We report paraneoplastic small fibre neuropathy followed by chorea associated with small cell lung cancer. A man aged 57 years with a 35 pack-year smoking history presented with painless subacute paresthesia and weight fluctuation...
August 29, 2016: BMJ Case Reports
Albrecht Günther, Julia Schubert, Dirk Brämer, Otto Wilhelm Witte
Autoimmune encephalitis, an inflammatory disease of the brain, is usually attributed to antibody-mediated damage and dysfunction of neuronal structures. A distinction is made between onconeuronal antibodies (directed against intracellular neuronal antigens with resulting paraneoplastic neurological syndromes) and antibodies directed against neuronal cell surface proteins (with resulting synaptic encephalopathies). Anti-NMDA-Receptor-Encephalitis, the most common form of autoimmune encephalopathy, is characterized by a phased course of disease...
August 2016: Deutsche Medizinische Wochenschrift
Carlo Selmi, Jobert G Barin, Noel R Rose
In the broad field of autoimmunity and clinical immunology, experimental evidence over the past few years have demonstrated several connections between the immune system and the nervous system, both central and peripheral, leading to the definition of neuroimmunology and of an immune-brain axis. Indeed, the central nervous system as an immune-privileged site, thanks to the blood-brain barrier, is no longer a dogma as the barrier may be altered during chronic inflammation with disruptive changes of endothelial cells and tight junctions, largely mediated by adenosine receptors and the expression of CD39/CD73...
August 18, 2016: Journal of Autoimmunity
Philipp Schwenkenbecher, Lisa Priya Chacko, Ulrich Wurster, Kaweh Pars, Refik Pul, Kurt-Wolfram Sühs, Martin Stangel, Thomas Skripuletz
BACKGROUND: Paraneoplastic syndromes are serious immune caused diseases of the peripheral and/or central nervous system associated with malignant neoplasm. Symptoms develop when antibodies against antigens expressed by tumor cells cross-react with neuronal proteins. Antineuronal antibodies are usually examined in patient's sera while examination of the cerebrospinal fluid (CSF) often fails. Furthermore, the few previous reports describing CSF data summarized different antineuronal antibodies and/or regarded patients with different neurological symptoms as one group...
2016: BMC Neurology
Charuwan Tantipalakorn, Atiwat Soontornpun, Tip Pongsuvareeyakul, Theera Tongsong
This report is aimed to describe a life-threatening case of anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis secondary to ovarian teratoma with rapid recovery in 1 day after the removal of the tumour. A 23-year-old woman presented with sudden headache, personality changes and seizure. After neurological assessment, limbic or herpes encephalitis was provisionally diagnosed and treated with intravenous immunoglobulin, acyclovir and steroids. The patient had progressive severe neurological symptoms, requiring prolonged intubation and mechanical ventilation...
2016: BMJ Case Reports
Misako Kaido, Yoshihito Yuasa, Tameyoshi Yamamoto, Satoru Munakata, Naohiro Tagawa, Keiko Tanaka
We report the case of a patient who had developed multiple cranial nerve palsies in the course of possible paraneoplastic neurological syndrome (PNS) associated with gallbladder cancer. Twelve days prior to visiting our hospital, a 69-year-old man began experiencing neurological symptoms, beginning with diplopia and progressing to ptosis of the left palpebra and subsequent complete closure of the eye within 8 days. Results of the initial medical examination indicated paresis of left oculomotor (III) and abducens (VI) nerves...
September 29, 2016: Rinshō Shinkeigaku, Clinical Neurology
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